The Journal of Family Practice

The abnormal upward curve to the fingernails was consistent with a diagnosis of koilonychia—otherwise known as spoon nails.

Koilonychia is an abnormal nail growth pattern where the distal nail matrix is depressed below its normal level, resulting in the spoon shape. The reverse, where the distal nail matrix is elevated in contrast to the proximal nail matrix, results in clubbing.¹

There are multiple factors and diseases that result in koilonychia, including lichen planus, psoriasis, nutritional deficiencies (including iron deficiency anemia), and endocrinopathies.¹ Lichen planus, which can cause koilonychia, often affects multiple nails and can also cause an associated central ridge pattern. Psoriasis may display a range of nail abnormalities; these include koilonychia, pitting onycholysis, and oil staining.

This patient did not have any signs or symptoms of psoriasis or lichen planus of her nails or skin. A review of her laboratory tests on file made no mention of anemia. Her chemistry profile—including liver tests, renal function tests, and protein levels—were all normal except for glucose levels, which was consistent with her prediabetes. Her thyroid function was also normal. No additional testing was performed since she had no symptoms, physical exam findings, or laboratory clues that pointed to other diseases or systemic processes.

The patient was advised to pick up over-the-counter nail strengtheners and to keep her fingernails trimmed short to minimize the likelihood of painful distal splitting that often occurs with brittle nails. Her physician advised her to follow up with the primary care team if she developed any new signs or symptoms.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

The abnormal upward curve to the fingernails was consistent with a diagnosis of koilonychia—otherwise known as spoon nails.

Koilonychia is an abnormal nail growth pattern where the distal nail matrix is depressed below its normal level, resulting in the spoon shape. The reverse, where the distal nail matrix is elevated in contrast to the proximal nail matrix, results in clubbing.¹

There are multiple factors and diseases that result in koilonychia, including lichen planus, psoriasis, nutritional deficiencies (including iron deficiency anemia), and endocrinopathies.¹ Lichen planus, which can cause koilonychia, often affects multiple nails and can also cause an associated central ridge pattern. Psoriasis may display a range of nail abnormalities; these include koilonychia, pitting onycholysis, and oil staining.

This patient did not have any signs or symptoms of psoriasis or lichen planus of her nails or skin. A review of her laboratory tests on file made no mention of anemia. Her chemistry profile—including liver tests, renal function tests, and protein levels—were all normal except for glucose levels, which was consistent with her prediabetes. Her thyroid function was also normal. No additional testing was performed since she had no symptoms, physical exam findings, or laboratory clues that pointed to other diseases or systemic processes.

The patient was advised to pick up over-the-counter nail strengtheners and to keep her fingernails trimmed short to minimize the likelihood of painful distal splitting that often occurs with brittle nails. Her physician advised her to follow up with the primary care team if she developed any new signs or symptoms.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

The abnormal upward curve to the fingernails was consistent with a diagnosis of koilonychia—otherwise known as spoon nails.

Koilonychia is an abnormal nail growth pattern where the distal nail matrix is depressed below its normal level, resulting in the spoon shape. The reverse, where the distal nail matrix is elevated in contrast to the proximal nail matrix, results in clubbing.¹

There are multiple factors and diseases that result in koilonychia, including lichen planus, psoriasis, nutritional deficiencies (including iron deficiency anemia), and endocrinopathies.¹ Lichen planus, which can cause koilonychia, often affects multiple nails and can also cause an associated central ridge pattern. Psoriasis may display a range of nail abnormalities; these include koilonychia, pitting onycholysis, and oil staining.

This patient did not have any signs or symptoms of psoriasis or lichen planus of her nails or skin. A review of her laboratory tests on file made no mention of anemia. Her chemistry profile—including liver tests, renal function tests, and protein levels—were all normal except for glucose levels, which was consistent with her prediabetes. Her thyroid function was also normal. No additional testing was performed since she had no symptoms, physical exam findings, or laboratory clues that pointed to other diseases or systemic processes.

The patient was advised to pick up over-the-counter nail strengtheners and to keep her fingernails trimmed short to minimize the likelihood of painful distal splitting that often occurs with brittle nails. Her physician advised her to follow up with the primary care team if she developed any new signs or symptoms.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

Although an elevated and pigmented lesion should be considered for possible melanoma, this one had prominent telangiectasias and was proven to be a basal cell carcinoma (BCC) on biopsy.

While the literature often focuses on light-colored skin types and the high risk of skin cancers, individuals with darker skin can also get melanoma and nonmelanoma skin cancer. Half of the BCCs in African American people are pigmented BCCs, compared to less than 10% for Caucasian individuals. Individuals who are Hispanic have twice the likelihood of pigmented BCCs as those who are Caucasian.¹ Pigmented BCCs manifest as darker lesions, as occurred in this individual. Nonpigmented BCCs tend to be pink or pale in color.

Typically, superficial and very small, nodular BCCs can be successfully treated with 2 cycles of electrodesiccation and curettage. EDC should, however, be avoided in low-risk BCCs when these lesions occur in areas of secondary hair growth, such as the beard or scalp. This is because the epidermis follows the hair follicle, and in sites with deep hair follicles, EDC would have to get down to the subcutis to effectively clear the tumor.

For larger, nodular BCCs, full-thickness excision with adequate margins is warranted. For high-risk types, and those in high-risk areas near the nose, eyes, mouth, and ears, Mohs micrographic surgery is recommended to maximize the likelihood of complete excision while minimizing the loss of normal tissue.

Since the physician suspected this was a pigmented BCC, he performed a superficial shave biopsy on a small representative area of the lesion for diagnosis. This patient’s biopsy confirmed a nodular-type pigmented BCC. The lesion was removed in the office with 5-mm margins oriented along the resting skin tension lines with good closure and cosmetic results.

The patient was advised to have routine skin evaluations every 6 months due to the high risk of additional cancers. He was also advised to take oral niacinamide 500 mg twice daily, which can reduce the risk of actinic keratoses and nonmelanoma skin cancers by 15% and 23%, respectively, in those who have had lesions.²

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

Although an elevated and pigmented lesion should be considered for possible melanoma, this one had prominent telangiectasias and was proven to be a basal cell carcinoma (BCC) on biopsy.

While the literature often focuses on light-colored skin types and the high risk of skin cancers, individuals with darker skin can also get melanoma and nonmelanoma skin cancer. Half of the BCCs in African American people are pigmented BCCs, compared to less than 10% for Caucasian individuals. Individuals who are Hispanic have twice the likelihood of pigmented BCCs as those who are Caucasian.¹ Pigmented BCCs manifest as darker lesions, as occurred in this individual. Nonpigmented BCCs tend to be pink or pale in color.

Typically, superficial and very small, nodular BCCs can be successfully treated with 2 cycles of electrodesiccation and curettage. EDC should, however, be avoided in low-risk BCCs when these lesions occur in areas of secondary hair growth, such as the beard or scalp. This is because the epidermis follows the hair follicle, and in sites with deep hair follicles, EDC would have to get down to the subcutis to effectively clear the tumor.

