LHERMITTE-DUCLOS-DISEASE - LITERATURE-REVIEW AND NOVEL TREATMENT STRATEGY

Background: Lhermitte-Duclos disease (LDD) is a rare pathologic entity involving the cerebellum. The fundamental nature of the entity and it s pathogenesis remain unknown, and considerable debate has centered on whether it represents a neoplastic, malformative or hamartomatous les ion. The cell or cells of origin remain incompletely defined. Previous reports of cases in the English literature have dealt predominantly w ith the clinical and pathological aspects yet few address issues of tr eatment. Methods: A case of Lhermitte-Duclos disease (LDD) in a 54-yea r-old female leading to local compressive symptoms and obstructive hyd rocephalus is presented. A craniectomy, in addition to a C1 laminectom y followed by a decompressive duroplasty (using autologous fascia lata graft) was performed. Results: The patient clinically improved and fo llow-up MRI 11 months post-operatively revealed improvement in hydroce phalus. Conclusion: The histological and immunohistochemical features of the lesion are described, emphasizing the role of an abnormal dyspl astic granule cell layer. The evidence in favor of each of the major t heories of pathogenesis, malformative and neoplastic is discussed. Bas ed on these facts a form of surgical intervention involving decompress ive duroplasty is proposed.