[P2-171] Frequent vomiting attacks in a patient with Lhermitte-Duclos disease: a neuronal connection-mediated pathophysiology?
Lhermitte-Duclos disease (LDD) is a neurological disease caused by a hamartomatous lesion in the cerebellum. Clinically, LDD is commonly associated with progressive space-occupying lesion (SOL)-effect in the posterior fossa; increasing intracranial pressure, occlusive hydrocephalus and focal neurological deficits of adjacent structures. In this article, we report a 10-year-old boy with LDD who had been suffering from frequent vomiting attacks (VAs). His VAs had been brief in duration but frequent, and resistant to antiemetic drugs since the early postnatal period. Magnetic resonance imaging (MRI) revealed the right cerebellar lesion with very little SOL-effect but its causal relationship with VAs was not evident at that point. He developed convulsive seizures at age of nine, and was referred to our epilepsy center. Initially, VAs were possibly considered as a part of seizures, but antiepileptic drugs were ineffective despite of remission of convulsive seizures. Video-EEG monitoring did not show any evolving ictal patterns associated with VAs. MRI detected no interval changes but Ictal Fluorodeoxyglucose-positron emission tomography (FDG-PET) identified hypermetabolism of the cerebellar lesion. There were no clinical symptoms and signs suggesting increasing intracranial pressure except vomiting. We speculated the causal relationship between the cerebellar lesion and VAs, and performed partial resection of the lesion. Postoperatively, VAs has been completely suppressed for two years. We hypothesize the pathophysiology of VAs in LDD include not only tumor-like space occupying effect but also neuronal connection mediated mechanisms of the central vomiting system.