Multiple Cerebral Infarctions and Intracranial Vessel Abnormalities

Abstract

Several months before being admitted to the hospital, a 65-yearold woman noted relapsing fevers, night sweats, fatigue, and a 16-kg weight loss. In the weeks before her hospital admission, she developed subacute cognitivedeteriorationand 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) (135mm/h), anemia (hemoglobin level, 9.5 g/dL), and high serum levels of soluble interleukin2 receptor (sIL2R) (36 551pg/mL), lactatedehydrogenase (LDH) (448 U/L), and β2-microglobulin (3.1 mg/L), with normal renal function, liver enzyme levels, and serum immunological andmicrobiological findings. Analysis of the cerebrospinal fluid (CSF) demonstrates amildly increased leukocyte count (11 × 10/L) and total protein concentration (0.10g/dL).Magnetic resonance imagingof the brain shows multiple infarctions, and digital subtraction angiography of the intracranialvesselsdemonstratesabnormalitiessuggestiveofvasculitis (Figure 1). An electrocardiogram, computed tomography scans of the chest and abdomen, 18-fluorodeoxyglucose positron emission tomography, transesophagealechocardiography, inspectionof theskin, and ophthalmologic evaluation are unremarkable. Quiz at jama.com A B Brain magnetic resonance imaging (MRI) Digital subtraction angiography