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JAMA Clinical Challenge
December 25, 2013

Multiple Cerebral Infarctions and Intracranial Vessel Abnormalities

Author Affiliations
  • 1Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center Utrecht, the Netherlands
JAMA. 2013;310(24):2668-2669. doi:10.1001/jama.2013.284467

Several months before being admitted to the hospital, a 65-year-old woman noted relapsing fevers, night sweats, fatigue, and a 16-kg weight loss. In the weeks before her hospital admission, she developed subacute cognitive deterioration and progressive headaches. On presentation she has difficulties initiating speech (nonfluent aphasia) and responds slowly and inconsistently to questions. Otherwise, her physical examination is unremarkable. Laboratory studies reveal an elevated erythrocyte sedimentation rate (ESR) (135 mm/h), anemia (hemoglobin level, 9.5 g/dL), and high serum levels of soluble interleukin 2 receptor (sIL2R) (36 551 pg/mL), lactate dehydrogenase (LDH) (448 U/L), and β2-microglobulin (3.1 mg/L), with normal renal function, liver enzyme levels, and serum immunological and microbiological findings. Analysis of the cerebrospinal fluid (CSF) demonstrates a mildly increased leukocyte count (11 × 106/L) and total protein concentration (0.10 g/dL). Magnetic resonance imaging of the brain shows multiple infarctions, and digital subtraction angiography of the intracranial vessels demonstrates abnormalities suggestive of vasculitis (Figure 1). An electrocardiogram, computed tomography scans of the chest and abdomen, 18-fluorodeoxyglucose positron emission tomography, transesophageal echocardiography, inspection of the skin, and ophthalmologic evaluation are unremarkable.