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Erik Greb joined the staff of Neurology Reviews in January 2012. Since then, he has attended scientific conferences, conducted video interviews, and written about clinical research in multiple sclerosis, epilepsy, Parkinson's disease, Alzheimer's disease, stroke, and other neurologic disorders. In addition to news articles, Erik has written investigative stories about multiple sclerosis, headache, and epilepsy. He previously wrote about pharmaceutical manufacturing, drug formulation and delivery, quality assurance, and regulation for Pharmaceutical Technology.
Children may develop prolonged headache after concussion
CHARLOTTE, N.C. – , according to research presented at the annual meeting of the Child Neurology Society. The headache may be migraine, chronic daily headache, tension-type headache, or a combination of these headaches.
“We strongly recommend that individuals who develop persistent headache after a concussion be evaluated and treated by a neurologist with experience in administering treatment for headache,” said Marcus Barissi, Weller Scholar at the Cleveland Clinic, and colleagues. “Using this approach, we hope that their prolonged headaches will be lessened.”
Few studies have examined prolonged pediatric postconcussion headache
The Centers for Disease Control and Prevention estimates that between 1.6 million and 3.8 million concussions occur annually during athletic and recreational activities in the United States. About 90% of concussions affect children or adolescents. The symptom most often reported after concussion is headache.
Few studies have focused on new persistent postconcussion headache (NPPCH) in children. Mr. Barissi and colleagues did not find any previous study that had examined prolonged headache following concussion in patients without prior chronic headache. They sought to ascertain the prognosis of patients with NPPCH and no history of prior headache, to describe this clinical entity, and to identify beneficial treatment methods.
The investigators retrospectively reviewed charts for approximately 2,000 patients who presented to the Cleveland Clinic pediatric neurology department between June 2017 and August 2018 for headaches. They identified 259 patients who received a diagnosis of concussion, 69 (27%) of whom had headaches for longer than 2 months after injury.
Mr. Barissi and colleagues emailed these patients, and 33 (48%) of them agreed to complete a questionnaire and participate in a 10-minute phone interview. Thirty-one patients (43%) could not be contacted, and eight (11%) declined to participate. All participants confirmed that they had not had consistent headache before the concussion and that chronic headache had arisen after concussion. To determine participants’ medical outcomes, the researchers compared participants’ initial assessment data with posttreatment data collected during the interview process.
Healthy behaviors increased after concussion
Of the 69 eligible participants, 38 (55%) were female. The population’s median age was 17. Twenty-eight (85%) of the 33 patients who completed the questionnaire considered the information and treatment that they had received to be beneficial. Twenty-five (78%) patients continued to have headache after several months, despite treatment.
Participants had withstood a mean of 1.72 concussions, and the mean age at first injury was 12.49 years. The most common cause of injury was a fall for males (36%) and an automobile accident for females (18%).
Forty-eight patients (70%) reported having two types of headache. Fifty-two patients (75%) had migraines, and 65 (94%) had chronic daily headache or tension-type headache. Forty-eight (70%) participants had a family history of headache.
In all, 64 patients (93%) had used a headache medication. The most common headache medications used were amitriptyline, topiramate, and cyproheptadine. Few patients were still taking these medications at several months after evaluation. The most common nonprescription medications used were Migravent (i.e., magnesium, riboflavin, coenzyme Q10, and butterbur), ondansetron, and melatonin. Furthermore, 61 patients (88%) participated in nonmedicinal therapy such as physical therapy, chiropractic therapy, and acupuncture.
After evaluation, patients engaged in several healthy behaviors (e.g., adequate exercise, proper use of over-the-counter medications, and drinking sufficient water) more frequently, but did not get adequate sleep. Sixty-five participants (94%) had undergone CT or MRI imaging, but the results did not improve understanding of headache etiology or treatment. Many patients missed several days of school, but average attendance improved after months of treatment.
Long-term outcomes
Thirty-one survey respondents (94%) reported that their emotional, cognitive, sleep, and somatic postconcussion symptoms had resolved. Nevertheless, a majority of participants still had headache. “The persistence of postconcussion symptoms is uncommon, but lasting headache is not,” said the researchers. “If patients are not properly educated, conditions may deteriorate, extending the duration of disability.” A longer study with a larger sample size could provide valuable information, said the researchers. Future work should examine objectively the efficacy of various medications used to treat NPPCH and determine the best methods of treatment for this syndrome, which “can cause prolonged pain, suffering, and lack of function,” they concluded.
The investigators did not report any study funding or disclosures.
SOURCE: Barissi M et al. CNS 2019, Abstract 95.
CHARLOTTE, N.C. – , according to research presented at the annual meeting of the Child Neurology Society. The headache may be migraine, chronic daily headache, tension-type headache, or a combination of these headaches.
“We strongly recommend that individuals who develop persistent headache after a concussion be evaluated and treated by a neurologist with experience in administering treatment for headache,” said Marcus Barissi, Weller Scholar at the Cleveland Clinic, and colleagues. “Using this approach, we hope that their prolonged headaches will be lessened.”
Few studies have examined prolonged pediatric postconcussion headache
The Centers for Disease Control and Prevention estimates that between 1.6 million and 3.8 million concussions occur annually during athletic and recreational activities in the United States. About 90% of concussions affect children or adolescents. The symptom most often reported after concussion is headache.
Few studies have focused on new persistent postconcussion headache (NPPCH) in children. Mr. Barissi and colleagues did not find any previous study that had examined prolonged headache following concussion in patients without prior chronic headache. They sought to ascertain the prognosis of patients with NPPCH and no history of prior headache, to describe this clinical entity, and to identify beneficial treatment methods.
The investigators retrospectively reviewed charts for approximately 2,000 patients who presented to the Cleveland Clinic pediatric neurology department between June 2017 and August 2018 for headaches. They identified 259 patients who received a diagnosis of concussion, 69 (27%) of whom had headaches for longer than 2 months after injury.
Mr. Barissi and colleagues emailed these patients, and 33 (48%) of them agreed to complete a questionnaire and participate in a 10-minute phone interview. Thirty-one patients (43%) could not be contacted, and eight (11%) declined to participate. All participants confirmed that they had not had consistent headache before the concussion and that chronic headache had arisen after concussion. To determine participants’ medical outcomes, the researchers compared participants’ initial assessment data with posttreatment data collected during the interview process.
Healthy behaviors increased after concussion
Of the 69 eligible participants, 38 (55%) were female. The population’s median age was 17. Twenty-eight (85%) of the 33 patients who completed the questionnaire considered the information and treatment that they had received to be beneficial. Twenty-five (78%) patients continued to have headache after several months, despite treatment.
Participants had withstood a mean of 1.72 concussions, and the mean age at first injury was 12.49 years. The most common cause of injury was a fall for males (36%) and an automobile accident for females (18%).
Forty-eight patients (70%) reported having two types of headache. Fifty-two patients (75%) had migraines, and 65 (94%) had chronic daily headache or tension-type headache. Forty-eight (70%) participants had a family history of headache.
In all, 64 patients (93%) had used a headache medication. The most common headache medications used were amitriptyline, topiramate, and cyproheptadine. Few patients were still taking these medications at several months after evaluation. The most common nonprescription medications used were Migravent (i.e., magnesium, riboflavin, coenzyme Q10, and butterbur), ondansetron, and melatonin. Furthermore, 61 patients (88%) participated in nonmedicinal therapy such as physical therapy, chiropractic therapy, and acupuncture.
After evaluation, patients engaged in several healthy behaviors (e.g., adequate exercise, proper use of over-the-counter medications, and drinking sufficient water) more frequently, but did not get adequate sleep. Sixty-five participants (94%) had undergone CT or MRI imaging, but the results did not improve understanding of headache etiology or treatment. Many patients missed several days of school, but average attendance improved after months of treatment.
Long-term outcomes
Thirty-one survey respondents (94%) reported that their emotional, cognitive, sleep, and somatic postconcussion symptoms had resolved. Nevertheless, a majority of participants still had headache. “The persistence of postconcussion symptoms is uncommon, but lasting headache is not,” said the researchers. “If patients are not properly educated, conditions may deteriorate, extending the duration of disability.” A longer study with a larger sample size could provide valuable information, said the researchers. Future work should examine objectively the efficacy of various medications used to treat NPPCH and determine the best methods of treatment for this syndrome, which “can cause prolonged pain, suffering, and lack of function,” they concluded.
The investigators did not report any study funding or disclosures.
SOURCE: Barissi M et al. CNS 2019, Abstract 95.
CHARLOTTE, N.C. – , according to research presented at the annual meeting of the Child Neurology Society. The headache may be migraine, chronic daily headache, tension-type headache, or a combination of these headaches.
“We strongly recommend that individuals who develop persistent headache after a concussion be evaluated and treated by a neurologist with experience in administering treatment for headache,” said Marcus Barissi, Weller Scholar at the Cleveland Clinic, and colleagues. “Using this approach, we hope that their prolonged headaches will be lessened.”
Few studies have examined prolonged pediatric postconcussion headache
The Centers for Disease Control and Prevention estimates that between 1.6 million and 3.8 million concussions occur annually during athletic and recreational activities in the United States. About 90% of concussions affect children or adolescents. The symptom most often reported after concussion is headache.
Few studies have focused on new persistent postconcussion headache (NPPCH) in children. Mr. Barissi and colleagues did not find any previous study that had examined prolonged headache following concussion in patients without prior chronic headache. They sought to ascertain the prognosis of patients with NPPCH and no history of prior headache, to describe this clinical entity, and to identify beneficial treatment methods.
The investigators retrospectively reviewed charts for approximately 2,000 patients who presented to the Cleveland Clinic pediatric neurology department between June 2017 and August 2018 for headaches. They identified 259 patients who received a diagnosis of concussion, 69 (27%) of whom had headaches for longer than 2 months after injury.
Mr. Barissi and colleagues emailed these patients, and 33 (48%) of them agreed to complete a questionnaire and participate in a 10-minute phone interview. Thirty-one patients (43%) could not be contacted, and eight (11%) declined to participate. All participants confirmed that they had not had consistent headache before the concussion and that chronic headache had arisen after concussion. To determine participants’ medical outcomes, the researchers compared participants’ initial assessment data with posttreatment data collected during the interview process.
Healthy behaviors increased after concussion
Of the 69 eligible participants, 38 (55%) were female. The population’s median age was 17. Twenty-eight (85%) of the 33 patients who completed the questionnaire considered the information and treatment that they had received to be beneficial. Twenty-five (78%) patients continued to have headache after several months, despite treatment.
Participants had withstood a mean of 1.72 concussions, and the mean age at first injury was 12.49 years. The most common cause of injury was a fall for males (36%) and an automobile accident for females (18%).
Forty-eight patients (70%) reported having two types of headache. Fifty-two patients (75%) had migraines, and 65 (94%) had chronic daily headache or tension-type headache. Forty-eight (70%) participants had a family history of headache.
In all, 64 patients (93%) had used a headache medication. The most common headache medications used were amitriptyline, topiramate, and cyproheptadine. Few patients were still taking these medications at several months after evaluation. The most common nonprescription medications used were Migravent (i.e., magnesium, riboflavin, coenzyme Q10, and butterbur), ondansetron, and melatonin. Furthermore, 61 patients (88%) participated in nonmedicinal therapy such as physical therapy, chiropractic therapy, and acupuncture.
After evaluation, patients engaged in several healthy behaviors (e.g., adequate exercise, proper use of over-the-counter medications, and drinking sufficient water) more frequently, but did not get adequate sleep. Sixty-five participants (94%) had undergone CT or MRI imaging, but the results did not improve understanding of headache etiology or treatment. Many patients missed several days of school, but average attendance improved after months of treatment.
Long-term outcomes
Thirty-one survey respondents (94%) reported that their emotional, cognitive, sleep, and somatic postconcussion symptoms had resolved. Nevertheless, a majority of participants still had headache. “The persistence of postconcussion symptoms is uncommon, but lasting headache is not,” said the researchers. “If patients are not properly educated, conditions may deteriorate, extending the duration of disability.” A longer study with a larger sample size could provide valuable information, said the researchers. Future work should examine objectively the efficacy of various medications used to treat NPPCH and determine the best methods of treatment for this syndrome, which “can cause prolonged pain, suffering, and lack of function,” they concluded.
The investigators did not report any study funding or disclosures.
SOURCE: Barissi M et al. CNS 2019, Abstract 95.
REPORTING FROM CNS 2019
AVXS-101 may result in long-term motor improvements in SMA
CHARLOTTE, N.C. – AVXS-101, the Food and Drug Administration–approved therapy for spinal muscular atrophy (SMA), yields rapid, sustained improvements in CHOP INTEND scores, better survival, and motor function improvements at long-term follow-up, according to an analysis presented at the annual meeting of the Child Neurology Society. The results provide a clinical demonstration of continuous expression of the SMN protein, according to the investigators. In addition, AVXS-101 is associated with reduced health care utilization in treated infants, which could decrease costs, lessen the burden on patients and caregivers, and improve quality of life.
