Surgical treatment of drug-resistant partial epilepsy in pediatric patients

been reported up to now [ 1]. Over the last 7 years, we have seen 9 patients affected by such a neuropathy. All patients had nerve conduction velocity (NCV) abnormalities suggestive of axonal involvement. In 7 of them, NCV abnormalities were further documented by sural nerve biopsy (5) or postmortem studies (2) which showed primary axonal changes with secondary myelin degeneration. In 3 children, symptoms were already severe at birth and 2 of them (2 brothers) died at 23 days and 3 months of age, respectively. Five of the other 6 patients had severe psychomotor retardation. In these 8 children, brain CT showed cerebral atrophy. This clinical picture is similar to the one seen in the patient reported by Guzzetta and Ferri~re in 1985 [1]. One patient had a different clinical picture in the sense that he had delayed motor milestones but normal intellectual development and a normal cerebral CT. Finally, extensive metabolic investigation was normal in the 5 children in whom it was done. Reference: [1] Guzzetta F, Ferri~re G. Congenital neuropathy with prevailing axonal changes. Acta Neuropathol 1985 ;68:185-90. 244. VIGABATRIN IN CHILDREN WITH UNCONTROLLED EPILEPSY A.A. Arzimanoglou, J. Aicardi, S. Mondragon, and J.P. Mumford, Paris, France

Forty-five patients, ages 2-21 years (mean age: 10.5 years) were included in an open, uncontrolled study of vigabatrin (GVG) as add-on therapy to pre-existing antiepileptic drugs. All children had severe refractory epilepsy. Thirty-four had partial seizures, with or without secondary generalization, 8 generalized seizures, including myoclonic seizures or absences and 3 children presented with Lennox-Gastaut syndrome. Initially, the recommended dose was 40-80 mg/kg/day. If tolerance allowed it, doses up to 5 gm/day (142 mg/kg/day) were occasionally used. Follow-up varied 2-62 months (mean period under GVG: 11 months). A 75-100% decrease in seizure frequency was observed in 11 children (24.5%). A further 17% (8 children) had a 50-75% decrease and in 6 others efficacy was moderate. Twenty-two suffered from partial epilepsies and 3 had generalized tonic-clonic fits. Four became seizure-free and monotherapy could be obtained in 3. Eleven of 25 were receiving GVG for more than 1 year at the time of this study (mean follow-up: 28.7 months) and no loss of efficacy was observed. However, in 7 other children with extremely resistant epilepsies an escape effect was observed, despite a very good response during the first 3-4 months following introduction of GVG. The use of GVG as an intermittent therapy for some of these patients should be studied further. In 20 children (44.5%) GVG was discontinued because of lack of efficacy (18 patients) or increase in seizure frequency (2 patients). No adverse reactions were observed in 33 children (73.3%). The remaining 12 had only transient secondary effects that disappeared either spontaneously or after reduction of GVG doses. Drowsiness, weight gain, hyperactivity or apathy, and transient limb stiffness in one patient were recorded. This study permitted confirmation of our initial impression that GVG is well tolerated and has a genuine antiepileptic action in severe childhood epilepsies, particularly against partial seizures. 245. CORPUS C A L L O S O T O M Y F O R INTRACTABLE SEIZURES IN CHILDREN A.A. Arzimanoglou, J. Aicardi, F. Goutieres, and J.J. Chevrie, Paris, France

The results of corpus callosotomy in 12 patients, 20 years of age and younger (mean age: 11.8 years), are presented. Our series included 6 total callosotomies (two as a second operation) and 8 partial (anterior 2/3) ones. Nine of our patients had apparently generalized seizures: tonic in 7, atonic in 6, myoclonic in 5, others in 5 (several types in same patient). Three children had partial seizures. Only 2 patients became seizure-free, but followup has been rather short. For the remaining 10 (follow-up: 3-6 years), 5 children had a reduction in seizure frequency of 7590%, 4 a reduction of 50-75%, and 1 had no change at all. From an EEG viewpoint, patients with clearly localized signs seemed to do far worse than those with diffuse or multifocal abnormalities. There was no mortality and morbidity was relatively mild. We did not find any correlation between age at operation, age at onset of epilepsy, and delay of operation on the one hand, and outcome on the other. However, all but 2 of our patients were moderately or severely retarded; therefore, we do not have detailed neuropsychologic evaluation for them. Following our limited experience, we would tend to use callosotomy mainly in children with sudden falls resulting in injury, preferably in patients without focal seizures and/or localized EEG abnormalities. We have had the impression that total callosotomy gave better results than partial operation. 246.

