Alan Guberman

LAMOTRIGINE-ASSOCIATED RASH: RISK/BENEFIT CONSIDERATIONS IN ADULTS AND CHILDREN

Summary: Purpose: Lamotrigine (LTG) is an antiepileptic drug (AED) recently released in several countries. It is effective for a variety of seizure types in adults and children both as an add‐on agent and in monotherapy, and is generally well tolerated. This report reviews the apparent risk factors for rash associated with LTG to determine whether and how the risk of serious rash can be minimized in practice.

Comparison of the psychosocial effects of epilepsy and narcolepsy/cataplexy: a controlled study.

Summary: A questionnaire survey compared the psychosocial effects of epilepsy in 60 patients without major organic pathology (selected cases with temporal lobe epilepsy or primary generalized epilepsy) with those of matched (duration of illness, sex) patients with narcolepsy/cataplexy and with those of age‐ and sex‐matched controls. Comparing epileptic patients with controls, we confirmed the well‐documented marked deleterious effects of epilepsy upon work, education, occupational and household accidents, recreation, personality, interpersonal relations, and other parameters. Comparisons of epileptic and narcoleptic patients, however, snowed that, in general, persons with narcolepsy are even more psychosocially impaired. The narcoleptic patients showed greater frequencies of disease‐attributed reduced performance at work, poorer driving records, higher accident rates from smoking, greater problems in planning recreation, and other significant differences. Rather dissimilar profiles of psychosocial impairment were found to characterize the two conditions, and these were largely understandable as a function of their symptoms. The only areas in which epileptic patients showed greater problems than those with narcolepsy were in educational achievement and in ability to maintain a driving licence. Most of the intergroup differences remained significant even for smaller groups matched also for age. The somewhat greater psychosocial impact of narcolepsy appears to be due to the continuous excessive daytime sleepiness that persists between the diagnostic attacks, whereas persons with epilepsy are relatively alert between seizures.

De Novo Aphasic Status Epilepticus

: The literature contains only a handful of reports of patients with aphasia as the principal or only obvious manifestation of partial status epilepticus. Even fewer patients of this type have been well documented both clinically and by ictal EEG monitoring. We studied an otherwise healthy woman with abrupt onset of aphasia initially thought to be the result of an infarct of the left temporoparietal area. We were able to document partial status epilepticus involving the left temporoparieta1 area with EEG/video monitoring and showed rapid reversal of the aphasic disorder with antiepileptic drug (AED) treatment. The case is presented with a review of previous reports to underscore the importance of considering this diagnosis in patients with abrupt onset of aphasia.

Quality of Life after Vagus Nerve Stimulation for Intractable Epilepsy: Is Seizure Control the Only Contributing Factor?

We assessed the impact of vagus nerve stimulation on a cohort of patients with intractable epilepsy. A 1-year prospective trial of vagus nerve stimulation for intractable epilepsy was done in 26 patients. Seizure frequency, anti-epileptic drugs, and quality of life were assessed using QOLIE-89, ELDQOL, and a Likert scale of impact of treatment. Seizures were reduced by more than 50% in 19% of the patients, by less than 50% in 46%, and were unchanged in 35% of them. Antiepileptic drugs were reduced in 43% of the patients. There was a significant improvement in the mean overall QOLIE-89 score and other measures of quality of life, but these did not correlate with changes in seizure frequency. Subjective improvement occurred in 84% of the patients. The quality of life improves in some patients following vagus nerve stimulation for intractable epilepsy. The favorable effects of this treatment may be attributable to additional factors besides seizure control which in this study was modest.

Clobazam in Long-Term Epilepsy Treatment: Sustained Responders Versus Those Developing Tolerance