For larger, nodular BCCs, full-thickness excision with adequate margins is warranted. For high-risk types, and those in high-risk areas near the nose, eyes, mouth, and ears, Mohs micrographic surgery is recommended to maximize the likelihood of complete excision while minimizing the loss of normal tissue.

Since the physician suspected this was a pigmented BCC, he performed a superficial shave biopsy on a small representative area of the lesion for diagnosis. This patient’s biopsy confirmed a nodular-type pigmented BCC. The lesion was removed in the office with 5-mm margins oriented along the resting skin tension lines with good closure and cosmetic results.

The patient was advised to have routine skin evaluations every 6 months due to the high risk of additional cancers. He was also advised to take oral niacinamide 500 mg twice daily, which can reduce the risk of actinic keratoses and nonmelanoma skin cancers by 15% and 23%, respectively, in those who have had lesions.²

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

Although an elevated and pigmented lesion should be considered for possible melanoma, this one had prominent telangiectasias and was proven to be a basal cell carcinoma (BCC) on biopsy.

While the literature often focuses on light-colored skin types and the high risk of skin cancers, individuals with darker skin can also get melanoma and nonmelanoma skin cancer. Half of the BCCs in African American people are pigmented BCCs, compared to less than 10% for Caucasian individuals. Individuals who are Hispanic have twice the likelihood of pigmented BCCs as those who are Caucasian.¹ Pigmented BCCs manifest as darker lesions, as occurred in this individual. Nonpigmented BCCs tend to be pink or pale in color.

Typically, superficial and very small, nodular BCCs can be successfully treated with 2 cycles of electrodesiccation and curettage. EDC should, however, be avoided in low-risk BCCs when these lesions occur in areas of secondary hair growth, such as the beard or scalp. This is because the epidermis follows the hair follicle, and in sites with deep hair follicles, EDC would have to get down to the subcutis to effectively clear the tumor.

For larger, nodular BCCs, full-thickness excision with adequate margins is warranted. For high-risk types, and those in high-risk areas near the nose, eyes, mouth, and ears, Mohs micrographic surgery is recommended to maximize the likelihood of complete excision while minimizing the loss of normal tissue.

Since the physician suspected this was a pigmented BCC, he performed a superficial shave biopsy on a small representative area of the lesion for diagnosis. This patient’s biopsy confirmed a nodular-type pigmented BCC. The lesion was removed in the office with 5-mm margins oriented along the resting skin tension lines with good closure and cosmetic results.

The patient was advised to have routine skin evaluations every 6 months due to the high risk of additional cancers. He was also advised to take oral niacinamide 500 mg twice daily, which can reduce the risk of actinic keratoses and nonmelanoma skin cancers by 15% and 23%, respectively, in those who have had lesions.²

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Professor and Chair, Department of Family and Community Medicine, Western Michigan University Homer Stryker, MD School of Medicine, Kalamazoo.

THE CASE

A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.

The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.

The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.

THE DIAGNOSIS

Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.

Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.¹ The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.¹ Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.

Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.² Usually white discolorations appear on the distal edges of the nail.³ It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.² However, the nails do not separate from the nail bed, and usually only the fingernails are involved.

Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.³ It also can be caused by medications and thyroid disease.^3,4

Continue to: Onychomadesis

Onychomadesis, sometimes considered a severe form of Beau’s line,^5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.⁷ When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.^6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.^5,7

In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.

DISCUSSION

Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.⁸ It is a potential late complication of infection, such as HFMD,⁹ and was first reported in children in Chicago in 2000.¹⁰ Since then, onychomadesis has been noted in children in many countries.⁸ Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.¹¹

Onychomadesis following hand-foot-mouth disease (HFMD) may be underreported in adults because HFMD is more common in children and symptoms in adults are minor.

Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.⁴ Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.⁴ Most known outbreaks seem to be caused by CVA6.⁴

No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”¹²

Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.

THE TAKEAWAY

The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.

CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]

THE CASE

A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.

The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.

The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.

THE DIAGNOSIS

Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.

Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.¹ The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.¹ Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.

Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.² Usually white discolorations appear on the distal edges of the nail.³ It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.² However, the nails do not separate from the nail bed, and usually only the fingernails are involved.

Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.³ It also can be caused by medications and thyroid disease.^3,4

Continue to: Onychomadesis

Onychomadesis, sometimes considered a severe form of Beau’s line,^5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.⁷ When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.^6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.^5,7

In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.

DISCUSSION

Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.⁸ It is a potential late complication of infection, such as HFMD,⁹ and was first reported in children in Chicago in 2000.¹⁰ Since then, onychomadesis has been noted in children in many countries.⁸ Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.¹¹

Onychomadesis following hand-foot-mouth disease (HFMD) may be underreported in adults because HFMD is more common in children and symptoms in adults are minor.

Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.⁴ Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.⁴ Most known outbreaks seem to be caused by CVA6.⁴

No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”¹²

Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.

THE TAKEAWAY

The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.

CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]

THE CASE

A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.

The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.

The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.

THE DIAGNOSIS

Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.

Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.¹ The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.¹ Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.

Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.² Usually white discolorations appear on the distal edges of the nail.³ It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.² However, the nails do not separate from the nail bed, and usually only the fingernails are involved.

Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.³ It also can be caused by medications and thyroid disease.^3,4

Continue to: Onychomadesis

Onychomadesis, sometimes considered a severe form of Beau’s line,^5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.⁷ When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.^6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.^5,7

In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.

DISCUSSION

Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.⁸ It is a potential late complication of infection, such as HFMD,⁹ and was first reported in children in Chicago in 2000.¹⁰ Since then, onychomadesis has been noted in children in many countries.⁸ Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.¹¹

Onychomadesis following hand-foot-mouth disease (HFMD) may be underreported in adults because HFMD is more common in children and symptoms in adults are minor.

Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.⁴ Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.⁴ Most known outbreaks seem to be caused by CVA6.⁴

No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”¹²

Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.

THE TAKEAWAY

The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.

CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]

Evidence summary

Mixed results, Yes, but no evidence of harm in continuing RAAS therapy

A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m² or a stable eGFR ≥ 60 mL/min/1.73 m² and an elevated urine microalbumin measurement.¹ In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).

A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.² Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).

A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.³ The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.

Recommendations from others

After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.⁴ Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.

In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.⁵ Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).

Editor’s takeaway

Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.

Evidence summary

Mixed results, Yes, but no evidence of harm in continuing RAAS therapy

A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m² or a stable eGFR ≥ 60 mL/min/1.73 m² and an elevated urine microalbumin measurement.¹ In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).

A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.² Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).

A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.³ The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.

Recommendations from others

After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.⁴ Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.

In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.⁵ Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).

Editor’s takeaway

Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.

