Edymar Jardim

Aspectos clinicos da neurocisticercose: análise de 500 casos

This was a retrospective study, made at the Hospital das Clinicas University of São Paulo Medical School--Ribeirão Preto from 1956 to 1979 in 500 patients with neurocysticercosis. To our knowledge, this represented the largest sample analysed so far. Clinical manifestations and complemmentary tests were studied. The neurocysticercosis is a serious endemic disease in our region and represented 2.7% of all clinical evaluations by the Neurology Department and 7.5% of all hospital admissions. The most frequent clinical presentations were the epileptic (64.8%), the intracranial hypertension (35.6%), and the meningitic (29%). Radiological study of the skull showed intracranial calcifications suggestive of cysticercosis in 47.6%. The cerebrospinal fluid was abnormal in 81.3%: pleocytosis in 60.9%, increased protein in 49.1% and increased pressure in 29%. The presence of eosinophils occurred in 41.9%, and the Weinberg test was positive in 62,6% of all the cases. It was discussed the utility of the different drugs and the results of the surgical treatment in neurocysticercosis drawing the conclusion that they show no efficacy when analysed at the end of a long follow-up. Since none of the drugs seems to be really efficient, only the sanitary education will be able to control this endemic disease that grasses among us with so somber perspectives.This was a retrospective study, made at the Hospital das Clinicas University of Sao Paulo Medical School - Ribeirao Preto from 1956 to 1979 in 500 patients with neurocysticercosis. To our knowledge, this represented the largest sample analysed so far. Clinical manifestations and complemmentary tests were studied. The neurocysticercosis is a serious endemic disease in our region and represented 2.7% of all clinical evaluations by the Neurology Department and 7.5% of all hospital admissions. The most frequent clinical presentations were the epileptic (64.8%), the intracranial hypertension (35.6%), and the meningitic (29%). Radiological study of the skull showed intracranial calcifications suggestive of cysticercosis in 47.6%. The cerebrospinal fluid was abnormal in 81.3%: pleocytosis in 60.9%, increased protein in 49.1% and increased pressure in 29%. The presence of eosinophils occurred in 41.9%, and the Weinberg test was positive in 62,6% of all the cases. It was discussed the utility of the different drugs and the results of the surgical treatment in neurocysticercosis drawing the conclusion that they show no efficacy when analysed at the end of a long follow-up. Since none of the drugs seems to be really efficient, only the sanitary education will be able to control this endemic disease that grasses among us with so somber perspectives.

Characterisation of the unstable expanded CAG repeat in the MJD1 gene in four Brazilian families of Portuguese descent with Machado-Joseph disease

Machado-Joseph disease (MJD) is an autosomal dominant neurodegenerative disorder which has been shown to result, in Japanese families, from the expansion of a CAG repeat in the MJD1 gene on chromosome 14q. We show that the same molecular mechanism is responsible for MJD in four large Brazilian kindreds of Portuguese descent. The behaviour of the mutation was evaluated in 28 affected and 19 asymptomatic gene carriers. The number of repeats in the expanded alleles ranged from 66 to 77 with a strong negative correlation with age at onset (r=0·79). A mean 1·6 repeats increase from generation to generation correlated with clinical anticipation. Instability of the CAG repeat was bidirectional, with expansions as well as contractions, and was more marked in paternal transmissions. Finally, linkage disequilibrium was complete at locus D14S280 in the four Portuguese-Brazilian kindreds and four previously reported French families with the same mutation, which suggests the existence of a common founder.

