Lhermitte-Duclos disease, H&E stained section

Last updated on Thursday, April 9 2009 by gliageek

Lhermitte-Duclos disease, H&E stained section

Lhermitte-Duclos diseases characterized by absence of recognizable Purkinje cells accompanied by enlargement of internal granular cell neurons separated by increased neuropil. The cerebellar molecular layer is often markedly thickened, with an abundance of radial processes consistent with granular cell overgrowth. Also known as dysplastic gangliocytoma of the cerebellum, this malformed lesion is part of Cowden disease, which may include multiple benign tumors of hair follicles (which may be quite subtle), breast cancer, and thyroid cancer. Therefore, screening of first degree relatives for this PTEN-related hamartoma syndrome should be done in all patients with Lhermitte-Duclos diseas.

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