I. History a. Symptomatic onset under 30 years of age.
b. Family history of a similar disorder.
c. Systemic diseases or other identifiable causes for features listed in Table 7.
d. Hallucinations unrelated to medication.
II. Physical examination a. DSM criteria for dementia.
b. Prominent slowing of vertical saccades or vertical supranuclear gaze plasy.a c. Evidence of focal cortical dysfunction such as aphasia, alien limb syndrome, and parietal dysfunction. III. Laboratory investigation
Metabolic, molecular, genetic, and imaging evidence of an alternative cause of feature listed in Table 7.
aIn practice, multiple-system atrophy is most frequently confused with Parkinson's disease or progressive supranuclear palsy (PSP). Mild limitation of upward gaze alone is nonspecific, whereas a prominent (>50%) limitation of upward gaze of any limitation of downward gaze suggests PSP. Before the onset of vertical gaze limitation, a clinically obvious slowing of voluntary vertical saccades is usually easily detectable in PSP and assists in the early differentiation of these two disorders.
DSM, Diagnostic and Statistical Manual of Psychiatric Disorders.
(Reprinted with permission from Gilman S, Low PA, Quinn N, et al. Consesus statement on the diagnosis of multiple system atrophy. J Neurol Sci 1999;163:94-98, and Elsevier.)
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