Epilepsia e doença de chagas cronica

A sindrome epileptica em chagasicos cronicos, foi referida raramente na literatura especializazda, nao tendo sido feito ate o momento, um estudo detalhado das suas manifestacoes. Partindo-se da premissa de que a molestia de Chagas tem por substrato anatomico uma destruicao neuronal, procurou-se comparar dois grupos de epilepticos, um dos quais com molestia de Chagas cronica. Foram estudados 167 pacientes epilepticos, dos quais 44 eram comprovadamente chagasicos. O estudo permitiu coletar dados referentes a procedencia dos pacientes, resultado soro-logico, sexo, idade, epoca de incidencia das manifestacoes epilepticas, elementos dos exames neurologicos, do liquido cefalorraqueano, eletrencefalografico e os resultados da terapeutica anticonvulsivante. Como resultados principais destacamos o inicio tardio da epilepsia nos chagasicos, e o predominio acentuado das crises parciais com sintomatologia elementar de tipo autonomico. O exame neurologico e o do liquido cefalorraqueano, apesar de apresentarem percentualmente nos seus resultados, taxas moderadamente mais elevadas na incidencia de alteracoes, nao caracterizaram sindromes neurologicas bem definidas. O exame eletrencefalografico, revelou alteracoes sugestivas de comprometimento orgânico cerebral difuso. A terapeutica anticonvulsivante, baseada na utilizacao de hidantoinatos, barbituricos, primidona e benzodiazepinicos, mostrou que o controle das crises foi mais dificil nos chagasicos, exigindo maiores quantidades de medicacao, com resultados menos satisfatorios.

Mielopatia por histoplasmose: registro de um caso

E relatado um caso de sindrome mielitica aguda por histoplasmose. A paciente apresentou um quadro de paraparesia aguda, com alteracoes da sensibilidade superficial e profunda em nivel de T11-T12, e perda do controle esfincterico. O exame do LCR revelou bloqueio total, confirmado pela mielografia. A mielografia e a laminectomia mostraram tratar-se de uma paquimeningite no nivel considerado, com intensa granulomatose da aracnoide. O exame histopatolo-gico revelou a presenca de granulomas por H. capsulatam. A terapeutica pela anfotericina B utilizada por via intrarraquidea e parenteral, nao condicionou resultados satisfatorios.

Forma nervosa crônica da doença de Chagas: estudo clínico evolutivo e anatomopatológico de um caso seguido durante vinte anos

As formas nervosas cronicas da doenca de Chagas comprometem tanto o sistema nervoso central (SNC) como o periferico, apesar de nao serem encontradas com a frequencia assinalada por Chagas em suas observacoes iniciais. O atual relato refere-se a uma paciente chagasica cronica desde a infância que, progressivamente, desenvolveu comprometimento da motricidade voluntaria, do tono muscular, da coordenacao, dos nervos cranianos. Sofria ainda de uma cardiopatia e de uma colopatia chagasicas, vindo a falecer no pos-operatorio de uma hemicolectomia. Foi necropsiada e o estudo histopatologico do SNC revelou desmielinizacao dos feixes espinocerebelares e dos cordoes posteriores, grande reducao do numero de celulas de Purkinje, grande destruicao neuronal da substância negra e do locus coeruleus, estado lacunar dos nucleos da base, infiltracao tecidual por corpusculos de Herring aberrantes, porencefalia e espessamento meningeo.

Moléstia de Chagas aguda experimental: parasitismo do hipotálamo

O autor procurou relacoes entre alteracoes funcionais atribuidas ao hipotalamo e lesoes orgânicas do mesmo, na vigencia da molestia de Chagas. Usou em seu trabalho ratos inoculados com T. cruzi por via intraperitonial. Realizou estudo histopatologico e quantitativo neuronal das varias regioes hipotalâmicas, em cortes seriados corados com a hematoxilina-eosina. Encontrou consideravel percentagem de parasitismo hipotalâmico (70%), sendo que a regiao anterior e mais parasitada que a media ou a posterior. A destruicao neuronal foi tambem mais evidente na regiao anterior. Sugere que as alteracoes do metabolismo glicidico, tireoidiano, da sudacao e da potassemia, devam ser consideradas como sequelas destas lesoes.

Moléstia de Hand-Schuller-Christian

A case of Hand-Schuller-Christian disease is reported. Radiologic and histopathological studies were done. Endocrine and neurologic impairments were not observed.

Riso na epilepsia

Three cases of temporal lobe epilepsy in which the seizures were initiated by outbursts of laughter are reported. The laughter was forced and uncontrollable. This symptom disappeared with the use of anticonvulsivant drugs. The mechanism of laughter is discussed.