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Superficial intracranial siderosis is a degenerative condition secondary to recurrent occult subarachnoid hemorrhage. Progressive sensorineural deafness, cerebellar 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.

Original publication

DOI

10.1097/WNO.0b013e3181e92c80

Type

Journal article

Journal

J Neuroophthalmol

Publication Date

12/2010

Volume

30

Pages

311 - 314

Keywords

Aged, Child, Female, Humans, Male, Middle Aged, Optic Nerve, Optic Nerve Diseases, Siderosis, Subarachnoid Hemorrhage