Tags

Type your tag names separated by a space and hit enter

A clinicopathologic study of acrodystrophic neuropathies.
Muscle Nerve. 1980 Nov-Dec; 3(6):491-501.MN

Abstract

Sixteen patients presenting with trophic changes associated with a peripheral neuropathy were investigated. Muscle power was normal in all patients, but neurogenic muscle atrophy was demonstrated in 4 of the 7 patients who had a muscle biopsy. Alcoholism was responsible for the neuropathy in 11 patients. In the other patients, one had primary hemochromatosis without diabetes and another a dominantly inherited primary hypertrophic neuropathy. Qualitative and quantitative light and electron microscopic studies, including teased nerve fiber preparations, showed axonal loss as the most salient feature. In the alcoholic patients, the large myelinated fibers were primarily involved, followed by small myelinated and unmyelinated fibers. The lesions were predominant distally as shown in patients who had a sural nerve biopsy at both calf and ankle levels. A mechanism of dying-back degeneration of the longest sensory fibers is the most plausible explanation for neurological and pathological abnormalities. In alcoholic neuropathy with trophic changes, loss of sensory fibers is more important than in alcoholic neuropathy without trophic changes. In familial and sporadic cases, axonal loss is more severe and unmyelinated fibers are more severely affected than in alcoholic acrodystrophic neuropathy. Patients with peripheral neuropathies who present with loss of pain sensation but have preserved muscle power are especially exposed to the development of trophic changes induced by usual trauma in insensitive tissues.

Authors

No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

6256624

Citation

Said, G. "A Clinicopathologic Study of Acrodystrophic Neuropathies." Muscle & Nerve, vol. 3, no. 6, 1980, pp. 491-501.
Said G. A clinicopathologic study of acrodystrophic neuropathies. Muscle Nerve. 1980;3(6):491-501.
Said, G. (1980). A clinicopathologic study of acrodystrophic neuropathies. Muscle & Nerve, 3(6), 491-501.
Said G. A Clinicopathologic Study of Acrodystrophic Neuropathies. Muscle Nerve. 1980 Nov-Dec;3(6):491-501. PubMed PMID: 6256624.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A clinicopathologic study of acrodystrophic neuropathies. A1 - Said,G, PY - 1980/11/1/pubmed PY - 1980/11/1/medline PY - 1980/11/1/entrez SP - 491 EP - 501 JF - Muscle & nerve JO - Muscle Nerve VL - 3 IS - 6 N2 - Sixteen patients presenting with trophic changes associated with a peripheral neuropathy were investigated. Muscle power was normal in all patients, but neurogenic muscle atrophy was demonstrated in 4 of the 7 patients who had a muscle biopsy. Alcoholism was responsible for the neuropathy in 11 patients. In the other patients, one had primary hemochromatosis without diabetes and another a dominantly inherited primary hypertrophic neuropathy. Qualitative and quantitative light and electron microscopic studies, including teased nerve fiber preparations, showed axonal loss as the most salient feature. In the alcoholic patients, the large myelinated fibers were primarily involved, followed by small myelinated and unmyelinated fibers. The lesions were predominant distally as shown in patients who had a sural nerve biopsy at both calf and ankle levels. A mechanism of dying-back degeneration of the longest sensory fibers is the most plausible explanation for neurological and pathological abnormalities. In alcoholic neuropathy with trophic changes, loss of sensory fibers is more important than in alcoholic neuropathy without trophic changes. In familial and sporadic cases, axonal loss is more severe and unmyelinated fibers are more severely affected than in alcoholic acrodystrophic neuropathy. Patients with peripheral neuropathies who present with loss of pain sensation but have preserved muscle power are especially exposed to the development of trophic changes induced by usual trauma in insensitive tissues. SN - 0148-639X UR - https://www.unboundmedicine.com/medline/citation/6256624/A_clinicopathologic_study_of_acrodystrophic_neuropathies_ L2 - https://doi.org/10.1002/mus.880030606 DB - PRIME DP - Unbound Medicine ER -