SMA1 is a progressive neurologic disease that causes loss of the lower motor neurons in the spinal cord and brainstem. Patients have increasing muscle weakness that leads to death or the need for permanent ventilation by age 2 years. The disease results from mutations in the SMN1 gene. AVXS-101 replaces the missing or nonfunctional SMN1 with a healthy copy of a human SMN gene.
AveXis, the company that developed the therapy, enrolled 12 patients with SMA1 in a phase 1/2a study between December 2014 and December 2015. All participants received one intravenous infusion of AVXS-101. Omar Dabbous, MD, vice president of global health economics, outcomes research, and real world evidence at AveXis in Bannockburn, Ill., and colleagues evaluated participants’ rates of event-free survival (i.e., absence of death or need for permanent ventilation), pulmonary or nutritional interventions, swallowing, hospitalization, and CHOP INTEND scores, as well as therapeutic safety at 2 years.
At study completion, all patients who had received a therapeutic dose had event-free survival. Seven participants did not need daily noninvasive ventilation. Eleven participants had stable or improved swallowing. All of the latter patients fed orally, and six fed exclusively by mouth. Eleven patients spoke.
Participants had a mean of 1.4 respiratory hospitalizations per year. Mean proportion of time participants spent hospitalized was 4.4%. Mean hospitalization rate per year was 2.1, and mean length of hospital stay was 6.7 days. In addition, participants’ CHOP INTEND scores increased from baseline by 9.8 points at 1 month and by 15.4 points at 3 months. Patients who received a therapeutic dose of AVXS-101 have maintained their motor milestones at long-term follow-up, which suggests that treatment effects persist over the long term. Adverse events included elevated serum aminotransferase levels, which were reduced by prednisolone.
Dr. Dabbous is an employee of AveXis, which developed AVXS-101.
SOURCE: Dabbous O et al. CNS 2019. Abstract 199.
CHARLOTTE, N.C. – AVXS-101, the Food and Drug Administration–approved therapy for spinal muscular atrophy (SMA), yields rapid, sustained improvements in CHOP INTEND scores, better survival, and motor function improvements at long-term follow-up, according to an analysis presented at the annual meeting of the Child Neurology Society. The results provide a clinical demonstration of continuous expression of the SMN protein, according to the investigators. In addition, AVXS-101 is associated with reduced health care utilization in treated infants, which could decrease costs, lessen the burden on patients and caregivers, and improve quality of life.
SMA1 is a progressive neurologic disease that causes loss of the lower motor neurons in the spinal cord and brainstem. Patients have increasing muscle weakness that leads to death or the need for permanent ventilation by age 2 years. The disease results from mutations in the SMN1 gene. AVXS-101 replaces the missing or nonfunctional SMN1 with a healthy copy of a human SMN gene.
AveXis, the company that developed the therapy, enrolled 12 patients with SMA1 in a phase 1/2a study between December 2014 and December 2015. All participants received one intravenous infusion of AVXS-101. Omar Dabbous, MD, vice president of global health economics, outcomes research, and real world evidence at AveXis in Bannockburn, Ill., and colleagues evaluated participants’ rates of event-free survival (i.e., absence of death or need for permanent ventilation), pulmonary or nutritional interventions, swallowing, hospitalization, and CHOP INTEND scores, as well as therapeutic safety at 2 years.
At study completion, all patients who had received a therapeutic dose had event-free survival. Seven participants did not need daily noninvasive ventilation. Eleven participants had stable or improved swallowing. All of the latter patients fed orally, and six fed exclusively by mouth. Eleven patients spoke.
Participants had a mean of 1.4 respiratory hospitalizations per year. Mean proportion of time participants spent hospitalized was 4.4%. Mean hospitalization rate per year was 2.1, and mean length of hospital stay was 6.7 days. In addition, participants’ CHOP INTEND scores increased from baseline by 9.8 points at 1 month and by 15.4 points at 3 months. Patients who received a therapeutic dose of AVXS-101 have maintained their motor milestones at long-term follow-up, which suggests that treatment effects persist over the long term. Adverse events included elevated serum aminotransferase levels, which were reduced by prednisolone.
Dr. Dabbous is an employee of AveXis, which developed AVXS-101.
SOURCE: Dabbous O et al. CNS 2019. Abstract 199.
CHARLOTTE, N.C. – AVXS-101, the Food and Drug Administration–approved therapy for spinal muscular atrophy (SMA), yields rapid, sustained improvements in CHOP INTEND scores, better survival, and motor function improvements at long-term follow-up, according to an analysis presented at the annual meeting of the Child Neurology Society. The results provide a clinical demonstration of continuous expression of the SMN protein, according to the investigators. In addition, AVXS-101 is associated with reduced health care utilization in treated infants, which could decrease costs, lessen the burden on patients and caregivers, and improve quality of life.
SMA1 is a progressive neurologic disease that causes loss of the lower motor neurons in the spinal cord and brainstem. Patients have increasing muscle weakness that leads to death or the need for permanent ventilation by age 2 years. The disease results from mutations in the SMN1 gene. AVXS-101 replaces the missing or nonfunctional SMN1 with a healthy copy of a human SMN gene.
AveXis, the company that developed the therapy, enrolled 12 patients with SMA1 in a phase 1/2a study between December 2014 and December 2015. All participants received one intravenous infusion of AVXS-101. Omar Dabbous, MD, vice president of global health economics, outcomes research, and real world evidence at AveXis in Bannockburn, Ill., and colleagues evaluated participants’ rates of event-free survival (i.e., absence of death or need for permanent ventilation), pulmonary or nutritional interventions, swallowing, hospitalization, and CHOP INTEND scores, as well as therapeutic safety at 2 years.
At study completion, all patients who had received a therapeutic dose had event-free survival. Seven participants did not need daily noninvasive ventilation. Eleven participants had stable or improved swallowing. All of the latter patients fed orally, and six fed exclusively by mouth. Eleven patients spoke.
Participants had a mean of 1.4 respiratory hospitalizations per year. Mean proportion of time participants spent hospitalized was 4.4%. Mean hospitalization rate per year was 2.1, and mean length of hospital stay was 6.7 days. In addition, participants’ CHOP INTEND scores increased from baseline by 9.8 points at 1 month and by 15.4 points at 3 months. Patients who received a therapeutic dose of AVXS-101 have maintained their motor milestones at long-term follow-up, which suggests that treatment effects persist over the long term. Adverse events included elevated serum aminotransferase levels, which were reduced by prednisolone.
Dr. Dabbous is an employee of AveXis, which developed AVXS-101.
SOURCE: Dabbous O et al. CNS 2019. Abstract 199.
REPORTING FROM CNS 2019
Stroke is diagnosed in about one-fifth of children with strokelike symptoms
CHARLOTTE, N.C. – (TIA), according to research presented at the annual meeting of the Child Neurology Society. Ischemic stroke and TIA were the second leading diagnoses among the stroke activations examined in the study, after seizure and Todd’s paralysis. “These data, in conjunction with previous studies, highlight the importance of developing protocols for early recognition and evaluation of children who present with strokelike symptoms,” said Tiffany Barkley, DO, a child neurology resident at Children’s Mercy Hospital in Kansas City, Mo., and colleagues.
Dr. Barkley and colleagues conducted their research to describe the demographic and other characteristics of patients who present with strokelike symptoms to their hospital. They undertook a descriptive, retrospective chart review of patients who came to Children’s Mercy Hospital from Sept. 1, 2016, to August 31, 2018, with concern for acute stroke. The investigators examined only patients for whom the Stroke Alert Process and power plan were activated.
“Power plans were created at Children’s Mercy Hospital to streamline and standardize care for children,” said Dr. Barkley. “While stroke order sets tend to be common practice in many adult hospitals, stroke order sets in pediatric hospitals are new.”
In all, 61 stroke activations occurred during the study period. Twelve patients (20%) had a final diagnosis of ischemic stroke or TIA. Among the patients with a final diagnosis of ischemic stroke, the most common presenting symptom was unilateral weakness. Two of these patients were candidates for intervention with mechanical thrombectomy, and none received tissue plasminogen activator. The average age of patients in all activations was 14 years, and the average age of patients with a final diagnosis of ischemic stroke or TIA was 4 years. About 37 (61%) subjects of activations were female, and the most common racial demographic was Caucasian.
Ischemic stroke or TIA was the second most common diagnosis of all activations (12 patients; 20%). Seizure or Todd’s paralysis (14 patients; 23%) was the leading diagnosis. Other common diagnoses included migraine (18%), psychogenic or conversion disorder (15%), oncologic process (3.0%), and complications of meningitis or encephalitis (1.6%). Children who presented with ischemic stroke secondary to Moyamoya disease were classified separately (two patients or 3%). It can be difficult to distinguish between stroke and stroke mimics based on neurologic examination alone, and imaging such as MRI often is needed, said Dr. Barkley. The researchers did not identify any intracranial hemorrhages in this patient population.
These findings are consistent with current reported literature, said the researchers. “Our study is one of the first to look at the demographics of children who present with strokelike symptoms,” said Dr. Barkley. “We hope that our study will not only help identify children who present with symptoms concerning for stroke, but also help us identify children who may be at risk for ischemic stroke before the stroke happens.”
The investigators did not have funding for this study and did not report any disclosures.
SOURCE: Barkley T et al. CNS 2019. Abstract 235.
CHARLOTTE, N.C. – (TIA), according to research presented at the annual meeting of the Child Neurology Society. Ischemic stroke and TIA were the second leading diagnoses among the stroke activations examined in the study, after seizure and Todd’s paralysis. “These data, in conjunction with previous studies, highlight the importance of developing protocols for early recognition and evaluation of children who present with strokelike symptoms,” said Tiffany Barkley, DO, a child neurology resident at Children’s Mercy Hospital in Kansas City, Mo., and colleagues.
Dr. Barkley and colleagues conducted their research to describe the demographic and other characteristics of patients who present with strokelike symptoms to their hospital. They undertook a descriptive, retrospective chart review of patients who came to Children’s Mercy Hospital from Sept. 1, 2016, to August 31, 2018, with concern for acute stroke. The investigators examined only patients for whom the Stroke Alert Process and power plan were activated.
“Power plans were created at Children’s Mercy Hospital to streamline and standardize care for children,” said Dr. Barkley. “While stroke order sets tend to be common practice in many adult hospitals, stroke order sets in pediatric hospitals are new.”
In all, 61 stroke activations occurred during the study period. Twelve patients (20%) had a final diagnosis of ischemic stroke or TIA. Among the patients with a final diagnosis of ischemic stroke, the most common presenting symptom was unilateral weakness. Two of these patients were candidates for intervention with mechanical thrombectomy, and none received tissue plasminogen activator. The average age of patients in all activations was 14 years, and the average age of patients with a final diagnosis of ischemic stroke or TIA was 4 years. About 37 (61%) subjects of activations were female, and the most common racial demographic was Caucasian.
Ischemic stroke or TIA was the second most common diagnosis of all activations (12 patients; 20%). Seizure or Todd’s paralysis (14 patients; 23%) was the leading diagnosis. Other common diagnoses included migraine (18%), psychogenic or conversion disorder (15%), oncologic process (3.0%), and complications of meningitis or encephalitis (1.6%). Children who presented with ischemic stroke secondary to Moyamoya disease were classified separately (two patients or 3%). It can be difficult to distinguish between stroke and stroke mimics based on neurologic examination alone, and imaging such as MRI often is needed, said Dr. Barkley. The researchers did not identify any intracranial hemorrhages in this patient population.
These findings are consistent with current reported literature, said the researchers. “Our study is one of the first to look at the demographics of children who present with strokelike symptoms,” said Dr. Barkley. “We hope that our study will not only help identify children who present with symptoms concerning for stroke, but also help us identify children who may be at risk for ischemic stroke before the stroke happens.”
The investigators did not have funding for this study and did not report any disclosures.
SOURCE: Barkley T et al. CNS 2019. Abstract 235.
CHARLOTTE, N.C. – (TIA), according to research presented at the annual meeting of the Child Neurology Society. Ischemic stroke and TIA were the second leading diagnoses among the stroke activations examined in the study, after seizure and Todd’s paralysis. “These data, in conjunction with previous studies, highlight the importance of developing protocols for early recognition and evaluation of children who present with strokelike symptoms,” said Tiffany Barkley, DO, a child neurology resident at Children’s Mercy Hospital in Kansas City, Mo., and colleagues.
Dr. Barkley and colleagues conducted their research to describe the demographic and other characteristics of patients who present with strokelike symptoms to their hospital. They undertook a descriptive, retrospective chart review of patients who came to Children’s Mercy Hospital from Sept. 1, 2016, to August 31, 2018, with concern for acute stroke. The investigators examined only patients for whom the Stroke Alert Process and power plan were activated.