Evidence summary

Mixed results, Yes, but no evidence of harm in continuing RAAS therapy

A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m² or a stable eGFR ≥ 60 mL/min/1.73 m² and an elevated urine microalbumin measurement.¹ In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).

A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.² Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).

A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.³ The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.

Recommendations from others

After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.⁴ Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.

In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.⁵ Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).

Editor’s takeaway

Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.

Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.¹ People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.²

Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).^3-6 

However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.⁷  In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.^7-9

Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.¹⁰The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.

Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.⁹ Recommend muscle-­strengthening activities be performed at least 2 days each week.⁹

Exercise recommendations for patients with Down syndrome

One in every 700 babies receives a diagnosis of Down syndrome.¹¹ Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities. 

Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-­participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.

Continue to: For patients with Down syndrome...

For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.

Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.

Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.¹²

An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.¹³ For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.¹³ Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.¹³Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.

Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.¹⁴ These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.¹⁵

Continue to: The complexity of pain assessment...

The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.¹⁵ In nonverbal patients, facial expressions are reliable indicators of pain.

Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.⁶ Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.¹⁶

Exercise recommendations for patients with cerebral palsy

Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.¹⁷ The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.¹⁸

Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.¹⁸ Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.

Consider sports and exercise a vital therapeutic modality— something beyond a form of recreation.

Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.¹⁷

Continue to: Special considerations

Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.¹⁹ Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health ­Questionnaire-9, and the Ask Suicide-­Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.

Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.^20-22

Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-­severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.

Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.⁵   Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.⁵ Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.²³ By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.

Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.^24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.

Continue to: Dance promotes functionality...

Dance promotes functionality and psychosocial adjustment.²⁶ Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.²⁷ Aquatic therapy also may increase muscle strength.²⁸

Sports for patients with autism spectrum disorder

Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.²⁹ Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.³⁰

Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury in patients with autism spectrum disorder.

Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.^31,32 

Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.³³ These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.³⁴ As such, these are solely screening tools. Final diagnosis is based on clinical judgment.

Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.²⁹ Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information 

Continue to: During the physical exam...

During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.

Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.³ Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.⁴

The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.^31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.³⁷ Additionally, patients with autism are much more likely to be overweight or obese.³² Physical activity offers those with autism health benefits similar to those for the general population.³²

Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.³⁸ Karate and swimming are among the most researched sports therapy options for patients with autism.^38-40 Both are shown to improve motor ability and reduce communication deficits. 

Summing up

The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/­sports involvement is maintained.

Exercise participation increases based on the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.

Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.⁴¹ Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.¹⁰

Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.

CORRESPONDENCE 
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]

Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.¹ People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.²

Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).^3-6 

However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.⁷  In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.^7-9

Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.¹⁰The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.

Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.⁹ Recommend muscle-­strengthening activities be performed at least 2 days each week.⁹

Exercise recommendations for patients with Down syndrome

One in every 700 babies receives a diagnosis of Down syndrome.¹¹ Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities. 

Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-­participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.

Continue to: For patients with Down syndrome...

For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.

Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.

Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.¹²

An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.¹³ For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.¹³ Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.¹³Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.

Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.¹⁴ These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.¹⁵

Continue to: The complexity of pain assessment...

The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.¹⁵ In nonverbal patients, facial expressions are reliable indicators of pain.

Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.⁶ Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.¹⁶

Exercise recommendations for patients with cerebral palsy

Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.¹⁷ The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.¹⁸

Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.¹⁸ Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.

Consider sports and exercise a vital therapeutic modality— something beyond a form of recreation.

Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.¹⁷

Continue to: Special considerations

Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.¹⁹ Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health ­Questionnaire-9, and the Ask Suicide-­Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.

Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.^20-22

Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-­severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.

Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.⁵   Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.⁵ Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.²³ By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.

Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.^24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.

Continue to: Dance promotes functionality...

Dance promotes functionality and psychosocial adjustment.²⁶ Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.²⁷ Aquatic therapy also may increase muscle strength.²⁸

Sports for patients with autism spectrum disorder

Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.²⁹ Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.³⁰

Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury in patients with autism spectrum disorder.

Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.^31,32 

Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.³³ These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.³⁴ As such, these are solely screening tools. Final diagnosis is based on clinical judgment.

Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.²⁹ Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information 

Continue to: During the physical exam...

During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.

Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.³ Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.⁴

The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.^31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.³⁷ Additionally, patients with autism are much more likely to be overweight or obese.³² Physical activity offers those with autism health benefits similar to those for the general population.³²

Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.³⁸ Karate and swimming are among the most researched sports therapy options for patients with autism.^38-40 Both are shown to improve motor ability and reduce communication deficits. 

Summing up

The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/­sports involvement is maintained.

Exercise participation increases based on the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.

Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.⁴¹ Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.¹⁰

Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.

CORRESPONDENCE 
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]

Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.¹ People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.²

Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).^3-6 

However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.⁷  In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.^7-9

Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.¹⁰The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.

Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.⁹ Recommend muscle-­strengthening activities be performed at least 2 days each week.⁹

Exercise recommendations for patients with Down syndrome

One in every 700 babies receives a diagnosis of Down syndrome.¹¹ Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities. 

Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-­participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.

Continue to: For patients with Down syndrome...

For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.

Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.

Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.¹²

An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.¹³ For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.¹³ Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.¹³Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.

Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.¹⁴ These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.¹⁵

Continue to: The complexity of pain assessment...

The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.¹⁵ In nonverbal patients, facial expressions are reliable indicators of pain.

Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.⁶ Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.¹⁶

Exercise recommendations for patients with cerebral palsy

Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.¹⁷ The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.¹⁸

Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.¹⁸ Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.

Consider sports and exercise a vital therapeutic modality— something beyond a form of recreation.

Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.¹⁷

Continue to: Special considerations

Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.¹⁹ Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health ­Questionnaire-9, and the Ask Suicide-­Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.

Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.^20-22

Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-­severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.

Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.⁵   Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.⁵ Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.²³ By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.

Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.^24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.

Continue to: Dance promotes functionality...

Dance promotes functionality and psychosocial adjustment.²⁶ Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.²⁷ Aquatic therapy also may increase muscle strength.²⁸

Sports for patients with autism spectrum disorder

Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.²⁹ Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.³⁰

Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury in patients with autism spectrum disorder.

Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.^31,32 

Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.³³ These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.³⁴ As such, these are solely screening tools. Final diagnosis is based on clinical judgment.

Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.²⁹ Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information 

Continue to: During the physical exam...

During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.

Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.³ Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.⁴

The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.^31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.³⁷ Additionally, patients with autism are much more likely to be overweight or obese.³² Physical activity offers those with autism health benefits similar to those for the general population.³²

Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.³⁸ Karate and swimming are among the most researched sports therapy options for patients with autism.^38-40 Both are shown to improve motor ability and reduce communication deficits. 

Summing up

The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/­sports involvement is maintained.

Exercise participation increases based on the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.

Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.⁴¹ Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.¹⁰

Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.

CORRESPONDENCE 
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]

THE CASE

Janice M* presented to the emergency department (ED) with worsening slurred speech. The 55-year-old patient’s history was significant for diabetes; hypertension; depression; sleep apnea; multiple transient ischemic attacks (TIAs) thought to be stress related; and left lower-extremity weakness secondary to prior infarct. Ms. M had been to the hospital multiple times in the previous 2 to 3 years for similar symptoms. Her most recent visit to the ED had been 2 months earlier.

