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In summary, this report is the first to present a RS patient who was diagnosed beyond age forty and has documented normal intelligence as an adult. Consistent with previous findings of grossly intact cognition on the basis of neurological examination in a minority of RS patients, the generally intact performance by this patient on neuropsychological tests provides further evidence that the disorder is not invariably associated with widespread cognitive impairment (Toelle et al., 2002) and can remain undiagnosed well into adulthood.