[Juvenile muscular atrophy of the distal upper limb--three decades of description and it's treatment].


This disorder has been separated clinically from motor neuron disease. Its clinical characteristics were clarified through our reports in 1957-1972. Muscular weakness and atrophy began insidiously in the growing generation, mostly in males, and were limited to the distal part of a unilateral upper limb or unilaterally-predominated upper limbs, showing a peculiar "oblique atrophy". The symptoms came to a halt spontaneously after a progressive course for several years. electromyography showed neurogenic changes in affected muscles innervated by the lower cervical segments, as well as in the corresponding muscles on a clinically unaffected side. The responsible lesion and pathogenesis had been unknown for decades until we reported pathological findings of the first autopsy case in 1987. The lesion was addressed to the bilateral anterior horns with a side-preponderance from the cord segment C5 to T1, most evident at C7 and C8, where we found necrotic changes presumably due to a local circulatory failure (ischemic myelopathy). On the other hand, we performed a radiological examination (myelography) using computerized tomography and magnetic resonance imagings, and found a dynamic compression of the spinal cord. In a posture of neck flexion, the posterior wall of the dural canal shifted anteriorly leaving the vertebral arch around the sixth cervical vertebrae, resulting in an antero-posterior compression of the cord segment from C7 to C8. The degree of the anterior shift was small in patients with longer duration, and had inverse correlation to the years from onset. In 1882 he was appointed as the first Professor of Neurology, under the strong support of Gambetta, Prime Minister of the third republican cabinet. Ackerknecth said that the Salpêtrière has became Medical Institution only by Revolution. An we remember that through the Enlightment of France, the spirit of "Philanthropy" was flowing concretely at the Salpêtrière, as seen in the figures of Pinel or Esquirol.(ABSTRACT TRUNCATED AT 250 WORDS)

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@article{Hirayama1993JuvenileMA, title={[Juvenile muscular atrophy of the distal upper limb--three decades of description and it's treatment].}, author={Kouichi Hirayama}, journal={Rinshō shinkeigaku = Clinical neurology}, year={1993}, volume={33 12}, pages={1235-43} }