In the ED, the patient’s NIH stroke score was 1 for the presence of dysarthria, and a code for emergency stroke management was initiated. Ms. M was alert and oriented x 3, with no focal motor or sensory deficits noted. Computed tomography (CT) and CT angiography were negative for any acute abnormality. Throughout the course of the ED visit, her NIH score improved to 0. Ms. M exhibited staccato/stuttering speech, but it was believed that this would likely improve over the next few days.

According to the hospital neurologist, the ED work-up suggested either a TIA, stress-induced psychiatric speech disorder, or conversion disorder. The patient was discharged home in stable condition and was asked to follow up with the outpatient neurologist in 1 week.

Ms. M was seen approximately 2 weeks later in the outpatient neurology stroke ­clinic. Her symptoms had resolved, and she did not report any new or worsening symptoms. An outpatient stroke work-up was initiated, including magnetic resonance imaging (MRI) of the brain, echocardiography, and measurement of low-density lipoprotein and hemoglobin A1C; all results were unremarkable. Given the timeline for symptom improvement and results of the work-up, the patient was given a diagnosis of conversion disorder. Ms. M was encouraged to follow up with her primary care physician (PCP) for further medical management.

● How would you proceed with this patient?

* The patient’s name has been changed to protect her identity.

According to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, text revision, conversion disorder (also known as functional neurological symptom disorder) is characterized as a somatic symptom and related disorder.¹ The prominent feature shared among disorders in this category is the presence of somatic symptoms that are associated with distress and impairment.

In conversion disorder, the focus is on symptoms that are neurologic in nature but are not due to underlying neurologic disease and are incongruent with typical patterns of presentation for any neurologic condition. Patients with conversion disorder may present with motor symptoms (eg, weakness, paralysis, tremor, dystonia), altered sensory or cognitive function, seizure-like symptoms, alterations in speech, or changes in swallowing.^1,2

For a diagnosis of conversion disorder, the following criteria must be met¹:

• The patient has 1 or more symptoms of altered voluntary motor or sensory function.
• Symptom presentation is incongruent with recognized neurologic or medical disease or conditions.
• Symptoms are not better explained by another medical or mental health condition.
• There is significant distress or impairment in functioning due to symptoms or the deficit.

The etiology of conversion disorder has not been firmly established. While the literature suggests that psychological stressors play a role,^3,4 an effort also has been made to better understand the underlying neural and biological basis. Specifically, studies have utilized brain imaging to explore brain pathways and mechanisms that could account for symptom presentation.^5,6

Prevalence rates for conversion disorder vary depending on the population studied. While it is estimated that 5% of patients in a general hospital setting meet full criteria for conversion disorder,⁷ higher rates may exist in specialty settings; 1 study found that 30% of patients in a neurology specialty clinic exhibited symptoms that were medically unexplained.⁸

Continue to: In primary care...

One study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.

In primary care, prevalence of conversion disorder can be difficult to pinpoint; however, 1 study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.⁹ Therefore, it is important for family physicians (FPs) to be familiar with the assessment and treatment of conversion disorder (and other disorders in which medically unexplained symptoms may be at the core of the patient presentation).

The differential: Neurologic and psychiatric conditions

Patients with conversion disorder may present with a variety of neurologic symptoms that can mimic those of organic disease. This can pose a diagnostic challenge, increase the chance of misdiagnosis, and delay treatment.

Motor symptoms may include paralysis, gait disturbance, dysphagia, or aphasia. Patients also may have sensory symptoms, such as blindness, deafness, or anesthesia.^10,11 As a result, it is important to rule out both urgent neurologic presentations, such as TIA, acute stroke, and brain tumor, and other chronic neurologic conditions, including multiple sclerosis, myasthenia gravis, and epilepsy.^11,12

Multiple sclerosis will demonstrate characteristic lesions on MRI that differentiate it from conversion disorder.

Myasthenia gravis is distinguished by positive findings on autoantibodies testing and on electrophysiologic studies.

Continue to: Epilepsy

Epilepsy. Patients with conversion disorder may present with unresponsiveness and abnormal movements, such as generalized limb shaking and hip thrusting, that mimic an epileptic seizure. In contrast to epileptic seizures, psychogenic nonepileptic seizures may last longer, symptoms may wax and wane, and patients generally do not have bowel or bladder incontinence or sustain injury as they would during an actual seizure.¹²

There are several psychiatric/­psychosocial conditions that also should be considered in the differential diagnosis of conversion disorder.

Somatic symptom disorder, like conversion disorder, produces somatic symptoms that can cause significant distress for patients. The difference in the 2 conditions is that symptoms of somatic symptom disorder may be compatible with a recognized neurologic or general medical condition, whereas in conversion disorder, the symptoms are not consistent with a recognized disease.^1,12

Factitious disorder, similar to conversion disorder, can involve neurologic symptoms that are not attributed to disease. However, patients with factitious disorder deliberately simulate symptoms to receive medical care. A thorough clinical interview and physical exam can help to distinguish conversion disorder from factitious disorder.

Malingering is not a psychiatric condition but a behavior that involves intentionally feigning symptoms for the purpose of personal or financial gain. There is no evidence that patients with conversion disorder simulate their symptoms.^12,13

Continue to: Negative results and positive signs point to the Dx

Negative results and positive signs point to the Dx

Conversion disorder is not a diagnosis of exclusion. Diagnosis requires detailed history taking and a thorough neurologic exam. Laboratory testing and neuroimaging are also important, and results will have to be negative to support the diagnosis.

Conversion disorder is not a diagnosis of exclusion; diagnosis requires detailed history taking and a thorough neurologic exam.

Neurologic deficits with conversion disorder do not follow a known neurologic insult.¹⁴ There are many tests that can be used to distinguish functional symptoms vs organic symptoms. Two of the most well-known tests are the Hoover sign and the abductor sign, which will be positive in conversion disorder. Both can be performed easily in an outpatient setting.

The Hoover sign is considered positive when there is weakness of voluntary hip extension in the presence of normal involuntary hip extension during contralateral hip flexion against resistance. According to a meta-­analysis of multiple studies of patients with conversion disorder, the overall estimated sensitivity of this test is 94% and the specificity, 99%.¹⁵

The abductor sign follows the same principle as the Hoover sign: When the patient abducts the nonparetic leg, both the nonparetic and “paretic” leg are strong. When the patient abducts just the “paretic” leg, both legs become weak.¹⁶

Other symptom evaluations. For patients who have functional seizures, video electroencephalography is helpful to distinguish functional seizures from “true” seizures.^17,18 In conversion disorder, functional dysarthria normally resembles a stutter or speech that is extremely slow with long hesitations that are hard to interrupt.¹⁸ Dysphonia and functional dysphagia are also very common functional symptoms. Usually after extensive work-up, no organic cause of the patient’s symptoms is ever found.¹⁸

Continue to: Treatment requires an integrated team approach

Treatment for conversion disorder can be difficult due to the complex and not fully understood etiology of the condition. Due to its multifaceted nature, an integrated team approach can be beneficial at each stage, including assessment and intervention.

