Mitch Kovacs

Case

A 39-year-old woman presented to the ED with a gradual onset of headache, which she rated a “2” on a pain scale of 0 to 10; tingling in the hands bilaterally; and floaters in her field of vision. She also stated that she had experienced slurred speech that lasted for approximately 5 minutes, resolving prior to her arrival. Several months earlier, the patient had been admitted to a tertiary care center where she was diagnosed with superior sagittal sinus (SSS) thrombosis and left frontal cerebral hemorrhage. During this inpatient visit, a thrombophilia workup revealed a homozygous 4G/4G genotype.

The patient’s past medical history included one miscarriage, as well as a papillary thyroid carcinoma with resection, which was discovered a few months before her presentation to the ED and after diagnosis of the initial SSS thrombosis.

Physical examination revealed a well-developed, mildly obese female. On arrival at the ED, the patient’s National Institutes of Health Stroke Scale score was 0. Her vital signs and ocular, neurological, and psychiatric examinations were all normal. The social history was negative for tobacco or alcohol use, and she had no family history of deep vein thrombosis (DVT) or pulmonary embolism.

A noncontrast computed tomography (CT) of the head demonstrated a hemorrhagic venous infarction involving the posterior right parietal lobe. Intracranial magnetic resonance venography (MRV) and brain magnetic resonance imaging (MRI) revealed thrombosis of the posterior third of the SSS as the source of the infarction. This sinus had been patent during the patient’s previous hospital admissions.

The patient’s international normalized ratio (INR) was therapeutic on presentation. Warfarin was discontinued, and she was started on an intravenous (IV) heparin drip. For anticoagulation, she was prescribed 20 mg rivaroxaban daily and 2,000 mg levetiracetam daily.

One week after discharge, the patient again presented to the ED with a recurrence of symptoms, including confusion, slurred speech, and headache, which she rated a “5” on a pain scale of 0 to 10. Similar to the previous ED visit, the slurred speech had resolved by the time of examination. The patient did not exhibit facial asymmetry but did complain of bilateral numbness and tingling in both hands. A noncontrast CT of the head showed no changes in the right parietal hemorrhagic venous infarct and intraparenchymal hemorrhage; however, there was an interval increase in edema compared to the prior CT. Rivaroxaban and levetiracetam were continued, and 20 mg simvastatin daily was prescribed.

Overview

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging. In 70% of cases, the SSS and lateral sinuses are individually involved, and in 30% of cases, both regions are affected simultaneously.¹ Only recently have clinicians been able to diagnose this condition antemortem.

Risk Factors and Etiology

Inherited and Acquired hypercoagulable states

Cerebral venous sinus thrombosis (CVST) and cerebrovascular accident (CVA) often result from a hypercoagulable state (HCS), and both acquired and inherited factors place patients at risk. Inherited factors are the most common cause of venous thromboembolism in patients younger than age 40 years. Acquired factors have a combined effect with inherited ones, leading to increased risk of CVST or CVA.²

The patient in this case possessed both acquired and inherited factors of an HCS. Inherited factors can be found through a thrombophilia evaluation. In general, acquired factors of thrombophilia include obesity, a prior history of thrombosis, pregnancy, and cancer and its treatment. A thrombophilia evaluation revealed the patient was homozygous for the 4G allele, which has been shown to increase concentration of plasminogen activator inhibitor (PAI-1) by 30%. An inhibitor to the pathway of fibrinolysis, PAI-1 is a major factor preventing the excessive presence and magnitude of blood clots.³

Pregnancy and the Puerperium

Cerebral vascular sinus thrombosis is most commonly seen in young to middle-aged women. High risk factors include pregnancy and the puerperium due to increased HCS during these periods.⁴ The incidence of CVST in this population is approximately 10 per 100,000 women.⁴

Oral Hormonal Contraceptives

In approximately 10% of CVST cases, oral hormonal contraceptive use in the presence of a coagulation disorder are frequently the cause—as observed in the incidence of DVT in this patient population.

Septic Cerebral Venous Sinus Thrombosis

Septic CVST occurs mainly in children and up to 18% of adult cases in developing countries. It is associated with localized infections (eg, mastoiditis, otitis media, sinusitis, meningitis).

Other Causes

Although rare, other causes of CVST include intracranial hypotension, hydrocephalus, and the use of certain drugs and supplements (eg, corticosteroids, high doses of vitamin A). Each of these potential causes also should be considered when evaluating for CVST.⁴

 

Symptoms and Signs

Common symptoms and signs of CVST include headache, nausea, vomiting, seizure, and focal neurological deficit. Papilledema is present in 40% of cases, primarily in patients with delayed diagnosis or a chronic course.

Neurological Deficits

Cerebral venous sinus thrombosis may not necessarily cause focal neurological deficits due to numerous pathways of venous drainage and the possibility of reversal of venous blood flow. However, the condition can lead to impaired resorption of CSF causing intracranial hypertension.

