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...
| Main Authors: | , , , , |
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| Format: | Journal article |
| Language: | English |
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2010
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| _version_ | 1797090513751375872 |
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| author | Painter, S Mathew, L Quaghebeur, G Esiri, M Elston, J |
| author_facet | Painter, S Mathew, L Quaghebeur, G Esiri, M Elston, J |
| author_sort | Painter, S |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T03:19:42Z |
| format | Journal article |
| id | oxford-uuid:b7028b93-0d03-49f7-9634-9fc88f904649 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T03:19:42Z |
| publishDate | 2010 |
| record_format | dspace |
| spelling | oxford-uuid:b7028b93-0d03-49f7-9634-9fc88f9046492022-03-27T04:45:17ZOptic neuropathy in superficial intracranial siderosis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:b7028b93-0d03-49f7-9634-9fc88f904649EnglishSymplectic Elements at Oxford2010Painter, SMathew, LQuaghebeur, GEsiri, MElston, JSuperficial 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. |
| spellingShingle | Painter, S Mathew, L Quaghebeur, G Esiri, M Elston, J Optic neuropathy in superficial intracranial siderosis. |
| title | Optic neuropathy in superficial intracranial siderosis. |
| title_full | Optic neuropathy in superficial intracranial siderosis. |
| title_fullStr | Optic neuropathy in superficial intracranial siderosis. |
| title_full_unstemmed | Optic neuropathy in superficial intracranial siderosis. |
| title_short | Optic neuropathy in superficial intracranial siderosis. |
| title_sort | optic neuropathy in superficial intracranial siderosis |
| work_keys_str_mv | AT painters opticneuropathyinsuperficialintracranialsiderosis AT mathewl opticneuropathyinsuperficialintracranialsiderosis AT quaghebeurg opticneuropathyinsuperficialintracranialsiderosis AT esirim opticneuropathyinsuperficialintracranialsiderosis AT elstonj opticneuropathyinsuperficialintracranialsiderosis |