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Other generalized idiopathic epilepsies
epileptogenic lesion is not always enough to cure the epilepsy; (c) how it is important to find the correlation between the cortical lesion and the primary epileptogenic zone; (d) how the presurgical monitoring with subdural electrodes is a safe procedure and of great value in the cortical functional mapping.
316. NEW THERAPEUTIC TRIAL FOR MITOCHONDRIAL ENCEPHALOMYOPATHIES WITH COMBINATION OF CYTOCHROME C AND VITAMINS B l AND B2 Toshisaburo Nagai, Junko Tanaka, Mano, Hirosi Arai, Taro Matsuoka, Ryuhei Kodaka, Yusuke Itagaki, Suzuki, Jiro Ono, Koji Inui, Hideo Yamauchi, Yu-ichi Goto, Ikuya Nonaka, and Shintaro Okada, Osaka and Tokyo, Japan Nine patients with mitochondrial encephalomyopathy (MEM) were treated with intravenous injection of Cardiocrome R (CDC), containing cytochrome C (cyt C) and vitamins B 1 and B2. Eight had significant clinical improvement, especially in easy fatigability, mobility, and stroke-like episodes. The decrease of both CSF protein concentrations and lactate levels was also recognized after CDC therapy. No favorable results were obtained by oral administration of cyt C with a similar combination of vitamins and also by intravenous injection of cyt C alone. CDC therapy was found to be beneficial for patients with MEM.
317. COMPARATIVE STUDY BETWEEN CONTINUOUS AND INTERMITTENT PHENOBARBITAL THERAPY FOR FEBRILE CONVULSIONS Boonsom Ratnasiri, Bangkok, Thailand A group of 270 children who were seen after a febrile convulsion at the Children's Hospital from November, 1986 to June, 1988 were randomly assigned to 2 treatment groups to evaluate the effectiveness of phenobarbital in preventing precurrent febrile convulsions. Group I: "continuous treatment" of phenobarbital was given orally (5 mg/kg/day) in 2 divided doses daily. Group II: "intermittent treatment" of phenobarbital was given orally (10 mg/kg/day) in 3 divided doses, with double doses on the first dose, and only at the continuation of fever. After 2 years of follow-up, we found no significant difference between the 2 treatment groups. One hundred nine patients (81.95%) in group I and 75 patients (72.82%) in group II were completely controlled without recurrent convulsions. Nineteen patients (14.29%) and 14 patients (13.59%), respectively, in each group had some repeated febrile convulsions but the treatment regimens were continued effectively (partial control). Only 5 patients (3.76%) in group I and 14 patients (13.59%) in group II had uncontrolled, recurrent febrile convulsions and required new treatment regimens.
318. OTHER GENERALIZED IDIOPATHIC EPILEPSIES Francisco X. Sanmarti, Gema Mons6, Juan Montalvo, and Emilio Fernfindez-Alvarez, Barcelona, Spain In a study conducted in 1991 of a group of 344 patients, ages 1 month to 6 years, we found that 37 children could only be classified in the ILAE International Classification group known as
168 PEDIATRICNEUROLOGY Vol. l l No. 2
"other generalized idiopathic epilepsies." The group consisted of 23 boys and 14 girls, with familial antecedents of epilepsy in 4 patients and febrile crises in 2. Six patients had suffered febrile crises. In 29 patients, the first crisis occurred in the first 3 years of life. In 5 patients the crises were tonic-clonic, in 11 clonic, in 12 tonic, in 1 myoclonic, in 3 tonic-clonic + tonic, in 2 tonic + absences, in 2 tonic + clonic, and in 1 clonic + tonic + myoclonic. Initial frequency of the crises was daily in 12 patients, weekly in 4, monthly in 12, and yearly in 9. Interictal EEG during wakefulness showed normal background activity in all patients. Paroxysmal events were focal in 2 patients, generalized in 9, and focal and generalized in 1. Sleep patterns were normal in all patients, generalized paroxysmal events were found in 9 patients, and generalized and focal paroxysmal events occurred in 1 patient. Absence was registered in 2 patients, eyelid myoclonia in l, and tonic-clonic in 1. In 33 patients, the evolution was favorable with monotherapy, while the crises persisted in 4 patients. IQs were normal in 33 and low in 4. In the first few years of life there are patients affected by primary generalized epilepsy who present with a favorable prognosis in spite of having tonic crises that might suggest the existence of Lennox-Gastaut syndrome. The classification of these epileptic conditions, therefore, should be individualized in order to facilitate diagnosis and prognosis.