“Power plans were created at Children’s Mercy Hospital to streamline and standardize care for children,” said Dr. Barkley. “While stroke order sets tend to be common practice in many adult hospitals, stroke order sets in pediatric hospitals are new.”
In all, 61 stroke activations occurred during the study period. Twelve patients (20%) had a final diagnosis of ischemic stroke or TIA. Among the patients with a final diagnosis of ischemic stroke, the most common presenting symptom was unilateral weakness. Two of these patients were candidates for intervention with mechanical thrombectomy, and none received tissue plasminogen activator. The average age of patients in all activations was 14 years, and the average age of patients with a final diagnosis of ischemic stroke or TIA was 4 years. About 37 (61%) subjects of activations were female, and the most common racial demographic was Caucasian.
Ischemic stroke or TIA was the second most common diagnosis of all activations (12 patients; 20%). Seizure or Todd’s paralysis (14 patients; 23%) was the leading diagnosis. Other common diagnoses included migraine (18%), psychogenic or conversion disorder (15%), oncologic process (3.0%), and complications of meningitis or encephalitis (1.6%). Children who presented with ischemic stroke secondary to Moyamoya disease were classified separately (two patients or 3%). It can be difficult to distinguish between stroke and stroke mimics based on neurologic examination alone, and imaging such as MRI often is needed, said Dr. Barkley. The researchers did not identify any intracranial hemorrhages in this patient population.
These findings are consistent with current reported literature, said the researchers. “Our study is one of the first to look at the demographics of children who present with strokelike symptoms,” said Dr. Barkley. “We hope that our study will not only help identify children who present with symptoms concerning for stroke, but also help us identify children who may be at risk for ischemic stroke before the stroke happens.”
The investigators did not have funding for this study and did not report any disclosures.
SOURCE: Barkley T et al. CNS 2019. Abstract 235.
REPORTING FROM CNS 2019
MS-related disability may be decreasing
STOCKHOLM – , according to an overview provided at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis. Data consistently indicate that the time that elapses before a patient requires a cane for ambulation has increased, and survival has likewise improved. “Some of the improvement can be attributed confidently to treatment effect,” said Ilya Kister, MD, associate professor of neurology at NYU Langone Health in New York. “We hope to see an even greater change with newer therapies.”
At the same time, neurologists appear to be diagnosing more cases of MS than they previously did, said Dr. Kister, which suggests that neurologists probably are diagnosing milder cases. The overall societal burden of MS remains high.
The relative prevalence of mild disability has increased
About 25 years have elapsed since the first disease-modifying treatment (DMT) for MS became available, and treatment has become widespread during that time. Dr. Kister and colleagues sought to determine whether the current clinical population of patients with MS, who for the most part receive DMTs, has less disability than do untreated patients or patients from natural history studies do. They identified the MS Severity Score (MSSS) as a measure with which to compare populations. The MSSS assigns a patient a ranking according to his or her level of disability, using a reference population of patients with the same disease duration for comparison. “MSSS can be conceptualized as rate of disability accumulation,” said Dr. Kister. “Lower MSSS corresponds to relatively slower disability accumulation, and higher MSSS to higher disability accumulation.”
The MSSS was developed using the Expanded Disability Status Scale (EDSS) score as a measure of disability. Because many neurologists do not routinely obtain EDSS scores for their patients, Dr. Kister and colleagues used the Patient-Determined Disease Steps (PDDS) to measure disability. As its name implies, the PDDS is a patient-reported outcome measure that mainly measures ambulation. It correlates strongly with EDSS, said Dr. Kister. He and colleagues used the PDDS to develop a reference table of MS disability, which they called the Patient-Derived MSSS.
The investigators examined a large sample of patients at NYU MS Center and Barnabas MS Center in Livingston, N.J. They grouped patients into sextiles according to their Patient-Derived MSSS. Dr. Kister and colleagues found that, rather than arriving at sextiles that contained equal numbers of patients, as would be expected if disability were distributed as in the reference population, they had significantly more patients in the two lowest sextiles and significantly fewer patients in the two highest sextiles. “This [result] suggests that the disability curve has indeed shifted toward the more benign end of the spectrum in the contemporary clinic population,” said Dr. Kister.
Other researchers have observed a similar phenomenon. George et al. published the results of a large, international collaboration in Neurology Genetics in 2016. After examining more than 7,000 patients, the investigators noted a similar overrepresentation of patients with milder severity scores and underrepresentation of patients with higher severity scores. These results support the hypothesis of a shift toward milder disability, said Dr. Kister.
Trend toward milder disability
The investigators next examined whether the rate of accumulation of disability among patients with MS had changed from year to year since DMTs were introduced. They conducted a univariate analysis of MSSS for 6,238 patients who were enrolled in the N.Y. State MS Consortium during 1996-2007. They found that patients who were enrolled in more recent years had significantly lower MSSS than patients who were enrolled in earlier years, regardless of disease duration. When Dr. Kister and colleagues replicated their analysis using EDSS, they found significantly lower levels of disability for patients enrolled in more recent years, except for patients with disease duration of 26-30 years. A multivariate analysis showed that the median MSSS of enrollees into the N.Y. State MS Consortium decreased from 5.04 in 1996 to 3.78 in 2006.
In a subsequent study, Dr. Kister and colleagues examined the age at which patients in the MSBase registry reached various disability milestones (e.g., EDSS of 6, which indicates the need of a cane to walk outdoors), according to their year of enrollment in the registry. They found a significant increase in age at milestone achievement with each subsequent calendar year. For example, for every consecutive year of enrollment, the age at which patients attained an EDSS of 6 increased by 0.38 years. These analyses were confirmed for the subgroups of patients diagnosed according to the Poser and McDonald criteria. The increase in age “is probably not just related to the shift in diagnostic criteria,” said Dr. Kister. When the researchers calculated the net average gains in years over the 13-year follow-up period, they found that patients who entered at the end of the enrollment period were 4.9 years older when they reached an EDSS of 6, compared with patients with an EDSS of 6 who entered at the beginning of the enrollment period.
International data show similar trends
Research conducted around the world shows similar trends, said Dr. Kister. In 2009, Veugelers et al. published the results of a study that included 1,752 patients with MS in Nova Scotia. Before the 1998 introduction of a drug insurance program that provides DMTs, the time to an EDSS of 6 was 14.4 years. After the introduction of this program, the time to EDSS of 6 was 18.6 years.
More recently, Capra et al. examined 1,324 patients with MS who attended an MS center in Brescia, Italy, during 1980-2010. They found that the age at which 50% of patients reached an EDSS of 6 was approximately 55 years in 1990. By 2010, the age at achieving this milestone had increased to approximately 63 years.
In a prospective study, Cree et al. examined the evolution of disability in 448 actively treated patients with relapsing-remitting MS and 69 patients with progressive MS. Approximately 45% of patients had no disability worsening during a 10-year follow-up period. Furthermore, a comparatively low 11% of patients had reached an EDSS of 6 at 10 years. The average disease duration of the cohort at that time was 17 years, said Dr. Kister. The results indicated that about 50% of patients would be expected to reach an EDSS of 6 after a disease duration of approximately 38 years, “which is much longer than in the natural history studies,” he added.
In 2019, Beiki et al. found that among patients with relapsing-remitting MS, the risk of reaching an EDSS of 6 decreased by 7% with each subsequent calendar year of diagnosis. The researchers did not observe a similar trend among patients with progressive MS. Their population-based, retrospective study included 7,331 patients in Sweden.
Two additional studies in Scandinavian populations add to the evidence of decreasing disability. In their examination of Swedish patients with MS who received a diagnosis of MS during 1968-2012, Burkill et al. found that the risk of death decreased over time. The hazard ratio of mortality for patients with MS, compared with a non-MS comparator group, decreased from 6.52 among those diagnosed during 1968-1980 to 2.08 for patients diagnosed during 2001-2012. The decrease in the risk of mortality was greater among patients with MS than in a matched comparator population. Similarly, in a nationwide, population-based study, Koch-Henriksen et al. found that all-cause excess mortality in Danish patients with MS decreased from 1950 through 1999.
The role of DMTs
The evidence suggests that DMTs are affecting the long-term progression of MS, said Dr. Kister. Palace et al. compared patients with MS in the UK who received treatment with interferon-beta with a modeled untreated cohort of patients in British Columbia. They found that treated patients reached an EDSS of 6 4 years later than did untreated patients.
Furthermore, an analysis by Brown et al. showed that the time to conversion to secondary progressive MS was longer among treated patients, compared with untreated patients. The risk of conversion was lower for patients treated with newer, more effective therapies (i.e., fingolimod, alemtuzumab, or natalizumab) than for those treated with glatiramer acetate or interferon beta.
Finally, Kingwell and colleagues examined the effect of treatment with interferon-beta on survival using an international cohort of approximately 6,000 patients with relapsing-remitting MS. They found that exposure to interferon-beta for more than 3 years was associated with a 32% reduction in the risk of mortality. They observed no similar risk reduction among patients exposed to interferon-beta for 6 months to 3 years.
Although these data are encouraging, other evidence indicates that the prevalence of MS in the United States has increased considerably in the past 40 years. Researchers estimate that 1 million Americans have MS, which “suggests that we are diagnosing many more mild cases,” said Dr. Kister. The burden of the disease remains high, he concluded.
Dr. Kister reported receiving consulting fees or research grants from Biogen, Roche, Genzyme and Genentech.
SOURCE: Kister I et al. ECTRIMS 2019. Abstract 281754.
STOCKHOLM – , according to an overview provided at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis. Data consistently indicate that the time that elapses before a patient requires a cane for ambulation has increased, and survival has likewise improved. “Some of the improvement can be attributed confidently to treatment effect,” said Ilya Kister, MD, associate professor of neurology at NYU Langone Health in New York. “We hope to see an even greater change with newer therapies.”
At the same time, neurologists appear to be diagnosing more cases of MS than they previously did, said Dr. Kister, which suggests that neurologists probably are diagnosing milder cases. The overall societal burden of MS remains high.
The relative prevalence of mild disability has increased
About 25 years have elapsed since the first disease-modifying treatment (DMT) for MS became available, and treatment has become widespread during that time. Dr. Kister and colleagues sought to determine whether the current clinical population of patients with MS, who for the most part receive DMTs, has less disability than do untreated patients or patients from natural history studies do. They identified the MS Severity Score (MSSS) as a measure with which to compare populations. The MSSS assigns a patient a ranking according to his or her level of disability, using a reference population of patients with the same disease duration for comparison. “MSSS can be conceptualized as rate of disability accumulation,” said Dr. Kister. “Lower MSSS corresponds to relatively slower disability accumulation, and higher MSSS to higher disability accumulation.”
The MSSS was developed using the Expanded Disability Status Scale (EDSS) score as a measure of disability. Because many neurologists do not routinely obtain EDSS scores for their patients, Dr. Kister and colleagues used the Patient-Determined Disease Steps (PDDS) to measure disability. As its name implies, the PDDS is a patient-reported outcome measure that mainly measures ambulation. It correlates strongly with EDSS, said Dr. Kister. He and colleagues used the PDDS to develop a reference table of MS disability, which they called the Patient-Derived MSSS.
The investigators examined a large sample of patients at NYU MS Center and Barnabas MS Center in Livingston, N.J. They grouped patients into sextiles according to their Patient-Derived MSSS. Dr. Kister and colleagues found that, rather than arriving at sextiles that contained equal numbers of patients, as would be expected if disability were distributed as in the reference population, they had significantly more patients in the two lowest sextiles and significantly fewer patients in the two highest sextiles. “This [result] suggests that the disability curve has indeed shifted toward the more benign end of the spectrum in the contemporary clinic population,” said Dr. Kister.
Other researchers have observed a similar phenomenon. George et al. published the results of a large, international collaboration in Neurology Genetics in 2016. After examining more than 7,000 patients, the investigators noted a similar overrepresentation of patients with milder severity scores and underrepresentation of patients with higher severity scores. These results support the hypothesis of a shift toward milder disability, said Dr. Kister.
Trend toward milder disability
The investigators next examined whether the rate of accumulation of disability among patients with MS had changed from year to year since DMTs were introduced. They conducted a univariate analysis of MSSS for 6,238 patients who were enrolled in the N.Y. State MS Consortium during 1996-2007. They found that patients who were enrolled in more recent years had significantly lower MSSS than patients who were enrolled in earlier years, regardless of disease duration. When Dr. Kister and colleagues replicated their analysis using EDSS, they found significantly lower levels of disability for patients enrolled in more recent years, except for patients with disease duration of 26-30 years. A multivariate analysis showed that the median MSSS of enrollees into the N.Y. State MS Consortium decreased from 5.04 in 1996 to 3.78 in 2006.
