[State of the neuromuscular apparatus in Friedreich's familial ataxia (clinico-electromyographic analysis)].

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.