WITHDRAWN.

247. HISTAMINE H1 RECEPTOR MAPPING BY USING P O S I T R O N EMISSION TOMOGRAPHY IN COMPLEX PARTIAL SEIZURES Kazuie Iinuma, Hiroyuki Yokoyama, Tsuneo Yagi, and Kazuhiko Yanai, Sendai, Japan

Histamine H1 receptors could be visualized in human brain by PET using 11C-doxepine (DOX) by Yanai et al. in 1991. This is the first report about histamine H1 receptor imaging applied to a specific disease. Six patients with complex partial seizures with ages ranging 12-26 years were included in this study. All cases were evaluated for clinical seizure patterns by repeated EEGs, MRIs, FDG-PET, and DOX-PET. Two patients showed old infarction in uni- or bilateral occipital areas. The interictal EEG focus was located in temporal area in 4 cases and spread to one hemisphere in one case, and spread to both hemispheres in the other. FDG-PET showed decreased cerebral metabolic rate for glucose (CMRglc) in the region of EEG focus in 5 of 6 cases; in the other case, however, the CMRglc demonstrated no specific localized changes. In DOX-PET, H1 receptors increased in the region where CMRglc was decreased in 5 of 6 cases. One case showed decreased H1 receptors in the reduced CMRglc area where old infarction was detected in MRI. It is suggested that in partial epilepsy, HI receptors increase in the epileptic focus like g-opiate receptors. 248. SURGICAL TREATMENT OF DRUG-RESISTANT PARTIAL EPILEPSY IN P E D I A T R I C PATIENTS Kochen Silvia and Betti Osvaldo, Buenos Aires, Argentina

In 1983, we started with the study of intractable drug-resistant partial epilepsy by means of stereo EEG (SEEG); 15 of 45 evaluated patients were younger than 16 years of age (range: 8-15 years). In this group evolution time for epileptic symptoms was 9.2 years. The adult patient group (age range: 17-42 years) had

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an evolution of seizure symptoms between 2-34 years. Neuroradiologic images (CT and MRI) and stereotactic stereoscopic carotid angiography and ventriculography were performed in all these patients. Scalp EEG, EP, and other neurophysiologic tests were also performed. The use of an invasive technique like the SEEG requires an accurate protocol. We employed the methodology of Bancaud and Talairach. On the basis of data obtained by noninvasive studies and the semiology of seizures, we hypothesized that there was a single epileptogenic focus. Highly suspected structures were implanted with intracerebral electrodes in order to determine the epileptogenic and lesional areas. The spontaneous seizure onset must be registered showing epileptogenicity. Variously located cortectomies were performed according to the SEEG results. The surgical outcome of all patients showed that in 85% of them the seizure frequency was significantly reduced (> 95%). Additionally, as a result of the successful surgical seizure control, the pediatric group had a better psychosocial adaptation, school performance, and behavior, An earlier indication of surgery would result in a higher surgical success in drug-resistant epilepsy. 249. CONGENITAL MYASTHENIC SYNDROME WITH ENDPLATE (EP) AChR DEFICIENCY AND SHORTCHANNEL OPEN TIME Hector A. Waisburg, Andrew G. Engel, A. Nagel, Analia Taratuto, and Alberto Dubrowsky, Buenos Aires, Argentina, and Rochester, Minnesota A 5-year-old girl had severe respiratory insufficiency and obstructive apnea since birth. She needed assisted ventilation during the first 14 days of life. She developed facial diplegia, ophthalmoparesis and fed poorly during the first year of life. She improved with anticholinesterases and steroids which currently are still administered. A decremental response was present at 2 Hz stimulation of the facial muscles. AChR antibody tests were negative and no immune complexes were detected at the EP. Miniature EP potentials and currents were abnormally small, but were increased with neostigmine. The AChR channel conductance was normal, but the channel open time was 30% shorter than in 9 normal controls (P < .001). The number of AChR/EP determined with 125I-bungarotoxin was severely reduced relative to EP size. Ultrastructural studies revealed decreased density of AChR (peroxidase-c~-bungarotoxin) on wellpreserved junctional folds. A mutation of AChR could account for both the kinetic abnormality and the AChR deficiency. 250. NEUROMUSCULAR DISEASE ASSOCIATED WITH VITAMIN E DEFICIENCY Marcela Garcia Erro, Hugo A. Molina, Guillermo Agosta, Isabel Badia, and Jorge Grippo, Buenos Aires, Argentina Vitamin E deficiency frequently induces CNS lesions; however, little is known about the neuromuscular involvement in this condition. Because chronic cholestasis is associated with vitamin E deficiency we have planned a prospective study with long-term follow-up of children with this syndrome. In this communication we present the results as follows: 7 of 14 cases of chronic cholestasis had vitamin E deficiency. Three cases had clinical abnormalities with reduced muscular strength and muscle wasting predominately distally in lower and upper limbs. All patients presented with absence of myotatic reflex and one of them had

