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Involvement of choroid plexus in mitochondrial encephalomyopathy (MELAS)

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Summary

Morphological study of the choroid plexuses of two patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) revealed an enormously increased number of mitochondria with structural abnormalities in almost all of the choroidal epithelial cells. The majority of the cells also showed loss of microvilli and collapsed or attenuated apical cytoplasmic processes with increased electron density and lysosome-like dense bodies. The blood vessels of the choroid plexus showed the features of mitochondrial angiopathy previously described in the pial arteries of the same patients. These findings are interpreted as the morphological expression of a primary biochemical defect of the mitochondrial function in the choroid plexus, and as the probable explanation for increased CSF lactate and pyruvate levels in this disease.

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Authors and Affiliations

  1. Department of Pathology, Brain Research Institute, Niigata University, 1 Asahimachi, 951, Niigata, Japan

    E. Ohama & F. Ikuta

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  1. E. Ohama
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  2. F. Ikuta
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Supported in part by grants from the Ministries of Education and Health and Welfare of Japan

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Ohama, E., Ikuta, F. Involvement of choroid plexus in mitochondrial encephalomyopathy (MELAS). Acta Neuropathol 75, 1–7 (1987). https://doi.org/10.1007/BF00686785

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  • DOI: https://doi.org/10.1007/BF00686785

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