Optic neuropathy in superficial intracranial siderosis.
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.
Department of Ophthalmology, Oxford Eye Hospital, Oxford, United Kingdom. sallylpainter@cantab.net
Sally L
SL
Painter
Liberty
L
Mathew
Gerardine
G
Quaghebeur
Margaret M
MM
Esiri
John S
JS
Elston
D000368:Aged; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009900:Optic Nerve / Q000473:pathology*; D009901:Optic Nerve Diseases / Q000209:etiology* / Q000473:pathology*; D012806:Siderosis / Q000150:complications* / Q000473:pathology*; D013345:Subarachnoid Hemorrhage / Q000150:complications / Q000473:pathology