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bilateral pes cavus. EMGs were recorded in the dcitoids, biceps, and tibialis anterior muscles. Fastest sensory and mntor conduction were studied in peroneal, median, ulnar, and ~ural nerves. Abnormalities were Iound in 4 patients. Signs o! dcncrvation were observed in the EMG and peripheral nerve ~tud? showed reduction of sensory amplitude and conduction ~ekwity. Pathologic studies were conducted in one patient, and Ihe muscle biopsy had evidence of denervation atrophy, fiber type grouping and ultrastructural study revealed accumulation of lipopigments. Decrease of myelin fiber density, active fiber degeneration, and demyelination were found in the sural nerve biopsy. Paranodal and segmental demyelination were more fi'equent than axonal degeneration in the teased fibers. The EM study confirmed the previous findings and deposits of lipofuscin and granules were also observed. Alter the treatment with vitamin E, the patients improved clinically, as well as the results of electmdiagnostic studies in one of them. 251. NEUROLOGIC INVOLVEMENT IN 70 HIV-POSITIVE INFANTS AND CHILDREN Liliana Czornyj, Luis Carniglia, Cecilia Procak-Psaros. Julia Redondo, Guillermo Rocatagliata, Susana Rodfiguez, Jos~ Torolla, Francisco Meli, and Natalio Fejerman, Buenos Aires. Argentina Starting in 1990, we followed-up 70 pediatric patients with positive serology for HIV. Sixty-six cases were due to vertical transmission and 4 appeared after blood transfusions. Fifty-nine (84%) had symptoms of AIDS and 41 (70%) presented with neurologic involvement/psychomotor development retardation (66%), pyramidal signs (56%), CT abnormalities (51%), arrest of head growth (39%), microcephaly (24%), seizures (20%), and ataxia (5%). No clinical signs of spinal cord or peripheral nerve lesions were found. Primary cerebral lymphoma was not found either. Every one of the 12 deceased patients had at least one neurologic sign. CT abnormalities included: cortical and/or central atrophy in 12 patients, basal ganglia calcifications in 6, and signs of opportunistic infections (confirmed later pathologically) in 3 (i.e., Chagas disease, toxoplasmosis, and histoplasmosis). We found that 70% of the children with AIDS and 100% of those who died developed signs of neurologic involvement.

252. ANTIHISTAMINE THERAPY CONTROLLING SEIZURES IN A NEW VARIANT OF EPIDERMAL NEVUS SYNDROME Liliana Czornyj, Adri~in Pierini, Marfa Palacios, and Natalio Fejerman, Buenos Aires, Argentina The epidermal nevus syndrome is a sporadic neurocutaneous disorder that consists of epidermal nevus with abnormalities of the CNS, eyes, and bone, other cutaneous alterations, and malignancies. The characteristic neurologic symptoms are mental retardation and seizures. We present a 12-year-old patient, first examined at 91/2 years due to moderate mental retardation, and partial and secondary generalized seizures which were refractory to antiepileptic treatment. EEG showed left occipital spikes. CT demonstrated moderate and dysmorphic enlargement of the ventricular system. He had a linear unilateral epidermal nevus, diagnosed through skin biopsy and present since the age of 6 months. He had recurrent infections without hyperthermia. Ab-