DEMENTIA AS A MANIFESTATION OF NEUROLUPUS - 2 CASES

Manifestations of dementia occurred in two young patients with dissemi nated lupus erythematosus who had no signs of focalized neurological d eficit. In case I followed for 8 years, a probably autonomous and fami lial psychiatric syndrome was complicated during a lupus flare-up by a prolonged dementia syndrome which regressed to a large extent. In the second patient with moderate dementia and familial hearing loss. seve re mental deterioration suddenly occurred with long-term degradation o f the cognitive capacity. In both of these cases with neurolupus, the frontal clinical signs, the neuropsychological deficits evidenced at t esting, and the lesions demonstrated at CT scan and magnetic resonance imaging favored a frontal and/or frontobasal type dementia. Treatment with cyclophosphamide was effective in case I after failure of cortic osteroid therapy.