Headache

In 70% of cases, headache is the initial symptom of CVST, and it is the only symptom in 16% of cases. With respect to headache presentation, it is important to remember that thunderclap headache is not exclusive to the diagnosis of subarachnoid hemorrhage (SAH). The absence of findings on workup to support the diagnosis of SAH should prompt investigation with MRV and evaluation of CVST.⁴

Seizure

Focal or generalized seizure on initial presentation occurs in 30% to 40% of cases of CVST. When smaller cerebral veins are involved, this can lead to focal edema, neurological deficits, venous infarction, and seizure. Focal deficits are determined by the localization of CVST and associated lesions. Other symptoms may include migraine headache, transient ischemic attack, cranial nerve palsies, and subarachnoid hemorrhage.⁴

Complications

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging.

Complications in patients with CVST occur when venous congestion increases and raises dural venous sinus and cerebral spinal fluid (CSF) pressure. Parenchymal edema with venous infarction and hemorrhage complicates up to 50% of venous sinus thromboses (as seen in this patient).¹ Unfortunately, little is known about long-term risk outcomes or recurrence of CVST.¹

As previously noted, the patient presented with transient slurred speech, mild headache, and bilateral hand tingling. On workup, she was found to have an SSS thrombosis with an associated right intraparenchymal hemorrhage that occurred despite therapeutic INR levels and the initiation of coumadin therapy prior to admission.

Evaluation and Diagnosis

D-Dimer Evaluation

There is a strong association between D-dimer levels above 500 ng/mL and acute CVST. Nevertheless, lower levels do not rule out the diagnosis in a patient presenting with headache.⁴

Imaging Techniques

Important imaging techniques in the evaluation of CVST include CT, MRV, MRI, and magnetic resonance angiography (MRA). The first imaging modality in evaluating a patient with neurological symptoms and headache in the ED is CT, which can show evidence of an infarction that does not respond to an arterial distribution. In the absence of a hemorrhagic component, however, infarct demonstration may be delayed for up to 72 hours.⁵ On contrast CT, an empty delta sign may be apparent due to enhancement of the collateral veins in the SSS walls surrounding a nonenhanced thrombus. The delta sign is not frequently present and may be false due to early division of the SSS.⁵

Computed tomography venography, CT angiography, and MRI can also be utilized to evaluate for CVST. Computed tomography venography is especially useful in identifying the cerebral veins and dural sinuses,⁶ and MRV is an excellent method for visualizing the dural venous sinuses and larger cerebral veins. Single-slice phase-contrast angiography is also a rapid and reliable test for CVST.⁷ Conventional angiography and direct venography should be considered if MR studies are nondiagnostic; however, this test is invasive with associated risks.⁵

Treatment

Heparin therapy should be initiated in patients presenting with dural sinus thrombosis even if pre-existing hemorrhage exists. Patients failing to respond to therapy with worsening neurological deficits may warrant local thrombolysis with tissue plasminogen activator. Identifying those in the acute state of disease is essential as they may have poor prognostic outcomes that may warrant more invasive intervention.¹

Conclusion

Cerebral venous sinus thrombosis is a rare condition with a diverse clinical presentation. As demonstrated in this case, some patients present with stroke-like symptoms of nontraumatic headache, slurred speech, and bilateral hand tingling, which, on workup, reveal SSS thrombosis associated right intraparenchymal hemorrhage.

This case draws attention to the importance of risk stratification in patients with a history of HCS and neurological complaints presenting to the ED. Dural sinus thrombosis may have a vague initial neurological presentation; therefore, early recognition and initiation of therapy will assist in reducing morbidity and mortality.

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.

Case

A 39-year-old woman presented to the ED with a gradual onset of headache, which she rated a “2” on a pain scale of 0 to 10; tingling in the hands bilaterally; and floaters in her field of vision. She also stated that she had experienced slurred speech that lasted for approximately 5 minutes, resolving prior to her arrival. Several months earlier, the patient had been admitted to a tertiary care center where she was diagnosed with superior sagittal sinus (SSS) thrombosis and left frontal cerebral hemorrhage. During this inpatient visit, a thrombophilia workup revealed a homozygous 4G/4G genotype.

The patient’s past medical history included one miscarriage, as well as a papillary thyroid carcinoma with resection, which was discovered a few months before her presentation to the ED and after diagnosis of the initial SSS thrombosis.

Physical examination revealed a well-developed, mildly obese female. On arrival at the ED, the patient’s National Institutes of Health Stroke Scale score was 0. Her vital signs and ocular, neurological, and psychiatric examinations were all normal. The social history was negative for tobacco or alcohol use, and she had no family history of deep vein thrombosis (DVT) or pulmonary embolism.

A noncontrast computed tomography (CT) of the head demonstrated a hemorrhagic venous infarction involving the posterior right parietal lobe. Intracranial magnetic resonance venography (MRV) and brain magnetic resonance imaging (MRI) revealed thrombosis of the posterior third of the SSS as the source of the infarction. This sinus had been patent during the patient’s previous hospital admissions.

The patient’s international normalized ratio (INR) was therapeutic on presentation. Warfarin was discontinued, and she was started on an intravenous (IV) heparin drip. For anticoagulation, she was prescribed 20 mg rivaroxaban daily and 2,000 mg levetiracetam daily.

