title: [State of the neuromuscular apparatus in Friedreich's familial ataxia (clinico-electromyographic analysis)].
abstract: The authors conducted a clinico-EMG study of 25 patients with Friedreich's familial ataxia. For this purpose with the aid of skin electrodes the distal muscles were studied. A supplementary study was made in 18 patients; the rate of the circulation of excitation along the motor fibres of the peripheral nerves and the monosynaptic-reflex were determined. In most of the studied patients there was a lesion of the peripheral motoneuron. The degree of the lesion depended mainly on the duration of the disease. The authors are of the opinion that the found disorders play a certain role in the mechanism of motor-coordinative disturbances in this disease.
mesh_terms: D000293:Adolescent; D000328:Adult; D004576:Electromyography; D005260:Female; D005621:Friedreich Ataxia / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009469:Neuromuscular Junction / Q000503:physiopathology*; D012023:Reflex, Monosynaptic; D012026:Reflex, Stretch
vernacular_title: Sostoianie nervo-myshechnogo apparata pri semeĭnoĭ ataksii Fridreĭkha (kliniko-élektromiograficheskiĭ analiz)