Subdural effusion with hydrocephalus in dentatorubral-pallidoluysian atrophy: A case report.

A 56-year-old woman with dentatorubral-pallidoluysian atrophy (DRPLA) was admitted with progressive disturbance of consciousness. Computed tomography of the head revealed bilateral subdural effusions compressing the cerebral hemispheres. The effusions enlarged despite conservative management, so burr-hole drainage was performed. Unexpectedly, marked hydrocephalus developed postoperatively, resulting in a poor neurological outcome. Subdural effusion with hydrocephalus (SDEH) is a condition that usually occurs secondary to severe head injury or neurosurgical procedures, but reports of its occurrence in association with neurodegenerative disorders are scarce. We speculate that repeated head injuries and periventricular parenchyma damage associated with DRPLA contributed to the development of SDEH. This case highlights the need for awareness of SDEH as a potential complication in neurodegenerative disorders such as DRPLA.