[Parkinsonian hemi-syndrome as the initial manifestation of supratentorial cystic hemangioblastoma in a patient with Von Hippel-Lindau disease].

INTRODUCTION

Retino-cerebellar hemangioblastomatosis or Von Hippel-Lindau (VHL) disease is a phacomatosis with a dominant autosomal pattern of inheritance, which is characterized by the presence of hemangioblastomas of the central nervous system (cerebellum and spinal medulla), retinal angiomas and tumors (pheochromocytoma, clear cell carcinoma) or cysts of the abdominal viscera.

CLINICAL CASE: We present the case of a 22 year old female with Von Hippel-Lindau disease, in whom a cystic hemangioblastoma of the basal ganglia of the left hemisphere was diagnosed when she complained of difficulty in carrying out fine movements of the right hand and tremor for some months. The supratenorial site of cystic hemangioblastomas in the clinical context of Von Hippel-Lindau disease is very rare and clinical presentation of a parkinsonian hemisyndrome is exceptional. In our search through the literature we have found tumors with many types of histology (meningiomas, glial tumors, craniopharyngioma, epidermoid cysts) in the origin of tumoral parkinsonism.

CONCLUSIONS

However, we have found no previous case of cystic hemangioblastomas. We also emphasize that there was full resolution of the condition after total removal of the tumour.