To determine whether the cognitive status of patients with dominantly inherited spinocerebellar ataxia (DSCA) might be related to neurologic severity, we administered a comprehensive neuropsychological test battery to 43 patients with DSCA, ranging in ataxia severity from mild to end-stage. As compared with the controls, the mildly ataxic patients scored normally or close to normal as a group on all of the neuropsychological tests. In contrast, approximately one-half of the moderately and all of the severely ataxic patients showed poor performance, independent of age, Hamilton Rating Scale for Depression score, or education, on the Wisconsin Card Sorting Test, suggesting impaired executive system function. In addition, a subgroup of these patients had a neuropsychological profile suggestive of mild generalized cognitive impairment. We conclude that DSCA is not a homogeneous group of disorders with respect to cognitive status and that the neurologic severity of the disorder is a major factor. Impaired executive system function could be explained by damage to olivopontocerebellar system control over cerebral cortical function or to damage to other neuronal systems (especially cholinergic) that degenerate in parallel with the olivopontocerebellar system.

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In acute multiple sclerosis, cranial computerized tomography (CT) may show periventricular and deep white matter contrast-enhanced lesions that are easier to see using 8 mm rather than 13 mm cranial CT sections. Follow-up studies show that the lesions evolve either to areas of density similar to the surrounding white matter or to low-density lesions. We presume they represent foci of active demyelination with extravasation of iodine through an altered blood-brain barrier. Enhanced cranial CT studies may be helpful in diagnosing acute multiple sclerosis and in following the course of the white matter lesions.