Epilepsy
of aminoacids. In some patients, a therapeutic trial with 66 vitamin PO or IV was also done. If any of these first tests was abnormal, gas chromatography of organic acids and/or HPLC of aminoacids were done in frozen samples of plasma and urine. Thirty-two children were included until now; from them, twenty-four showed abnormalities in the initial tests. In this sample of 24 selected patients, we found a case of glutaric acidemia type I and a case of maple syrup urine disease variant intermittent. We have also diagnosed two other cases through additional investigation (skin and hepatic biopsies): a patient with glycogenosis type Ill and other with neuronal ceroid lipofuscinosis. Among the 5 patients who received 86 vitamin, there was a case of pyridoxine deficiency. Nineteen children remain undiagnosed, however, in 6 of them, the blood and urinary HPLC of aminoacids was abnormal. Abnormalities found included different quantities of various aminoacids not characteristic of any clear disorder. Despite the absence of diagnosis in the majority of these patients, we concluded that the high frequency of aminoacids abnormalities is an indication of the existence of possible underlying metabolic disorders, perhaps causally related to epilepsy in these children. (FAPERGS)
I1 17 14
Prevalence of neurocysticercosis in individuals affected with epilepsy in Antioquia, Colombia
I. Jimenez, G. Palacio, J.L. Sanchez, M.E. Jimenez, I. Canasteros, M. Giraldo, M. Arcos-Burgos, V. Tsang, J. Pilcher, for the Antioquian Group Neuroepidemiological Research. Antioquian League Against Epilepsy (ALA& Medelin, Colombia
of
In the aim of to determine the frequency of neurocysticercosis (NC) as a cause of Juvenil or Late Epilepsy a clinical trial of prevalence was carried out in patients affected with Epilepsy consulting to the ALAE. From a fixed sampling strategy, 757 epileptic individuals were ascertained according with the order of arrival. They were submitted to Computarfzed Tomography (CT). 26.6% of them, fulfill de CT criteria for NC diagnosis. From the all set, only 551 individuals were submitted to Western Blot for cysticercosis (EITB). The latter was able to detect 12.15% positive individuals for NC. Taking as a gold standard EITB we conclude that CT have a sensibility of 63.3% and an specificity of 73.9% in relation to EITB. The bivariate analysis penit us to establish association between positive EITB with factors as belong to the masculine gender, and to have multiple and mixed lesions in CT. Thus far, the prevalence of NC in individuals affected with epilepsy is approximately 15% and therefore we conclude that all patients affected with Epilepsy belonging to this geographical region should be screened for NC through EITB and CT.
l-l 7-l 5
Idiopathic epilepsy: Is the syndromatic classification able to discriminate genetic heterogeneity? Results from comdex segregation analysis applied to some types of generalized and partial idiopathic epilepsies
I. Jimenez, J. Sanchez, M. Jimenez, G. Palacio, 0. Mora, G. L6pez, L. Zuluaga, R. Isaza, C.S. Uribe, A. Villa, M. Arcos-Burgos. Antioquian League Against Epilepsy; Medellh, Colombia In the aim of discriminate modes of inheritance for Generalized and Partial Idiopathic epilepsies we are canted out complex segregation analysis for affected with Generalized Tonic Clonic Epilepsies’of the awakening type (GTCS), Childhood Absence Epilepsies (CAE) and Benign Focal Epilep-sies (BFE), classified according with the syndrome suffered bythe index-case. First analysis were applied on pedigrees partitioned according to each syndrome. Results shown that different modes of inheritance explain the familiar susceptibility for develop every one of the syndromes. GTCS show a mixed model of inheritance with a major locus codominant plus low multifactorial effects. CAE are explained for a threshold multifactorial model and BFE fit to a mixed model with a major recessive locus and very low muftffactorial effects. Contrary to partitioned analysis, when all unpartftioned set of pedigrees was tested, the model with best fitting was of the major gene only, without multifactorial component. A significant deficit of transmission in the heterozygote at major gene component, compatible with variations in gene expression more than gen heterogeneity was found. Lastly, a test of heterogeneity using the results from the complex segregation analysis between unpartitioned v/s partitioned set showed that different subtypes of syndromes are susceptible of genetic discrimination. We conclude that syndromic classification is able of discriminate between subtypes of Idiopathic Epilepsy showing familial conglomeration. Contrarily, fit of only major gen model without multifactorial component when all unpartitioned set of pedigrees is analyzed could be explained in bias terms associated to ascertainment misspecification derived from a soft clinical diagnosis.