Explain the diagnosis clearly. An essential initial step in the treatment of conversion disorder is careful explanation of the diagnosis. Clear explanation of the terminology and presentation of conversion disorder may prevent the patient from misinterpreting their diagnosis as a suggestion that they are feigning or malingering symptoms or feeling that their symptoms or concerns are being dismissed.² Understanding the condition can help improve the likelihood of the patient accepting the treatment plan and help decrease the likelihood of unnecessary testing, health care visits, and consultations. Developing a strong rapport with the patient is key when explaining the diagnosis.

Recommend cognitive behavioral therapy (CBT). In a meta-analysis of 15 randomized controlled trials, CBT significantly reduced somatic, anxious, and depressive symptoms and improved physical functioning in patients with somatoform disorders and medically unexplained symptoms.¹⁹ Another study, utilizing a case series, demonstrated significant improvement in social, emotional, and behavioral functioning in children and adolescents with functional neurologic symptoms (conversion disorder) post–CBT intervention.²⁰

Given that research supports CBT’s effectiveness in the management of conversion disorder, it is beneficial to engage a behavioral health professional as a part of the treatment team to focus on factors such as stress management, development of coping skills, and treatment of underlying psychiatric conditions.

Consider these other options. The addition of medication management can be considered for patients with comorbid psychiatric disorders. Evidence suggests that physical therapy is helpful in the treatment of motor and gait dysfunction seen in conversion disorder.^21,22 The role of hypnosis in the management of conversion disorder has also been studied, but more randomized clinical trials are needed to further explore this treatment.^2,23,24

Continue to: The FP's role in coordination of care

The FP’s role in coordination of care

Conversion disorder can be challenging to diagnose and often involves a multidisciplinary approach. Patients with conversion disorder may see multiple clinicians as they undergo evaluation for their symptoms, but they usually are referred back to their PCP for management and coordination of care. Thus, the FP’s understanding of how the condition is diagnosed and appropriately managed is beneficial.

The prognosis of conversion disorder is associated with symptom duration; thus, consultation between FPs and mental health providers is essential.

Open and effective communication among all members of the health care team can ensure consistency in treatment, a strong patient–provider relationship, favorable prognosis, and prevention of symptom relapse. FPs, by establishing a good rapport with patients, can help them understand the condition and the mind-body connection. Once other diagnoses have been ruled out, the FP can provide reassurance to patients and minimize further diagnostic testing.

The prognosis of conversion disorder is associated with symptom duration²⁵; thus, consultation between FPs and mental health providers is essential. The FP also can be integral in the recognition of psychiatric comorbidities, such as anxiety and depression, helping to ensure that these conditions also are treated appropriately.^25,26

THE CASE

Ms. M was referred to a neuropsychologist for further assessment, and the diagnosis of conversion disorder was confirmed. She was then referred to a family medicine behavioral health psychologist for CBT. The initial consult indicated that psychological stressors were contributing to symptoms, and Ms. M was diagnosed with depression and anxiety as well as conversion disorder.

Treatment started with patient education. The treatment framework was carefully explained to Ms. M, with a focus on identifying possible symptom triggers, helping her build a more effective stress response, increasing skills to more effectively manage stressors, and managing underlying psychiatric disorders (ie, depression, anxiety).

Ms. M continued regular visits with the family medicine behavioral health psychologist for CBT and followed up with her PCP as needed to manage chronic health conditions and stroke risk factors. The patient was able to implement skills discussed in treatment sessions, including identifying triggers and implementing coping skills (eg, managing negative thoughts that contribute to symptoms, setting boundaries) to manage stressors.

Her depressive and anxious symptoms improved, as indicated by symptom measurement tools and self-report. The frequency and severity of episodes of slurred speech and muscle weakness decreased, and the patient reported only 1 ED visit related to speech difficulties in the 2 years while following up with the behavioral health psychologist.

CORRESPONDENCE
Kristen J. Alston, PhD, University of Mississippi Medical Center, 2400 North State Street, Jackson, MS 39216; [email protected]

THE CASE

Janice M* presented to the emergency department (ED) with worsening slurred speech. The 55-year-old patient’s history was significant for diabetes; hypertension; depression; sleep apnea; multiple transient ischemic attacks (TIAs) thought to be stress related; and left lower-extremity weakness secondary to prior infarct. Ms. M had been to the hospital multiple times in the previous 2 to 3 years for similar symptoms. Her most recent visit to the ED had been 2 months earlier.

In the ED, the patient’s NIH stroke score was 1 for the presence of dysarthria, and a code for emergency stroke management was initiated. Ms. M was alert and oriented x 3, with no focal motor or sensory deficits noted. Computed tomography (CT) and CT angiography were negative for any acute abnormality. Throughout the course of the ED visit, her NIH score improved to 0. Ms. M exhibited staccato/stuttering speech, but it was believed that this would likely improve over the next few days.

According to the hospital neurologist, the ED work-up suggested either a TIA, stress-induced psychiatric speech disorder, or conversion disorder. The patient was discharged home in stable condition and was asked to follow up with the outpatient neurologist in 1 week.

Ms. M was seen approximately 2 weeks later in the outpatient neurology stroke ­clinic. Her symptoms had resolved, and she did not report any new or worsening symptoms. An outpatient stroke work-up was initiated, including magnetic resonance imaging (MRI) of the brain, echocardiography, and measurement of low-density lipoprotein and hemoglobin A1C; all results were unremarkable. Given the timeline for symptom improvement and results of the work-up, the patient was given a diagnosis of conversion disorder. Ms. M was encouraged to follow up with her primary care physician (PCP) for further medical management.

● How would you proceed with this patient?

* The patient’s name has been changed to protect her identity.

According to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, text revision, conversion disorder (also known as functional neurological symptom disorder) is characterized as a somatic symptom and related disorder.¹ The prominent feature shared among disorders in this category is the presence of somatic symptoms that are associated with distress and impairment.

In conversion disorder, the focus is on symptoms that are neurologic in nature but are not due to underlying neurologic disease and are incongruent with typical patterns of presentation for any neurologic condition. Patients with conversion disorder may present with motor symptoms (eg, weakness, paralysis, tremor, dystonia), altered sensory or cognitive function, seizure-like symptoms, alterations in speech, or changes in swallowing.^1,2

For a diagnosis of conversion disorder, the following criteria must be met¹:

• The patient has 1 or more symptoms of altered voluntary motor or sensory function.
• Symptom presentation is incongruent with recognized neurologic or medical disease or conditions.
• Symptoms are not better explained by another medical or mental health condition.
• There is significant distress or impairment in functioning due to symptoms or the deficit.