In a subsequent study, Dr. Kister and colleagues examined the age at which patients in the MSBase registry reached various disability milestones (e.g., EDSS of 6, which indicates the need of a cane to walk outdoors), according to their year of enrollment in the registry. They found a significant increase in age at milestone achievement with each subsequent calendar year. For example, for every consecutive year of enrollment, the age at which patients attained an EDSS of 6 increased by 0.38 years. These analyses were confirmed for the subgroups of patients diagnosed according to the Poser and McDonald criteria. The increase in age “is probably not just related to the shift in diagnostic criteria,” said Dr. Kister. When the researchers calculated the net average gains in years over the 13-year follow-up period, they found that patients who entered at the end of the enrollment period were 4.9 years older when they reached an EDSS of 6, compared with patients with an EDSS of 6 who entered at the beginning of the enrollment period.
International data show similar trends
Research conducted around the world shows similar trends, said Dr. Kister. In 2009, Veugelers et al. published the results of a study that included 1,752 patients with MS in Nova Scotia. Before the 1998 introduction of a drug insurance program that provides DMTs, the time to an EDSS of 6 was 14.4 years. After the introduction of this program, the time to EDSS of 6 was 18.6 years.
More recently, Capra et al. examined 1,324 patients with MS who attended an MS center in Brescia, Italy, during 1980-2010. They found that the age at which 50% of patients reached an EDSS of 6 was approximately 55 years in 1990. By 2010, the age at achieving this milestone had increased to approximately 63 years.
In a prospective study, Cree et al. examined the evolution of disability in 448 actively treated patients with relapsing-remitting MS and 69 patients with progressive MS. Approximately 45% of patients had no disability worsening during a 10-year follow-up period. Furthermore, a comparatively low 11% of patients had reached an EDSS of 6 at 10 years. The average disease duration of the cohort at that time was 17 years, said Dr. Kister. The results indicated that about 50% of patients would be expected to reach an EDSS of 6 after a disease duration of approximately 38 years, “which is much longer than in the natural history studies,” he added.
In 2019, Beiki et al. found that among patients with relapsing-remitting MS, the risk of reaching an EDSS of 6 decreased by 7% with each subsequent calendar year of diagnosis. The researchers did not observe a similar trend among patients with progressive MS. Their population-based, retrospective study included 7,331 patients in Sweden.
Two additional studies in Scandinavian populations add to the evidence of decreasing disability. In their examination of Swedish patients with MS who received a diagnosis of MS during 1968-2012, Burkill et al. found that the risk of death decreased over time. The hazard ratio of mortality for patients with MS, compared with a non-MS comparator group, decreased from 6.52 among those diagnosed during 1968-1980 to 2.08 for patients diagnosed during 2001-2012. The decrease in the risk of mortality was greater among patients with MS than in a matched comparator population. Similarly, in a nationwide, population-based study, Koch-Henriksen et al. found that all-cause excess mortality in Danish patients with MS decreased from 1950 through 1999.
The role of DMTs
The evidence suggests that DMTs are affecting the long-term progression of MS, said Dr. Kister. Palace et al. compared patients with MS in the UK who received treatment with interferon-beta with a modeled untreated cohort of patients in British Columbia. They found that treated patients reached an EDSS of 6 4 years later than did untreated patients.
Furthermore, an analysis by Brown et al. showed that the time to conversion to secondary progressive MS was longer among treated patients, compared with untreated patients. The risk of conversion was lower for patients treated with newer, more effective therapies (i.e., fingolimod, alemtuzumab, or natalizumab) than for those treated with glatiramer acetate or interferon beta.
Finally, Kingwell and colleagues examined the effect of treatment with interferon-beta on survival using an international cohort of approximately 6,000 patients with relapsing-remitting MS. They found that exposure to interferon-beta for more than 3 years was associated with a 32% reduction in the risk of mortality. They observed no similar risk reduction among patients exposed to interferon-beta for 6 months to 3 years.
Although these data are encouraging, other evidence indicates that the prevalence of MS in the United States has increased considerably in the past 40 years. Researchers estimate that 1 million Americans have MS, which “suggests that we are diagnosing many more mild cases,” said Dr. Kister. The burden of the disease remains high, he concluded.
Dr. Kister reported receiving consulting fees or research grants from Biogen, Roche, Genzyme and Genentech.
SOURCE: Kister I et al. ECTRIMS 2019. Abstract 281754.
STOCKHOLM – , according to an overview provided at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis. Data consistently indicate that the time that elapses before a patient requires a cane for ambulation has increased, and survival has likewise improved. “Some of the improvement can be attributed confidently to treatment effect,” said Ilya Kister, MD, associate professor of neurology at NYU Langone Health in New York. “We hope to see an even greater change with newer therapies.”
At the same time, neurologists appear to be diagnosing more cases of MS than they previously did, said Dr. Kister, which suggests that neurologists probably are diagnosing milder cases. The overall societal burden of MS remains high.
The relative prevalence of mild disability has increased
About 25 years have elapsed since the first disease-modifying treatment (DMT) for MS became available, and treatment has become widespread during that time. Dr. Kister and colleagues sought to determine whether the current clinical population of patients with MS, who for the most part receive DMTs, has less disability than do untreated patients or patients from natural history studies do. They identified the MS Severity Score (MSSS) as a measure with which to compare populations. The MSSS assigns a patient a ranking according to his or her level of disability, using a reference population of patients with the same disease duration for comparison. “MSSS can be conceptualized as rate of disability accumulation,” said Dr. Kister. “Lower MSSS corresponds to relatively slower disability accumulation, and higher MSSS to higher disability accumulation.”
The MSSS was developed using the Expanded Disability Status Scale (EDSS) score as a measure of disability. Because many neurologists do not routinely obtain EDSS scores for their patients, Dr. Kister and colleagues used the Patient-Determined Disease Steps (PDDS) to measure disability. As its name implies, the PDDS is a patient-reported outcome measure that mainly measures ambulation. It correlates strongly with EDSS, said Dr. Kister. He and colleagues used the PDDS to develop a reference table of MS disability, which they called the Patient-Derived MSSS.
The investigators examined a large sample of patients at NYU MS Center and Barnabas MS Center in Livingston, N.J. They grouped patients into sextiles according to their Patient-Derived MSSS. Dr. Kister and colleagues found that, rather than arriving at sextiles that contained equal numbers of patients, as would be expected if disability were distributed as in the reference population, they had significantly more patients in the two lowest sextiles and significantly fewer patients in the two highest sextiles. “This [result] suggests that the disability curve has indeed shifted toward the more benign end of the spectrum in the contemporary clinic population,” said Dr. Kister.
Other researchers have observed a similar phenomenon. George et al. published the results of a large, international collaboration in Neurology Genetics in 2016. After examining more than 7,000 patients, the investigators noted a similar overrepresentation of patients with milder severity scores and underrepresentation of patients with higher severity scores. These results support the hypothesis of a shift toward milder disability, said Dr. Kister.
Trend toward milder disability
The investigators next examined whether the rate of accumulation of disability among patients with MS had changed from year to year since DMTs were introduced. They conducted a univariate analysis of MSSS for 6,238 patients who were enrolled in the N.Y. State MS Consortium during 1996-2007. They found that patients who were enrolled in more recent years had significantly lower MSSS than patients who were enrolled in earlier years, regardless of disease duration. When Dr. Kister and colleagues replicated their analysis using EDSS, they found significantly lower levels of disability for patients enrolled in more recent years, except for patients with disease duration of 26-30 years. A multivariate analysis showed that the median MSSS of enrollees into the N.Y. State MS Consortium decreased from 5.04 in 1996 to 3.78 in 2006.
In a subsequent study, Dr. Kister and colleagues examined the age at which patients in the MSBase registry reached various disability milestones (e.g., EDSS of 6, which indicates the need of a cane to walk outdoors), according to their year of enrollment in the registry. They found a significant increase in age at milestone achievement with each subsequent calendar year. For example, for every consecutive year of enrollment, the age at which patients attained an EDSS of 6 increased by 0.38 years. These analyses were confirmed for the subgroups of patients diagnosed according to the Poser and McDonald criteria. The increase in age “is probably not just related to the shift in diagnostic criteria,” said Dr. Kister. When the researchers calculated the net average gains in years over the 13-year follow-up period, they found that patients who entered at the end of the enrollment period were 4.9 years older when they reached an EDSS of 6, compared with patients with an EDSS of 6 who entered at the beginning of the enrollment period.
International data show similar trends
Research conducted around the world shows similar trends, said Dr. Kister. In 2009, Veugelers et al. published the results of a study that included 1,752 patients with MS in Nova Scotia. Before the 1998 introduction of a drug insurance program that provides DMTs, the time to an EDSS of 6 was 14.4 years. After the introduction of this program, the time to EDSS of 6 was 18.6 years.
More recently, Capra et al. examined 1,324 patients with MS who attended an MS center in Brescia, Italy, during 1980-2010. They found that the age at which 50% of patients reached an EDSS of 6 was approximately 55 years in 1990. By 2010, the age at achieving this milestone had increased to approximately 63 years.
In a prospective study, Cree et al. examined the evolution of disability in 448 actively treated patients with relapsing-remitting MS and 69 patients with progressive MS. Approximately 45% of patients had no disability worsening during a 10-year follow-up period. Furthermore, a comparatively low 11% of patients had reached an EDSS of 6 at 10 years. The average disease duration of the cohort at that time was 17 years, said Dr. Kister. The results indicated that about 50% of patients would be expected to reach an EDSS of 6 after a disease duration of approximately 38 years, “which is much longer than in the natural history studies,” he added.
In 2019, Beiki et al. found that among patients with relapsing-remitting MS, the risk of reaching an EDSS of 6 decreased by 7% with each subsequent calendar year of diagnosis. The researchers did not observe a similar trend among patients with progressive MS. Their population-based, retrospective study included 7,331 patients in Sweden.
Two additional studies in Scandinavian populations add to the evidence of decreasing disability. In their examination of Swedish patients with MS who received a diagnosis of MS during 1968-2012, Burkill et al. found that the risk of death decreased over time. The hazard ratio of mortality for patients with MS, compared with a non-MS comparator group, decreased from 6.52 among those diagnosed during 1968-1980 to 2.08 for patients diagnosed during 2001-2012. The decrease in the risk of mortality was greater among patients with MS than in a matched comparator population. Similarly, in a nationwide, population-based study, Koch-Henriksen et al. found that all-cause excess mortality in Danish patients with MS decreased from 1950 through 1999.
The role of DMTs
The evidence suggests that DMTs are affecting the long-term progression of MS, said Dr. Kister. Palace et al. compared patients with MS in the UK who received treatment with interferon-beta with a modeled untreated cohort of patients in British Columbia. They found that treated patients reached an EDSS of 6 4 years later than did untreated patients.
Furthermore, an analysis by Brown et al. showed that the time to conversion to secondary progressive MS was longer among treated patients, compared with untreated patients. The risk of conversion was lower for patients treated with newer, more effective therapies (i.e., fingolimod, alemtuzumab, or natalizumab) than for those treated with glatiramer acetate or interferon beta.
Finally, Kingwell and colleagues examined the effect of treatment with interferon-beta on survival using an international cohort of approximately 6,000 patients with relapsing-remitting MS. They found that exposure to interferon-beta for more than 3 years was associated with a 32% reduction in the risk of mortality. They observed no similar risk reduction among patients exposed to interferon-beta for 6 months to 3 years.
Although these data are encouraging, other evidence indicates that the prevalence of MS in the United States has increased considerably in the past 40 years. Researchers estimate that 1 million Americans have MS, which “suggests that we are diagnosing many more mild cases,” said Dr. Kister. The burden of the disease remains high, he concluded.
Dr. Kister reported receiving consulting fees or research grants from Biogen, Roche, Genzyme and Genentech.
SOURCE: Kister I et al. ECTRIMS 2019. Abstract 281754.
EXPERT ANALYSIS FROM ECTRIMS 2019
Fetal MRI may change pregnancy management
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. This imaging technique and neurologic consultation complement the information that prenatal ultrasound and obstetric consultations provide and may influence pregnancy management and infant neurologic care significantly.
The fetal diagnosis of posterior fossa abnormalities can be challenging. The prognosis can vary greatly, depending on the diagnosis. Sarah Mulkey, MD, PhD, director of the fetal and neonatal fellowship and a fetal and neonatal neurologist at Children’s National in Washington, and colleagues conducted an analysis to evaluate whether fetal MRI and neurology consultation produce alternative diagnoses for maternal-fetal dyads who are referred to a fetal neurology program because of concern for a fetal posterior fossa anomaly. The researchers also sought to determine how often the postnatal evaluation differed from the fetal diagnosis.
Dr. Mulkey and colleagues retrospectively analyzed cases referred to the Fetal Medicine Institute at Children’s National from January 2012 to June 2018. They included the referral diagnoses of Dandy-Walker continuum, cerebellar hypoplasia, vermis hypoplasia, Blake’s pouch cyst, mega cisterna magna, and other posterior fossa anomalies in their study.
