Late-onset hereditary myopathy with abnormal mitochondria and progressive dementia |
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| Authors: | S T Vilming P Dietrichson M M Isachsen L Løvvik A Heiberg |
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| Institution: | Departments of Neurology, Oslo, Norway;Pathology, Ullevaal Hospital, University of Oslo, Norway;Department of Medical Genetics, Oslo, Norway |
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| Abstract: | 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 encephalomyopathies. |
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| Keywords: | dementia hereditary mitochondrial encephalomyopathy |
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