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Late‐onset hereditary myopathy with abnormal mitochondria and progressive dementia
Author(s) -
Vilming S. T.,
Dietrichson P.,
Isachsen M. M.,
Løvvik L.,
Heiberg A.
Publication year - 1986
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1986.tb04594.x
Subject(s) - dementia , myopathy , mitochondrial myopathy , medicine , mitochondrion , pediatrics , pathology , disease , biology , genetics , mitochondrial dna , gene
A family with autosomal dominant late‐onset progressive dementia and myopathy is described. Electron microscopy of muscle revealed abnormal mitochondria in the proband. Thus, the disease may be classified as a “mitochondrial encephalomyopathy”. The cases are unique because dementia was a dominating feature and because the symptoms developed late in life. These cases may represent a new subgroup of the mitochondrial ence‐phalomyopathies.