S27
1 l-1 7-16 1 The estimation of risk degree and possibility preventive treatment of epilepsy V. Karlov, L. Zenkov, I. Zhidkova. Stomatological Institute, Moscow,
Department Russia
of Neurology,
of
Medical
The new diagnostic system was developed based on the clinico-electroencephalographical examinations of 105 patients with epilepsy and 120 patients from other diagnostic groups for the estimation of risk degree of epilepsy for the preventive treatment. According to our system the significant diagnostical signs are given to the definite statistically verified diagnostical coefficients (P < 0.01 corresponds 1 ball, P 4 0.001 - 10 ball), by summing them up we can calculate 3 indexes: 1) clinical, 2) EEG, 3) clinico-EEG. The degree of epilepsy risk is estimated by the digit of indexes (O-l-2-3). The third degree corresponds to the diagnosis of epilepsy. Developed system was applied to the group of 120 patients with different diagnosis, including panic attacks, convalsive forms of syncope, pseudoseizures, the single nonprovoked epileptic fit. The high reliability and differential diagnostic possibility were revealed. The estimation of risk degree of epilepsy in patients having risk factors, but not suffering from epilepsy, allows to select the correct tactics and if necessary to prescribe the preventive anticonvalsive treatment.
1l-l 7-17 1 Carbamazepine monotherapy child-bearing age V. Karlov, P. Vlasov, N. Kuchlinsky, A. Sokolov. Moscow Medical Institute, Moscow, Russia
in epileptic Department
women
of Neurology
There were studied serum fluctuations of Carbamazepine (CBZ) concentrations during mkffollicular and midluteal periods of menstrual cycle in 34 epileptic women with cryptogenic localization-related (mainly temporal lobe) epilepsy 21-30 y.o. without epileptic seizures for more than 6 months. In fifteen epileptic pregnant women (21-30 y.o. 62 f 3.6 kg) CBZ, estradiol and progesterone serum concentrations were evaluated monthly throughout pregnancy too. Daily doses of CBZ were from 400 mg to 1200 mg (most frequently 600 mg/day). In 113 observations background CBZ concentrations in follicular and luteal periods differ more than 33%. Background CBZ follicular period concentrations were hiohlv in comparison to luteal period and statistically different (P c 0.03) (nonparametric signs test). There was determined daily dose I serum correlations r = 0.56 (P < 0.02) in follicular period too (rank correlations test). CBZ concentrations in. women with catamenial epileptic seizures (9 cases) don’t differ from other serum CBZ concentrations. Decrease of CBZ concentrations during luteal period of menstrual cycle is suggested with opposite increase of summary level sex steroid hormones. This data required to monitore serum CBZ concentrations in luteal period of menstrual cycle. Preliminary results revealed dose-dependent connections between sex steroid hormones and CBZ during pregnancy. In cases with CBZ up to 600 mg/day estradiol and progesterone concentrations did not differentiate from population data throughout pregnancy. Doses 600-600 mg/day showed preferable tendency to low estradiol concentrations on first and third trimesters of pregnancy, and stable progesterone concentrations within gestation period. Doses 900 and over (muday) revealed stable low estradiol concentrations in all periods of pregnancy and tendency to low progesterone concentrations in third trimester.
l-l 7-l 6
Visual evoked potentials in patients with epilepsy prior to antieplleptic therapy and after introduction of antiipileptic therapy
G. Kiteva-Trencevska, R. Naumovski, Clinical Center Skopje, Macedonia
E. Cvetkovska.
Clinic of Neurolog):
The aim is to analyze the visual evoked potentials (VEPs) in patients with newly diagnosed epilepsy prior to introduction of antiepileptic therapy (AET) with carbamazepine (CZ) and to analyze the influence of AET with CZ on VEPs one month after achieving the therapeutic plasma concentration of CZ. Pattern shift whole field VEP was done in 25 patients with newly diagnosed epilepsy prior to introduction of AET with CZ and one month after the therapeutic plasma concentration of CZ was achieved. Each patient was his own control in comparing the VEPs prior to AET and after introduction of AET. The group of 25 patients consisted of 13 males and 12 females, the mean age was 26.6 years, standard deviation 11.23. Seven patients manifested generalized tonic clonic seizures, 11 patients partial seizures secondarily generalized, 3 patients complex partial seizures and 4 unclassified seizures. Somatoneurological examination and CT brain scans were normal in all patients. VEPs done prior to AET showed normal findings considering the shape, latency and amplitude of the VEP. VEPs done one month after achieving the therapeutic plasma concentration of CZ showed normal findings considering the shape, latency and amplitude of the VEP and no differences of VEPs compared with the findings of VEPs before AET. In conclusion, the functional state of the visual sensory system could be evalu-
S28
Epilepsy
ated using VEP in patients administration of AET.
