Idiopathic superficial siderosis: a case report.

ABSTRACT

Superficial siderosis of the central nervous system (SSCN) is a very rare disorder. The clinical syndrome of SSNC consists of sensorineural hearing loss, cerebellar ataxia and myelopathy. The clinical syndrome together with the typical appearance on magnetic resonance imaging (MRI) of hyposignal intensity along the leptomeninges in T2 sequence permit the diagnosis of SSCN. A 58 year-old man who has a history of chronic progressive hearing loss and gait instability for 5 years is presented. The neurological examination revealed bilateral sensorineural hearing loss, cerebellar ataxia and mild spasticity of the lower extremities. MRI showed classical superficial siderosis in the form of hyposignal intensity along the leptomeninges in T2 sequence. The prominent sites of hemosiderin deposition in this case were cerebellar vermis, trigeminal nerves, vestibulocochlear nerves, around the brain stem and spinal cord surface. Cerebrospinal fluid findings confirmed chronic subarachnoid hemorrhage but bleeding site could not be demonstrated. There is no specific treatment available for idiopathic SSCN.