RESUMO
This 9-year-old boy was admitted at the age of 2 with a diagnosis of congenital hemiparesis while the rest of physical and neurological examination was normal. His score in the Wechsler intelligence scale was 80. Right fronto-parietal cortical dysplasia with hemisphere atrophy was evident by computerized tomography scanning and magnetic resonance imaging. The latter, also disclosed abnormal thick cortex which was interpreted as polymicrogyria or pachygyria. Karyotype was normal. He had a hemifacial motor seizure at the age of 7. At the age of 8 frequent atonic or inhibitory seizures were presented. Asymmetric bilateral spike discharges with high voltage in the right hemisphere during the EEG recording were found. His mother, a 35-year-old woman (Full scale; Adult intelligence scale: 85) also had congenital hemiparesis. She never had seizures and her EEG was normal. Magnetic resonance imaging disclosed right fronto-parietal cortical dysplasia with ipsilateral hemisphere atrophy. Karyotype was normal. Our cases should be interpreted as a familial presentation of the anomaly, probably with autosomal-dominant transmission.
Assuntos
Córtex Cerebral/anormalidades , Paresia/etiologia , Adulto , Atrofia/etiologia , Atrofia/patologia , Córtex Cerebral/diagnóstico por imagem , Córtex Cerebral/patologia , Córtex Cerebral/fisiopatologia , Criança , Eletroencefalografia , Feminino , Lobo Frontal/anormalidades , Lobo Frontal/diagnóstico por imagem , Lobo Frontal/patologia , Genes Dominantes , Humanos , Cariotipagem , Imageamento por Ressonância Magnética , Masculino , Lobo Parietal/anormalidades , Lobo Parietal/diagnóstico por imagem , Lobo Parietal/patologia , Convulsões/etiologia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To evaluate clinical and EEG features, as well as treatment and progression in fifteen patients with a diagnosis of acquired epileptic aphasia. PATIENTS AND METHODS: The population comprised nine male and six female patients, whose mean age was 14.5 (r = 8.11-20 years). All were on routine antiepileptic drugs. Inclusion criteria were acute, subacute or chronic aphasia, lacking signs of motor deficit or demonstrable brain lesion, but displaying bilateral spikes or generalized spike-wave discharges. Throughout, sleep and waking EEG, neuroimaging, brainstem auditory evoked potentials and neuro-psychological evaluations were performed. Cortical brainstem auditory evoked potentials were carried out in ten cases. RESULTS: Median age at onset of verbal auditory agnosia was 5.6 years (r = 1.1-8.6 years), which eleven cases developed epileptic seizures at a median age of five years. Waking and sleep EEG were abnormal but brainstem auditory evoked potentials were normal throughout. Cortical brainstem auditory evoked potentials in ten patients displayed P300 wave and vertex potential alterations. Five cases received 1-3 mg/kg/day prednisone during 6-12 months, with almost complete speech recovery in four. At the last follow-up, language impairment was mild in five patients, moderate in five and severe in three, while two children recovered normal speech. CONCLUSIONS: Early acquired epileptic aphasia treatment is advisable with valproic acid, benzodiazepines or ethosuximide alone or in combinations, supplementary with corticoids for at least six months in the absence of clinical response and/or EEG improvement. Globally, seven out of fifteen patients overcame their speech disorder.
Assuntos
Afasia/complicações , Afasia/diagnóstico , Epilepsia Generalizada/complicações , Adolescente , Adulto , Nível de Alerta/fisiologia , Encéfalo/anatomia & histologia , Encéfalo/diagnóstico por imagem , Criança , Doença Crônica , Eletroencefalografia , Epilepsia Generalizada/diagnóstico , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Testes Neuropsicológicos , Índice de Gravidade de Doença , Sono REM/fisiologia , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Partial benign epilepsy of childhood with occipital paroxysms (EPBI-O) described by Gastaut in 1982, has been recognized in the latest Classification of Epilepsy and Epileptic Syndromes as being a form of idiopathic partial epilepsy. In 1989 Panayiotopoulos described a different form of idiopathic occipital epilepsy. OBJECTIVE: We analyzed the electro-clinical characteristics of EPBI-O in order to identify the different forms of idiopathic occipital epilepsy. MATERIAL AND METHODS: This prospective study included 74 patients who fulfilled the diagnostic criteria of EPBI-O. The patients were evaluated between January 1990 and June 1996 by means of clinical and electro-encephalic examinations during a follow-up period of between 6 months and 6.5 years. RESULTS: We identified two groups. Group I: eighteen patients with visual crises and post-ictal migrainous episodes, with or without motor crises, predominantly when awake and with the onset of these crises at 8.7 years old. On EEG there were P-O occipital paroxysms which reacted to eyelid opening and group II: fifty-six patients with crises of vomiting followed by oculo-encephalic version, predominantly during sleep and with an average age of 4.9 years when these crises started to occur. On EEG there were occipital spikes with identical morphology to that of the benign epilepsy of childhood with spikes of Roland (EPBI-R). Two patients had associated crises of EPBI-O of Group II and EPBI-R with independent occipital and Roland spikes. These formed a third group. CONCLUSIONS: This study confirms the existence of two variantes of EPBI-O; the 'Gastaut' type and the 'Panayiotopoulos' type with a more benign and more frequent course.
Assuntos
Eletroencefalografia , Epilepsias Parciais/fisiopatologia , Lobo Occipital/fisiopatologia , Adolescente , Criança , Pré-Escolar , Epilepsias Parciais/diagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Sono REM/fisiologia , Tomografia Computadorizada por Raios X , Vigília/fisiologiaRESUMO
INTRODUCTION: Magnetic resonance has permitted the recognition of cortical dysplasias in patients with congenital hemiparesia and epilepsy. OBJECTIVE: To study the clinic-EEG characteristics and course of epilepsy in patients with congenital hemiparesia and unilateral polymicrogyria. METHODS AND RESULTS: We analyzed the clinical histories of 11 patients seen between 1990 and 1996. We studied 6 girls and 5 boys aged between 5 and 13 years, with a follow-up period of from 1 to 6 years. The epilepsy began at between 1 and 6 years old with partial motor seizures. On EEG there were frontotemporal spikes in 9 cases, temporooccipital in 1 and parieto-occipital in another. All 11 patients had hemiparesia, with slight mental retardation in 9 patients and moderate mental retardation in 2. The CT/MR brain scan showed unilateral polymicrogyria. At between 2 and 8 years of age, all 11 patients developed subintrant atonic crises with a pseudo-ataxic gait, absences in 7 patients and myoclonia in 3. Awake EEG showed bilateral asymmetrical spikes. During sleep 7 patients had continuous spikewave discharges and 4 had frequent asymmetrical bilateral spikes. Four patients relapsed. Five patients are free of crises, five have sporadic crises and one continues to have daily crises. CONCLUSIONS: These patients had hemiparetic cerebral paralysis, slight mental retardation and epilepsy. At about the age of 6 a peculiar electro-clinical condition developed. Response to treatment was satisfactory, although the follow-up period is still not long.