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Progressive cerebral poliodystrophy – Alpers' disease
  1. U. Sandbank,
  2. P. Lerman
  1. J. Casper Institute of Pathology, Laboratory of Neuropathology, Israel
  2. The EEG Laboratory, Beilinson Hospital, Israel
  3. Department of Pediatrics, Beilinson Hospital, Israel
  4. The Aviv University Medical School, Tel Aviv, Israel

    Abstract

    Three siblings who suffered from progressive mental retardation, seizures, and rigidity showed degeneration of the cerebral cortex. This was manifested by severe to complete neuronal loss with astrogliosis and microgliosis. In one child a brain biopsy was performed at the age of 3 months. The only lesion found was large disorganized perinuclear mitochondria in the neurones. The possibility that the cerebral poliodystrophy is due to an inherited mitochondrial disorder is discussed.

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    Disorganized giant neuronal mitochondria on electron microscopy

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