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Lhermitte-Duclos syndrome

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A rare pathologic entity with progrediating, diffuse hypertrophy chiefly of the stratum granulosum of the cerebellum. The main clinical signs are headache, movement disorders and tremor, visual disturbances, enlarged head suggesting hydrocephalus, and abnormal EEG. Autosomal dominant inheritance demonstrated in some families. Authors disagree as to whether this represents a neoplastic, malformative or hamartomatous lesion.


  • J. Lhermitte, P. Duclos:
    Sur un ganglioneurome diffuse du cortex du cervelet.
    Bulletin de l'Association francaise pour l'etude du cancer, Paris, 1920, 9: 99-107.
  • M. Ambler, et al:
    Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum). Pathological analysis of the first familial cases.
    Journal of Neuropathology and Experimental Neurology, Lawrence, Kansas, 1969, 28: 622-647.

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