The investigators identified 188 cases that had undergone fetal MRI and neurology consultation. The average gestational age at evaluation was 25 weeks, and the average maternal age was 30 years. Approximately 43% of referrals resulted from a concern regarding Dandy-Walker malformation, and 21% of referrals resulted from a suspicion of mega cisterna magna.
Fetal MRI and neurology consultation resulted in a change from the referral diagnosis or additional information about the fetus in 124 (66%) cases. For example, after imaging and consultation, 15% of referrals were diagnosed with Dandy-Walker malformation, as opposed to the 43% who were suspected of having it. Most referrals with a diagnosis of vermis hypoplasia had a better prognosis after fetal MRI. Fetal MRI and consultation also resulted in new diagnoses of Joubert syndrome and rhombencephalosynapsis. About 19% of referrals were considered normal. “A considerable number of these referrals ended up being for conditions that would have a good outcome,” said Dr. Mulkey.
In addition, the researchers obtained the postnatal diagnosis for 60 of 138 (43%) live-born infants. The fetal diagnosis of Dandy-Walker continuum was confirmed post natally in six of six (100%) cases. Of the 13 cases of fetally diagnosed vermis hypoplasia, 7 (54%) had stable findings, 3 (23%) normalized, and diagnosis changed in 3 (23%). Of the 17 fetally diagnosed Blake’s pouch cysts, 8 (47%) remained stable, 5 (29%) normalized, and diagnosis changed in 4 (24%). Four of nine (44%) cases of fetally diagnosed mega cisterna magna remained stable, two (22%) normalized, and diagnosis changed in three (33%). Overall, prognosis did not change after postnatal imaging.
“There is a high degree of correlation between fetal and postnatal diagnoses for Dandy-Walker continuum, cerebellar hypoplasia, cyst, and ‘other’ diagnoses,” said Dr. Mulkey. “Vermis hypoplasia and Blake’s pouch cyst diagnoses were less consistent.”
The investigators reported no disclosures.
SOURCE: Schlatterer S et al. CNS 2019, Abstract 158.
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. This imaging technique and neurologic consultation complement the information that prenatal ultrasound and obstetric consultations provide and may influence pregnancy management and infant neurologic care significantly.
The fetal diagnosis of posterior fossa abnormalities can be challenging. The prognosis can vary greatly, depending on the diagnosis. Sarah Mulkey, MD, PhD, director of the fetal and neonatal fellowship and a fetal and neonatal neurologist at Children’s National in Washington, and colleagues conducted an analysis to evaluate whether fetal MRI and neurology consultation produce alternative diagnoses for maternal-fetal dyads who are referred to a fetal neurology program because of concern for a fetal posterior fossa anomaly. The researchers also sought to determine how often the postnatal evaluation differed from the fetal diagnosis.
Dr. Mulkey and colleagues retrospectively analyzed cases referred to the Fetal Medicine Institute at Children’s National from January 2012 to June 2018. They included the referral diagnoses of Dandy-Walker continuum, cerebellar hypoplasia, vermis hypoplasia, Blake’s pouch cyst, mega cisterna magna, and other posterior fossa anomalies in their study.
The investigators identified 188 cases that had undergone fetal MRI and neurology consultation. The average gestational age at evaluation was 25 weeks, and the average maternal age was 30 years. Approximately 43% of referrals resulted from a concern regarding Dandy-Walker malformation, and 21% of referrals resulted from a suspicion of mega cisterna magna.
Fetal MRI and neurology consultation resulted in a change from the referral diagnosis or additional information about the fetus in 124 (66%) cases. For example, after imaging and consultation, 15% of referrals were diagnosed with Dandy-Walker malformation, as opposed to the 43% who were suspected of having it. Most referrals with a diagnosis of vermis hypoplasia had a better prognosis after fetal MRI. Fetal MRI and consultation also resulted in new diagnoses of Joubert syndrome and rhombencephalosynapsis. About 19% of referrals were considered normal. “A considerable number of these referrals ended up being for conditions that would have a good outcome,” said Dr. Mulkey.
In addition, the researchers obtained the postnatal diagnosis for 60 of 138 (43%) live-born infants. The fetal diagnosis of Dandy-Walker continuum was confirmed post natally in six of six (100%) cases. Of the 13 cases of fetally diagnosed vermis hypoplasia, 7 (54%) had stable findings, 3 (23%) normalized, and diagnosis changed in 3 (23%). Of the 17 fetally diagnosed Blake’s pouch cysts, 8 (47%) remained stable, 5 (29%) normalized, and diagnosis changed in 4 (24%). Four of nine (44%) cases of fetally diagnosed mega cisterna magna remained stable, two (22%) normalized, and diagnosis changed in three (33%). Overall, prognosis did not change after postnatal imaging.
“There is a high degree of correlation between fetal and postnatal diagnoses for Dandy-Walker continuum, cerebellar hypoplasia, cyst, and ‘other’ diagnoses,” said Dr. Mulkey. “Vermis hypoplasia and Blake’s pouch cyst diagnoses were less consistent.”
The investigators reported no disclosures.
SOURCE: Schlatterer S et al. CNS 2019, Abstract 158.
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. This imaging technique and neurologic consultation complement the information that prenatal ultrasound and obstetric consultations provide and may influence pregnancy management and infant neurologic care significantly.
The fetal diagnosis of posterior fossa abnormalities can be challenging. The prognosis can vary greatly, depending on the diagnosis. Sarah Mulkey, MD, PhD, director of the fetal and neonatal fellowship and a fetal and neonatal neurologist at Children’s National in Washington, and colleagues conducted an analysis to evaluate whether fetal MRI and neurology consultation produce alternative diagnoses for maternal-fetal dyads who are referred to a fetal neurology program because of concern for a fetal posterior fossa anomaly. The researchers also sought to determine how often the postnatal evaluation differed from the fetal diagnosis.
Dr. Mulkey and colleagues retrospectively analyzed cases referred to the Fetal Medicine Institute at Children’s National from January 2012 to June 2018. They included the referral diagnoses of Dandy-Walker continuum, cerebellar hypoplasia, vermis hypoplasia, Blake’s pouch cyst, mega cisterna magna, and other posterior fossa anomalies in their study.
The investigators identified 188 cases that had undergone fetal MRI and neurology consultation. The average gestational age at evaluation was 25 weeks, and the average maternal age was 30 years. Approximately 43% of referrals resulted from a concern regarding Dandy-Walker malformation, and 21% of referrals resulted from a suspicion of mega cisterna magna.
Fetal MRI and neurology consultation resulted in a change from the referral diagnosis or additional information about the fetus in 124 (66%) cases. For example, after imaging and consultation, 15% of referrals were diagnosed with Dandy-Walker malformation, as opposed to the 43% who were suspected of having it. Most referrals with a diagnosis of vermis hypoplasia had a better prognosis after fetal MRI. Fetal MRI and consultation also resulted in new diagnoses of Joubert syndrome and rhombencephalosynapsis. About 19% of referrals were considered normal. “A considerable number of these referrals ended up being for conditions that would have a good outcome,” said Dr. Mulkey.
In addition, the researchers obtained the postnatal diagnosis for 60 of 138 (43%) live-born infants. The fetal diagnosis of Dandy-Walker continuum was confirmed post natally in six of six (100%) cases. Of the 13 cases of fetally diagnosed vermis hypoplasia, 7 (54%) had stable findings, 3 (23%) normalized, and diagnosis changed in 3 (23%). Of the 17 fetally diagnosed Blake’s pouch cysts, 8 (47%) remained stable, 5 (29%) normalized, and diagnosis changed in 4 (24%). Four of nine (44%) cases of fetally diagnosed mega cisterna magna remained stable, two (22%) normalized, and diagnosis changed in three (33%). Overall, prognosis did not change after postnatal imaging.
“There is a high degree of correlation between fetal and postnatal diagnoses for Dandy-Walker continuum, cerebellar hypoplasia, cyst, and ‘other’ diagnoses,” said Dr. Mulkey. “Vermis hypoplasia and Blake’s pouch cyst diagnoses were less consistent.”
The investigators reported no disclosures.
SOURCE: Schlatterer S et al. CNS 2019, Abstract 158.
REPORTING FROM CNS 2019
Delay in EEG monitoring associated with increased seizure duration in pediatric refractory status epilepticus
CHARLOTTE, NC – , according to a multicenter study that was presented at the annual meeting of the Child Neurology Society. Delays in initiating EEG monitoring are associated with longer seizure duration in this patient population.
Neurologists are advised to initiate continuous EEG monitoring rapidly for all cases of pediatric refractory status epilepticus. Little information is available, however, about patterns in the timing of EEG placement. In addition, the relationship between delays in the initiation of continuous EEG and outcomes of refractory status epilepticus are unknown. Dmitry Tchapyjnikov, MD, assistant professor of child neurology at Duke University in Durham, N.C., and colleagues evaluated trends in the time to continuous EEG initiation and examined whether delays are associated with longer seizure duration in children with refractory status epilepticus.
A retrospective analysis of pSERG data
Dr. Tchapyjnikov and colleagues analyzed data from 11 hospitals participating in the Pediatric Status Epilepticus Research Group (pSERG), a prospective, observational cohort. They focused on pediatric patients who were admitted from 2011 to 2017 with refractory status epilepticus, which they defined as a seizure that persisted after treatment with two or more antiseizure medications (ASMs), one of which had to be a nonbenzodiazepine ASM, or a continuous infusion. Eligible patients were between 1 month and 21 years old and had convulsive seizures at onset. Patients who had EEG placement before seizure onset were excluded.
The investigators included in their study 121 patients who had seizure durations of 3 or more hours. Based on an exploratory analysis of various time-point cutoffs, Dr. Tchapyjnikov and colleagues defined delayed continuous EEG placement as placement at more than 5 hours after seizure onset. They used the Kaplan–Meier estimator to assess time to continuous EEG and used covariate-adjusted proportional hazards models to examine the association between delay in continuous EEG placement and seizure duration.
EEG placement overall was delayed
The median time to continuous EEG placement after seizure onset was 9 hours. Approximately 4% of the children had continuous EEG placed within 1 hour, and 74% had it placed within 24 hours.
The investigators found that seizure onset outside the study hospital was associated with a higher likelihood of delayed time to EEG placement. “Females seemed to be more likely to have timely EEG placement,” said Dr. Tchapyjnikov. “I don’t have a physiological explanation for that.” The researchers saw no difference in treatment between patients who had timely EEG placement and those who had delayed EEG placement.
About 68% of children were having seizures at the time of continuous EEG placement. A presumed seizure etiology of CNS infection was associated with a higher likelihood of being in status epilepticus at the time of EEG placement. A history of epilepsy, developmental delay, or home ASM use, however, was associated with a lower likelihood of being in status epilepticus at time of EEG placement.
Dr. Tchapyjnikov’s group found that the 24-hour cumulative probability of seizure resolution was lower among patients who did not have continuous EEG initiation within 5 hours, compared with those who did (56% vs.70%). The association remained significant after the investigators adjusted the data for covariates that were independently associated with 24-hour seizure resolution (hazard ratio, 0.31).
The investigators included in their analysis patients who had seizure resolution before EEG placement, because restricting the analysis to patients who have persistent status epilepticus would have overemphasized the benefits of EEG, according to Dr. Tchapyjnikov. “Looking at the overall hazard ratios is a more conservative way of looking at these data.”
The study was not supported by external funding. Dr. Tchapyjnikov had no relevant disclosures.
CHARLOTTE, NC – , according to a multicenter study that was presented at the annual meeting of the Child Neurology Society. Delays in initiating EEG monitoring are associated with longer seizure duration in this patient population.
Neurologists are advised to initiate continuous EEG monitoring rapidly for all cases of pediatric refractory status epilepticus. Little information is available, however, about patterns in the timing of EEG placement. In addition, the relationship between delays in the initiation of continuous EEG and outcomes of refractory status epilepticus are unknown. Dmitry Tchapyjnikov, MD, assistant professor of child neurology at Duke University in Durham, N.C., and colleagues evaluated trends in the time to continuous EEG initiation and examined whether delays are associated with longer seizure duration in children with refractory status epilepticus.
A retrospective analysis of pSERG data
Dr. Tchapyjnikov and colleagues analyzed data from 11 hospitals participating in the Pediatric Status Epilepticus Research Group (pSERG), a prospective, observational cohort. They focused on pediatric patients who were admitted from 2011 to 2017 with refractory status epilepticus, which they defined as a seizure that persisted after treatment with two or more antiseizure medications (ASMs), one of which had to be a nonbenzodiazepine ASM, or a continuous infusion. Eligible patients were between 1 month and 21 years old and had convulsive seizures at onset. Patients who had EEG placement before seizure onset were excluded.
The investigators included in their study 121 patients who had seizure durations of 3 or more hours. Based on an exploratory analysis of various time-point cutoffs, Dr. Tchapyjnikov and colleagues defined delayed continuous EEG placement as placement at more than 5 hours after seizure onset. They used the Kaplan–Meier estimator to assess time to continuous EEG and used covariate-adjusted proportional hazards models to examine the association between delay in continuous EEG placement and seizure duration.
