Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus
A 17-year-old Korean female was brought to the pediatric department because of new-onset, right-sided weakness that was first noted 3 weeks ago when it affected her writing ability. Her speech was slurred and she complained tongue numbness. Her symptoms seemed to improve for a while at first, but afterward she did not completely recover for 1 month. There was no evidence of trauma, flu-like symptoms, or no report of nausea, vomiting, headache, or convulsions. Evaluating the patient's medical history, there were no complications at birth, and her development had been age appropriate. There was no family history of stroke in young individuals.\nIn September 2008, at 15 years of age, she was diagnosed with lupus nephritis. Initial clinical course showed severe manifestations with microangiopathic hemolytic anemia, thrombocytopenia, and hypertension. The renal biopsy finding indicated the lupus nephritis ISN/RPS Class IV-G(A). After the initial period of 6 months, her clinical symptoms were well controlled with treatment of azathioprine, prednisolone and antihypertensive drugs. However, after 22 months, new-onset right-sided weakness developed.\nOn admission, vital signs were stable. Neurological examination revealed asymmetric motor weakness with the grade of 4/5 in the right arm and leg. She was mentally alert, but had a subtle dysarthric speech despite of normal cranial nerves. Reflexes for both knees were symmetric and normal. Otherwise, her systemic examination was unremarkable.\nThe laboratory tests revealed the followings: leukocyte, 5,100/mm3; hemoglobin, 14.8 g/dL; platelet, 266,000/mm3; erythrocyte sedimentation rate, 2 mm/hr (0-20); C-reactive protein level, 0.01 mg/dL (0-0.5); C3, 64 mg/dL (79-152); C4, 12.4 mg/dL (10-40); and CH50, 37.6 U/mL (23-46). Electrolytes, renal and hepatic profiles, and lipid profile were normal. Anti-thrombin III, prothrombin time, activated partial thromboplastin time, protein C (120%), protein S (70%), and lipid profiles were all within normal limits. Urine analysis showed no proteinuria or microscopic hematuria. Immunologic evaluation revealed antinuclear antibody (ANA) of 1:80, antids-DNA immunoglobulin G (IgG)/IgM 7 (0.25)/14 (0.20) IU/mL, anticardiolipin IgG/IgM (-/-), lupus anticoagulant (-), antiphospholipid antibody (-), anti-Ro/anti-La (-/-), and venereal disease research laboratory test (VDRL) (-). Chest X-ray, electrocardiogram, and echocardiogram findings were all unremarkable. There was no evidence of aggravation of SLE activity.\nBrain magnetic resonance imaging (MRI) revealed no evidence of cerebral infarction or ischemic change (). Magnetic resonance angiography of the brain revealed stenosis of the supraclinoid portion of the left ICA and the right anterior cerebral artery (ACA). Conventional angiography showed severe narrowing of the distal portion of left ICA, the proximal portion of left middle cerebral artery (MCA), ACA and the right ACA (). These findings were compatible with MMD.\nThe patient was started aspirin, however, her right-sided weakness and dysarthria continued unabated. One week later, left encephalo-duro-arterio-synangiosis bypass surgery was performed. Postoperative MRI showed no interval change of right MCA branch and no further progression with new lesion. She has since recovered without new ischemic symptoms. No further neurological deficits occurred during a 14-month follow-up. Right-side surgery will be scheduled depending on follow-up neuroimaging results and the patient's neurological symptoms.