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The peroneal muscular atrophy syndrome. Clinical, genetic, electrophysiological and nerve biopsy studies. Part 2. Observations on pathological changes in sural nerve biopsies.
J Neurol Sci. 1977 May; 32(1):91-122.JN

Abstract

The light-and electron-microscopic, single teased nerve and morphometric studies of a series of 17 sural nerve biopsies from patients with personeal muscular atrophy are presented. The cases are divided into the following groups according to the criteria of Davis, Bradley and Madrid (1977): Hypertrophic Neuropathy Group; Intermediate Group; Neuronal Sensorimotor Group; Neuronal Motor Group. The Hypertrophic Neuropathy Group had nerve hypertrophy and marked segmental demyelination and onion bulb formation. The Intermediate Group also had segmental demyelilination and onion bulb formation, but nerve hypertrophy was not seen, and axonal degeneration and regeneration were prominent. The Neuronal Sensorimotor Group cases were all sporadic, and showed some onion bulbs, paranodal demyelination and evidence of axonal degeneration and regeneration. The sensory nerve biopsy in the Neuronal Motor Group showed no major abnormality apart from some cluster formation indicating axonal regeneration. The data tend to support the classification of peroneal muscular atrophy proposed by Davis et al. (1977), though there was overlap between the groups in individual pathological parameters.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

864493

Citation

Madrid, R, et al. "The Peroneal Muscular Atrophy Syndrome. Clinical, Genetic, Electrophysiological and Nerve Biopsy Studies. Part 2. Observations On Pathological Changes in Sural Nerve Biopsies." Journal of the Neurological Sciences, vol. 32, no. 1, 1977, pp. 91-122.
Madrid R, Bradley WG, Davis CJ. The peroneal muscular atrophy syndrome. Clinical, genetic, electrophysiological and nerve biopsy studies. Part 2. Observations on pathological changes in sural nerve biopsies. J Neurol Sci. 1977;32(1):91-122.
Madrid, R., Bradley, W. G., & Davis, C. J. (1977). The peroneal muscular atrophy syndrome. Clinical, genetic, electrophysiological and nerve biopsy studies. Part 2. Observations on pathological changes in sural nerve biopsies. Journal of the Neurological Sciences, 32(1), 91-122.
Madrid R, Bradley WG, Davis CJ. The Peroneal Muscular Atrophy Syndrome. Clinical, Genetic, Electrophysiological and Nerve Biopsy Studies. Part 2. Observations On Pathological Changes in Sural Nerve Biopsies. J Neurol Sci. 1977;32(1):91-122. PubMed PMID: 864493.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The peroneal muscular atrophy syndrome. Clinical, genetic, electrophysiological and nerve biopsy studies. Part 2. Observations on pathological changes in sural nerve biopsies. AU - Madrid,R, AU - Bradley,W G, AU - Davis,C J, PY - 1977/5/1/pubmed PY - 1977/5/1/medline PY - 1977/5/1/entrez SP - 91 EP - 122 JF - Journal of the neurological sciences JO - J. Neurol. Sci. VL - 32 IS - 1 N2 - The light-and electron-microscopic, single teased nerve and morphometric studies of a series of 17 sural nerve biopsies from patients with personeal muscular atrophy are presented. The cases are divided into the following groups according to the criteria of Davis, Bradley and Madrid (1977): Hypertrophic Neuropathy Group; Intermediate Group; Neuronal Sensorimotor Group; Neuronal Motor Group. The Hypertrophic Neuropathy Group had nerve hypertrophy and marked segmental demyelination and onion bulb formation. The Intermediate Group also had segmental demyelilination and onion bulb formation, but nerve hypertrophy was not seen, and axonal degeneration and regeneration were prominent. The Neuronal Sensorimotor Group cases were all sporadic, and showed some onion bulbs, paranodal demyelination and evidence of axonal degeneration and regeneration. The sensory nerve biopsy in the Neuronal Motor Group showed no major abnormality apart from some cluster formation indicating axonal regeneration. The data tend to support the classification of peroneal muscular atrophy proposed by Davis et al. (1977), though there was overlap between the groups in individual pathological parameters. SN - 0022-510X UR - https://www.unboundmedicine.com/medline/citation/864493/The_peroneal_muscular_atrophy_syndrome__Clinical_genetic_electrophysiological_and_nerve_biopsy_studies__Part_2__Observations_on_pathological_changes_in_sural_nerve_biopsies_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0022-510X(77)90042-9 DB - PRIME DP - Unbound Medicine ER -