[Charcot-Marie-Tooth disease. Electromyographic studies in 45 patients]. / Doença de Charcot-Marie-Tooth. Estudos eletromiográficos em 45 pacientes.

The electrophysiological studies of 45 patients with Charcot-Marie-Tooth disease (CMT) are presented. The nerve conduction of the motor median and ulnar nerves permitted us to separate our patients in two types: type I (demyelinating) with motor nerve conduction (MNC) below 38 m/s (11 cases) and type II with MNC normal or above 38 m/s (34 cases). In type I there was no correlation between reduction in MNC and clinical severity. It was not possible to classify the disease on the sural nerve sensory action potential (SAP). They were unobtainable in most cases. In many patients with CMT type II the MNC was normal. In the cases the sural SAP was absent or reduced. We concluded that the MNC study is the best useful test to classify CMT disease in type I and type II.

Neuropatia diabética: I. Conceito, epidemiologia, classificaçäo, quadro clínico e eletroneuromiográfico: estudo de 210 casos / Diabetic neuropathy: I. Definition, epidemiology, classification, clinical features and electrophysiologic changes: study of 210 patients

Os autores fazem revisäo da neuropatia diabética, seu conceito, sua prevalência e citam as várias classificaçöes existentes. Referem-se aos principais quadros clínicos apresentando 210 pacientes com neuropatia diabética estudados num período de quatro anos (1987-1991). As polineuropatias compreendem 184 casos. A forma sensitiva foi a predominante ocorrendo em 135 enfermos. Seguiram-se com menor número as formas sensitivo-motora, atáxica, motora e autonômica. Os distúrbios autonômicos estiveram presentes 63 vezes, sendo as alteraçöes pupilares as mais freqüentes. Os nervos cranianos foram acometidos 20 vezes, predominando a paralisia do VII e III nervos. O nervo mediano foi acometido em 20 oportunidades e a plexopatia lombar unilateral ocorreu em 10 pacientes. A eletroneuromiografia foi realizada em 63 pacientes, predominando a lesäo axonal sensitivo-motora (43 vezes)

Neuropatia diabetica: I-Conceito, epidemiologia, classificacao, quadro clinico e eletroneuromiografico. Estudo de 210 casos

Os autores fazem revisao da neuropatia diabetica, seu conceito, sua prevalencia e citam as varias classificacoes existentes. Referem-se aos principais quadros clinicos apresentado 210 pacientes com neuropatia diabetica estudados num periodo de quatro anos (1987-1991). As polineuropatias compreendem 184 casos. A forma sensitiva foi a predominante ocorrendo em 135 enfermos. Seguiram-se com menor numero as formas sensitivo-motora, ataxica, motora e autonomica. Os disturbios autonomicos estiveram presentes 63 vezes, sendo as alteracoes pupilares as mais frequentes. Os nervos cranianos foram acometidos 20 vezes, predominando a paralisia do VII e III nervos. O nervo mediano foi acometido em 20 oportunidades e a plexopatia lombar unilateral ocorreu em 10 pacientes. A eletroneuromiografia foi realizada em 63 pacientes, predominando a lesao axonal sensitivo-motora (43 vezes).

[Polyneuropathy caused by trichlorfon: report of a case with electrophysiologic and histopathologic study of the sural nerve]. / Polineuropatia por triclorfon: registro de um caso com estudo eletrofisiológico e histopatológico do nervo sural.

The authors observed a patient who worked in a farm and suffered an organophosphate intoxication (trichlorfon). The immediate effect was manifested by vomiting and abdominal cramps. Three months later he presented a distal symmetric sensorimotor (predominantly motor) neuropathy with distal muscle atrophy. Electromyography has revealed denervation changes in every muscle studied and the sensory and motor nerve conduction was slowed in arms and legs. The sural nerve biopsy specimen studied by light microscopy with semi-thin section and teased fiber preparation showed axonal degeneration. The ultrastructural studies of the axonal alterations consisted of degeneration of the neurofilaments and the neurotubules with granular appearance of the axoplasm.

[Familial myasthenia gravis: report of 2 brothers]. / Miastenia gravis familiar: registro de dois irmãos.

The familial form of myasthenia gravis is a relatively rare condition, occurring in about 3.4 per cent of myasthenic patients. Two familial cases with ocular myasthenia gravis are reported. They had a third brother who died probably with the same disease. Their parents are cousins. The authors made a brief approach of genetic, clinical, statistical and therapeutic aspects of the disease.