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A negative electroretinogram (ERG) in a case of probable multiple system atrophy (MSA).
Barnes, Claire S; Yan, Jiong; Wilmot, George R.
Afiliación
  • Barnes CS; Department of Ophthalmology, Emory University, 1365B Clifton Rd NE, Atlanta, GA 30322, USA. cbarne3@emory.edu
Doc Ophthalmol ; 118(3): 247-56, 2009 Jun.
Article en En | MEDLINE | ID: mdl-19023607
Recent articles have described negative ERGs in a small number of patients with cerebellar degeneration. Five of the previously reported seven cases were hereditary (2/5 had spinocerebellar ataxia-1 (SCA-1) gene mutations) and the other two were sporadic. We report a negative ERG in a case of cerebellar degeneration that differs significantly from earlier cases. The 65-year-old man had a 5-year history of ataxia, unsteady gait, orthostatic hypotension, and bladder and erectile dysfunction, with no family history of neurological or retinal disease. Visual acuity was 20/30 OD, 20/40 OS, but reportedly was never 20/20. His fundus exam showed optic nerve pallor, but otherwise was normal. Visual fields had enlarged blind spots but no central scotomas. Autofluorescence was normal. Photopic flash and 30-Hz ERG responses were normal. Rod b-waves were reduced and delayed. Standard flash a-waves were normal, but the b-waves were smaller than the a-waves. Blood tests were negative for Leber's hereditary optic neuropathy, dominant optic atrophy, and for expansions in SCA genes including SCA-1. This is only the third reported case of sporadic ataxia with a negative ERG. The patient's prominent autonomic dysfunction differs from the previous cases, and meets the clinical criteria for probable multiple system atrophy (MSA). This introduces another possible diagnosis in cases of negative ERGs with ataxia, and suggests that the visual system may be affected in MSA.
Asunto(s)

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Atrofia de Múltiples Sistemas / Electrorretinografía Tipo de estudio: Diagnostic_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Doc Ophthalmol Año: 2009 Tipo del documento: Article País de afiliación: Estados Unidos

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Atrofia de Múltiples Sistemas / Electrorretinografía Tipo de estudio: Diagnostic_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Doc Ophthalmol Año: 2009 Tipo del documento: Article País de afiliación: Estados Unidos