Neurosarcoidosis causing ventricular loculation, hydrocephalus, and death

Ten years after a diagnosis of sarcoidosis, a 33-year-old woman presented with a severe headache of 5 days' duration. Neuroradiologic evaluation revealed a large cystic lesion of the left temporal lobe, causing a mass effect. An exploratory operation proved the lesion to be a loculated portion of the temporal horn of the lateral ventricle. Drainage of the loculated ventricle relieved the patient's cephalgia. Within 2 months, however, pain in the head recurred and an unsteady, broad-based gait appeared. Reevaluation disclosed hydrocephalus for which a ventriculoperitoneal shunt was inserted. After this procedure, the patient did well neurologically for 1 year, after which seizures, personality changes, incontinence, and disturbance of gait developed. Death occurred after revision of the shunt, and widespread granulomatous disease was found at autopsy. Neurosarcoidosis, with emphasis on intracranial mass lesions in sarcoidosis, is discussed; the role of surgical treatment in some of these lesions, and in hydrocephalus, is stressed.