Mitochondrial Abnormalities of Late Motor Neuron Degeneration Following Poliomyelitis and Other Neurogenic Muscular Atrophies

Overview

Journal J Neurol

Specialty Neurology

Date 1979 Sep 1

PMID 91673

Citations 2

Authors

D Schiffer

L Palmucci

A Bertolotto

G Monga

Affiliations

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Abstract

A case of late motor neuron degeneration following poliomyelitis with abnormal mitochondria in muscle fibers is presented with two additional cases of systemic neurogenic muscular atrophy (Charcot-Marie-Tooth disease). Muscle biopsy revealed a neurogenic pattern of variable severity in all cases. Subsarcolemmal zones of hyperactivity and hyperpositive intermyofibrillar collections of granular material present in a variable proportion of type I fibers were demonstrated by oxidative enzymes. Ultrastructurally they corresponded to abnormal mitochondria, with paracrystalline inclusions in one case. The finding is discussed in the light of the previous literature on mitochondrial myopathies. Mitochondrial alterations are not specific and their significance in neurogenic conditions is debated.

Citing Articles

Focal deficiency of cytochrome-c-oxidase in skeletal muscle of patients with progressive external ophthalmoplegia. Cytochemical-fine-structural study.

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PMID: 6318426 DOI: 10.1007/BF00695049.

Mixed nemaline-mitochondrial "myopathy".

Kornfeld M Acta Neuropathol. 1980; 51(3):185-9.

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