One week after discharge, the patient again presented to the ED with a recurrence of symptoms, including confusion, slurred speech, and headache, which she rated a “5” on a pain scale of 0 to 10. Similar to the previous ED visit, the slurred speech had resolved by the time of examination. The patient did not exhibit facial asymmetry but did complain of bilateral numbness and tingling in both hands. A noncontrast CT of the head showed no changes in the right parietal hemorrhagic venous infarct and intraparenchymal hemorrhage; however, there was an interval increase in edema compared to the prior CT. Rivaroxaban and levetiracetam were continued, and 20 mg simvastatin daily was prescribed.

Overview

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging. In 70% of cases, the SSS and lateral sinuses are individually involved, and in 30% of cases, both regions are affected simultaneously.¹ Only recently have clinicians been able to diagnose this condition antemortem.

Risk Factors and Etiology

Inherited and Acquired hypercoagulable states

Cerebral venous sinus thrombosis (CVST) and cerebrovascular accident (CVA) often result from a hypercoagulable state (HCS), and both acquired and inherited factors place patients at risk. Inherited factors are the most common cause of venous thromboembolism in patients younger than age 40 years. Acquired factors have a combined effect with inherited ones, leading to increased risk of CVST or CVA.²

The patient in this case possessed both acquired and inherited factors of an HCS. Inherited factors can be found through a thrombophilia evaluation. In general, acquired factors of thrombophilia include obesity, a prior history of thrombosis, pregnancy, and cancer and its treatment. A thrombophilia evaluation revealed the patient was homozygous for the 4G allele, which has been shown to increase concentration of plasminogen activator inhibitor (PAI-1) by 30%. An inhibitor to the pathway of fibrinolysis, PAI-1 is a major factor preventing the excessive presence and magnitude of blood clots.³

Pregnancy and the Puerperium

Cerebral vascular sinus thrombosis is most commonly seen in young to middle-aged women. High risk factors include pregnancy and the puerperium due to increased HCS during these periods.⁴ The incidence of CVST in this population is approximately 10 per 100,000 women.⁴

Oral Hormonal Contraceptives

In approximately 10% of CVST cases, oral hormonal contraceptive use in the presence of a coagulation disorder are frequently the cause—as observed in the incidence of DVT in this patient population.

Septic Cerebral Venous Sinus Thrombosis

Septic CVST occurs mainly in children and up to 18% of adult cases in developing countries. It is associated with localized infections (eg, mastoiditis, otitis media, sinusitis, meningitis).

Other Causes

Although rare, other causes of CVST include intracranial hypotension, hydrocephalus, and the use of certain drugs and supplements (eg, corticosteroids, high doses of vitamin A). Each of these potential causes also should be considered when evaluating for CVST.⁴

 

Symptoms and Signs

Common symptoms and signs of CVST include headache, nausea, vomiting, seizure, and focal neurological deficit. Papilledema is present in 40% of cases, primarily in patients with delayed diagnosis or a chronic course.

Neurological Deficits

Cerebral venous sinus thrombosis may not necessarily cause focal neurological deficits due to numerous pathways of venous drainage and the possibility of reversal of venous blood flow. However, the condition can lead to impaired resorption of CSF causing intracranial hypertension.

Headache

In 70% of cases, headache is the initial symptom of CVST, and it is the only symptom in 16% of cases. With respect to headache presentation, it is important to remember that thunderclap headache is not exclusive to the diagnosis of subarachnoid hemorrhage (SAH). The absence of findings on workup to support the diagnosis of SAH should prompt investigation with MRV and evaluation of CVST.⁴

Seizure

Focal or generalized seizure on initial presentation occurs in 30% to 40% of cases of CVST. When smaller cerebral veins are involved, this can lead to focal edema, neurological deficits, venous infarction, and seizure. Focal deficits are determined by the localization of CVST and associated lesions. Other symptoms may include migraine headache, transient ischemic attack, cranial nerve palsies, and subarachnoid hemorrhage.⁴

Complications

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging.

Complications in patients with CVST occur when venous congestion increases and raises dural venous sinus and cerebral spinal fluid (CSF) pressure. Parenchymal edema with venous infarction and hemorrhage complicates up to 50% of venous sinus thromboses (as seen in this patient).¹ Unfortunately, little is known about long-term risk outcomes or recurrence of CVST.¹

As previously noted, the patient presented with transient slurred speech, mild headache, and bilateral hand tingling. On workup, she was found to have an SSS thrombosis with an associated right intraparenchymal hemorrhage that occurred despite therapeutic INR levels and the initiation of coumadin therapy prior to admission.

Evaluation and Diagnosis

D-Dimer Evaluation

There is a strong association between D-dimer levels above 500 ng/mL and acute CVST. Nevertheless, lower levels do not rule out the diagnosis in a patient presenting with headache.⁴

Imaging Techniques

Important imaging techniques in the evaluation of CVST include CT, MRV, MRI, and magnetic resonance angiography (MRA). The first imaging modality in evaluating a patient with neurological symptoms and headache in the ED is CT, which can show evidence of an infarction that does not respond to an arterial distribution. In the absence of a hemorrhagic component, however, infarct demonstration may be delayed for up to 72 hours.⁵ On contrast CT, an empty delta sign may be apparent due to enhancement of the collateral veins in the SSS walls surrounding a nonenhanced thrombus. The delta sign is not frequently present and may be false due to early division of the SSS.⁵

