Silk road occlusion

Abstract

In November, 2009, a 19-year-old woman presented to us with a 3-month history of several episodes of numbness of her left limbs lasting 2–3 min, and one episode of weakness lasting 2·5 h. She also complained of anorexia because of paroxysmal upper abdominal pain, which had been present for 1 week before admission and had a 5-year history of intermittent stomach discomfort.Our patient had had an average of ten episodes of recurrent oral ulcers per year, and two episodes of genital ulcers since she was 4 years old. She did not report any history of hypertension, diabetes, coronary artery disease, smoking, or drug or alcohol misuse. On physical examination there was an absence of bilateral carotid pulses. Neurological and ophthalmological examinations were normal. The pathergy test was positive. Laboratory tests suggested an infl ammatory condition with a high ESR 37 mm/h, and C-reactive protein 91·4 mg/L. Blood cell count, lipid profi le, liver and kidney function, blood glucose concentration, urine and stool tests, and cerebrospinal fl uid examination were normal. Serum immunoglobulin, complement components (C3, C4), protein C, protein S, and tests for antithrombin III and antistreptolycin O were also normal. She was HIV negative. Endoscopy showed multifocal oesophageal ulcers. MRI of the brain showed a remote lacunar infarction at the anterior horn of the right lateral ventricle. Carotid angiography showed complete occlusion of the bilateral common carotid arteries but the external and internal carotid arteries were patent and perfused by collateral circulation, mainly from the bilateral vertebral arteries (fi gure A and B). Connective tissue disease was excluded because of the absence of rheumatoid factor and relevant autoantibodies.