Hema Patel

Nonepileptic Seizures in Children

Purpose: To determine if the clinical characteristics of nonepileptic seizures (NES) are different in children younger than 13 years age as compared to adolescents.

Presurgical evaluation and surgical outcome of temporal lobe epilepsy.

The authors analyzed 22 patients younger than 18 years of age with temporal lobe epilepsy (TLE) treated surgically. Patients underwent a comprehensive presurgical evaluation, including video-electroencephalogram. Fifty-five percent had a history of febrile seizures. Eighty-two percent had auraes and most exhibited oroalimentary and gestural automatisms. Contralateral dystonic posturing was present in 36% and postictal dysphasia in 54% of patients with left-sided resections. Cranial magnetic resonance imaging (MRI) was abnormal in 59% of patients. MRI revealed changes consistent with mesial temporal sclerosis in 8 (47%) of 17 patients without lesions. Fluorodeoxyglucose-positron emission tomography (PET) scans revealed ipsilateral temporal hypometabolism (PET-TH) in 12 (85.7%) of 14 patients. The intracarotid amobarbital procedure revealed impaired memory of the epileptogenic side in 59% of patients. Seventeen patients underwent en-bloc resections and five lesionectomies and resection of the epileptogenic area. There was no surgical morbidity or mortality. Forty-three percent had hippocampal sclerosis, 28.5% gliosis, 14% low-grade tumors, 9.5% cavernous angiomas, and 5% had no pathologic findings. Follow-up (6 months to 12 years) was available for 21 patients; 76% became seizure free, 19% had rare seizures, and 5% had a worthwhile improvement. TLE can be safely treated surgically in younger patients with excellent results. The clinical manifestations were similar to adult patients. PET-TH was present even at a younger age, suggesting that the focal functional deficits appear early in patients with medically refractory TLE, which may help in the early identification of these patients.

Spontaneous extracranial carotid artery dissection in children

Dissection of cerebral arteries as a cause of stroke is rarely recognized in children. Two patients with stroke due to extracranial carotid artery dissection are reported. A 7-year-old girl with a 2-week history of right arm chorea had a left basal ganglia infarct and is receiving haloperidol for persistent chorea. The second patient, a 15-year-old boy, developed aphasia and right hemiparesis a day before admission during a football game without obvious trauma. He had a large left middle cerebral artery infarct and died of cerebral edema and herniation. We believe that strokes due to arterial dissection are more common than currently recognized, partly because of a lack of history of trauma, and suggest that cerebral artery dissection be considered as an etiology of childhood strokes. Greater awareness of arterial dissection as a cause of stroke and availability of noninvasive techniques like magnetic resonance angiography should result in a more accurate diagnosis and improved prognosis in these patients.

Antiphospholipid and glutamic acid decarboxylase antibodies in patients with focal epilepsy

Autoantibodies to glutamic acid decarboxylase (GAD-A) have been associated with focal epilepsy.1 GAD catalyzes the conversion of l-glutamic acid to gamma aminobutyric acid (GABA). GABA is an inhibitory neurotransmitter and indirect suppression of GABA via GAD-A, in theory, could favor the development of seizures. Although originally implicated in the pathogenesis of type I diabetes and stiff-person syndrome,2 GAD-A have been identified in 8 of 51 (12%) patients with focal epilepsy due to conditions including hippocampal sclerosis.1 We attempted to further investigate the presence of GAD-A in a sample of patients with medically refractory focal epilepsy with mesial temporal sclerosis (MTS). Antiphospholipid antibodies (aPL) have been associated with thromboembolic events, although some have found these antibodies in the serum of patients with seizures unrelated to thromboembolic events.3 A lupus anticoagulant associated with late onset epilepsy was reported in four adults,3 …

Neurologic complications of pediatric liver transplantation

The neurologic complications of 24 children, ages 5 months to 18 years, following orthotopic liver transplantation at the Indiana University hospitals are reported. Biliary atresia (14 patients) was the most common cause for orthotopic liver transplantation. Three children died. Seventeen children (70%) had no neurologic deficit on follow-up 6 months or longer after transplantation. Eleven children (46%), including 4 of 16 patients (25%) who had received OKT3, had neurologic complications. Seven children (29%) had new-onset seizures; 4 of these patients had status epilepticus. Two children had intracranial hemorrhage. Seizures occurred later in children than in adults following orthotopic liver transplantation and were not associated with poor prognosis. Longer term follow-up is indicated to assess subtle, cognitive deficits following liver transplantation in children.

