Peripheral neuropathy associated with hereditary and sporadic inclusion body myositis: confirmation by electron microscopy and morphometry.
Inclusion body myositis (IBM) is a disabling myopathy affecting proximal and distal muscle groups. The involvement of peripheral nerves in IBM is still a controversial matter. In a previous morphometric study at the light microscopic level only, we described a peripheral neuropathy in sural nerve biopsies of eight patients with sporadic IBM (s-IBM). Here we present a larger series of 14 cases in which a combined muscle and nerve biopsy was available for additional electron microscopic investigation. In two of the new cases, the IBM had a hereditary background (h-IBM). The presence of neuropathy was confirmed in all 14 cases studied. Morphometry using an optic-electronic, digital evaluation system showed large variation of severity presumably due to age and coincidal factors such as diabetes mellitus or lymphoma. Ultrastructural analysis revealed a variety of changes considered to be non-specific. Signs of axonal damage predominated. In addition, there were numerous changes in Schwann cells and myelin sheaths. Neither inflammatory changes nor tubulofilamentous inclusions were detectable in the sural nerves. Peripheral neuropathy, although occasionally without apparent clinical manifestation, appears to be a common and aggravating feature in IBM; its pathogenesis, however, remains elusive.
Hermanns
B
B
Institut für Neuropathologie, Universitätsklinikum der Rheinisch-Westfälischen Technischen Hochschule Aachen, Pauwelsstrasse 30, D-52074, Aachen, Germany.
Molnar
M
M
Schröder
J M
JM
D000293:Adolescent; D000328:Adult; D000368:Aged; D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D018979:Myositis, Inclusion Body / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D009410:Nerve Degeneration / Q000473:pathology; D009413:Nerve Fibers, Myelinated / Q000473:pathology / Q000648:ultrastructure; D010375:Pedigree; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology / Q000648:ultrastructure