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01-06-2009 | Case Report

A negative electroretinogram (ERG) in a case of probable multiple system atrophy (MSA)

Authors: Claire S. Barnes, Jiong Yan, George R. Wilmot

Published in: Documenta Ophthalmologica | Issue 3/2009

Abstract

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.

This flash luminance for the bright flashes in the dark and the photopic flashes and flicker fell well within the ‘standard flash’ range recommended in the 2004 version of the ISCEV ERG Standard [25], but is lower than the recommended ‘Scotopic 3.0 ERG’ or ‘Photopic 3.0 ERG’ luminance in the 2008 Standard [26]. The dim white flashes correspond to the ‘Scotopic 0.01 ERG’ luminance in the 2008 Standard.

Consistent with earlier reports [e.g., 28, 29], the responses from the two oldest control subjects (84 and 86 years of age) tended to show smaller amplitudes and longer implicit times than the responses from the other controls (ages 55 and under). Comparison of the patient’s results with the limits from only the 11 younger subjects showed the identical pattern of results, except that the SF a-wave implicit time recorded from his right eye was then slightly longer than normal (17.0 ms vs. 16.7 ms). Therefore the patient’s results overall were not just determined to be ‘normal’ or ‘abnormal’ due to the inclusion of the oldest control subjects.

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Title: A negative electroretinogram (ERG) in a case of probable multiple system atrophy (MSA)
Authors: Claire S. Barnes
Jiong Yan
George R. Wilmot
Publication date: 01-06-2009
Publisher: Springer-Verlag
Published in: Documenta Ophthalmologica / Issue 3/2009
Print ISSN: 0012-4486
Electronic ISSN: 1573-2622
DOI: https://doi.org/10.1007/s10633-008-9156-3

At a glance: The STEP trials

Springer Medicine

A negative electroretinogram (ERG) in a case of probable multiple system atrophy (MSA)

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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