319. LANGUAGE EVOLUTION UNDER VIGABATRIN IN CHILDREN WITH SEVERE LANGUAGE DYSFUNCTION B. Echenne and R. Cheminal, Montpellier, France Neuropsycholinguistic tests were repeated twice or more for 6 months to 2 years in children without clinical seizures, but with Landau-Kleffner syndrome (2 patients) or developmental dysphasia with epileptic discharges on sleep EEG (i.e., continuous spike-and-waves activity [2 patients] or spike-and-waves discharges during 20-85% of the recordings [4 patients]), treated by vigabatrin (VGV). In 2 patients with dysphasia and sleep EEG epileptic discharges, marked improvement of language abilities was seen with VGV monotherapy. In 6 of 8 patients, in whom VGV was used after a brief, long-acting ACTH treatment, the language performances markedly improved, with EEG normalization or improvement. The results emphasize the question of the beneficial effects of VGV on language abilities in children with severe language impairment related to sleep EEG epileptic abnormalities, and suggest to do more large studies concerning these effects because of the inefficiency of classic antiepileptic drugs in these patients.
320. NEUROPSYCHOLOGIC PROFILE IN ULLRICHTURNER SYNDROME IN COMPARISON TO SIBS: PATTERN OF SLIGHT IMPAIRMENT AND SELECTIVE DEFICIENCIES F. Haverkamp, M. Noeker, and D. D6rholt, Bonn, Germany Most studies analyzing intellectual performance in UllrichTurner syndrome (UTS) reported subtle deficits in verbal intelligence and reduced performance intelligence, suggesting impaired right hemisphere thought processes. For a better control of additional genetic and familial influences we investigated 97
316. NEW THERAPEUTIC TRIAL FOR MITOCHONDRIAL ENCEPHALOMYOPATHIES WITH COMBINATION OF CYTOCHROME C AND VITAMINS B l AND B2 Toshisaburo Nagai, Junko Tanaka, Mano, Hirosi Arai, Taro Matsuoka, Ryuhei Kodaka, Yusuke Itagaki, Suzuki, Jiro Ono, Koji Inui, Hideo Yamauchi, Yu-ichi Goto, Ikuya Nonaka, and Shintaro Okada, Osaka and Tokyo, Japan Nine patients with mitochondrial encephalomyopathy (MEM) were treated with intravenous injection of Cardiocrome R (CDC), containing cytochrome C (cyt C) and vitamins B 1 and B2. Eight had significant clinical improvement, especially in easy fatigability, mobility, and stroke-like episodes. The decrease of both CSF protein concentrations and lactate levels was also recognized after CDC therapy. No favorable results were obtained by oral administration of cyt C with a similar combination of vitamins and also by intravenous injection of cyt C alone. CDC therapy was found to be beneficial for patients with MEM.
317. COMPARATIVE STUDY BETWEEN CONTINUOUS AND INTERMITTENT PHENOBARBITAL THERAPY FOR FEBRILE CONVULSIONS Boonsom Ratnasiri, Bangkok, Thailand A group of 270 children who were seen after a febrile convulsion at the Children's Hospital from November, 1986 to June, 1988 were randomly assigned to 2 treatment groups to evaluate the effectiveness of phenobarbital in preventing precurrent febrile convulsions. Group I: "continuous treatment" of phenobarbital was given orally (5 mg/kg/day) in 2 divided doses daily. Group II: "intermittent treatment" of phenobarbital was given orally (10 mg/kg/day) in 3 divided doses, with double doses on the first dose, and only at the continuation of fever. After 2 years of follow-up, we found no significant difference between the 2 treatment groups. One hundred nine patients (81.95%) in group I and 75 patients (72.82%) in group II were completely controlled without recurrent convulsions. Nineteen patients (14.29%) and 14 patients (13.59%), respectively, in each group had some repeated febrile convulsions but the treatment regimens were continued effectively (partial control). Only 5 patients (3.76%) in group I and 14 patients (13.59%) in group II had uncontrolled, recurrent febrile convulsions and required new treatment regimens.