The etiology of conversion disorder has not been firmly established. While the literature suggests that psychological stressors play a role,^3,4 an effort also has been made to better understand the underlying neural and biological basis. Specifically, studies have utilized brain imaging to explore brain pathways and mechanisms that could account for symptom presentation.^5,6

Prevalence rates for conversion disorder vary depending on the population studied. While it is estimated that 5% of patients in a general hospital setting meet full criteria for conversion disorder,⁷ higher rates may exist in specialty settings; 1 study found that 30% of patients in a neurology specialty clinic exhibited symptoms that were medically unexplained.⁸

Continue to: In primary care...

One study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.

In primary care, prevalence of conversion disorder can be difficult to pinpoint; however, 1 study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.⁹ Therefore, it is important for family physicians (FPs) to be familiar with the assessment and treatment of conversion disorder (and other disorders in which medically unexplained symptoms may be at the core of the patient presentation).

The differential: Neurologic and psychiatric conditions

Patients with conversion disorder may present with a variety of neurologic symptoms that can mimic those of organic disease. This can pose a diagnostic challenge, increase the chance of misdiagnosis, and delay treatment.

Motor symptoms may include paralysis, gait disturbance, dysphagia, or aphasia. Patients also may have sensory symptoms, such as blindness, deafness, or anesthesia.^10,11 As a result, it is important to rule out both urgent neurologic presentations, such as TIA, acute stroke, and brain tumor, and other chronic neurologic conditions, including multiple sclerosis, myasthenia gravis, and epilepsy.^11,12

Multiple sclerosis will demonstrate characteristic lesions on MRI that differentiate it from conversion disorder.

Myasthenia gravis is distinguished by positive findings on autoantibodies testing and on electrophysiologic studies.

Continue to: Epilepsy

Epilepsy. Patients with conversion disorder may present with unresponsiveness and abnormal movements, such as generalized limb shaking and hip thrusting, that mimic an epileptic seizure. In contrast to epileptic seizures, psychogenic nonepileptic seizures may last longer, symptoms may wax and wane, and patients generally do not have bowel or bladder incontinence or sustain injury as they would during an actual seizure.¹²

There are several psychiatric/­psychosocial conditions that also should be considered in the differential diagnosis of conversion disorder.

Somatic symptom disorder, like conversion disorder, produces somatic symptoms that can cause significant distress for patients. The difference in the 2 conditions is that symptoms of somatic symptom disorder may be compatible with a recognized neurologic or general medical condition, whereas in conversion disorder, the symptoms are not consistent with a recognized disease.^1,12

Factitious disorder, similar to conversion disorder, can involve neurologic symptoms that are not attributed to disease. However, patients with factitious disorder deliberately simulate symptoms to receive medical care. A thorough clinical interview and physical exam can help to distinguish conversion disorder from factitious disorder.

Malingering is not a psychiatric condition but a behavior that involves intentionally feigning symptoms for the purpose of personal or financial gain. There is no evidence that patients with conversion disorder simulate their symptoms.^12,13

Continue to: Negative results and positive signs point to the Dx

Negative results and positive signs point to the Dx

Conversion disorder is not a diagnosis of exclusion. Diagnosis requires detailed history taking and a thorough neurologic exam. Laboratory testing and neuroimaging are also important, and results will have to be negative to support the diagnosis.

Conversion disorder is not a diagnosis of exclusion; diagnosis requires detailed history taking and a thorough neurologic exam.

Neurologic deficits with conversion disorder do not follow a known neurologic insult.¹⁴ There are many tests that can be used to distinguish functional symptoms vs organic symptoms. Two of the most well-known tests are the Hoover sign and the abductor sign, which will be positive in conversion disorder. Both can be performed easily in an outpatient setting.

The Hoover sign is considered positive when there is weakness of voluntary hip extension in the presence of normal involuntary hip extension during contralateral hip flexion against resistance. According to a meta-­analysis of multiple studies of patients with conversion disorder, the overall estimated sensitivity of this test is 94% and the specificity, 99%.¹⁵

The abductor sign follows the same principle as the Hoover sign: When the patient abducts the nonparetic leg, both the nonparetic and “paretic” leg are strong. When the patient abducts just the “paretic” leg, both legs become weak.¹⁶

Other symptom evaluations. For patients who have functional seizures, video electroencephalography is helpful to distinguish functional seizures from “true” seizures.^17,18 In conversion disorder, functional dysarthria normally resembles a stutter or speech that is extremely slow with long hesitations that are hard to interrupt.¹⁸ Dysphonia and functional dysphagia are also very common functional symptoms. Usually after extensive work-up, no organic cause of the patient’s symptoms is ever found.¹⁸

Continue to: Treatment requires an integrated team approach

Treatment for conversion disorder can be difficult due to the complex and not fully understood etiology of the condition. Due to its multifaceted nature, an integrated team approach can be beneficial at each stage, including assessment and intervention.

Explain the diagnosis clearly. An essential initial step in the treatment of conversion disorder is careful explanation of the diagnosis. Clear explanation of the terminology and presentation of conversion disorder may prevent the patient from misinterpreting their diagnosis as a suggestion that they are feigning or malingering symptoms or feeling that their symptoms or concerns are being dismissed.² Understanding the condition can help improve the likelihood of the patient accepting the treatment plan and help decrease the likelihood of unnecessary testing, health care visits, and consultations. Developing a strong rapport with the patient is key when explaining the diagnosis.

Recommend cognitive behavioral therapy (CBT). In a meta-analysis of 15 randomized controlled trials, CBT significantly reduced somatic, anxious, and depressive symptoms and improved physical functioning in patients with somatoform disorders and medically unexplained symptoms.¹⁹ Another study, utilizing a case series, demonstrated significant improvement in social, emotional, and behavioral functioning in children and adolescents with functional neurologic symptoms (conversion disorder) post–CBT intervention.²⁰

Given that research supports CBT’s effectiveness in the management of conversion disorder, it is beneficial to engage a behavioral health professional as a part of the treatment team to focus on factors such as stress management, development of coping skills, and treatment of underlying psychiatric conditions.

Consider these other options. The addition of medication management can be considered for patients with comorbid psychiatric disorders. Evidence suggests that physical therapy is helpful in the treatment of motor and gait dysfunction seen in conversion disorder.^21,22 The role of hypnosis in the management of conversion disorder has also been studied, but more randomized clinical trials are needed to further explore this treatment.^2,23,24

Continue to: The FP's role in coordination of care

The FP’s role in coordination of care

Conversion disorder can be challenging to diagnose and often involves a multidisciplinary approach. Patients with conversion disorder may see multiple clinicians as they undergo evaluation for their symptoms, but they usually are referred back to their PCP for management and coordination of care. Thus, the FP’s understanding of how the condition is diagnosed and appropriately managed is beneficial.

