[Clinicoelectroneuromyographic characteristics of neuralgic amyotrophy]

Clinical and genealogical examinations of 155 patients with neural amyotrophies were carried out. Use was made of general and local electromyography, determinations of the speed of impulse transmission along the efferent fibres of the peripheral nerves, and examinations of the H-reflex. The examinations revealed a great diversity of the clinical forms of the neural amyotrophies, each forms having a definite clinical picture, type of hereditary transmission, and characteristic electrophysiological changes. The electroneuromyographic examinations showed that in cases of Charcot-Marie-Tooth's classical neural amyotrophy neuritic changes were prevailing in rapidly-progressing autosomo-dominant neural amyotrophies and autosomo-recessive infantile neural amyotrophy the peripheral efferent neuron was affected over its entire length. A complex examination of Roussy-Levy's syndrome revealed a pronounced dissociation between the relatively unimpaired motor function and the considerable delay of the impulse transmission along the efferent fibres of the peripheral nerves. In heterozygotic carries signs of mononeuron dysfunction were determined.