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Ultrastructural and Morphometric Observations on Nerve Fibers, Mitochondria, and Dense-Cored Vesicles

Claudia Sommer, MD; J. Michael Schröder, MD

Arch Neurol. 1989;46(9):973-977.

Abstract
• Nerve biopsy specimens from three cases of hereditary motor and sensory neuropathy with optic atrophy were studied by light and electron microscopy and by morphometry. All cases had a chronic neuropathy of the neuronal/axonal type with little, presumably secondary, demyelination. There was predominant reduction of the large-caliber population of myelinated and unmyelinated nerve fibers. The number of dense-cored vesicles in unmyelinated and small myelinated fibers was increased. Abnormal mitochondria in Schwann cells with paracrystalline inclusions, prominent cristae including paracrystalline material (cases 1 and 2), and axonal mitochondria with presumable hydroxyapatite crystals (case 3) were found. The morphologic results suggest that hereditary motor and sensory neuropathy with optic atrophy should be regarded as a separate entity within the hereditary motor and sensory neuropathy group.

Author Affiliations
From the Institute of Neuropathology, Hospitals of the Rheinisch Westfälische Technische Hochschule Aachen, Aachen, West Germany.

Footnotes
Accepted for publication January 3, 1989.

Presented, in part, at the Arbeitstagung der Deutschen Gesellschaft für Neurologie, Essen, West Germany, September 1987.

Reprint requests to Institut für Neuropathologie, Klinikum der Rheinisch Westfälische Technische Hochschule Aachen, Pauwelsstrasse, D-4 5100 Aachen, West Germany (Dr Schröder).

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