A 60‐year‐old woman with headache, confusion, and hallucinations

Abstract

History of the present illnessA 60-year-old woman, healthy and highly functioning at baseline, was in her usual state of health when she developed new-onset headache in a bilateral temporoparietal distribution. This was gradual in onset, associated with nausea and vomiting, and without photophobia or phonophobia. 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 tomography (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 meningoencephalitis. 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 reaction (PCR) was negative. Electroencephalogram (EEG) showed right temporal and parietal sharp waves but no epileptogenic activity. Blood, CSF, and urine cultures returned negative, after which antibiotics were discontinued. She was treated symptomatically and headaches improved, 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 lumbar 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 enterovirus 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, antineutrophil cytoplasmic antibodies, lupus anticoagulant, and antiphospholipid antibodies. Chest/abdomen/pelvis CT and mammogram were also normal. The patient subsequently developed auditory hallucin...

Cerebral amyloid angiopathy associated with inflammation: A systematic review of clinical and imaging features and outcome

“…The search (Figure 2) identified 186 abstracts, and a further 44 from reference lists, giving a total of 230. After inclusion and exclusion criteria were applied, 104 publications were included which described 213 distinct pathologically proven cases of CAARI/ABRA, 4,7…”

Identifying diagnostic and prognostic factors in cerebral amyloid angiopathy‐related inflammation: A systematic analysis of published and seven new cases

“…Notably, elevated CSF protein concentration was decreased following immunosuppression in 80.0% (8/10) [18, 32, 41, 52, 54, 57–59], whereas it increased upon disease progression [8, 60–63] or relapse [54, 64, 65]. The CSF white blood cell count changed in parallel with protein concentration in most reports [8, 32, 52, 57–60, 65] with few exceptions [54, 62–64]. The CSF white blood cell count was decreased following immunosuppression in 87.5% (7/8).…”

“…In addition, CSF pleocytosis tended to be associated with LE (53.8% in those with LE vs 32.0% in those without; p = 0.087). Notably, elevated CSF protein concentration was decreased following immunosuppression in 80.0% (8/10) [18, 32, 41, 52, 54, 57–59], whereas it increased upon disease progression [8, 60–63] or relapse [54, 64, 65]. The CSF white blood cell count changed in parallel with protein concentration in most reports [8, 32, 52, 57–60, 65] with few exceptions [54, 62–64].…”

“…Macrophages are abundant in the infiltrate (HE, J), frequently with Aβ-immunopositive cytoplasm (arrowheads, Aβ, K), and in the microinfarcts (arrowheads, Aβ, L).concentration was decreased following immunosuppression in 80.0% (8/10)[18,32,41,52,54,[57][58][59], whereas it increased upon disease progression[8,[60][61][62][63] or relapse[54,64,65]. The CSF white blood cell count changed in parallel with protein concentration in most reports[8,32,52,[57][58][59][60]65] with few exceptions[54,[62][63][64]. The CSF white blood cell count was decreased following immunosuppression in 87.5%(7/8…”

“…The perivascular infiltrates apparent through (A)-(K) (black-filled arrowheads) contain several T lymphocytes (CD3, G), including cytotoxic T cells (CD8, H), and a few B cells (CD20, I). Macrophages are abundant in the infiltrate (HE, J), frequently with Aβ-immunopositive cytoplasm (arrowheads, Aβ, K), and in the microinfarcts (arrowheads, Aβ, L).concentration was decreased following immunosuppression in 80.0% (8/10)[18,32,41,52,54,[57][58][59], whereas it increased upon disease progression[8,[60][61][62][63] or relapse[54,64,65]. The CSF white blood cell count changed in parallel with protein concentration in most reports[8,32,52,[57][58][59][60]65] with few exceptions[54,[62][63][64].…”