Computed tomography venography, CT angiography, and MRI can also be utilized to evaluate for CVST. Computed tomography venography is especially useful in identifying the cerebral veins and dural sinuses,⁶ and MRV is an excellent method for visualizing the dural venous sinuses and larger cerebral veins. Single-slice phase-contrast angiography is also a rapid and reliable test for CVST.⁷ Conventional angiography and direct venography should be considered if MR studies are nondiagnostic; however, this test is invasive with associated risks.⁵

Treatment

Heparin therapy should be initiated in patients presenting with dural sinus thrombosis even if pre-existing hemorrhage exists. Patients failing to respond to therapy with worsening neurological deficits may warrant local thrombolysis with tissue plasminogen activator. Identifying those in the acute state of disease is essential as they may have poor prognostic outcomes that may warrant more invasive intervention.¹

Conclusion

Cerebral venous sinus thrombosis is a rare condition with a diverse clinical presentation. As demonstrated in this case, some patients present with stroke-like symptoms of nontraumatic headache, slurred speech, and bilateral hand tingling, which, on workup, reveal SSS thrombosis associated right intraparenchymal hemorrhage.

This case draws attention to the importance of risk stratification in patients with a history of HCS and neurological complaints presenting to the ED. Dural sinus thrombosis may have a vague initial neurological presentation; therefore, early recognition and initiation of therapy will assist in reducing morbidity and mortality.

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.

Case

A 39-year-old woman presented to the ED with a gradual onset of headache, which she rated a “2” on a pain scale of 0 to 10; tingling in the hands bilaterally; and floaters in her field of vision. She also stated that she had experienced slurred speech that lasted for approximately 5 minutes, resolving prior to her arrival. Several months earlier, the patient had been admitted to a tertiary care center where she was diagnosed with superior sagittal sinus (SSS) thrombosis and left frontal cerebral hemorrhage. During this inpatient visit, a thrombophilia workup revealed a homozygous 4G/4G genotype.

The patient’s past medical history included one miscarriage, as well as a papillary thyroid carcinoma with resection, which was discovered a few months before her presentation to the ED and after diagnosis of the initial SSS thrombosis.

Physical examination revealed a well-developed, mildly obese female. On arrival at the ED, the patient’s National Institutes of Health Stroke Scale score was 0. Her vital signs and ocular, neurological, and psychiatric examinations were all normal. The social history was negative for tobacco or alcohol use, and she had no family history of deep vein thrombosis (DVT) or pulmonary embolism.

A noncontrast computed tomography (CT) of the head demonstrated a hemorrhagic venous infarction involving the posterior right parietal lobe. Intracranial magnetic resonance venography (MRV) and brain magnetic resonance imaging (MRI) revealed thrombosis of the posterior third of the SSS as the source of the infarction. This sinus had been patent during the patient’s previous hospital admissions.

The patient’s international normalized ratio (INR) was therapeutic on presentation. Warfarin was discontinued, and she was started on an intravenous (IV) heparin drip. For anticoagulation, she was prescribed 20 mg rivaroxaban daily and 2,000 mg levetiracetam daily.

One week after discharge, the patient again presented to the ED with a recurrence of symptoms, including confusion, slurred speech, and headache, which she rated a “5” on a pain scale of 0 to 10. Similar to the previous ED visit, the slurred speech had resolved by the time of examination. The patient did not exhibit facial asymmetry but did complain of bilateral numbness and tingling in both hands. A noncontrast CT of the head showed no changes in the right parietal hemorrhagic venous infarct and intraparenchymal hemorrhage; however, there was an interval increase in edema compared to the prior CT. Rivaroxaban and levetiracetam were continued, and 20 mg simvastatin daily was prescribed.

Overview

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging. In 70% of cases, the SSS and lateral sinuses are individually involved, and in 30% of cases, both regions are affected simultaneously.¹ Only recently have clinicians been able to diagnose this condition antemortem.

Risk Factors and Etiology

Inherited and Acquired hypercoagulable states

Cerebral venous sinus thrombosis (CVST) and cerebrovascular accident (CVA) often result from a hypercoagulable state (HCS), and both acquired and inherited factors place patients at risk. Inherited factors are the most common cause of venous thromboembolism in patients younger than age 40 years. Acquired factors have a combined effect with inherited ones, leading to increased risk of CVST or CVA.²

The patient in this case possessed both acquired and inherited factors of an HCS. Inherited factors can be found through a thrombophilia evaluation. In general, acquired factors of thrombophilia include obesity, a prior history of thrombosis, pregnancy, and cancer and its treatment. A thrombophilia evaluation revealed the patient was homozygous for the 4G allele, which has been shown to increase concentration of plasminogen activator inhibitor (PAI-1) by 30%. An inhibitor to the pathway of fibrinolysis, PAI-1 is a major factor preventing the excessive presence and magnitude of blood clots.³

Pregnancy and the Puerperium

Cerebral vascular sinus thrombosis is most commonly seen in young to middle-aged women. High risk factors include pregnancy and the puerperium due to increased HCS during these periods.⁴ The incidence of CVST in this population is approximately 10 per 100,000 women.⁴

Oral Hormonal Contraceptives

In approximately 10% of CVST cases, oral hormonal contraceptive use in the presence of a coagulation disorder are frequently the cause—as observed in the incidence of DVT in this patient population.

