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September 1971

Disseminated Lupus Erythematosus Presenting as Chorea Gravidarum

Author Affiliations

Christchurch, New Zealand
From the Medical Unit, the Princess Margaret Hospital, Christchurch, New Zealand.

Arch Neurol. 1971;25(3):240-244. doi:10.1001/archneur.1971.00490030066007

Chorea gravidarum occurred due to disseminated lupus erythematosus. This diagnosis should be suspected, especially in women with chorea in the second or third decade, in whom there is not clear evidence of rheumatic fever. There is frequently no other clinical evidence of lupus for several years. Although the duration of choreiform attacks is similar to that of rheumatic fever, the tendency for recurrence is probably less. There is a high incidence of clinical involvement of other parts of the nervous system at some time during the disease. Results of cerebrospinal fluid examination are most frequently normal. The typical autopsy finding is widespread involvement of the small vessels of the cerebral cortex with fibrinoid necrosis and adjacent microinfarcts, but without demonstrable lesions in the basal ganglia. There has been at least a 20% mortality at six months.