PMC4723134_01
Summary of clinical presentation. (A) Head CT on days 1 and 2 showed extensive vasogenic edema in the right temporal-parietal region, a focal hypodensity in the right frontal region associated with mass effect, and subfalcine herniation to the left with early central downward herniation. Therefore, an urgent right-sided craniectomy was performed to relieve the pressure and obtain a biopsy of the right frontal region. (B) Chest x-ray on day 2 showed patchy congestion consistent with a right lower lobe pneumonia as well as pulmonary edema. Later, autopsy results at day 5 confirmed these findings and also showed mucoid purulent secretions in the bronchial tree. Myocarditis was also noted at autopsy with a primary neutrophilic reaction. Nasopharyngeal swab was negative for enteroviruses and respiratory viruses via PCR. Cold agglutinins were positive at a titer of 64 when tested against adult cells at 4 C.
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F1
Head CT showed right frontal edema with mass effect (figure 1A). Nevertheless, given his lung x-ray showed consolidation (figure 1B), he was placed on the following antimicrobials: ceftriaxone 2 g IV twice a day, vancomycin 1 g IV every 8 hours, and acyclovir 1 g IV every 8 hours (HSV status at the time was pending).
Gross autopsy findings. A full Autopsy was performed after the patient died on Day 5 and the brain weight was 1600 grams. There is evidence of a previous right frontal biopsy site. On external examination of the brain there was hemorrhagic necrosis and swelling noted along the right lateral edge of the frontal/temporal/parietal area (arrows, A, C-F, H) with evidence of flattening of the right olfactory tract secondary to increase intracranial pressure (arrow, B). There was subfalcine herniation to the left, with herniation of the cingulate gyrus underneath the edge of the falx cerebri (arrow, G). There was also transtentorial herniation on the right with early signs of Duret's hemorrhages in the pons (arrows, I, J). A normal pons is shown for comparison (I). There was also cerebellar tonsillar herniation noted on the right which was likely the fatal complication.
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F2
A head CT showed extensive vasogenic edema in the right frontal-parietal region, a focal hypodensity in the right frontal area associated with mass effect, and subfalcine herniation to the left with early central downward herniation (figure 2).
Initial brain biopsy and autopsy microscopic findings. Initial brain biopsy on day 2 of the right frontal region showed acute cerebritis. There was a marked acute inflammatory cell reaction most consistent with an acute infectious process noted in lower (A) and higher (B) magnification. The neutrophil predominance was determined to be consistent with a pyogenic process, although the Gram stain was negative. Silver stain was also negative for fungus. There was no neoplasm, granulomatous inflammation, or vasculitis present. (C, D) Hematoxylin & eosin stain at autopsy showed punctate, ring-shaped foci of perivascular hemorrhage and edema, localized to the white matter. This was surrounded by inflammation with a predominantly macrophage reaction in the right and left orbital frontal areas as well as right frontal-parietal sections. (E, F) Trichrome stain confirmed fibrinoid necrosis in multiple vessels in the right orbital frontal and frontal-parietal areas. There was no lymphocytic infiltration and no microglial nodules as seen in vasculitis and viral encephalitis, respectively. There was no inflammation in the vessel walls. Thromboemboli, viral cytopathologic effects, foreign abscesses, and granulomas were not seen.
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Initial brain biopsy of the right frontal region on day 2 showed acute cerebritis (figure 3).