MRI of Guillain-Barré syndrome.

We describe the results of MRI of the spine in a case of Guillain-Barré syndrome. The main finding was abnormal enhancement of the nerve roots in the region of the conus medullaris and cauda equina.

Clinical correlation of periodic lateralized epileptiform discharges in children

Fifteen children with periodic lateralized epileptiform discharges are reported and clinical and radiologic features and outcome are presented. Both structural cerebral lesions and metabolic factors were associated with periodic lateralized epileptiform discharges. Although all patients had seizures, 8 had status epilepticus. Seven patients survived and 8 patients died. Six of the 7 survivors had residual seizures. Periodic lateralized epileptiform discharges in children are associated with acute encephalopathies and there is a high incidence of subsequent epilepsy.

Psychogenic Nonepileptic Seizures (Pseudoseizures)

1. Hema Patel, MD* 2. David W. Dunn, MD*† 3. Joan K. Austin, PhD, RN§ 4. Julia L. Doss, PsyD‡ 5. W. Curt LaFrance Jr, MD, MPH** 6. Sigita Plioplys, MD§§ 7. Rochelle Caplan, MD†† 1. *Section of Child Neurology, Department of Neurology, Indiana University School of Medicine, Indianapolis, IN. 2. †Section of Child Psychiatry, Department of Psychiatry, Indiana University School of Medicine, Indianapolis, IN. 3. §Indiana University School of Nursing, Indianapolis, IN. 4. ‡Minnesota Epilepsy Group, St. Paul, MN. 5. **Department of Neuropsychiatry and Behavioral Neurology, Brown Medical School, Rhode Island Hospital, Providence, RI. 6. §§Department of Psychiatry and Behavioral Sciences, Northwestern Universitys Feinberg School of Medicine, Chicago, IL. 7. ††Semel Institute for Neuroscience and Human Behavior, UCLA, Los Angeles, CA. After completing this article, readers should be able to: 1. Recognize the antecedent stressors associated with psychogenic nonepileptic seizures in children. 2. Identify pediatric nonepileptic seizures clinically. 3. Distinguish psychogenic nonepileptic seizures from epileptic seizures and other paroxysmal nonepileptic events. 4. Be aware of the comprehensive assessment needed to evaluate the child who has possible psychogenic nonepileptic seizures. 5. Become familiar with the management approaches used to treat psychogenic nonepileptic seizures in children. Nonepileptic seizures are episodic behavioral events that mimic epileptic seizures but are not associated with abnormal cortical electrical discharges. Psychogenic nonepileptic seizures (PNES) are related to an underlying psychological stressor or conflict and differ from other paroxysmal nonepileptic events. A variety of terms have been used in the literature to describe these events, including hysterical epilepsy, hysteroepilepsy, psychogenic seizures, pseudoepileptic seizures, pseudoseizures, and nonphysiologic or functional seizures. The term psychogenic nonepileptic seizure is preferred because it is nonpejorative and neutral, although there is continuing discussion regarding the most appropriate terminology. PNES are common. Although population data are limited, one report suggested a prevalence of 2 to 33 per 100,000, basing the estimate on an assumption that 10% to 20% of patients seen in an epilepsy center would be found to have PNES. (1) Reviewing video-electroencephalography (EEG) monitoring records, Patel and associates (2) and Wyllie and colleagues (3) found that 3.5% and 7% of children, respectively, seen in clinic for assessment of persistent seizures had PNES. PNES occur in both elementary-age children and in adolescents as well as in all age groups of adults. (2)(3)(4)(5)(6) A female preponderance has been …

Episodic coma in a new leukodystrophy

Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.

Intractable Seizures, Developmental Delay, and the Ketogenic Diet

Glucose transporter type 1 (GLUT1) deficiency syndrome is a rare, treatable cause of developmental delay and seizures. It must be considered in the differential diagnosis of infants with intractable seizures. The finding of a low glucose level in the cerebrospinal fluid with normal level in the blood in the absence of pleocytosis or other cerebrospinal fluid abnormalities identifies the condition. Genetic analysis for confirmation is available. Treatment with antiepileptic medications often is unsuccessful, and the ketogenic diet is the favored treatment for seizure control. Early identification and initiation of treatment may prevent or lessen the severity of developmental delay.