Summary: Clobazam (CLB) is a structurally unique benzodiazepine (BZD) that has anticonvulsant activity in all types of refractory seizures. The main drawback to CLB, as to other BZDs, is the occurrence of tolerance. To date, there has been no way to predict which patients will develop tolerance. We compared clinical features and treatment variables between two groups of patients whose seizures were initially well controlled with CLB: patients with a sustained response and patients who developed tolerance. We retrospectively identified a group of 50 very good responders from among 173 consecutive patients with uncontrolled epilepsy treated with CLB. Very good responders were defined as patients with >75% reduction in seizures after the addition of CLB who continued CLB treatment for at least 1 month. At a mean follow‐up of 37.5 ± 12.8 months, 25 patients continued to respond and 25 developed tolerance (mean follow‐up 17.0 ± 15.7 months).Tolerance was defined as a relapse to a level <50% of pre‐CLB seizure frequency after an initial very good response for a minimum period of 1 month, despite constant CLB dose and, when available, serum levels. There was no change in concomitant medication. Significant differences were noted between the two groups. The sustained response group had a shorter duration of epilepsy (mean 16.5 vs.24.5 years, p = 0.015), a greater proportion of individuals with a known etiology for their epilepsy (48 vs.16%, p = 0.006), and higher CLB levels (0.50 vs.0.22 μM, p = 0.017), but no significant difference in N‐desmethyl‐CLB levels. Certain factors apparently may influence the likelihood of developing tolerance to the antiepileptic effects of clobazam.

Primary Reading Epilepsy: Investigation of Critical Seizure-Provoking Stimuli

Summary: Precipitating stimuli were investigated in a 24‐year‐old woman with primary reading epilepsy. Reading material was presented on a microcomputer video display monitor under controlled conditions while the patient underwent EEG radiotelemetry/video monitoring. We examined the relative contribution of the following variables utilizing a factorial design: eye movements, reading aloud versus reading silently, linguistic complexity, and concentration. None of these factors acted solely as the critical stimulus in provoking seizures. Seizures were most readily elicited when the patient read aloud, using material of medium or high linguistic complexity with the usual scanning eye movements for reading.

Pseudohypoparathyroidism and epilepsy: diagnostic value of computerized cranial tomography.

Summary: Computerized cranial tomograms (CCTs) unexpectedly showed bilateral symmetrical calcifications in the basal ganglia and frontal areas in two unrelated epileptic patients 12 and 13 years of age. The patients presented with a variety of seizures, some with focal features; these seizures were resistant to medication in the first case. Subsequent testing revealed hypocalcemia and other biochemical and radiologic features of pseudohypoparathyroidism, despite absence of the usual phenotypic features, tetanic symptoms, and positive family history. The CCT scan may provide the first clue to an underlying hypocalcemic disorder in an epileptic patient even when the skull X‐ray is, normal. Early detection of this metabolic condition by CCT scanning allows specific treatment to restore serum calcium levels to normal, which usually eliminates seizures and favors optimal cerebral functioning. Serial CCT scanning also provides a useful means for following the intracerebral calcifications, which remained unchanged after 1 and 2 years of normocalcemia in our 2 patients.

Epileptogenesis due to Peripheral Injury as a Cause of Focal Epilepsy

Summary:u2002 Purpose: To report three patients in whom focal epilepsy developed shortly after painful soft tissue injuries to their hands.

Epilepsy and crossed cerebellar diaschisis with persistent cerebellar syndrome.

A 24-year-old man with developmental delay and epilepsy since the age of 11 presented with one day history of secondarily generalized tonic clonic status epilepticus. He recently had focal motor seizures involving his left face and arm five times per day and generalized and complex partial seizures once per month despite two prior surgeries including a right temporal lobe resection. (Figures 1A, B) The pathology from his surgical resection revealed non specific gliosis. He had tried multiple anti epileptic drugs (AEDS) but his epilepsy remained intractable. He remained on divalproex sodium, clobazam and topiramate at the time of his admission. Upon admission to the emergency room (ER), he was intubated for airway protection and treated with intravenous phenytoin. Computed tomogram head revealed known encephalomalacia in the right temporo-parieto-occipital lobe and a new area of hypodensity in the left cerebellar hemisphere. An initial magnetic resonance imaging (MRI) showed T2 hyperintensities in the right cerebral cortex and left cerebellar hemisphere consistent with crossed cerebellar diaschisis (CCD). (Figure 1C) After extubation, he had new left sided hemiparesis and left upper extremity dysmetria and dysdiadochokinesis. A second MRI two weeks later revealed CCD in similar appearance to the initial scan. He continued to have daily left sided focal motor seizures despite AED therapy. His deficits improved with daily physiotherapy but remained upon his discharge. A third MRI performed two months after the initial presentation demonstrated complete resolution of the increased fluid attenuation inversion recovery (FLAIR) signal in the cerebellum with no perfusion abnormalities. His left sided hemiparesis and cerebellar deficits remained however.