Septic Cerebral Venous Sinus Thrombosis

Septic CVST occurs mainly in children and up to 18% of adult cases in developing countries. It is associated with localized infections (eg, mastoiditis, otitis media, sinusitis, meningitis).

Other Causes

Although rare, other causes of CVST include intracranial hypotension, hydrocephalus, and the use of certain drugs and supplements (eg, corticosteroids, high doses of vitamin A). Each of these potential causes also should be considered when evaluating for CVST.⁴

 

Symptoms and Signs

Common symptoms and signs of CVST include headache, nausea, vomiting, seizure, and focal neurological deficit. Papilledema is present in 40% of cases, primarily in patients with delayed diagnosis or a chronic course.

Neurological Deficits

Cerebral venous sinus thrombosis may not necessarily cause focal neurological deficits due to numerous pathways of venous drainage and the possibility of reversal of venous blood flow. However, the condition can lead to impaired resorption of CSF causing intracranial hypertension.

Headache

In 70% of cases, headache is the initial symptom of CVST, and it is the only symptom in 16% of cases. With respect to headache presentation, it is important to remember that thunderclap headache is not exclusive to the diagnosis of subarachnoid hemorrhage (SAH). The absence of findings on workup to support the diagnosis of SAH should prompt investigation with MRV and evaluation of CVST.⁴

Seizure

Focal or generalized seizure on initial presentation occurs in 30% to 40% of cases of CVST. When smaller cerebral veins are involved, this can lead to focal edema, neurological deficits, venous infarction, and seizure. Focal deficits are determined by the localization of CVST and associated lesions. Other symptoms may include migraine headache, transient ischemic attack, cranial nerve palsies, and subarachnoid hemorrhage.⁴

Complications

Cerebral venous sinus thrombosis is a rare condition with an often varied clinical presentation—the symptoms of which can take hours to weeks to evolve, thus making the diagnosis challenging.

Complications in patients with CVST occur when venous congestion increases and raises dural venous sinus and cerebral spinal fluid (CSF) pressure. Parenchymal edema with venous infarction and hemorrhage complicates up to 50% of venous sinus thromboses (as seen in this patient).¹ Unfortunately, little is known about long-term risk outcomes or recurrence of CVST.¹

As previously noted, the patient presented with transient slurred speech, mild headache, and bilateral hand tingling. On workup, she was found to have an SSS thrombosis with an associated right intraparenchymal hemorrhage that occurred despite therapeutic INR levels and the initiation of coumadin therapy prior to admission.

Evaluation and Diagnosis

D-Dimer Evaluation

There is a strong association between D-dimer levels above 500 ng/mL and acute CVST. Nevertheless, lower levels do not rule out the diagnosis in a patient presenting with headache.⁴

Imaging Techniques

Important imaging techniques in the evaluation of CVST include CT, MRV, MRI, and magnetic resonance angiography (MRA). The first imaging modality in evaluating a patient with neurological symptoms and headache in the ED is CT, which can show evidence of an infarction that does not respond to an arterial distribution. In the absence of a hemorrhagic component, however, infarct demonstration may be delayed for up to 72 hours.⁵ On contrast CT, an empty delta sign may be apparent due to enhancement of the collateral veins in the SSS walls surrounding a nonenhanced thrombus. The delta sign is not frequently present and may be false due to early division of the SSS.⁵

Computed tomography venography, CT angiography, and MRI can also be utilized to evaluate for CVST. Computed tomography venography is especially useful in identifying the cerebral veins and dural sinuses,⁶ and MRV is an excellent method for visualizing the dural venous sinuses and larger cerebral veins. Single-slice phase-contrast angiography is also a rapid and reliable test for CVST.⁷ Conventional angiography and direct venography should be considered if MR studies are nondiagnostic; however, this test is invasive with associated risks.⁵

Treatment

Heparin therapy should be initiated in patients presenting with dural sinus thrombosis even if pre-existing hemorrhage exists. Patients failing to respond to therapy with worsening neurological deficits may warrant local thrombolysis with tissue plasminogen activator. Identifying those in the acute state of disease is essential as they may have poor prognostic outcomes that may warrant more invasive intervention.¹

Conclusion

Cerebral venous sinus thrombosis is a rare condition with a diverse clinical presentation. As demonstrated in this case, some patients present with stroke-like symptoms of nontraumatic headache, slurred speech, and bilateral hand tingling, which, on workup, reveal SSS thrombosis associated right intraparenchymal hemorrhage.

This case draws attention to the importance of risk stratification in patients with a history of HCS and neurological complaints presenting to the ED. Dural sinus thrombosis may have a vague initial neurological presentation; therefore, early recognition and initiation of therapy will assist in reducing morbidity and mortality.

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.

Case

A 22-year-old white man presented to the ED after waking from sleep and experiencing painless bilateral lower extremity weakness that ascended to the right upper extremity. This weakness left the patient unable to walk. He denied incontinence and was otherwise healthy with no prior history of similar symptoms. A family history of the presenting symptomatology was denied, and there was no family history of diabetes or kidney disease. The patient did state he had upper respiratory symptoms with a sore throat and runny nose 2 weeks earlier. He denied use of tobacco, alcohol, and illicit drugs.