318. OTHER GENERALIZED IDIOPATHIC EPILEPSIES Francisco X. Sanmarti, Gema Mons6, Juan Montalvo, and Emilio Fernfindez-Alvarez, Barcelona, Spain In a study conducted in 1991 of a group of 344 patients, ages 1 month to 6 years, we found that 37 children could only be classified in the ILAE International Classification group known as
168 PEDIATRICNEUROLOGY Vol. l l No. 2
"other generalized idiopathic epilepsies." The group consisted of 23 boys and 14 girls, with familial antecedents of epilepsy in 4 patients and febrile crises in 2. Six patients had suffered febrile crises. In 29 patients, the first crisis occurred in the first 3 years of life. In 5 patients the crises were tonic-clonic, in 11 clonic, in 12 tonic, in 1 myoclonic, in 3 tonic-clonic + tonic, in 2 tonic + absences, in 2 tonic + clonic, and in 1 clonic + tonic + myoclonic. Initial frequency of the crises was daily in 12 patients, weekly in 4, monthly in 12, and yearly in 9. Interictal EEG during wakefulness showed normal background activity in all patients. Paroxysmal events were focal in 2 patients, generalized in 9, and focal and generalized in 1. Sleep patterns were normal in all patients, generalized paroxysmal events were found in 9 patients, and generalized and focal paroxysmal events occurred in 1 patient. Absence was registered in 2 patients, eyelid myoclonia in l, and tonic-clonic in 1. In 33 patients, the evolution was favorable with monotherapy, while the crises persisted in 4 patients. IQs were normal in 33 and low in 4. In the first few years of life there are patients affected by primary generalized epilepsy who present with a favorable prognosis in spite of having tonic crises that might suggest the existence of Lennox-Gastaut syndrome. The classification of these epileptic conditions, therefore, should be individualized in order to facilitate diagnosis and prognosis.
319. LANGUAGE EVOLUTION UNDER VIGABATRIN IN CHILDREN WITH SEVERE LANGUAGE DYSFUNCTION B. Echenne and R. Cheminal, Montpellier, France Neuropsycholinguistic tests were repeated twice or more for 6 months to 2 years in children without clinical seizures, but with Landau-Kleffner syndrome (2 patients) or developmental dysphasia with epileptic discharges on sleep EEG (i.e., continuous spike-and-waves activity [2 patients] or spike-and-waves discharges during 20-85% of the recordings [4 patients]), treated by vigabatrin (VGV). In 2 patients with dysphasia and sleep EEG epileptic discharges, marked improvement of language abilities was seen with VGV monotherapy. In 6 of 8 patients, in whom VGV was used after a brief, long-acting ACTH treatment, the language performances markedly improved, with EEG normalization or improvement. The results emphasize the question of the beneficial effects of VGV on language abilities in children with severe language impairment related to sleep EEG epileptic abnormalities, and suggest to do more large studies concerning these effects because of the inefficiency of classic antiepileptic drugs in these patients.
320. NEUROPSYCHOLOGIC PROFILE IN ULLRICHTURNER SYNDROME IN COMPARISON TO SIBS: PATTERN OF SLIGHT IMPAIRMENT AND SELECTIVE DEFICIENCIES F. Haverkamp, M. Noeker, and D. D6rholt, Bonn, Germany Most studies analyzing intellectual performance in UllrichTurner syndrome (UTS) reported subtle deficits in verbal intelligence and reduced performance intelligence, suggesting impaired right hemisphere thought processes. For a better control of additional genetic and familial influences we investigated 97