A 60-Year-Old Woman With Headache, Confusion, and Hallucinations ASHIMA MAKOL, JOSEPH E. PARISI, GEORGE W. PETTY, ROBERT E. WATSON, AND KENNETH J. WARRINGTON CASE PRESENTATION History of the present illness A 60-year-old woman, healthy and highly functioning at baseline, was in her usual state of health when she devel- oped new-onset headache in a bilateral temporoparietal distribution. This was gradual in onset, associated with nausea and vomiting, and without photophobia or phono- phobia. She was noted to be disoriented to time, place, and person by her family. She also rapidly developed auditory hallucinations, which prompted admission to an outside hospital. She was afebrile and hemodynamically stable. Kernig’s and Brudzinski’s signs were negative. Neurologic examination was nonfocal. A noncontrast computed to- mography (CT) scan of the brain showed hypodensities with mild mass effect involving both posterior temporal lobes and left frontal subcortical white matter. Magnetic resonance imaging (MRI) of the brain (Figure 1) showed signal changes in the same locations with smaller areas of signal abnormality and localized mass effect involving the subcortical white matter of the frontal lobes and superior cerebellar vermis. Gradient-echo imaging demonstrated multiple tiny punctate areas of hemosiderin deposition in the brain bilaterally without focal intracranial hemorrhage or ischemia. She was empirically treated with acyclovir, ceftriaxone, and vancomycin for suspected meningoen- cephalitis. Cerebrospinal fluid (CSF) analysis showed 3 white blood cells (WBCs)/high-power field (hpf), 15 red blood cells (RBCs)/hpf, protein 16 mg/dl, and glucose 48 mg/dl. Herpes simplex virus (HSV) polymerase chain re- action (PCR) was negative. Electroencephalogram (EEG) showed right temporal and parietal sharp waves but no epileptogenic activity. Blood, CSF, and urine cultures re- turned negative, after which antibiotics were discontin- ued. She was treated symptomatically and headaches im- proved, but did not resolve completely. She was dismissed home and returned to work 2 weeks later. Four days later, she relapsed with severe headaches and disorientation. She was hospitalized and a repeat MRI (Figure 1) showed areas of leptomeningeal enhancement and multiple white matter lesions with T2 hyperintensity, patchy enhancement, and restricted diffusion. Repeat lum- bar puncture showed 1 WBC/hpf, 8 RBCs/hpf, glucose 52 mg/dl, and protein 65 mg/dl. CSF studies were negative or normal for angiotensin-converting enzyme level, syphilis, Whipple’s disease, Lyme, HSV, West Nile virus, and en- terovirus PCR. Serologic studies for Western, Eastern, and California equine viral encephalitis were negative, as were CSF bacterial, mycobacterial, and fungal cultures. The erythrocyte sedimentation rate was 26 mm/hour and the C-reactive protein level was 1 mg/liter. Laboratory studies were normal or negative, including a complete blood cell count, liver and kidney functions, antinuclear antibody (ANA), extractable nuclear antigen (ENA) panel, antineu- trophil cytoplasmic antibodies, lupus anticoagulant, and antiphospholipid antibodies. Chest/abdomen/pelvis CT and mammogram were also normal. The patient subse- quently developed auditory hallucinations again and had new findings of peripheral vision loss on the left side and formed visual hallucinations. A neuroophthalmology eval- uation confirmed left homonymous hemianopia. EEG showed periodic lateralized epileptiform discharges over the right temporoparietal areas. Although hemodynami- cally stable, her confusion and disorientation continued to worsen. She underwent a right temporal lobe brain biopsy that was interpreted as being suggestive of vasculitis. She was treated with methylprednisolone intravenously 1 gm daily for 3 days with a remarkable resolution of all of her clinical symptoms. She was also started on levetiracetam for abnormalities on EEG and trimethoprim/sulfamethoxa- zole for Pneumocystis jiroveci pneumonia prophylaxis. She returned to her baseline and was dismissed home on an oral steroid taper a week later. She did well clinically for 2 months until she was down to prednisone 10 mg Ashima Makol, MD, Joseph E. Parisi, MD, George W. Petty, MD, Robert E. Watson, MD, PhD, Kenneth J. War- rington, MD: Mayo Clinic, Rochester, Minnesota. Dr. Parisi receives royalties for Principles and Practice of Neuropathology, 2nd Edition. Address correspondence to Ashima Makol, MD, Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905. E-mail: makol.ashima@mayo.edu. Submitted for publication May 15, 2011; accepted June 17, 2011. Arthritis Care & Research Vol. 63, No. 10, October 2011, pp 1486 –1494 DOI 10.1002/acr.20536 © 2011, American College of Rheumatology CLINICOPATHOLOGIC CONFERENCE 1486