Physical examination revealed abnormal deep tendon reflexes, absent patellar and achilles reflexes on the right side, reduced patellar and achilles reflexes of the left side, and diminished bilateral brachioradialis reflexes. Examination of the lower extremities revealed motor strength at 2/5 on the left and 1/5 on the right. His upper extremities demonstrated bilateral 4/5 motor strength. No ptosis was observed. All pulses of the extremities were normal with good capillary refill. His vital signs were: blood pressure, 131/75 mm Hg; pulse, 75 beats/minute; respiratory rate, 18 breaths/minute; and temperature, 97.2°F

Brain and cervical spine magnetic resonance imaging, computed tomography of the head, and blood cultures were ordered; the results of each were unremarkable. A lumbar puncture (LP) was attempted in the ED, but was unsuccessful due to difficulty in patient positioning secondary to profound weakness. An LP was later succesfully performed under fluoroscopy and revealed no abnormal findings. An electrocardiogram (ECG) demonstrated normal sinus rhythm with ST-segment flattening. The differential diagnosis for rapidly ascending weakness included Guillain-Barré syndrome, transverse myelitis, toxic metabolic syndromes, and myelopathies.

A basic metabolic panel showed that the patient was profoundly hypokalemic, with a potassium level of 1.9 mEq/L. After further blood analysis, mild hypomagnesemia was observed, with a level of 1.4 mEq/L. Whle in the ED, he was given 40 mEq potassium chloride intravenously (IV) and orally, and 2 g IV magnesium sulfate in the ED. The patient was evaluated by a neurologist in the ED. No respiratory compromise was present and the patient was admitted to neurology service with consultations from both nephrology and endocrinology.

Within 12 hours of starting potassium and magnesium supplementation, the patient’s potassium and magnesium levels returned to normal and his weakness concurrently subsided. The final diagnosis of the patient was hypokalemic periodic paralysis.

Discussion

Hypokalemic periodic paralysis (HPP), a rare autosomal dominant disorder with a prevalence of one in 100,000, is characterized by muscle weakness with accompanying hypokalemia. The first onset of symptoms is usually in the first or second decade of life, with a quarter of cases presenting before the age 10 years, and half before age 16 years. Being a typically-inherited autosomal dominant disorder, approximately two-thirds of cases present with a family history—unlike this patient case in which there was no family history of the disease. It is three to four times more common in men than women.

Hypokalemic periodic paralysis typically occurs upon waking from sleep or during rest following exercise. It may also be triggered by high-carbohydrate or high-salt meals, or from alcohol consumption. The muscles of the extremeties are usually affected, while those of the eyes, face, and sphincters are typically spared. If untreated, an attack can last from several hours to several days. Tendon and cutaneous reflexes can also be reduced or absent.¹ The manifestation of HPP can, at first impression, lead the differential diagnosis in the direction of the many other conditions that cause weakness of the extremities. Guillain-Barré syndrome and transverse myelitis are the more common diagnoses for which HPP can be mistaken. It is therefore important that the EP consider HPP in the differential so that an unusual presentation such as the one in this case may be diagnosed.

When presented with extraneous complications (eg, when the condition is encountered perioperatively or suffered comorbidly with similar conditions such as Guillain-Barré syndrome), HPP can be especially difficult to recognize.^2,3 Although a relatively uncommon condition, it can be potentially life-threatening if not recognized and treated appropriately. Severe hypokalemia may cause sequela such as respiratory failure and cardiac arhythmia. Thus, ECG and cardiorespiratory monitoring are essential in patients with HPP and hypokalemia as very severe hypokalemia may also cause paralysis of bulbar and cranial nerve musculature. Electocardiographic changes are common, and may include ST-segment sagging and flattening, U waves, and T-wave inversion. Other causes in patients presenting with HPP must also be considered, as hypokalemic paralysis of a sporadic nature may be associated with conditions including barium poisoning, hyperthyroidism, renal disorders, certain endocrinopathies, and gastrointestinal potassium losses.⁴

 

The pathogenesis of HPP is not completely understood; however, alteration in potassium regulation are well documented. Treatment of HPP includes both oral and IV potassium supplementation. Prophylaxis against recurrent attacks has been successful with various modalities including spiranolactone daily and acetazolamide.⁴ Care must be taken to consider thyrotoxic periodic paralysis, which most commonly presents in Asian men, as hyperthyroid symptoms may be subtle. Treatment focuses on reversal of the thyrotoxic state. β-Adrenergic blocking agents reduce the frequency and severity of attacks and should be started while measures to control thyrotoxicosis are being instituted.⁴

Before a diagnosis of HPP is made, other causes of hypokalemic paralysis must first be excluded.

Conclusion

This case is important because it demonstrates an unusual presentation of HPP in an emergency setting. This perspective of HPP can help the EP in recognizing and differentiating the condsition from similar disorders. 

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.

Case

A 22-year-old white man presented to the ED after waking from sleep and experiencing painless bilateral lower extremity weakness that ascended to the right upper extremity. This weakness left the patient unable to walk. He denied incontinence and was otherwise healthy with no prior history of similar symptoms. A family history of the presenting symptomatology was denied, and there was no family history of diabetes or kidney disease. The patient did state he had upper respiratory symptoms with a sore throat and runny nose 2 weeks earlier. He denied use of tobacco, alcohol, and illicit drugs.

