PHOTO QUIZ

Philip A. Mackowiak, Section Editor

A 53-Year-Old Woman with Rapidly Progressive Altered Mental Status

and Ataxia
(See pages 629–630 for the Answer to the Photo Quiz.)

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Figure 1. Axial fluid attenuation inversion recovery (FLAIR) magnetic resonance imaging of the patient’s brain demonstrating hyperintensity involving
the periventricular and periatrial white matter with patchy areas of enhancement.

A 53-year-old white woman with relapsed acute myeloid
leukemia after a T cell–depleted sibling allogenic stem cell trans-
plant was admitted to the hospital with a 1-week history of
expressive aphasia, weakness, and ataxia leading to multiple
falls at home. She also reported a 1-month history of inter-
mittent low-grade fevers, rhinorrhea, and cough. Two days be-
fore hospital admission, her neurologic symptoms progressed
such that she was unable to ambulate. On hospital day 2, she
had a rapid decline in her mental status, and magnetic reso-
nance imaging revealed fluid attenuation inversion recovery
(FLAIR) hyperintensity in the right cerebellum and bilateral
periventricular and periatrial white matter (Figure 1). Cere-
brospinal fluid (CSF) obtained by lumbar puncture revealed a
white blood cell count of 37 cells/mm3 (74% lymphocytes, 20%
neutrophils, and 6% monocytes), a red blood cell count of 12
cells/mm3, a protein level of 100 mg/dL, and a glucose level of

Clinical Infectious Diseases 2010; 51(5):575–576

 2010 by the Infectious Diseases Society of America. All rights reserved.
1058-4838/2010/5105-0014$15.00
DOI: 10.1086/655690

PHOTO QUIZ • CID 2010:51 (1 September) • 575


Figure 2. Hematoxylin and eosin stain (A) and indirect immunofluorescence staining (B ) of brain autopsy specimen (original magnification, ⫻1000).
97 mg/dL. CSF cultures did not grow bacteria or fungus, and
polymerase chain reaction tests for herpes simplex virus, var-
icella zoster virus, Epstein-Barr virus, and JC virus had negative
results. Because of concerns for possible post-transplant lym-
phoproliferative disorder, treatment with dexamethasone and
methotrexate was initiated. On hospital day 4, she had a seizure
and was intubated. Over the next several days, her neurologic
status continued to decline, and she became comatose with
evidence of worsening cerebral edema and herniation on fol-
low-up computed tomography. She died on hospital day 8, and
576 • CID 2010:51 (1 September) • PHOTO QUIZ
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a brain-only autopsy was performed. Gross examination re-
vealed extensive hemorrhagic infiltrative lesions involving the
gray and white matter within the cerebrum and cerebellum, as
well as linear pontine hemorrhages. Microscopic examination
revealed large, discohesive cells with vacuolated cytoplasm and
eccentric nuclei with prominent nucleoli present both within
the parenchyma and in a perivascular distribution (Figure 2A).
Indirect immunofluorescence was performed (Figure 2B).
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