Muscle Nerve. 1989 Nov;12(11):875-82.

AAEE case report #20: hereditary motor and sensory neuropathy, type I.

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Department of Neurology, University of Massachusetts Medical Center, Worcester 01655.

Abstract

A case study is reported regarding a 32-year-old man with classic clinical and electrophysiologic features of hereditary motor and sensory neuropathy, type I (HMSN I), a slowly progressive autosomal dominant condition marked by slow motor and sensory velocities and generalized segmental demyelination. Another clinically similar autosomal dominant neuropathy (HMSN II) is distinguished from HMSN I by nearly normal nerve conduction velocity. Acquired demyelinating neuropathy may occasionally resemble HMSN I clinically, but the former demonstrates electrophysiologic features not seen in the latter such as conduction block, dispersed compound muscle action potentials, and differential slowing of conduction velocity. Neuropathologic studies of HMSN I suggest that both neuronal and Schwann cell distrubances play a role in pathogenesis.

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Muscle Nerve. 1991 Jan;14(1):85-6.

PMID:: 2608082; [PubMed - indexed for MEDLINE]

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AAEE case report #20: hereditary motor and sensory neuropathy, type I.
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AAEE case report #20: hereditary motor and sensory neuropathy, type I.

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