Immunohistochemistry and proliferative activity in Lhermitte-Duclos disease

We have evaluated a recurrence of Lhermitte-Duclos disease by immunohistochemistry for Purkinje cell markers and proliferative activity (proliferating cell nuclear antigen), by electron microscopy and for DNA ploidy (image analysis). While most of the abnormal neurons in the lesion appear to be derived from granule cells, several Purkinje cell specific polyclonal and monoclonal antibodies, including L7, PEP 19 and calbindin, labeled a minor subpopulation. Staining with monoclonal antibodies to proliferating cell nuclear antigen and measuring cell DNA index and ploidy with a cell image analyzer revealed no proliferative activity. Electron microscopy findings were similar to those previously reported. In spite of its recurrence, our findings support the notion that Lhermitte-Duclos disease is malformative, not neoplastic, and that the characteristic neurons are derived predominantly but not exclusively from a non-Purkinje cell source, probably the granule cell.