Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy

Sagun Tuli, John P. Provias and Mark Bernstein

Abstract: Background: Lhermitte-Duclos disease (LDD) is a rare pathologic entity involving the cerebellum. The fundamental nature of the entity and its pathogenesis remain unknown, and considerable debate has centered on whether it represents a neoplastic, malformative or hamartomatous lesion. The cell or cells of origin remain incompletely defined. Previous reports of cases in the English literature have dealt predominantly with the clinical and pathological aspects yet few address issues of treatment. Methods: A case of Lhermitte-Duclos disease (LDD) in a 54-year-old female leading to local compressive symptoms and obstructive hydrocephalus is presented. A craniectomy, in addition to a C1 laminectomy followed by a decompressive duroplasty (using autologous fascia lata graft) was performed. Results: The patient clinically improved and follow-up MRI 11 months post-operatively revealed improvement in hydrocephalus. Conclusion: The histological and immunohistochemical features of the lesion are described, emphasizing the role of an abnormal dysplastic granule cell layer. The evidence in favor of each of the major theories of pathogenesis, malformative and neoplastic is discussed. Based on these facts a form of surgical intervention involving decompressive duroplasty is proposed.

Can. J. Neurol. Sci. 1997; 24: 155-160