There have been 87 cases of superficial siderosis of the CNS reported in the world literature
and 63 cases had developed the clinical syndrome with sufficient details to be reviewed. It is
a distinct clinical syndrome characterized by sensorineural deafness (95%), cerebellar
ataxia (88%) and pyramidal signs (76%). Other features include dementia (24%), bladder
disturbance (24%), anosmia (at least 17%), aniscoria (at least 10%) and sensory signs
(13%). Less frequent features are extra-ocular motor palsies, neck or backache, bilateral …

Objective Superficial siderosis of the central nervous system is a rare intractable disease
induced by chronic subarachnoid hemorrhage. Neurological deficits, such as cerebellar
ataxia and hearing difficulties, gradually progress if left undiagnosed. Hemosiderin
deposition is irreversible because standard medical treatment has not yet been established.
Interventions at the source of bleeding may be the key to a preferable outcome of treatment
for chronic subarachnoid hemorrhage; however, the source is not clear in many cases …