title: Lhermitte-Duclos disease. An immunohistochemical study of the cerebellar cortex.
abstract: Immunocytochemical studies were carried out on two previously reported autopsy cases of Lhermitte-Duclos disease. The unaffected cerebellar cortex adjacent to the lesions served as control. The findings supported the view, previously expressed by one of the authors, of a heterogeneous neuronal structure of the lesion, consisting of at least two cell types. No further light was thrown on the predominant medium-sized cells, believed to represent hypertrophic internal granular neurons. On the other hand the large cells shared a number of features with Purkinje cells. In particular they were recognized by the pan-T-cell antibody anti-Leu-4, were surrounded by axosomatic synapses visualized by the antisynaptic vesicle glycoprotein antibody SV2, and contained both non-phosphorylated and phosphorylated neurofilament epitopes. It is suggested that these cells represent dysplastic Purkinje cells. The lesion therefore appears to be a complex hamartoma rather than a simple hypertrophy of the internal granular neurons.
authors.affiliation: Department of Pathology, Veterans Administration Medical Center, Palo Alto, CA 94304.
authors.forename: R A
authors.initials: RA
authors.lastname: Shiurba
authors.forename: E C
authors.initials: EC
authors.lastname: Gessaga
authors.forename: L F
authors.initials: LF
authors.lastname: Eng
authors.forename: L A
authors.initials: LA
authors.lastname: Sternberger
authors.forename: N H
authors.initials: NH
authors.lastname: Sternberger
authors.forename: H
authors.initials: H
authors.lastname: Urich
mesh_terms: D000911:Antibodies, Monoclonal; D002525:Cerebellar Cortex / Q000378:metabolism* / Q000473:pathology; D002528:Cerebellar Neoplasms / Q000378:metabolism* / Q000473:pathology; D003598:Cytoskeletal Proteins / Q000378:metabolism; D006222:Hamartoma / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D006984:Hypertrophy; D007124:Immunoenzyme Techniques; D007382:Intermediate Filaments / Q000378:metabolism / Q000473:pathology; D008870:Microtubules / Q000378:metabolism / Q000473:pathology; D013572:Synaptic Vesicles / Q000378:metabolism / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D003598:Cytoskeletal Proteins