Hereditary Distal Dominant Amyotrophy Followed bySpastic Paraplegia.

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概要

• 論文の詳細を見る

• Clinical, neurophysiological and neuropathological investigations were performed on five patients from two families with autosomal dominant distal amyotrophy followed by spastic paraplegia and with a positive history in two generations of these two families. All cases in the two families had a benign clinical course, although two mothers could not walk without support at around 60 years old. Neurophysiological studies revealed normal maximum conduction velocities of peripheral sensory and motor nerves, and the central spinal sensory pathway. Distribution of motor nerve conduction velocities in the ulnar nerve had a normal pattern except for one patient who had severe deformities of the cervical vertebrae. The biopsied sural nerve disclosed no distinct abnormalities in any cases. From these results, we confirmed preservation of the myelinated nerve fibers of motor and sensory peripheral nerves.(Internal Medicine 32: 825-831, 1993)

• 社団法人　日本内科学会の論文

著者

• SUNOHARA Nobuhiko

  the Department of Neurology, National Center of Neurology and Psychiatry

関連論文

• Hereditary Distal Dominant Amyotrophy Followed bySpastic Paraplegia.

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