EEG placement overall was delayed
The median time to continuous EEG placement after seizure onset was 9 hours. Approximately 4% of the children had continuous EEG placed within 1 hour, and 74% had it placed within 24 hours.
The investigators found that seizure onset outside the study hospital was associated with a higher likelihood of delayed time to EEG placement. “Females seemed to be more likely to have timely EEG placement,” said Dr. Tchapyjnikov. “I don’t have a physiological explanation for that.” The researchers saw no difference in treatment between patients who had timely EEG placement and those who had delayed EEG placement.
About 68% of children were having seizures at the time of continuous EEG placement. A presumed seizure etiology of CNS infection was associated with a higher likelihood of being in status epilepticus at the time of EEG placement. A history of epilepsy, developmental delay, or home ASM use, however, was associated with a lower likelihood of being in status epilepticus at time of EEG placement.
Dr. Tchapyjnikov’s group found that the 24-hour cumulative probability of seizure resolution was lower among patients who did not have continuous EEG initiation within 5 hours, compared with those who did (56% vs.70%). The association remained significant after the investigators adjusted the data for covariates that were independently associated with 24-hour seizure resolution (hazard ratio, 0.31).
The investigators included in their analysis patients who had seizure resolution before EEG placement, because restricting the analysis to patients who have persistent status epilepticus would have overemphasized the benefits of EEG, according to Dr. Tchapyjnikov. “Looking at the overall hazard ratios is a more conservative way of looking at these data.”
The study was not supported by external funding. Dr. Tchapyjnikov had no relevant disclosures.
CHARLOTTE, NC – , according to a multicenter study that was presented at the annual meeting of the Child Neurology Society. Delays in initiating EEG monitoring are associated with longer seizure duration in this patient population.
Neurologists are advised to initiate continuous EEG monitoring rapidly for all cases of pediatric refractory status epilepticus. Little information is available, however, about patterns in the timing of EEG placement. In addition, the relationship between delays in the initiation of continuous EEG and outcomes of refractory status epilepticus are unknown. Dmitry Tchapyjnikov, MD, assistant professor of child neurology at Duke University in Durham, N.C., and colleagues evaluated trends in the time to continuous EEG initiation and examined whether delays are associated with longer seizure duration in children with refractory status epilepticus.
A retrospective analysis of pSERG data
Dr. Tchapyjnikov and colleagues analyzed data from 11 hospitals participating in the Pediatric Status Epilepticus Research Group (pSERG), a prospective, observational cohort. They focused on pediatric patients who were admitted from 2011 to 2017 with refractory status epilepticus, which they defined as a seizure that persisted after treatment with two or more antiseizure medications (ASMs), one of which had to be a nonbenzodiazepine ASM, or a continuous infusion. Eligible patients were between 1 month and 21 years old and had convulsive seizures at onset. Patients who had EEG placement before seizure onset were excluded.
The investigators included in their study 121 patients who had seizure durations of 3 or more hours. Based on an exploratory analysis of various time-point cutoffs, Dr. Tchapyjnikov and colleagues defined delayed continuous EEG placement as placement at more than 5 hours after seizure onset. They used the Kaplan–Meier estimator to assess time to continuous EEG and used covariate-adjusted proportional hazards models to examine the association between delay in continuous EEG placement and seizure duration.
EEG placement overall was delayed
The median time to continuous EEG placement after seizure onset was 9 hours. Approximately 4% of the children had continuous EEG placed within 1 hour, and 74% had it placed within 24 hours.
The investigators found that seizure onset outside the study hospital was associated with a higher likelihood of delayed time to EEG placement. “Females seemed to be more likely to have timely EEG placement,” said Dr. Tchapyjnikov. “I don’t have a physiological explanation for that.” The researchers saw no difference in treatment between patients who had timely EEG placement and those who had delayed EEG placement.
About 68% of children were having seizures at the time of continuous EEG placement. A presumed seizure etiology of CNS infection was associated with a higher likelihood of being in status epilepticus at the time of EEG placement. A history of epilepsy, developmental delay, or home ASM use, however, was associated with a lower likelihood of being in status epilepticus at time of EEG placement.
Dr. Tchapyjnikov’s group found that the 24-hour cumulative probability of seizure resolution was lower among patients who did not have continuous EEG initiation within 5 hours, compared with those who did (56% vs.70%). The association remained significant after the investigators adjusted the data for covariates that were independently associated with 24-hour seizure resolution (hazard ratio, 0.31).
The investigators included in their analysis patients who had seizure resolution before EEG placement, because restricting the analysis to patients who have persistent status epilepticus would have overemphasized the benefits of EEG, according to Dr. Tchapyjnikov. “Looking at the overall hazard ratios is a more conservative way of looking at these data.”
The study was not supported by external funding. Dr. Tchapyjnikov had no relevant disclosures.
REPORTING FROM CNS 2019
Does AED prophylaxis delay seizure onset in children with brain tumors?
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. Levetiracetam, oxcarbazepine, and phenytoin are the most common initial prophylactic AEDs administered to children with brain tumors, the researchers said.
The literature indicates that between 20% and 35% of children with brain tumors have seizures, and up to half of these patients have seizure as their presenting symptom. Common practice is to prescribe antiseizure medication after a child has had a first seizure, because the risk for recurrence is high. In 2000, the American Academy of Neurology discouraged prophylactic use of AEDs in children, citing a lack of evidence for efficacy. Most of the data that it reviewed, however, came from adults.
Michelle Yun, a medical student at Weill Cornell Medical College, New York, and colleagues used national Medicaid claims data that had been collected between 2009 and 2012 for children with seizures to conduct a retrospective, observational, case-control study. They included children aged 0-20 years with a diagnosis of brain tumor, a seizure diagnosis within 6 months after brain tumor diagnosis, an AED prescription, and 12 continuous months of Medicaid coverage following brain tumor diagnosis in their analysis. The investigators defined seizure prophylaxis as AED prescription within 30 days after brain tumor diagnosis but before a first seizure diagnosis.
The exposure in the study was AED prescription within 45 days of diagnosis, and the outcome was the time to first seizure. Ms. Yun and colleagues also analyzed the most common initial prophylactic AEDs and the proportion of cases with first seizure diagnosis after prophylactic AED discontinuation, which was defined as a treatment gap longer than 30 days. The study covariates included age, sex, race, ethnicity, and medical comorbidities.
In all, 218 children were included in the study; 40 received AED prophylaxis and 26 received it within 45 days of brain tumor diagnosis. Patients with and without AED prophylaxis were well matched on all covariates.
At 1 year, Ms. Yun and colleagues saw no difference in time to first seizure between the two groups. The median time to first seizure was 75 days in the prophylaxis group and 80 days in the no-prophylaxis group. The researchers observed a transient separation between the two groups, however, in the early months after brain tumor diagnosis. When they examined children who had a seizure during the first 6 months of follow-up, the median time to diagnosis of first seizure was 68 days in children with prophylaxis and 34 days in the no-prophylaxis group. The difference between groups was statistically significant. “When we added all the covariates of interest, we found that there was a protective effect in these children with early seizures,” said Ms. Yun.
Among the study limitations that Ms. Yun acknowledged were its observational, retrospective design and its small sample size. Medicaid data themselves are limited, since states do not report them in a uniform manner, and the data do not include much clinical information. “Something that would be helpful is a prospective clinical study,” Ms. Yun concluded.
The Weill Cornell Clinical and Translational Science Center and the American Academy of Neurology provided funding for the study. The Pediatric Epilepsy Research Foundation provided the Medicaid data. Ms. Yun had no relevant disclosures.
SOURCE: Yun M et al. CNS 2019, Abstract PL2-1.
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. Levetiracetam, oxcarbazepine, and phenytoin are the most common initial prophylactic AEDs administered to children with brain tumors, the researchers said.
The literature indicates that between 20% and 35% of children with brain tumors have seizures, and up to half of these patients have seizure as their presenting symptom. Common practice is to prescribe antiseizure medication after a child has had a first seizure, because the risk for recurrence is high. In 2000, the American Academy of Neurology discouraged prophylactic use of AEDs in children, citing a lack of evidence for efficacy. Most of the data that it reviewed, however, came from adults.
Michelle Yun, a medical student at Weill Cornell Medical College, New York, and colleagues used national Medicaid claims data that had been collected between 2009 and 2012 for children with seizures to conduct a retrospective, observational, case-control study. They included children aged 0-20 years with a diagnosis of brain tumor, a seizure diagnosis within 6 months after brain tumor diagnosis, an AED prescription, and 12 continuous months of Medicaid coverage following brain tumor diagnosis in their analysis. The investigators defined seizure prophylaxis as AED prescription within 30 days after brain tumor diagnosis but before a first seizure diagnosis.
The exposure in the study was AED prescription within 45 days of diagnosis, and the outcome was the time to first seizure. Ms. Yun and colleagues also analyzed the most common initial prophylactic AEDs and the proportion of cases with first seizure diagnosis after prophylactic AED discontinuation, which was defined as a treatment gap longer than 30 days. The study covariates included age, sex, race, ethnicity, and medical comorbidities.
In all, 218 children were included in the study; 40 received AED prophylaxis and 26 received it within 45 days of brain tumor diagnosis. Patients with and without AED prophylaxis were well matched on all covariates.
At 1 year, Ms. Yun and colleagues saw no difference in time to first seizure between the two groups. The median time to first seizure was 75 days in the prophylaxis group and 80 days in the no-prophylaxis group. The researchers observed a transient separation between the two groups, however, in the early months after brain tumor diagnosis. When they examined children who had a seizure during the first 6 months of follow-up, the median time to diagnosis of first seizure was 68 days in children with prophylaxis and 34 days in the no-prophylaxis group. The difference between groups was statistically significant. “When we added all the covariates of interest, we found that there was a protective effect in these children with early seizures,” said Ms. Yun.
Among the study limitations that Ms. Yun acknowledged were its observational, retrospective design and its small sample size. Medicaid data themselves are limited, since states do not report them in a uniform manner, and the data do not include much clinical information. “Something that would be helpful is a prospective clinical study,” Ms. Yun concluded.
The Weill Cornell Clinical and Translational Science Center and the American Academy of Neurology provided funding for the study. The Pediatric Epilepsy Research Foundation provided the Medicaid data. Ms. Yun had no relevant disclosures.
SOURCE: Yun M et al. CNS 2019, Abstract PL2-1.
CHARLOTTE, N.C. – according to research presented at the annual meeting of the Child Neurology Society. Levetiracetam, oxcarbazepine, and phenytoin are the most common initial prophylactic AEDs administered to children with brain tumors, the researchers said.
The literature indicates that between 20% and 35% of children with brain tumors have seizures, and up to half of these patients have seizure as their presenting symptom. Common practice is to prescribe antiseizure medication after a child has had a first seizure, because the risk for recurrence is high. In 2000, the American Academy of Neurology discouraged prophylactic use of AEDs in children, citing a lack of evidence for efficacy. Most of the data that it reviewed, however, came from adults.
Michelle Yun, a medical student at Weill Cornell Medical College, New York, and colleagues used national Medicaid claims data that had been collected between 2009 and 2012 for children with seizures to conduct a retrospective, observational, case-control study. They included children aged 0-20 years with a diagnosis of brain tumor, a seizure diagnosis within 6 months after brain tumor diagnosis, an AED prescription, and 12 continuous months of Medicaid coverage following brain tumor diagnosis in their analysis. The investigators defined seizure prophylaxis as AED prescription within 30 days after brain tumor diagnosis but before a first seizure diagnosis.
The exposure in the study was AED prescription within 45 days of diagnosis, and the outcome was the time to first seizure. Ms. Yun and colleagues also analyzed the most common initial prophylactic AEDs and the proportion of cases with first seizure diagnosis after prophylactic AED discontinuation, which was defined as a treatment gap longer than 30 days. The study covariates included age, sex, race, ethnicity, and medical comorbidities.
In all, 218 children were included in the study; 40 received AED prophylaxis and 26 received it within 45 days of brain tumor diagnosis. Patients with and without AED prophylaxis were well matched on all covariates.
At 1 year, Ms. Yun and colleagues saw no difference in time to first seizure between the two groups. The median time to first seizure was 75 days in the prophylaxis group and 80 days in the no-prophylaxis group. The researchers observed a transient separation between the two groups, however, in the early months after brain tumor diagnosis. When they examined children who had a seizure during the first 6 months of follow-up, the median time to diagnosis of first seizure was 68 days in children with prophylaxis and 34 days in the no-prophylaxis group. The difference between groups was statistically significant. “When we added all the covariates of interest, we found that there was a protective effect in these children with early seizures,” said Ms. Yun.