Physical examination revealed abnormal deep tendon reflexes, absent patellar and achilles reflexes on the right side, reduced patellar and achilles reflexes of the left side, and diminished bilateral brachioradialis reflexes. Examination of the lower extremities revealed motor strength at 2/5 on the left and 1/5 on the right. His upper extremities demonstrated bilateral 4/5 motor strength. No ptosis was observed. All pulses of the extremities were normal with good capillary refill. His vital signs were: blood pressure, 131/75 mm Hg; pulse, 75 beats/minute; respiratory rate, 18 breaths/minute; and temperature, 97.2°F

Brain and cervical spine magnetic resonance imaging, computed tomography of the head, and blood cultures were ordered; the results of each were unremarkable. A lumbar puncture (LP) was attempted in the ED, but was unsuccessful due to difficulty in patient positioning secondary to profound weakness. An LP was later succesfully performed under fluoroscopy and revealed no abnormal findings. An electrocardiogram (ECG) demonstrated normal sinus rhythm with ST-segment flattening. The differential diagnosis for rapidly ascending weakness included Guillain-Barré syndrome, transverse myelitis, toxic metabolic syndromes, and myelopathies.

A basic metabolic panel showed that the patient was profoundly hypokalemic, with a potassium level of 1.9 mEq/L. After further blood analysis, mild hypomagnesemia was observed, with a level of 1.4 mEq/L. Whle in the ED, he was given 40 mEq potassium chloride intravenously (IV) and orally, and 2 g IV magnesium sulfate in the ED. The patient was evaluated by a neurologist in the ED. No respiratory compromise was present and the patient was admitted to neurology service with consultations from both nephrology and endocrinology.

Within 12 hours of starting potassium and magnesium supplementation, the patient’s potassium and magnesium levels returned to normal and his weakness concurrently subsided. The final diagnosis of the patient was hypokalemic periodic paralysis.

Discussion

Hypokalemic periodic paralysis (HPP), a rare autosomal dominant disorder with a prevalence of one in 100,000, is characterized by muscle weakness with accompanying hypokalemia. The first onset of symptoms is usually in the first or second decade of life, with a quarter of cases presenting before the age 10 years, and half before age 16 years. Being a typically-inherited autosomal dominant disorder, approximately two-thirds of cases present with a family history—unlike this patient case in which there was no family history of the disease. It is three to four times more common in men than women.

Hypokalemic periodic paralysis typically occurs upon waking from sleep or during rest following exercise. It may also be triggered by high-carbohydrate or high-salt meals, or from alcohol consumption. The muscles of the extremeties are usually affected, while those of the eyes, face, and sphincters are typically spared. If untreated, an attack can last from several hours to several days. Tendon and cutaneous reflexes can also be reduced or absent.¹ The manifestation of HPP can, at first impression, lead the differential diagnosis in the direction of the many other conditions that cause weakness of the extremities. Guillain-Barré syndrome and transverse myelitis are the more common diagnoses for which HPP can be mistaken. It is therefore important that the EP consider HPP in the differential so that an unusual presentation such as the one in this case may be diagnosed.

When presented with extraneous complications (eg, when the condition is encountered perioperatively or suffered comorbidly with similar conditions such as Guillain-Barré syndrome), HPP can be especially difficult to recognize.^2,3 Although a relatively uncommon condition, it can be potentially life-threatening if not recognized and treated appropriately. Severe hypokalemia may cause sequela such as respiratory failure and cardiac arhythmia. Thus, ECG and cardiorespiratory monitoring are essential in patients with HPP and hypokalemia as very severe hypokalemia may also cause paralysis of bulbar and cranial nerve musculature. Electocardiographic changes are common, and may include ST-segment sagging and flattening, U waves, and T-wave inversion. Other causes in patients presenting with HPP must also be considered, as hypokalemic paralysis of a sporadic nature may be associated with conditions including barium poisoning, hyperthyroidism, renal disorders, certain endocrinopathies, and gastrointestinal potassium losses.⁴

 

The pathogenesis of HPP is not completely understood; however, alteration in potassium regulation are well documented. Treatment of HPP includes both oral and IV potassium supplementation. Prophylaxis against recurrent attacks has been successful with various modalities including spiranolactone daily and acetazolamide.⁴ Care must be taken to consider thyrotoxic periodic paralysis, which most commonly presents in Asian men, as hyperthyroid symptoms may be subtle. Treatment focuses on reversal of the thyrotoxic state. β-Adrenergic blocking agents reduce the frequency and severity of attacks and should be started while measures to control thyrotoxicosis are being instituted.⁴

Before a diagnosis of HPP is made, other causes of hypokalemic paralysis must first be excluded.

Conclusion

This case is important because it demonstrates an unusual presentation of HPP in an emergency setting. This perspective of HPP can help the EP in recognizing and differentiating the condsition from similar disorders. 

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.

Case

A 22-year-old white man presented to the ED after waking from sleep and experiencing painless bilateral lower extremity weakness that ascended to the right upper extremity. This weakness left the patient unable to walk. He denied incontinence and was otherwise healthy with no prior history of similar symptoms. A family history of the presenting symptomatology was denied, and there was no family history of diabetes or kidney disease. The patient did state he had upper respiratory symptoms with a sore throat and runny nose 2 weeks earlier. He denied use of tobacco, alcohol, and illicit drugs.

