BASAL GANGLIONIC ANGIOLEIOMYOMA

A rare case of basal ganglionic angioleiomyoma in a 12-year-old girl i s reported. She presented with features of raised intracranial tension , multifocal seizures, left hemidystonia and apraxia of eyelid closure . Cranial CT scan showed a large hypodense lesion with an enhancing mu ral nodule in the region of head of the caudate nucleus on the right s ide. The lesion was extending to the posterior limb of internal capsul e postero-medially and to globus pallidus laterally. Another small, hy perdense, enhancing lesion was seen in the region of globus pallidus o n the left side. The right basal ganglionic mass was excised through f rontal craniotomy. Histopathological, Immunocytochemical and Ultrastru ctural examination of the right basal ganglionic mass showed features characteristic of angioleiomyoma. Following surgery the patient improv ed initially with respect to dystonia but worsened later on. Repeat cr anial CT, 20 months after surgery, showed bilateral frontotemporal atr ophy without any tumor recurrence while the lesion on the left side re mained quiescent.