A 66-year-old woman presented with a recurrent psychotic disorder with central manifestations that initially suggested a delusional disorder. Peripheral stiffness was at first attributed to a dystonic reaction secondary to neuroleptic treatment. The atypical presentation led to suspicion of collagen vascular disease. Both the psychotic disorder and peripheral involvement responded to cortisone. Findings are discussed in light of recent information on the pathogenesis and neuropsychiatric manifestations of systemic lupus erythematosus, progressive systemic scleroderma and limited scleroderma variants (CREST, unclassifiable connective tissue disease) with positive anticentromere titers, including a discussion of the vasculitis hypothesis of collagen vascular cerebral involvement.
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