Lhermitte duclos disease: what to expect during surgery?

Abstract

Lhermitte and Duclos first described the dysplastic gangliocytoma of the cerebellum in 1920. In the last 100 years, its clinical presentation, radiological features, pathological characteristics, and association with Cowden syndrome have been well described. However, documentation of surgical experiences is lagging. We here describe intraoperative experience during the removal of the tumor, which could help the operating surgeon plan and mental makeup.

Lhermitte-Duclos disease: A rare case of cerebellar tumor with successful surgical treatment

“…It was first described by Lhermitte and Duclos in 1920, and since then, only a few hundred cases have been reported worldwide. [ 9 , 12 ] LDD is characterized by enlarged and disorganized cerebellar folia, which is composed of hypertrophic and dysplastic ganglion cells. [ 6 ] The disease can present with a wide range of neurological symptoms, including headache, dizziness, ataxia, and visual disturbances, and diagnosis is typically made through neuroimaging and histopathological examination.…”

“…However, the optimal surgical approach remains controversial, as complete resection is often difficult to achieve and may be associated with significant morbidity. [ 9 ] In this case report, we present the case of a 54-year-old male with LDD who presented with a range of neurological symptoms. Through this case, we aim to highlight the importance of surgery as a method of resolving the patient’s clinical condition in the treatment of LDD.…”