In a retrospective study we assessed the outcome of the criptogenic and symptomatic forms of West syndrome and evaluated the efficacy of adrenocorticotropic hormone, vigabatrin, prednisone, valproate and nitrazepam in the spasms control. Seventy patients were follwed up by 2 years. Twelve (17%) were criptogenics and 58 (83%) symptomatics. In criptogenic group significantly more patients were in regular school classes and with normal motor development, better control of seizure, less tendency to evoluate to Lennox Gastaut syndrome and 83. 3% had control of spasms (72.4% of patients from symptomatic group had control of spasms). Adrenocorticotropic hormone and vigabatrin were the most efective drugs, with 68.75% and 60% of spasms control, respectivelly, when used as first line of therapy and 75% and 50%, respectivelly, as second line of therapy.
The authors describe a family (mother, son and two daughters) with mitochondrial myopathy. The mother was asymptomatic. Two daughters had lactic acidosis and myoclonic epilepsy, mild dementia, ataxia, weakness and sensory neuropathy. The son suffered one acute hemiplegic episode due to an ischemic infarct in the right temporal region. All the patients studied had hypertension. EEG disclosed photomyoclonic response in the proband patient. Muscle biopsy disclosed ragged-red fibers and abnormal mitochondria by electron microscopy. Biochemical analysis showed a defect of cytochrome C oxidase in mitochondria isolated from skeletal muscle. Several clinical and genetic aspects of the mitochondrial encephalomyopathies are discussed.
RESUMO -Os autores observaram, no período de agosto-1978 a outubro-1988, 7 meninas com síndrome de Rett, cujo diagnóstico foi feito entre as idades de 2 e 10 anos, sendo seguidas por período médio de 5 anos e 9 meses. O início da regressão do desenvolvimento psicomotor ocorreu entre 5 e 24 meses. Microcefalia adquirida estava presente em 6 casos e hiperven¬ tilação, em 3. As convulsões tônico-clônicas generalizadas foram as mais freqüentes (6 pacientes) e tiveram início entre 1 e 7 anos de idade, a maioria medicada com carbamazepina. O EEG mostrou-se; lento difuso em 4 pacientes; com atividade irritativa em 5; normal em 1. As alterações no EEG, embora não específicas podem auxiliar no diagnóstico. A etiologia da síndrome permanece ainda desconhecida, sendo discutidos os papéis de possíveis causas genéticas e de neuro-transmissores.Rett's syndrome: report of 7 cases.
RESUMO -Relatamos as características clínicas e eletrencefalográficas de 13 pacientes com focos irritativos de linha média (LM) e parassagitais (PS) ou ambos. Puderam ser demonstradas manifestações clinicas tônieo-clônicas generalizadas, parciais complexas e parciais com generalização secundária.O padrão da manifestação epileptiforme é imprevisível; contudo, verificamos que a convulsão tônico-clônica generalizada foi o tipo mais comum (60%). Infelizmente, este dado não tem significado estatístico.O exame neurológico foi normal na maioria dos pacientes. Em relação à idade, há nítida predominância de crianças (84%) com LM e/ou PS. O sono constitui o principal método de ativação (73%) na pesquisa deste tipo de atividade epileptiforme. Embora a incidência de focos de LM e/ou PS seia muito baixa (0,4%), a elevada potencialidade epileptogênica que têm justifica a avaliação cuidadosa e adequada dessas regiões. Atenção particular deve ser dada aos complexos normais do sono, especialmente em crianças, pois podem por vezes simular atividade epileptiforme verdadeira. Midliner parasagitai foci: clinical and electroencephalographic features.SUMMARY -We report the clinical and electroencephalographic characteristics of thirteen patients with midline spikes (MS), parasagital foci (PF), or both. Numerous clinical manifestations, including generalized tonic-clonic, complex partial and partial with secondary generalization could be demonstrated.The pattern of the epileptiform manifestation is unpredictable; however, we find generalized tonic-clonic seizure the commonest type (60%). Unfortunately, this data has no statistic value. Neurologic examination was normal in the majority of the patients. Regarding age, there is a marked predominance of children (84%) with MS and/or PF. Sleep constitutes the main activation method (73%) in the search for this kind of epileptiform activity.In conclusion, although the incidence of the MS/PF is quite low (0.4%), the high epileptogenic potencial (70%) of them justifies a careful and adequate evaluation of these regions. Special attention must be paid to the normal sleep complexes, mainly in children, which sometimes can mimic true epileptiform activity.Os focos irritativos de linha média (Fz, Cz e Pz) e regiões parassagitais (F3F4, C3C4 e P3P4) constituem por vezes elementos eletrográficos de difícil caracterização, dado a peculiaridades de sua apresentação, principalmente em crianças e em sono (fase II), em que grafo-elementos normais assumem aspecto extremamente agudo que pode gerar alguma confusão.A literatura oferece algumas orientações segundo as quais seria possível definir se o achado em questão é fisiológico ou não.Apesar de adequadas teoricamente, estas determinações perdem em aplicabilidade e praticidade na interpretação rotineira do eletrencefalograma (EEG).A par disto, a literatura
A case of a 65 years-old black man with chronic renal failure, treated with chronic haemodialysis, in which a dialysis dementia syndrome developed is reported. In the initial phase the electroencephalogram had the typical burst of sharp and slow waves, waning with haloperidol and diazepam treatment. The withdrawal of this medication induced reappearance of the symptoms and EEG. abnormalities. As soon as the patient came back to the diazepine (potassium clorazepate), the mental symptoms subsided, but few weeks later he died of a cerebral haemorrhage. The electroencephalographic features are described and a brief comment about this disease is made.
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