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Cerebellar Ataxia With Extreme Photophobia Associated With Anti-SOX1 Antibodies

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dc.contributor.author Alessandro, Lucas
dc.contributor.author Schachter, Daniel
dc.contributor.author Farez, Mauricio Franco
dc.contributor.author Varela, Francisco José
dc.date.accessioned 2020-02-04T13:45:44Z
dc.date.available 2020-02-04T13:45:44Z
dc.date.issued 2019-07
dc.identifier.citation Alessandro, L., Schachter, D., Farez, M.F., Varela, F. Cerebellar Ataxia With Extreme Photophobia Associated With Anti-SOX1 Antibodies. Neurohospitalist. 2019;9(3):165–168. doi:10.1177/1941874418802130. en_US
dc.identifier.uri https://doi.org/10.1177/1941874418802130
dc.identifier.uri https://repositorio.fleni.org.ar/handle/123456789/172
dc.description.abstract Anti-SOX1 antibodies are associated with diverse neurological syndromes, targeting both the central (paraneoplastic cerebellar degeneration) and peripheral nervous systems (Lambert Eaton myasthenic syndrome, paraneoplastic neuropathy). Although the pathogenic role of these antibodies remains unclear, their strong association with underlying neoplastic disease (mainly small-cell lung cancer) has designated them as onconeural antibodies. Here, we present a case of cerebellar ataxia with marked photophobia, with severe atrophy of the cerebellum and brain stem, associated with anti-SOX1 antibodies without evidence of an underlying malignancy. Although anti-SOX1-associated cerebellar syndrome is infrequent, investigation of these antibodies should be considered as a part of the diagnostic algorithm if more common causes have been ruled out. Extensive brain stem lesions causing disruption of the trigeminal pathway and its connections with the pretectal area might explain the underlying mechanism of the associated photophobia. Early recognition of anti-SOX1 antibodies, exclusion of underlying neoplasm, and prompt initiation of immunotherapy are essential to achieve a better outcome. en_US
dc.language.iso eng en_US
dc.publisher Sage en_US
dc.rights info:eu-repo/semantics/openAccess
dc.rights.uri https://creativecommons.org/licenses/by/2.5/ar/
dc.subject SOX1 protein en_US
dc.subject Proteína SOX1 en_US
dc.subject Cerebellar Ataxia en_US
dc.subject Ataxia Cerebelosa en_US
dc.subject Paraneoplastic Cerebellar Degeneration en_US
dc.subject Degeneración Cerebelosa Paraneoplásica en_US
dc.title Cerebellar Ataxia With Extreme Photophobia Associated With Anti-SOX1 Antibodies en_US
dc.type info:eu-repo/semantics/publishedVersion
dc.type info:eu-repo/semantics/article en_US
dc.description.fil Fil: Alessandro, Lucas. Fleni. Departamento de Neurología; Argentina.
dc.description.fil Fil: Schachter, Daniel. Rush University Medical Center. Neurology Department; Estados Unidos.
dc.description.fil Fil: Farez, Mauricio Franco. Fleni. Centro para la Investigación de Enfermedades Neuroinmunológicas; Argentina.
dc.description.fil Fil: Varela, Francisco José. Fleni. Departamento de Neurología; Argentina.
dc.relation.ispartofVOLUME 9
dc.relation.ispartofNUMBER 3
dc.relation.ispartofPAGINATION 165-168
dc.relation.ispartofCOUNTRY Estados Unidos
dc.relation.ispartofCITY Thousand Oaks
dc.relation.ispartofTITLE The Neurohospitalist
dc.relation.ispartofISSN 1941-8752
dc.type.snrd info:ar-repo/semantics/artículo es_ES


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