Neuropsychiatric systemic lupus erythematosus: a diagnostic challenge.
A 58-year-old woman presented to neuropsychiatric services with increased frequency of confusional episodes and intermittent psychotic symptoms. She had a 19-year history of atypical epileptic seizures and cognitive decline. Detailed review of history and clinical investigations revealed that she had accumulated sufficient features to meet diagnostic criteria for systemic lupus erythematosus (SLE). She had previously had lymphopenia and a malar rash; she had positive antinuclear, anti-Ro (anti-Sjögren's-syndrome-related antigen A) and anti-SM (anti-Smith Antibody) antibodies, and elevated erythrocyte sedimentation rate. The seizures, cognitive impairment and psychosis were attributable to neuropsychiatric SLE. Treatment with immune-modulating therapy, cyclophosphamide, resulted in significant improvement in subjective and objective clinical presentation. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures, psychotic symptoms or cognitive decline. A detailed clinical evaluation with review of the medical history and appropriate laboratory analyses allows this diagnosis to be made, and appropriate treatment to be initiated.
Division of Psychiatry, University College London, London, UK.
Andrew
A
Sommerlad
Department of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.
John
J
Duncan
National Hospital for Neurology and Neurosurgery, London, UK.
Michael P T
MP
Lunn
Department of Neuropsychiatry, National Hospital for Neurology and Neurosurgery, London, UK.
Jacqueline
J
Foong
D003072:Cognition Disorders / Q000209:etiology; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020945:Lupus Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008875:Middle Aged; D011618:Psychotic Disorders / Q000209:etiology; D012640:Seizures / Q000209:etiology; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide