Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies
Tóm tắt
A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.
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Tài liệu tham khảo
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