Lhermitte-Duclos Disease in a Sudden Death: An autopsy case

ABSTRACT

Lhermitte-Duclos disease of the cerebellum manifests itself as a slowly growing mass formed by abnormal neurons that appear more malformative than tumoral. Although most cases show satisfactory clinical evolution, several cases, not recognized in life, died abruptly, usually from respiratory arrest. We recently experienced a 19 year old male, who died suddenly during alcohol drinking. At autopsy, the cerebellum revealed thickening of the folia over two limited areas. Microscopically, the lesion was characterized by proliferation of abnormal neuronal cells with excessive myelination of their axons, disappearance of Purkinie cells and greatly reduced white mater. Theories regarding the pathogenesis of Lhermitte-Duclos disease were reviewed and discussed.