Sulphasalazine-induced cerebral lupus erythematosus.

Case report In November 1980, a 19-year-old girl presented with a 5-day history of right frontal headache and a 3-day history of deteriorating conscious level. Three years earlier, she had been diagnosed as having ulcerative colitis on clinical, radiological and sigmoidoscopic criteria, and had been treated with sulphasalazine, 1-5 g daily. There was no family history of ulcerative colitis or connective tissue disease. On examination, she was apyrexial but anaemic; there was no neck stiffness or evidence of skin or joint involvement. She did not speak or obey commands. Although she moved all 4 limbs to painful stimuli, there was less response on the right side. There was no reflex asymmetry but both plantar responses were extensor. Initially, fundoscopy was normal but she developed transient bilateral papilloedema during the first week of her illness. Initial investigations showed a haemoglobin of 7-6 g/dl, a hypochromic, microcytic blood film and a white count of 9-3 x 109/l, with a normal differential count. The erythrocyte sedimentation rate was raised at 52 mm in the first hour. Plasma urea, electrolytes and liver enzymes were normal. Com puterized axial tomography (CT) brain scan showed evidence of diffuse low density in the region of both basal ganglia. Electroencephalogram (EEG) showed a right temporal delta focus of high amplitude but, on arousal, there developed diffuse delta activity. Cerebrospinal fluid (CSF) examination showed a raised protein level (4 7 g/dl), with a normal glucose content (3-2 mmol/l) and a white cell count of < 1I0 x 109/l. In view ofthe possibility of acute viral encephalitis, treatment was started with dexamethasone, 30 mg daily, and acyclovir, 500 mg 8-hourly via i.v. infusion. However, aright frontal brain biopsy showed no evidence of viral infection, vasculitis or tumour and further viral studies on brain tissue, CSF and serum were all negative. Over the following 10 days, her conscious level began to improve and she was later able to move her right arm in response to commands. She developed a left homonymous hemianopia and spasticity in both arms and legs. Acyclovir was stopped after 10 days and steroids were gradually reduced. A repeat CT scan showed evidence of diffuse low density throughout the right hemisphere with moderate ventricular enlargement, compatible with cerebral infarction. No further sulphasalazine was given after admission to hospital because of the possibility of a drug-induced lupus syndrome. Immunological investigations 2 weeks after admission showed (normal ranges in brackets): Antinuclear antibody (IgG) was positive-1560 u./ml. Anti-DNA antibody (Crithidia luciliae, immunofluorescence) was negative -Kit 1* (N< 10 u./ml)-10 u./ml, Kit 2t (N< 160 u./ml)-positive, 750 u./ml. Anti-ribonucleoprotein antibody was negative. Circulating immune complexes, platelet aggregation titre (1/20) was positive-