Faculty Bibliography

The authors investigated cognition in juvenile myoclonic epilepsy (JME), focusing on frontal functions as suggested by maximal spatial distribution of epileptiform activity seen over frontocentral regions. Fifteen patients with JME (mean age, 34.3 years; mean estimated IQ 101) were administered a battery of tests sensitive to frontal dysfunction. The number of patients with impaired test performance and the frequency of impairment per test were calculated. Performance on selected tests was compared with that of 15 patients with temporal lobe epilepsy (TLE) who were matched for estimated IQ using paired t-tests. Although the performance of the group with JME was not uniform--some patients showed marked impairment whereas others showed little or no deficit--a high frequency of impairment was found on tests of concept formation-abstract reasoning and mental flexibility, cognitive speed, and planning and organization. Significant differences were found between the group with JME and the group with TLE on tests requiring mental flexibility and concept formation-abstract reasoning. In conjunction with studies demonstrating intractable seizures in approximately 20% of patients, the results from this study suggest that JME is not a uniformly benign condition. Frontal deficits may have maladaptive behavioral consequences suggestive of personality dysfunction, as described anecdotally by previous investigators

There is considerable variability among epilepsy centers in the methods and interpretations of the intracarotid amobarbital procedure. Prominent among these differences is the determination of language representation and assessment of language functions. Some centers rely on speech arrest following amobarbital injection as a marker for language representation, whereas other centers examine verbal output for the presence of aphasic errors. The present study assessed the pattern of language recovery following amobarbital injection in epilepsy patients who were candidates for temporal lobectomy. Language recovery from dominant hemisphere injection (left or right) followed a stereotypical progression, with 71.8% of patients showing return of vocalization followed by return of naming and comprehension. Repetition deficits with paraphasic errors persisted the longest (mean = 12'30'), with a conduction aphasia persisting after the acute global aphasia resolved. Although two patients interpreted as left hemisphere language dominant were mute following right hemisphere injection, all language functions were intact immediately upon resumption of vocalization and they showed no other signs of aphasia such as paraphasias or anomia. Possible explanations for serial language recovery and persistent conduction aphasia are discussed. These findings have significant implications for the determination of cerebral language dominance

Although gabapentin (GPN) is commonly used as adjunctive therapy to treat recurrent self-limited seizures, little is known about its potential efficacy in treating prolonged partial seizures. We report two cases of refractory focal status epilepticus successfully treated with oral GPN. Ln both cases, GPN precluded the need for pentobarbital coma. We suggest that GPN may be an effective treatment for focal status epilepticus and should be studied further for its potential efficacy in this condition. (C) 1997 by Elsevier Science Inc. All rights reserved

Transient paralysis is an uncommon seizure symptom. We report two new cases of inhibitory motor status and review 24 previously cases of inhibitory seizures. Among the 22 adult patients, 14 (64%) had a frontoparietal lesion (tumor, 7; stroke, 7); 5 (23%) had mesiotemporal sclerosis (MTS), and 3 (14%) had no identified lesion. In contrast, all 4 pediatric patients had no identified brain lesions. Inhibitory motor seizures were associated most commonly with lesions in frontoparietal primary and supplementary motor-sensory area and, less often, in the mesial temporal lobe. Inhibitory motor seizures arising from frontoparietal foci are often more prolonged (>2-3 min) than those arising from the mesial temporal area (<1.5 min). Patients with temporal lobe seizure foci manifest ictal flaccidity of an extremity during a complex partial seizure (CPS), which may represent motor neglect rather than ictal weakness since strength cannot be accurately assessed when consciousness is impaired. Inhibitory motor seizures from sensorimotor cortex seizure foci are probably more common than is recognized. (C) 1997 by Elsevier Science Inc

We report three patients with focal prolonged myoclonic jerking movements suggestive of epilepsia partialis continua (EPC) but produced on a psychogenic basis [nonepileptic seizures-EPS (NES-EPC)]. NES-EPC was identified by (1) variability in the areas of motor activity in the same patient, (2) distribution and spread of motor activity without neuroanatomic basis, (3) transient abolition of movements with distraction (e.g., when other body parts are engaged in effortful tasks), (4) eliciting and abolishing typical episodes with suggestive techniques, (5) absence of motor weakness during or in between episodes, (6) absence of electroencephalogram (EEG) correlate of motor activity, and (7) absence of hyperperfusion on ictal SPECT scan. (C) 1997 by Elsevier Science Inc. All rights reserved

We attempted to evaluate the efficacy of Gabapentin (GBP) monotherapy as a treatment for partial epilepsy. We reviewed the medical records of 30 consecutive patients treated. with GBP monotherapy at our epilepsy center. Twenty-nine of these patients had previously been treated with antiepileptic drugs (AEDs) (median number of two drugs). Only one patient had taken GBP as an adjunctive medication Median duration of GBP monotherapy was 6 months, and the median daily dose was 1200 mg. During treatment with GBP, 17 patients (57%) experienced a greater than or equal to 25% reduction in seizure frequency, with nine patients reporting a greater than or equal to 90% reduction. Twelve patients reported no change in seizure frequency; however, six of these patients had experienced less than one seizure every 6 months prior to treatment with GBP, and therefore maintained fairly good control. Six patients discontinued GBP due to poorly controlled seizures. Adverse effects were experienced by 13 patients, but led to discontinuation of GBP in only four cases. Our results suggest that monotherapy with GBP tan be effective in partial epilepsy and is often well tolerated. (C) 1997 by Elsevier Science inc. All rights reserved

Multiple subpial transections (MST) were made in language cortex in 13 patients with medically refractory partial epilepsy. Transections were made in posterior language cortex in 12 patients and in both anterior and posterior language areas in 1. Eleven patients had additional resective surgery in the anterior temporal (n = 10) or anterior temporal and frontopolar (n = 1) regions. Eleven of the 13 patients had at least 90% reduction in seizure frequency. Dysnomia was present >6 months postoperatively in 9 patients (mild, 6; moderate, 3). Other deficits included impaired memory (mild, 3; moderate, 1) and reading (mild, 2). We compared the postoperative neuropsychologic findings in 7 patients with MST and temporal lobectomy with those in 14 patients who underwent only dominant temporal lobectomy. MST patients had significantly (p < 0.05) poorer postoperative naming, verbal fluency, and oral reading than patients with dominant temporal resections that spared language functions. MST in language areas can contribute to or cause language disorders that persist >1 year postoperatively. (C) 1997 by Elsevier Science Inc. All rights reserved

We studied 21 patients who underwent multiple subpial transections (MST) for the surgical treatment of intractable partial-onset seizures in whom the epileptogenic focus overlapped with primary sensorimotor or language cortex. All patients had intracranial EEG localization of seizures and functional mapping with electrical stimulation before surgery. Eighteen patients had cortical resections in addition to MST, At an average follow-up of 21 months, 13 patients either are seizure-free or are experiencing auras or simple partial seizures (SPS) only, 6 patients have had greater than 50% reduction in seizure frequency, 1 patient has had a modest reduction in seizures, and 1 patient is unchanged. MST in the 3 patients who did not undergo cortical resections reduced seizure frequency in all 3, although none are seizure-free. MST, combined with cortical resection, appears to be a safe and effective alternative to subtotal resection of the epileptogenic zone. MST alone, although not curative, reduced seizure frequency in all patients who underwent the procedure. (C) 1997 Elsevier Science Inc

This is the first randomized, double-blind, parallel-group, multicenter trial that evaluated the efficacy of divalproex sodium monotherapy by comparing seizure frequency in 143 patients with poorly controlled partial epilepsy randomly assigned to high (80 to 150 mu g/mL; 555 to 1,040 mu mol/L) or low (25 to 50 mu g/mL; 175 to 345 mu mol/L) plasma valproate groups, There was a statistically significant reduction from baseline in the 8-week frequency of complex partial (p = 0.001) and secondarily generalized tonic-clonic seizures (p = 0.018) for patients in the high, compared with the low, plasma valproate group. Compared with baseline, there was a 30% median reduction in complex partial seizures for patients in the high group and a 19% increase for those in the low group. The median reduction for secondarily generalized tonic-clonic seizures was 70% for patients in the high group compared with a 22% increase in the low group. Adverse events that occurred significantly more frequently in the high group included tremors, thrombocytopenia, alopecia, asthenia, diarrhea, vomiting, and anorexia. This study demonstrates the efficacy of divalproex sodium as monotherapy for the treatment of partial-onset seizures and supports its role as one of the first-line antiepileptic drug treatments for patients with partial epilepsy