Among the study limitations that Ms. Yun acknowledged were its observational, retrospective design and its small sample size. Medicaid data themselves are limited, since states do not report them in a uniform manner, and the data do not include much clinical information. “Something that would be helpful is a prospective clinical study,” Ms. Yun concluded.
The Weill Cornell Clinical and Translational Science Center and the American Academy of Neurology provided funding for the study. The Pediatric Epilepsy Research Foundation provided the Medicaid data. Ms. Yun had no relevant disclosures.
SOURCE: Yun M et al. CNS 2019, Abstract PL2-1.
REPORTING FROM CNS 2019
Researchers seek to characterize pediatric new daily persistent headache
CHARLOTTE, N.C. – according to research presented at the 48th national meeting of the Child Neurology Society. Most children with NDPH fulfill criteria for its migraine subtype, and one-third of pediatric patients with NDPH have comorbid medication overuse headache (MOH).
NDPH is defined as a daily, unremitting headache that lasts for at least 3 months. “Not many studies on NDPH focus on pediatrics,” said Emily Pierce, from Children’s National Medical Center in Washington. NDPH “is considered to be one of the most intractable headaches in children. Children are able to tell that they’ve had this different type of headache because there’s some kind of onset that is very memorable.”
Ms. Pierce and colleagues conducted an observational study to describe the characteristics of NDPH in pediatric patients who presented to a headache program at a tertiary referral center. The researchers included pediatric patients who visited the headache clinic at Children’s National Medical Center between 2016 and 2018 in their analysis. All patients were enrolled in patient registry that had been approved by an independent review board. Ms. Pierce and colleagues queried the registry for NDPH and reviewed these records to examine participants’ clinical presentations.
The investigators identified 3,260 patient encounters during the study period. Of these encounters, 454 patients (13.9%) were identified as having NDPH. Patients with NDPH were predominantly female (78%) and white (72%). The median age of the sample was 14.8 years.
The frontal head region was the most common location of headache pain, which often had a throbbing quality and was associated with photophobia, phonophobia, nausea, and decreased activity. The median pain intensity was 6 of 10. Approximately 72% of patients had tried abortive medication, and 56% of patients had failed at least one abortive medication. Excedrin, ibuprofen, and acetaminophen were among the common failed abortive medications.
Furthermore, 36% of patients were diagnosed with MOH. The most commonly overused medication was ibuprofen. MOH “is also considered to be intractable for patients with NDPH,” said Ms. Pierce. “Typically, if the patient stops overusing that medication, they’ll find relief from their headaches. However, with our NDPH patients, when they stop overusing that medication, they still are having headaches associated with NDPH.”
The data indicated “a strong difference between our male and female patients,” said Ms. Pierce. Female patients reported significantly more instances of photophobia, phonophobia, nausea, and dizziness than did male patients. Overall, 78% of participants had a diagnosis of an additional comorbidity, such as head trauma (18%), anxiety (14%), depression (8%), or other (37%).
Observational studies of pediatric NDPH offer “a better way for our providers to diagnose these patients, and also to better understand them and help them figure out a treatment that may work,” said Ms. Pierce. In future research, she and her colleagues intend to examine blood work and potential genetic associations in pediatric patients with NDPH.
The study was not supported by funding, and the investigators had no disclosures.
SOURCE: Pierce E et al. CNS 2019, Abstract 100.
CHARLOTTE, N.C. – according to research presented at the 48th national meeting of the Child Neurology Society. Most children with NDPH fulfill criteria for its migraine subtype, and one-third of pediatric patients with NDPH have comorbid medication overuse headache (MOH).
NDPH is defined as a daily, unremitting headache that lasts for at least 3 months. “Not many studies on NDPH focus on pediatrics,” said Emily Pierce, from Children’s National Medical Center in Washington. NDPH “is considered to be one of the most intractable headaches in children. Children are able to tell that they’ve had this different type of headache because there’s some kind of onset that is very memorable.”
Ms. Pierce and colleagues conducted an observational study to describe the characteristics of NDPH in pediatric patients who presented to a headache program at a tertiary referral center. The researchers included pediatric patients who visited the headache clinic at Children’s National Medical Center between 2016 and 2018 in their analysis. All patients were enrolled in patient registry that had been approved by an independent review board. Ms. Pierce and colleagues queried the registry for NDPH and reviewed these records to examine participants’ clinical presentations.
The investigators identified 3,260 patient encounters during the study period. Of these encounters, 454 patients (13.9%) were identified as having NDPH. Patients with NDPH were predominantly female (78%) and white (72%). The median age of the sample was 14.8 years.
The frontal head region was the most common location of headache pain, which often had a throbbing quality and was associated with photophobia, phonophobia, nausea, and decreased activity. The median pain intensity was 6 of 10. Approximately 72% of patients had tried abortive medication, and 56% of patients had failed at least one abortive medication. Excedrin, ibuprofen, and acetaminophen were among the common failed abortive medications.
Furthermore, 36% of patients were diagnosed with MOH. The most commonly overused medication was ibuprofen. MOH “is also considered to be intractable for patients with NDPH,” said Ms. Pierce. “Typically, if the patient stops overusing that medication, they’ll find relief from their headaches. However, with our NDPH patients, when they stop overusing that medication, they still are having headaches associated with NDPH.”
The data indicated “a strong difference between our male and female patients,” said Ms. Pierce. Female patients reported significantly more instances of photophobia, phonophobia, nausea, and dizziness than did male patients. Overall, 78% of participants had a diagnosis of an additional comorbidity, such as head trauma (18%), anxiety (14%), depression (8%), or other (37%).
Observational studies of pediatric NDPH offer “a better way for our providers to diagnose these patients, and also to better understand them and help them figure out a treatment that may work,” said Ms. Pierce. In future research, she and her colleagues intend to examine blood work and potential genetic associations in pediatric patients with NDPH.
The study was not supported by funding, and the investigators had no disclosures.
SOURCE: Pierce E et al. CNS 2019, Abstract 100.
CHARLOTTE, N.C. – according to research presented at the 48th national meeting of the Child Neurology Society. Most children with NDPH fulfill criteria for its migraine subtype, and one-third of pediatric patients with NDPH have comorbid medication overuse headache (MOH).
NDPH is defined as a daily, unremitting headache that lasts for at least 3 months. “Not many studies on NDPH focus on pediatrics,” said Emily Pierce, from Children’s National Medical Center in Washington. NDPH “is considered to be one of the most intractable headaches in children. Children are able to tell that they’ve had this different type of headache because there’s some kind of onset that is very memorable.”
Ms. Pierce and colleagues conducted an observational study to describe the characteristics of NDPH in pediatric patients who presented to a headache program at a tertiary referral center. The researchers included pediatric patients who visited the headache clinic at Children’s National Medical Center between 2016 and 2018 in their analysis. All patients were enrolled in patient registry that had been approved by an independent review board. Ms. Pierce and colleagues queried the registry for NDPH and reviewed these records to examine participants’ clinical presentations.
The investigators identified 3,260 patient encounters during the study period. Of these encounters, 454 patients (13.9%) were identified as having NDPH. Patients with NDPH were predominantly female (78%) and white (72%). The median age of the sample was 14.8 years.
The frontal head region was the most common location of headache pain, which often had a throbbing quality and was associated with photophobia, phonophobia, nausea, and decreased activity. The median pain intensity was 6 of 10. Approximately 72% of patients had tried abortive medication, and 56% of patients had failed at least one abortive medication. Excedrin, ibuprofen, and acetaminophen were among the common failed abortive medications.
Furthermore, 36% of patients were diagnosed with MOH. The most commonly overused medication was ibuprofen. MOH “is also considered to be intractable for patients with NDPH,” said Ms. Pierce. “Typically, if the patient stops overusing that medication, they’ll find relief from their headaches. However, with our NDPH patients, when they stop overusing that medication, they still are having headaches associated with NDPH.”
The data indicated “a strong difference between our male and female patients,” said Ms. Pierce. Female patients reported significantly more instances of photophobia, phonophobia, nausea, and dizziness than did male patients. Overall, 78% of participants had a diagnosis of an additional comorbidity, such as head trauma (18%), anxiety (14%), depression (8%), or other (37%).
Observational studies of pediatric NDPH offer “a better way for our providers to diagnose these patients, and also to better understand them and help them figure out a treatment that may work,” said Ms. Pierce. In future research, she and her colleagues intend to examine blood work and potential genetic associations in pediatric patients with NDPH.
The study was not supported by funding, and the investigators had no disclosures.
SOURCE: Pierce E et al. CNS 2019, Abstract 100.
REPORTING FROM CNS 2019
Many children who present to headache clinics have joint hypermobility
CHARLOTTE, N.C. – according to data presented at the 48th national meeting of the Child Neurology Society. Furthermore, patients with joint hypermobility have a high rate of headache disability, while patients without joint hypermobility have less headache disability, according to Dhwani Sahjwani, MD, a resident at Inova Fairfax Hospital in Falls Church, Va., and colleagues.
While conducting research in the headache clinic at Children’s National Hospital in Washington, D.C., Dr. Sahjwani saw several children with joint hypermobility and a diagnosis of a disorder such as Ehlers-Danlos syndrome. She and her colleagues began analyzing patients to evaluate the potential association between joint hypermobility and headache disability in children. The investigators included pediatric patients examined in the headache clinic at Children’s National Medical Center between October 2018 and January 2019 in their study. All headache clinic patients were enrolled in a patient registry that had been approved by an independent review board.
Dr. Sahjwani and colleagues measured patients’ headache disability with the Headache Impact Test–6 (HIT-6) questionnaire. Scores of 60 or greater on this questionnaire indicate severe headache disability. The researchers assessed joint hypermobility using the Beighton scoring system. In this system, scores greater than 4 indicate joint hypermobility.
Dr. Sahjwani’s group scored 76 patients using the Beighton system and HIT-6 questionnaire. Participants’ median age was 13.7 years. Approximately 26% of patients had Beighton scores that indicated joint hypermobility. About 65% of the patients with joint hypermobility had a diagnosis of migraine without aura. In addition, 80% of patients with joint hypermobility had severe headache disability, according to the HIT-6 disability criteria. The average pain intensity in patients with hypermobile joints was 6.1 out of 10. Among participants without significant joint hypermobility, 90% had mild headache disability.
Patients with joint hypermobility and increased tissue elasticity “tend to have a lower threshold for pain, in general, in all parts of their bodies,” said Dr. Sahjwani. Greater headache severity might be expected in this population, “because they have more pain if they have hypermobile joints or tissue.”
Headache treatments for this population are based solely on the type of headache that each patient has. Patients with joint hypermobility and migraine, for example, are candidates for rescue medication and long-term prophylactic medications. “I don’t think the joint hypermobility is going to change how you manage their headaches,” said Dr. Sahjwani.
The study results suggest that, when children present with severely debilitating headaches, a neurologist should consider examining them for joint hypermobility “to see if they have another diagnosis, such as Ehlers-Danlos syndrome ... that has to be managed in addition to their headaches,” Dr. Sahjwani concluded.
The study was not supported by funding. The authors did not report any disclosures.
SOURCE: Sahjwani D et al. CNS 2019, Abstract 101.
CHARLOTTE, N.C. – according to data presented at the 48th national meeting of the Child Neurology Society. Furthermore, patients with joint hypermobility have a high rate of headache disability, while patients without joint hypermobility have less headache disability, according to Dhwani Sahjwani, MD, a resident at Inova Fairfax Hospital in Falls Church, Va., and colleagues.
While conducting research in the headache clinic at Children’s National Hospital in Washington, D.C., Dr. Sahjwani saw several children with joint hypermobility and a diagnosis of a disorder such as Ehlers-Danlos syndrome. She and her colleagues began analyzing patients to evaluate the potential association between joint hypermobility and headache disability in children. The investigators included pediatric patients examined in the headache clinic at Children’s National Medical Center between October 2018 and January 2019 in their study. All headache clinic patients were enrolled in a patient registry that had been approved by an independent review board.
Dr. Sahjwani and colleagues measured patients’ headache disability with the Headache Impact Test–6 (HIT-6) questionnaire. Scores of 60 or greater on this questionnaire indicate severe headache disability. The researchers assessed joint hypermobility using the Beighton scoring system. In this system, scores greater than 4 indicate joint hypermobility.
Dr. Sahjwani’s group scored 76 patients using the Beighton system and HIT-6 questionnaire. Participants’ median age was 13.7 years. Approximately 26% of patients had Beighton scores that indicated joint hypermobility. About 65% of the patients with joint hypermobility had a diagnosis of migraine without aura. In addition, 80% of patients with joint hypermobility had severe headache disability, according to the HIT-6 disability criteria. The average pain intensity in patients with hypermobile joints was 6.1 out of 10. Among participants without significant joint hypermobility, 90% had mild headache disability.
Patients with joint hypermobility and increased tissue elasticity “tend to have a lower threshold for pain, in general, in all parts of their bodies,” said Dr. Sahjwani. Greater headache severity might be expected in this population, “because they have more pain if they have hypermobile joints or tissue.”
