Magnetic Resonance Imaging in the Diagnosis and Treatment of Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum)

Abstract

ABSTRACT Lhermitte-Duclos disease is a rare disorder characterized by a slowly enlarging mass lesion in the cerebellum. The pathophysiology of the disease is poorly understood, but recent reports have suggested that a clinically significant mass may recur many years after total gross removal of the tumor. Computed tomography and angiography have been relatively insensitive in imaging the tumor either at presentation or during the postoperative period. The authors present three patients with histologically proven Lhermitte-Duclos disease who have undergone magnetic resonance imaging (MRI). In the first case, preoperative T1- and T2-weighted images clearly demonstrated the location and extent of the lesion, which was verified at operation. In the other two cases, only postoperative images were obtained. These demonstrated the extent of residual tumor and indicate a high probability of lesion expansion during periods of 4 and 10 years, respectively. We conclude that MRI is an important tool in both the diagnosis and the treatment of patients with Lhermitte-Duclos disease.