l-l 7-l 9 V. Klimmek, Kirschberg,
with epilepsy
prior to AET and during continuous
Brain atrophy and polyneuropathy-syndrom valproic-acid monotherapy, a case report Ch. Lefebre. H&/and Weimar. Germany
K/iniken GmBH Neurologische
during Ktinik, Am
We report about a young female (f, 26) seizurefree patient who developed a hypesthesia in legs and hands, a slight depression and anxiety during monotherapy with valproic-acid because of a generalized epilepsy. After 3 years of this treatment (serum levels above 100 pgram/ml) the first signs of hypesthesia were discovered. The electrophysiological examinations of the peripheral nerves revealed a beginning neuropathy marked by axonal degenerations. Repeated MRI examinations of the brain showed a progressive diffuse cortical atrophy in the lapse of 3 years. Generalized spike-slow-wave complexes, which were unchanged in the course of the last 3 years could be detected in the EEG. Clinically there were no signs of epileptic activity. No other toxic substances, no metabolic changes and no other remarkable abnormalities could be found to explain the described impairments. We consider the remarkable progressive brain atrophy as a possible side effect of the high dose long term valproic-acid therapy in our patient although only 3 similar cases are reported in the literature up to now, because our otherwise healthy suffered from other known side effects during the drug therapy as described above, too. It is not known so far wether this brain atrophy is reversible after withdrawal. It is highly recommended to discuss a change in the anticonvulsant drug treatment if young young patients develop a progressive brain atrophy during valproic-acid treatment.
I1 17 20
Spike-and-waves complexes seizures by Carbamazepine
and exacerbation
of
S. Kochen, S. Oddo, B. Giagante, P. Said6n, W. Silva, D. Consalvo. Municipal Center of Epilepsy, Neutvtogy Division, Ramos Mejia Hospital, Bs. As., Argentina Purpose: Analysis of a number of patients in whom CBZ therapy adversely affected the EEG and produced seizure exacerbation. Identification of risk factors for this phenomena. Method: From a population of 2115 patients (p) of our Epilepsy Center, 736 were seen in the last two years, we founded 102~ (4.8%) that developed spike-wave complexes (SW) after CBZ therapy. Results: Twenty-five p. (24.5%) had Generalized Syndrome (GS), and 77p (75.5%) had Partial Syndrome (PS). In the group with GS before CBZ therapy, there were no SW. After CBZ treatment, 14 p. (56%) developed generalized spike slow wave (GSSW); lp. (4%) presented generalized 3 Hz SW; 2 p. (8%) continuous SW; and 6 p. (32%) focal spike slow wave (FSSW). In all of them there were a seizure exacerbation. After this events CBZ was discontinued, SW discharges disappeared in 23 p. (92%) persists GSSW in 1 p. (4%) and FSSW in 1 p. (4%). All p. showed a clinical improved. In the group with PS before CBZ, there were no SW. Four p. (40%) with Frontal Syndrome (FS) presented focal or generalized spike-slow wave. After CBZ treatment 54 p. (70.1%) developed FSSW; 20 p. (26%) developed GSSW. Three p. (3.9%) with FS development generalized 3 Hz SW. In p. with FSSW there were no seizure exacerbation. Remain p. presented an increase frequency and modified seizure type. When CBZ was stopped, in all p. disappeared SW and all had a clinical improve. Conclusion: CBZ is an excellent AED. In our experience, this problem was seen in a few cases. Basic research which manipulates intracortical GABA systems, show a variety of syndromes which may involve GABA B receptors independents GABA A receptors. Our findings may indicate that CBZ would excitate GABA 6 receptors in some of our patients. When changes occurred in the EEG or seizures exacerbated, CBZ should be discontinued immediately.
1 l-l 7-21 1 Pseudo seizures in epileptics-study Navneet Kumar, V. Anand, Akhilesh Gupta, R. Chandra, Neuro/qy Unit, GSVM Medical College, Kanpuc India 330 Patients of epilepsy were studied, out of 155 were females. 36 cases were found to have (NEAD). It was seen in 25 females (age 15-25 10-16 years). The commonest presentation was on the ground (20 patients). 8 patients complained mindedness lasting for about 30 minutes or more.
in 330 cases R.N. Dwivedi.
