PMC9900111_01
Case 1, course of illness and clinical studies. (A) A graphical time course of events. Brain biopsies are indicated by vertical dotted lines. Periods of glucocorticoid and mycophenolate mofetil immunosuppressive therapy are indicated by light and dark green lines, respectively. Active meningoencephalitis is indicated by the blue line. MRI scans are indicated by the coronal brain icon (red = active meningoencephalitis and gray = stable/no enhancement or worsening of the encephalitic process). Black dots indicate clinical CSF values for white blood count (WBC, ref. <=5 cells/muL), protein (ref. <=60 mg/dL), and glucose levels (ref. >=50 and <=80 mg/dL). Colored areas between dots have been added to aid visual assessment. The vertical light blue outlined box indicates a period of acute illness secondary to a confirmed ventriculoperitoneal (VP) shunt infection resulting in a 2-month interruption in mycophenolate mofetil treatment and transient CSF abnormalities thought to be unrelated to the autoimmune meningoencephalitis. (B) Axial T1 post-gadolinium MRIs demonstrate that the right temporal lobe and insular leptomeningeal enhancement present at initial presentation (arrows, left image) had resolved at a follow-up appointment after immunosuppressive therapy (right image). (C) Histopathology findings: hematoxylin and eosin (H&E) staining of a biopsy of the meninges revealed a dense, mixed inflammatory infiltrate. Immunohistochemistry of the meninges identified scattered CD3+ T cells and scattered CD20+ B cells. A separate biopsy of the brain parenchyma revealed perivascular and intraparenchymal collections of CD43+ inflammatory cells. Scale bars = 50 mum.
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F1
He had lived in the San Francisco Bay Area for over 20 years and was an avid gardener. He did not have a history of incarceration, homelessness, or international travel (Figure 1A). T2/FLAIR hyperintensity, and patchy enhancement, and without abnormal susceptibility weighted imaging (Figure 1B). The imaging was consistent with meningoencephalitis, and serial lumbar punctures (LP) revealed a neutrophil-predominant pleocytosis (WBC: 260-756/muL with 12-70% neutrophils), mild hypoglycorrhachia (nadir 33 mg/dL), and elevated protein (76-95 mg/dL). T cells (Figure 1C), suggestive of an infectious etiology. The underlying cortical brain tissue was also biopsied and showed no significant pathological abnormality.
PMC9900111_02