Headache treatments for this population are based solely on the type of headache that each patient has. Patients with joint hypermobility and migraine, for example, are candidates for rescue medication and long-term prophylactic medications. “I don’t think the joint hypermobility is going to change how you manage their headaches,” said Dr. Sahjwani.
The study results suggest that, when children present with severely debilitating headaches, a neurologist should consider examining them for joint hypermobility “to see if they have another diagnosis, such as Ehlers-Danlos syndrome ... that has to be managed in addition to their headaches,” Dr. Sahjwani concluded.
The study was not supported by funding. The authors did not report any disclosures.
SOURCE: Sahjwani D et al. CNS 2019, Abstract 101.
CHARLOTTE, N.C. – according to data presented at the 48th national meeting of the Child Neurology Society. Furthermore, patients with joint hypermobility have a high rate of headache disability, while patients without joint hypermobility have less headache disability, according to Dhwani Sahjwani, MD, a resident at Inova Fairfax Hospital in Falls Church, Va., and colleagues.
While conducting research in the headache clinic at Children’s National Hospital in Washington, D.C., Dr. Sahjwani saw several children with joint hypermobility and a diagnosis of a disorder such as Ehlers-Danlos syndrome. She and her colleagues began analyzing patients to evaluate the potential association between joint hypermobility and headache disability in children. The investigators included pediatric patients examined in the headache clinic at Children’s National Medical Center between October 2018 and January 2019 in their study. All headache clinic patients were enrolled in a patient registry that had been approved by an independent review board.
Dr. Sahjwani and colleagues measured patients’ headache disability with the Headache Impact Test–6 (HIT-6) questionnaire. Scores of 60 or greater on this questionnaire indicate severe headache disability. The researchers assessed joint hypermobility using the Beighton scoring system. In this system, scores greater than 4 indicate joint hypermobility.
Dr. Sahjwani’s group scored 76 patients using the Beighton system and HIT-6 questionnaire. Participants’ median age was 13.7 years. Approximately 26% of patients had Beighton scores that indicated joint hypermobility. About 65% of the patients with joint hypermobility had a diagnosis of migraine without aura. In addition, 80% of patients with joint hypermobility had severe headache disability, according to the HIT-6 disability criteria. The average pain intensity in patients with hypermobile joints was 6.1 out of 10. Among participants without significant joint hypermobility, 90% had mild headache disability.
Patients with joint hypermobility and increased tissue elasticity “tend to have a lower threshold for pain, in general, in all parts of their bodies,” said Dr. Sahjwani. Greater headache severity might be expected in this population, “because they have more pain if they have hypermobile joints or tissue.”
Headache treatments for this population are based solely on the type of headache that each patient has. Patients with joint hypermobility and migraine, for example, are candidates for rescue medication and long-term prophylactic medications. “I don’t think the joint hypermobility is going to change how you manage their headaches,” said Dr. Sahjwani.
The study results suggest that, when children present with severely debilitating headaches, a neurologist should consider examining them for joint hypermobility “to see if they have another diagnosis, such as Ehlers-Danlos syndrome ... that has to be managed in addition to their headaches,” Dr. Sahjwani concluded.
The study was not supported by funding. The authors did not report any disclosures.
SOURCE: Sahjwani D et al. CNS 2019, Abstract 101.
REPORTING FROM CNS 2019
Researchers identify common reasons for misdiagnosis of ALS
AUSTIN – Lack of upper motor neuron signs on examination, presence of sensory symptoms, and absence of tongue fasciculations are common causes of amyotrophic lateral sclerosis (ALS) misdiagnosis, according to an investigation presented at the annual meeting of the American Association of Neuromuscular and Electrodiagnostic Medicine.
Because its initial presenting symptoms vary, ALS can be difficult for clinicians to diagnose. A diagnostic error may prompt clinicians and patients to pursue ineffective and potentially harmful medical or surgical interventions. Research suggests that surgery, for example, hastens the progression of ALS.
Catherine Rodriguez, a medical student at University of Missouri in Columbia, and colleagues conducted a study to identify the clinical factors and types of cognitive errors that can result in misdiagnosis of ALS. The researchers analyzed electronic medical records of 88 patients with a diagnosis of ALS who were receiving treatment at the University of Missouri Hospital during 2011-2017 with at least 1 year of follow-up. They collected demographic information and clinical characteristics (e.g., ALS Functional Rating Scale and site of symptom onset) for each patient. If a patient received an incorrect diagnosis, Ms. Rodriguez and colleagues recorded the number of physicians he or she had seen, the incorrect diagnosis, the treatment, the type of diagnostic error, the clinical factors contributing to the misdiagnosis, and the type of physician who gave the incorrect diagnosis.
The investigators classed diagnostic errors according to the four categories of cognitive bias of the Patient Safety Network. The categories are availability heuristic (i.e., the diagnosis of a current patient is biased by the clinician’s experience with previous cases), anchoring heuristic (i.e., relying on the initial impression despite the emergence of evidence that may contradict it), framing effects (i.e., subtle cues and collateral information bias the diagnosis), and blind obedience (i.e., undue reliance on test results or expert opinion). Ms. Rodriguez and colleagues used Fisher’s exact test to perform a statistical analysis of the data.
Thirty-four (39%) of the 88 patients were female, and the populations average age was about 60 years. Eighty patients (91%) were white, six (7%) were black, and two (2%) were Hispanic. Twenty patients (23%) received an incorrect diagnosis. Common incorrect diagnoses included spinal abnormality, Bell’s palsy, myasthenia gravis, ulnar neuropathy, autoimmune motor neuropathy, and stroke.
The investigators observed significant differences in the reasons for misdiagnosis, depending on patient characteristics. Veterans were misdiagnosed because of the availability heuristic, while nonveterans were misdiagnosed because of the anchoring heuristic. Lower-limb onset was most commonly misdiagnosed because of the anchoring heuristic. Bulbar onset was most commonly misdiagnosed because of the availability heuristic. Surgical intervention was the most common treatment for an incorrect diagnosis.
The data serve as a reminder of the prevalence of cognitive biases, said Ms. Rodriguez. “Common things are common, so we tend to stick with those [diagnoses]. Especially with ALS, nobody wants to give anyone that diagnosis.” Clinicians should “recognize that incorrect diagnoses have equally bad outcomes for those patients,” she concluded.
The study was supported by a University of Missouri School of Medicine Summer Research Fellowship Program.
SOURCE: Rodriguez C et al. AANEM 2019. Abstract 10. Diagnostic errors and the implications for amyotrophic lateral sclerosis patients.
AUSTIN – Lack of upper motor neuron signs on examination, presence of sensory symptoms, and absence of tongue fasciculations are common causes of amyotrophic lateral sclerosis (ALS) misdiagnosis, according to an investigation presented at the annual meeting of the American Association of Neuromuscular and Electrodiagnostic Medicine.
Because its initial presenting symptoms vary, ALS can be difficult for clinicians to diagnose. A diagnostic error may prompt clinicians and patients to pursue ineffective and potentially harmful medical or surgical interventions. Research suggests that surgery, for example, hastens the progression of ALS.
Catherine Rodriguez, a medical student at University of Missouri in Columbia, and colleagues conducted a study to identify the clinical factors and types of cognitive errors that can result in misdiagnosis of ALS. The researchers analyzed electronic medical records of 88 patients with a diagnosis of ALS who were receiving treatment at the University of Missouri Hospital during 2011-2017 with at least 1 year of follow-up. They collected demographic information and clinical characteristics (e.g., ALS Functional Rating Scale and site of symptom onset) for each patient. If a patient received an incorrect diagnosis, Ms. Rodriguez and colleagues recorded the number of physicians he or she had seen, the incorrect diagnosis, the treatment, the type of diagnostic error, the clinical factors contributing to the misdiagnosis, and the type of physician who gave the incorrect diagnosis.
The investigators classed diagnostic errors according to the four categories of cognitive bias of the Patient Safety Network. The categories are availability heuristic (i.e., the diagnosis of a current patient is biased by the clinician’s experience with previous cases), anchoring heuristic (i.e., relying on the initial impression despite the emergence of evidence that may contradict it), framing effects (i.e., subtle cues and collateral information bias the diagnosis), and blind obedience (i.e., undue reliance on test results or expert opinion). Ms. Rodriguez and colleagues used Fisher’s exact test to perform a statistical analysis of the data.
Thirty-four (39%) of the 88 patients were female, and the populations average age was about 60 years. Eighty patients (91%) were white, six (7%) were black, and two (2%) were Hispanic. Twenty patients (23%) received an incorrect diagnosis. Common incorrect diagnoses included spinal abnormality, Bell’s palsy, myasthenia gravis, ulnar neuropathy, autoimmune motor neuropathy, and stroke.
The investigators observed significant differences in the reasons for misdiagnosis, depending on patient characteristics. Veterans were misdiagnosed because of the availability heuristic, while nonveterans were misdiagnosed because of the anchoring heuristic. Lower-limb onset was most commonly misdiagnosed because of the anchoring heuristic. Bulbar onset was most commonly misdiagnosed because of the availability heuristic. Surgical intervention was the most common treatment for an incorrect diagnosis.
The data serve as a reminder of the prevalence of cognitive biases, said Ms. Rodriguez. “Common things are common, so we tend to stick with those [diagnoses]. Especially with ALS, nobody wants to give anyone that diagnosis.” Clinicians should “recognize that incorrect diagnoses have equally bad outcomes for those patients,” she concluded.
The study was supported by a University of Missouri School of Medicine Summer Research Fellowship Program.
SOURCE: Rodriguez C et al. AANEM 2019. Abstract 10. Diagnostic errors and the implications for amyotrophic lateral sclerosis patients.
AUSTIN – Lack of upper motor neuron signs on examination, presence of sensory symptoms, and absence of tongue fasciculations are common causes of amyotrophic lateral sclerosis (ALS) misdiagnosis, according to an investigation presented at the annual meeting of the American Association of Neuromuscular and Electrodiagnostic Medicine.
Because its initial presenting symptoms vary, ALS can be difficult for clinicians to diagnose. A diagnostic error may prompt clinicians and patients to pursue ineffective and potentially harmful medical or surgical interventions. Research suggests that surgery, for example, hastens the progression of ALS.
Catherine Rodriguez, a medical student at University of Missouri in Columbia, and colleagues conducted a study to identify the clinical factors and types of cognitive errors that can result in misdiagnosis of ALS. The researchers analyzed electronic medical records of 88 patients with a diagnosis of ALS who were receiving treatment at the University of Missouri Hospital during 2011-2017 with at least 1 year of follow-up. They collected demographic information and clinical characteristics (e.g., ALS Functional Rating Scale and site of symptom onset) for each patient. If a patient received an incorrect diagnosis, Ms. Rodriguez and colleagues recorded the number of physicians he or she had seen, the incorrect diagnosis, the treatment, the type of diagnostic error, the clinical factors contributing to the misdiagnosis, and the type of physician who gave the incorrect diagnosis.
The investigators classed diagnostic errors according to the four categories of cognitive bias of the Patient Safety Network. The categories are availability heuristic (i.e., the diagnosis of a current patient is biased by the clinician’s experience with previous cases), anchoring heuristic (i.e., relying on the initial impression despite the emergence of evidence that may contradict it), framing effects (i.e., subtle cues and collateral information bias the diagnosis), and blind obedience (i.e., undue reliance on test results or expert opinion). Ms. Rodriguez and colleagues used Fisher’s exact test to perform a statistical analysis of the data.
Thirty-four (39%) of the 88 patients were female, and the populations average age was about 60 years. Eighty patients (91%) were white, six (7%) were black, and two (2%) were Hispanic. Twenty patients (23%) received an incorrect diagnosis. Common incorrect diagnoses included spinal abnormality, Bell’s palsy, myasthenia gravis, ulnar neuropathy, autoimmune motor neuropathy, and stroke.
The investigators observed significant differences in the reasons for misdiagnosis, depending on patient characteristics. Veterans were misdiagnosed because of the availability heuristic, while nonveterans were misdiagnosed because of the anchoring heuristic. Lower-limb onset was most commonly misdiagnosed because of the anchoring heuristic. Bulbar onset was most commonly misdiagnosed because of the availability heuristic. Surgical intervention was the most common treatment for an incorrect diagnosis.
The data serve as a reminder of the prevalence of cognitive biases, said Ms. Rodriguez. “Common things are common, so we tend to stick with those [diagnoses]. Especially with ALS, nobody wants to give anyone that diagnosis.” Clinicians should “recognize that incorrect diagnoses have equally bad outcomes for those patients,” she concluded.
The study was supported by a University of Missouri School of Medicine Summer Research Fellowship Program.
SOURCE: Rodriguez C et al. AANEM 2019. Abstract 10. Diagnostic errors and the implications for amyotrophic lateral sclerosis patients.