Optic Neuropathy in Superficial Intracranial Siderosis

Optic Neuropathy in Superficial Intracranial Siderosis Sally L. Painter, MB, BChir, MA, Liberty Mathew, MB, ChB, MRCP, Gerardine Quaghebeur, FRCR, Margaret M. Esiri, DM, FRCPath, John S. Elston, MD, FRCOphth Superficial intracranial siderosis is a degenera- tive condition secondary to recurrent occult subarachnoid hemorrhage. Progressive sensorineural deafness, cere- bellar ataxia, and pyramidal signs are well-documented clinical manifestations, but optic neuropathy is not a recognized feature. We describe 2 patients with clinical and electrophysiological evidence of optic nerve/chiasm dysfunction and MRI signal abnormalities consistent with hemosiderin staining of the anterior visual pathway. In a third case, neuropathological examination of the optic chiasm showed demyelination attributed to hemosiderin deposition. We suggest that anterior visual pathway damage may be underrecognized in this condition.

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