The prognosis of conversion disorder is associated with symptom duration; thus, consultation between FPs and mental health providers is essential.

Open and effective communication among all members of the health care team can ensure consistency in treatment, a strong patient–provider relationship, favorable prognosis, and prevention of symptom relapse. FPs, by establishing a good rapport with patients, can help them understand the condition and the mind-body connection. Once other diagnoses have been ruled out, the FP can provide reassurance to patients and minimize further diagnostic testing.

The prognosis of conversion disorder is associated with symptom duration²⁵; thus, consultation between FPs and mental health providers is essential. The FP also can be integral in the recognition of psychiatric comorbidities, such as anxiety and depression, helping to ensure that these conditions also are treated appropriately.^25,26

THE CASE

Ms. M was referred to a neuropsychologist for further assessment, and the diagnosis of conversion disorder was confirmed. She was then referred to a family medicine behavioral health psychologist for CBT. The initial consult indicated that psychological stressors were contributing to symptoms, and Ms. M was diagnosed with depression and anxiety as well as conversion disorder.

Treatment started with patient education. The treatment framework was carefully explained to Ms. M, with a focus on identifying possible symptom triggers, helping her build a more effective stress response, increasing skills to more effectively manage stressors, and managing underlying psychiatric disorders (ie, depression, anxiety).

Ms. M continued regular visits with the family medicine behavioral health psychologist for CBT and followed up with her PCP as needed to manage chronic health conditions and stroke risk factors. The patient was able to implement skills discussed in treatment sessions, including identifying triggers and implementing coping skills (eg, managing negative thoughts that contribute to symptoms, setting boundaries) to manage stressors.

Her depressive and anxious symptoms improved, as indicated by symptom measurement tools and self-report. The frequency and severity of episodes of slurred speech and muscle weakness decreased, and the patient reported only 1 ED visit related to speech difficulties in the 2 years while following up with the behavioral health psychologist.

CORRESPONDENCE
Kristen J. Alston, PhD, University of Mississippi Medical Center, 2400 North State Street, Jackson, MS 39216; [email protected]

THE CASE

Janice M* presented to the emergency department (ED) with worsening slurred speech. The 55-year-old patient’s history was significant for diabetes; hypertension; depression; sleep apnea; multiple transient ischemic attacks (TIAs) thought to be stress related; and left lower-extremity weakness secondary to prior infarct. Ms. M had been to the hospital multiple times in the previous 2 to 3 years for similar symptoms. Her most recent visit to the ED had been 2 months earlier.

In the ED, the patient’s NIH stroke score was 1 for the presence of dysarthria, and a code for emergency stroke management was initiated. Ms. M was alert and oriented x 3, with no focal motor or sensory deficits noted. Computed tomography (CT) and CT angiography were negative for any acute abnormality. Throughout the course of the ED visit, her NIH score improved to 0. Ms. M exhibited staccato/stuttering speech, but it was believed that this would likely improve over the next few days.

According to the hospital neurologist, the ED work-up suggested either a TIA, stress-induced psychiatric speech disorder, or conversion disorder. The patient was discharged home in stable condition and was asked to follow up with the outpatient neurologist in 1 week.

Ms. M was seen approximately 2 weeks later in the outpatient neurology stroke ­clinic. Her symptoms had resolved, and she did not report any new or worsening symptoms. An outpatient stroke work-up was initiated, including magnetic resonance imaging (MRI) of the brain, echocardiography, and measurement of low-density lipoprotein and hemoglobin A1C; all results were unremarkable. Given the timeline for symptom improvement and results of the work-up, the patient was given a diagnosis of conversion disorder. Ms. M was encouraged to follow up with her primary care physician (PCP) for further medical management.

● How would you proceed with this patient?

* The patient’s name has been changed to protect her identity.

According to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, text revision, conversion disorder (also known as functional neurological symptom disorder) is characterized as a somatic symptom and related disorder.¹ The prominent feature shared among disorders in this category is the presence of somatic symptoms that are associated with distress and impairment.

In conversion disorder, the focus is on symptoms that are neurologic in nature but are not due to underlying neurologic disease and are incongruent with typical patterns of presentation for any neurologic condition. Patients with conversion disorder may present with motor symptoms (eg, weakness, paralysis, tremor, dystonia), altered sensory or cognitive function, seizure-like symptoms, alterations in speech, or changes in swallowing.^1,2

For a diagnosis of conversion disorder, the following criteria must be met¹:

• The patient has 1 or more symptoms of altered voluntary motor or sensory function.
• Symptom presentation is incongruent with recognized neurologic or medical disease or conditions.
• Symptoms are not better explained by another medical or mental health condition.
• There is significant distress or impairment in functioning due to symptoms or the deficit.

The etiology of conversion disorder has not been firmly established. While the literature suggests that psychological stressors play a role,^3,4 an effort also has been made to better understand the underlying neural and biological basis. Specifically, studies have utilized brain imaging to explore brain pathways and mechanisms that could account for symptom presentation.^5,6

Prevalence rates for conversion disorder vary depending on the population studied. While it is estimated that 5% of patients in a general hospital setting meet full criteria for conversion disorder,⁷ higher rates may exist in specialty settings; 1 study found that 30% of patients in a neurology specialty clinic exhibited symptoms that were medically unexplained.⁸

Continue to: In primary care...

One study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.

In primary care, prevalence of conversion disorder can be difficult to pinpoint; however, 1 study indicated that physicians identified medically unexplained symptoms as the main presenting problem for nearly 20% of patients in a primary care setting.⁹ Therefore, it is important for family physicians (FPs) to be familiar with the assessment and treatment of conversion disorder (and other disorders in which medically unexplained symptoms may be at the core of the patient presentation).

The differential: Neurologic and psychiatric conditions

Patients with conversion disorder may present with a variety of neurologic symptoms that can mimic those of organic disease. This can pose a diagnostic challenge, increase the chance of misdiagnosis, and delay treatment.

Motor symptoms may include paralysis, gait disturbance, dysphagia, or aphasia. Patients also may have sensory symptoms, such as blindness, deafness, or anesthesia.^10,11 As a result, it is important to rule out both urgent neurologic presentations, such as TIA, acute stroke, and brain tumor, and other chronic neurologic conditions, including multiple sclerosis, myasthenia gravis, and epilepsy.^11,12

Multiple sclerosis will demonstrate characteristic lesions on MRI that differentiate it from conversion disorder.

Myasthenia gravis is distinguished by positive findings on autoantibodies testing and on electrophysiologic studies.

Continue to: Epilepsy

Epilepsy. Patients with conversion disorder may present with unresponsiveness and abnormal movements, such as generalized limb shaking and hip thrusting, that mimic an epileptic seizure. In contrast to epileptic seizures, psychogenic nonepileptic seizures may last longer, symptoms may wax and wane, and patients generally do not have bowel or bladder incontinence or sustain injury as they would during an actual seizure.¹²

There are several psychiatric/­psychosocial conditions that also should be considered in the differential diagnosis of conversion disorder.

