MR imaging of Lhermitte-Duclos disease: a case report.

Abstract

margins. Mass effect on the brainstem was clearly shown, as were the effects of the posterior fossa. Hydrocephalus was again noted. On the basis of the imaging studies a diagnosis of Lhermitte-Ouclos disease was postulated, although an atypical appearing glioma could not be entirely excluded. The patient underwent surgery for decompression and partial resection . The lesion at surgery appeared as a swoll en mass with associated flattened gyri and compressed su lci. The mass was diffi­ cult to distinguish from adjacent and deep normal cerebellum . This relatively hypovascular lesion had a poor cleavage plane, and it was resected in fragments up to a total of 3 x 3 cm. Pathology demonstrated a thickened molecular layer with hyper­ myelination, an abnormal granular layer, and an absence of Purkinje cells consistent with Lhermitte-Ouclos disease. The patient did well postoperatively with resolution of the head­ aches and disequilibrium; however, the left visual findings remain relatively unchanged and are probably secondary to a postcompres­ sive optic neuropathy.