PARKINSONIAN-SYNDROME COMPLICATING SYSTEMIC LUPUS-ERYTHEMATOSUS

Two girls with florid extrapyramidal parkinsonism complicating systemi c lupus erythematosus (SLE) are reported. One patient (15 years old) p resented with extreme rigidity, irritability, and mutism initially dia gnosed as acute psychosis. Examination revealed severe extrapyramidal akinetic mutism, along with marked restlessness. CT and MRI imaging of the brain were unremarkable, EEG revealed moderate generalized distur bance of background activity. Tc-99m-HmPAO SPECT cerebral scanning det ected decreased regional cerebral blood flow at the basal ganglia, Dop amine-agonist drugs led to complete recovery after 3 months, along wit h normalization of EEG and SPECT alterations. The second patient (16 y ears old) was assessed for progressive bradykinesia and apathy impedin g her active daily activities, and she was suspected to have developed depression. Neurologic assessment revealed a parkinsonian syndrome th at was less severe than that of the first patient. The EEG showed mild disturbance of background activity, and Tc-99m-HmPAO SPECT demonstrat ed impaired regional cerebral blood flow over the basal ganglia. A par kinsonian extrapyramidal syndrome complicating SLE should therefore be taken into account in any patient with SLE presenting with marked beh avioral alterations, rigidity, or akinetic mutism, (C) 1998 by Elsevie r Science Inc. All rights reserved.