Somatic symptom disorder, like conversion disorder, produces somatic symptoms that can cause significant distress for patients. The difference in the 2 conditions is that symptoms of somatic symptom disorder may be compatible with a recognized neurologic or general medical condition, whereas in conversion disorder, the symptoms are not consistent with a recognized disease.^1,12

Factitious disorder, similar to conversion disorder, can involve neurologic symptoms that are not attributed to disease. However, patients with factitious disorder deliberately simulate symptoms to receive medical care. A thorough clinical interview and physical exam can help to distinguish conversion disorder from factitious disorder.

Malingering is not a psychiatric condition but a behavior that involves intentionally feigning symptoms for the purpose of personal or financial gain. There is no evidence that patients with conversion disorder simulate their symptoms.^12,13

Continue to: Negative results and positive signs point to the Dx

Negative results and positive signs point to the Dx

Conversion disorder is not a diagnosis of exclusion. Diagnosis requires detailed history taking and a thorough neurologic exam. Laboratory testing and neuroimaging are also important, and results will have to be negative to support the diagnosis.

Conversion disorder is not a diagnosis of exclusion; diagnosis requires detailed history taking and a thorough neurologic exam.

Neurologic deficits with conversion disorder do not follow a known neurologic insult.¹⁴ There are many tests that can be used to distinguish functional symptoms vs organic symptoms. Two of the most well-known tests are the Hoover sign and the abductor sign, which will be positive in conversion disorder. Both can be performed easily in an outpatient setting.

The Hoover sign is considered positive when there is weakness of voluntary hip extension in the presence of normal involuntary hip extension during contralateral hip flexion against resistance. According to a meta-­analysis of multiple studies of patients with conversion disorder, the overall estimated sensitivity of this test is 94% and the specificity, 99%.¹⁵

The abductor sign follows the same principle as the Hoover sign: When the patient abducts the nonparetic leg, both the nonparetic and “paretic” leg are strong. When the patient abducts just the “paretic” leg, both legs become weak.¹⁶

Other symptom evaluations. For patients who have functional seizures, video electroencephalography is helpful to distinguish functional seizures from “true” seizures.^17,18 In conversion disorder, functional dysarthria normally resembles a stutter or speech that is extremely slow with long hesitations that are hard to interrupt.¹⁸ Dysphonia and functional dysphagia are also very common functional symptoms. Usually after extensive work-up, no organic cause of the patient’s symptoms is ever found.¹⁸

Continue to: Treatment requires an integrated team approach

Treatment for conversion disorder can be difficult due to the complex and not fully understood etiology of the condition. Due to its multifaceted nature, an integrated team approach can be beneficial at each stage, including assessment and intervention.

Explain the diagnosis clearly. An essential initial step in the treatment of conversion disorder is careful explanation of the diagnosis. Clear explanation of the terminology and presentation of conversion disorder may prevent the patient from misinterpreting their diagnosis as a suggestion that they are feigning or malingering symptoms or feeling that their symptoms or concerns are being dismissed.² Understanding the condition can help improve the likelihood of the patient accepting the treatment plan and help decrease the likelihood of unnecessary testing, health care visits, and consultations. Developing a strong rapport with the patient is key when explaining the diagnosis.

Recommend cognitive behavioral therapy (CBT). In a meta-analysis of 15 randomized controlled trials, CBT significantly reduced somatic, anxious, and depressive symptoms and improved physical functioning in patients with somatoform disorders and medically unexplained symptoms.¹⁹ Another study, utilizing a case series, demonstrated significant improvement in social, emotional, and behavioral functioning in children and adolescents with functional neurologic symptoms (conversion disorder) post–CBT intervention.²⁰

Given that research supports CBT’s effectiveness in the management of conversion disorder, it is beneficial to engage a behavioral health professional as a part of the treatment team to focus on factors such as stress management, development of coping skills, and treatment of underlying psychiatric conditions.

Consider these other options. The addition of medication management can be considered for patients with comorbid psychiatric disorders. Evidence suggests that physical therapy is helpful in the treatment of motor and gait dysfunction seen in conversion disorder.^21,22 The role of hypnosis in the management of conversion disorder has also been studied, but more randomized clinical trials are needed to further explore this treatment.^2,23,24

Continue to: The FP's role in coordination of care

The FP’s role in coordination of care

Conversion disorder can be challenging to diagnose and often involves a multidisciplinary approach. Patients with conversion disorder may see multiple clinicians as they undergo evaluation for their symptoms, but they usually are referred back to their PCP for management and coordination of care. Thus, the FP’s understanding of how the condition is diagnosed and appropriately managed is beneficial.

The prognosis of conversion disorder is associated with symptom duration; thus, consultation between FPs and mental health providers is essential.

Open and effective communication among all members of the health care team can ensure consistency in treatment, a strong patient–provider relationship, favorable prognosis, and prevention of symptom relapse. FPs, by establishing a good rapport with patients, can help them understand the condition and the mind-body connection. Once other diagnoses have been ruled out, the FP can provide reassurance to patients and minimize further diagnostic testing.

The prognosis of conversion disorder is associated with symptom duration²⁵; thus, consultation between FPs and mental health providers is essential. The FP also can be integral in the recognition of psychiatric comorbidities, such as anxiety and depression, helping to ensure that these conditions also are treated appropriately.^25,26

THE CASE

Ms. M was referred to a neuropsychologist for further assessment, and the diagnosis of conversion disorder was confirmed. She was then referred to a family medicine behavioral health psychologist for CBT. The initial consult indicated that psychological stressors were contributing to symptoms, and Ms. M was diagnosed with depression and anxiety as well as conversion disorder.

Treatment started with patient education. The treatment framework was carefully explained to Ms. M, with a focus on identifying possible symptom triggers, helping her build a more effective stress response, increasing skills to more effectively manage stressors, and managing underlying psychiatric disorders (ie, depression, anxiety).

Ms. M continued regular visits with the family medicine behavioral health psychologist for CBT and followed up with her PCP as needed to manage chronic health conditions and stroke risk factors. The patient was able to implement skills discussed in treatment sessions, including identifying triggers and implementing coping skills (eg, managing negative thoughts that contribute to symptoms, setting boundaries) to manage stressors.

Her depressive and anxious symptoms improved, as indicated by symptom measurement tools and self-report. The frequency and severity of episodes of slurred speech and muscle weakness decreased, and the patient reported only 1 ED visit related to speech difficulties in the 2 years while following up with the behavioral health psychologist.

CORRESPONDENCE
Kristen J. Alston, PhD, University of Mississippi Medical Center, 2400 North State Street, Jackson, MS 39216; [email protected]