which 175 were males and non-epileptic attack disorder years) and 10 males (age drop attacks in from of falling of forgetfulness and absent 7 Patients presented with the
generalised shaking of the body. These 7 patients were already on anti-epileptic drugs for epilepsy and attacks appeared to be a conversion reaction since they could hear during unconsciousness and there was no injury, tongue bite & incontinence. EEG was abnormal in 20 patients out of 35. However 7 of these cases were already receiving anti-epileptic drug with controlled seizures. 80 of 35 cases responded to psychotherapy while remaining 7 had to be sent for psychiatric follow up. It is concluded that there is a high incidence (Approx. 10%) of non-epileptic attack disorder in patients attending epilepsy clinic. It is also noteworthy that epileptics are often prone to suffer with NEAD. the diagnosis of which may be suspected when epilepsy is controlled and serum drug levels are adequate. A high degree of suspicion and witnessing an attack are a prelude for its diagnosis.
1l-1 7-22 1 High expression of multidrug resistance gene (MD&l) and persistant low levels of phenytoin (PHT) on a patient with refractary epilepsy due to tuberous sclerosis (TS) A. Lazarowski, D. Riveros, G. Sevlever, M. Massaro, A.L. Rabinowicz. Departments of Neurology Child Neurology, Pathology and Medical Laboratory; lnstituto de lnvestigaciones Neurologicas ‘Raul Carrea”, FLENI, Bs. As., Argentina Objectives: To report the presence of MDR-1 phenotype and low plasma levels of PHT on a patient with TS and refractaty seizures. Methods: We report a 4 month old female with poorly controlled seizures since birth and a brain lesion proven on pathology to be consistent with TS. She received several i.v loading doses of PHT (IO-16 mg/kg) throughout her hospitalization. PHT blood levels were assessed by HLPC (Abbott-TDX). In order to estimate PHT half life, serial blood levels were drawn at 30 min and 2, 4.5 and 11.5 hours following the loading dose. Gp170 was investigated in the brain tissue by immunohistochemistry using monoclonal antibodies. Results: PHT blood levels were always subtherapeutic (ji: 2.4 us/ml, range: 0.45-3.50). PHT half life was 5 hours. lmmunohistochemical analysis disclosed overexpression for Gp-170, thus suggesting positivity for MDR-1. Conclusions: The expression of MDR-1 gene and persistant low levels of PHT, might be linked with a possible mechanism accounting for our patient drug resistant epilepsy.
1 l-l 7-23 1 {zgr$c
generalized
epilepsy of late onset.
J. Loiseau ‘, B. Duche ‘, M.-C. Picot *, N. Ayrivie ‘, A. Crespel 3, L. Letenneur4, P. Loiseau ‘, P. Jallon s. ‘Dept. of Neurology; 4Dept. of Epidemiolog)! University Hospital Bordeaux, France, *Medical Information Dept., 3 Epilepsy Unit, University Hospital Montpellie~ France, 5 Clinical Epilepsy Unit, HUG, Geneva, Switzerland Rationale: Idiopathic epilepsies are defined by age-related onset, clinical and EEG characteristics, and a presumed genetic etiology. Most of them start in childhood and adolescence. A second incidence peak in presenium was hypothesized (Luef et al. 1996). This study addressed the question of a later onset, i.e. after age 60. Methods: Available literature and personal data banks were analyzed, looking for patients with (1) generalized convulsive or nonconvulsive seizures with an onset after age 60: (2) bilateral spike-wave complexes on the EEG. Results: As regards GTCS, only 4 reports of patients were found in the literature, and none among 877 patients with a first seizure after 60 years. Nonconvulsive status epilepticus is not rare, but appears to be a heterogenous group. Conclusion: Convulsive idiopathic generalized epilepsies should be compared to the Loch Ness monster: some authors have seen them. Conversely, some nonconvulsive status epilepticus might be a late consequence of a genetic predisposition triggered by other epileptogenic factors such as psychotropic drug withdrawal, or metabolic/toxic disorders (Thomas et al. 1992, 1993).
1 l-1 7-24 1 Risk of recurrence after a first unprovoked Study of elderly outpatients V. Bertol, M.I. Perez, B. Gras, L. Santolarfa, A. Oliveros. Neurolog)! Migue/ Servet Hospital, Zaragoza, Spain
Department
seizure. of
Purpose and Methods: A follow-up of a mean duration of 18 months (range l-tl) from the day of their first unprovoked seizures was carried out on 49 patients, aged 60 years or older. All seizures were categorized as partial or generalized according to criteria recommended by the ILAE. The patient’s seizures were categorized by etiology as idiopathic or remote symptomatic. The cumulative risk of recurrence (RR) was determined by life-table methods with event defined as recurrence of an unprovoked seizure.