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AAEE case report #20: hereditary motor and sensory neuropathy, type I.
Muscle Nerve. 1989 Nov; 12(11):875-82.MN

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.

Authors+Show Affiliations

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

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

2608082

Citation

Chad, D A.. "AAEE Case Report #20: Hereditary Motor and Sensory Neuropathy, Type I." Muscle & Nerve, vol. 12, no. 11, 1989, pp. 875-82.
Chad DA. AAEE case report #20: hereditary motor and sensory neuropathy, type I. Muscle Nerve. 1989;12(11):875-82.
Chad, D. A. (1989). AAEE case report #20: hereditary motor and sensory neuropathy, type I. Muscle & Nerve, 12(11), 875-82.
Chad DA. AAEE Case Report #20: Hereditary Motor and Sensory Neuropathy, Type I. Muscle Nerve. 1989;12(11):875-82. PubMed PMID: 2608082.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - AAEE case report #20: hereditary motor and sensory neuropathy, type I. A1 - Chad,D A, PY - 1989/11/1/pubmed PY - 1989/11/1/medline PY - 1989/11/1/entrez SP - 875 EP - 82 JF - Muscle & nerve JO - Muscle Nerve VL - 12 IS - 11 N2 - 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. SN - 0148-639X UR - https://www.unboundmedicine.com/medline/citation/2608082/AAEE_case_report_ L2 - https://doi.org/10.1002/mus.880121102 DB - PRIME DP - Unbound Medicine ER -