We report the characteristics of three patients with spinocerebellar degeneration (SCD) with negative-type electroretinograms (ERGs). None of the patients showed retinal degeneration, but all had severe cerebellar ataxia, and brain MRIs showed cerebellar atrophy. Negative-type ERGs have been implicated in the selective functional impairment of the inner retinal layer, but few studies have reported dysfunction of the inner nuclear layer in SCD patients.
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