Physical examination revealed abnormal deep tendon reflexes, absent patellar and achilles reflexes on the right side, reduced patellar and achilles reflexes of the left side, and diminished bilateral brachioradialis reflexes. Examination of the lower extremities revealed motor strength at 2/5 on the left and 1/5 on the right. His upper extremities demonstrated bilateral 4/5 motor strength. No ptosis was observed. All pulses of the extremities were normal with good capillary refill. His vital signs were: blood pressure, 131/75 mm Hg; pulse, 75 beats/minute; respiratory rate, 18 breaths/minute; and temperature, 97.2°F

Brain and cervical spine magnetic resonance imaging, computed tomography of the head, and blood cultures were ordered; the results of each were unremarkable. A lumbar puncture (LP) was attempted in the ED, but was unsuccessful due to difficulty in patient positioning secondary to profound weakness. An LP was later succesfully performed under fluoroscopy and revealed no abnormal findings. An electrocardiogram (ECG) demonstrated normal sinus rhythm with ST-segment flattening. The differential diagnosis for rapidly ascending weakness included Guillain-Barré syndrome, transverse myelitis, toxic metabolic syndromes, and myelopathies.

A basic metabolic panel showed that the patient was profoundly hypokalemic, with a potassium level of 1.9 mEq/L. After further blood analysis, mild hypomagnesemia was observed, with a level of 1.4 mEq/L. Whle in the ED, he was given 40 mEq potassium chloride intravenously (IV) and orally, and 2 g IV magnesium sulfate in the ED. The patient was evaluated by a neurologist in the ED. No respiratory compromise was present and the patient was admitted to neurology service with consultations from both nephrology and endocrinology.

Within 12 hours of starting potassium and magnesium supplementation, the patient’s potassium and magnesium levels returned to normal and his weakness concurrently subsided. The final diagnosis of the patient was hypokalemic periodic paralysis.

Discussion

Hypokalemic periodic paralysis (HPP), a rare autosomal dominant disorder with a prevalence of one in 100,000, is characterized by muscle weakness with accompanying hypokalemia. The first onset of symptoms is usually in the first or second decade of life, with a quarter of cases presenting before the age 10 years, and half before age 16 years. Being a typically-inherited autosomal dominant disorder, approximately two-thirds of cases present with a family history—unlike this patient case in which there was no family history of the disease. It is three to four times more common in men than women.

Hypokalemic periodic paralysis typically occurs upon waking from sleep or during rest following exercise. It may also be triggered by high-carbohydrate or high-salt meals, or from alcohol consumption. The muscles of the extremeties are usually affected, while those of the eyes, face, and sphincters are typically spared. If untreated, an attack can last from several hours to several days. Tendon and cutaneous reflexes can also be reduced or absent.¹ The manifestation of HPP can, at first impression, lead the differential diagnosis in the direction of the many other conditions that cause weakness of the extremities. Guillain-Barré syndrome and transverse myelitis are the more common diagnoses for which HPP can be mistaken. It is therefore important that the EP consider HPP in the differential so that an unusual presentation such as the one in this case may be diagnosed.

When presented with extraneous complications (eg, when the condition is encountered perioperatively or suffered comorbidly with similar conditions such as Guillain-Barré syndrome), HPP can be especially difficult to recognize.^2,3 Although a relatively uncommon condition, it can be potentially life-threatening if not recognized and treated appropriately. Severe hypokalemia may cause sequela such as respiratory failure and cardiac arhythmia. Thus, ECG and cardiorespiratory monitoring are essential in patients with HPP and hypokalemia as very severe hypokalemia may also cause paralysis of bulbar and cranial nerve musculature. Electocardiographic changes are common, and may include ST-segment sagging and flattening, U waves, and T-wave inversion. Other causes in patients presenting with HPP must also be considered, as hypokalemic paralysis of a sporadic nature may be associated with conditions including barium poisoning, hyperthyroidism, renal disorders, certain endocrinopathies, and gastrointestinal potassium losses.⁴

 

The pathogenesis of HPP is not completely understood; however, alteration in potassium regulation are well documented. Treatment of HPP includes both oral and IV potassium supplementation. Prophylaxis against recurrent attacks has been successful with various modalities including spiranolactone daily and acetazolamide.⁴ Care must be taken to consider thyrotoxic periodic paralysis, which most commonly presents in Asian men, as hyperthyroid symptoms may be subtle. Treatment focuses on reversal of the thyrotoxic state. β-Adrenergic blocking agents reduce the frequency and severity of attacks and should be started while measures to control thyrotoxicosis are being instituted.⁴

Before a diagnosis of HPP is made, other causes of hypokalemic paralysis must first be excluded.

Conclusion

This case is important because it demonstrates an unusual presentation of HPP in an emergency setting. This perspective of HPP can help the EP in recognizing and differentiating the condsition from similar disorders. 

Dr Orlik is a resident, department of emergency medicine, Akron General Medical Center, Ohio. Mr Kovacs is a student and summer research fellow, department of emergency medicine, Akron General Medical Center, Ohio. Dr Simon is the emergency medicine research director, department of emergency medicine, Akron General Medical Center, Northeast Ohio Medical University.