Open AccessAn Autopsy Case of Mitochondrial Encephalomyopathy (MELAS) with SpeciaI Reference to Extra‐neuromuscular AbnormaIities
Author(s) -
Ban Shinichi,
Mori Naoyoshi,
Saito Kiyoshi,
Mizukami Katsuyoshi,
Suzuki Toshihito,
Shiraishi Hiroyasu
Publication year - 1992
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1992.tb01883.x
Subject(s) - mitochondrial encephalomyopathy , melas syndrome , mitochondrial myopathy , medicine , pathology , autopsy , mitochondrial disease , neuroscience , mitochondrial dna , biology , genetics , gene
An autopsy case of a 37 year old man with mitochondrial encephalomyopathy is reported. Ragged red fibers and crystalline inclusions in mitochondria were revealed by biopsy of the striated muscle of the patient. Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke‐like episodes (MELAS) was diagnosed clinically. In addition to severe atrophy and degeneration of the generalized striated muscles and many foci of laminar necrosis of the cerebral cortex, the following abnormalities were observed: 1) hypertrophy of the myocardium, 2) fatty change of the liver, 3) focal sclerosis of the glomeruli and dilatation of the tubules of the kidneys, 4) hyalinous degeneration of the Langerhans' islands of the pancreas and 5) wavy change of the smooth muscle fibers of the muscularis propria of the gastrointestinal tract. We suggest that mitochondrial encephalomyopathy affects various organs and tissues, among which susceptibility of the muscular tissues —skeletal muscle, myocardium and smooth muscle—is high. Acta Pathol Jpn 42: 818–825, 1992.