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Washington University Experience | NEOPLASMS (GLIAL) | Diffuse midline glioma, H3 K27-altered | 2A0 Case 2 History
Case 2 History ---- In May 2019 this 20 year old female patient was admitted at an outside hospital for right-sided waxing and waning weakness, confusion, and short term memory loss. MRI showed a solitary FLAIR hyperintense pontine lesion, concerning for demyelination. In September 2019 she had altered mental status which resolved to her baseline in 3-5 days with corticosteroids and a course of doxycycline. Two weeks later, she experienced a second spell identical to her first, but now involved the left side of her body which again resolved in 3-5 days treated with doxycycline. Prior to her admission in September 2019, she had complaints of progressive fatigue and intermittent headaches for which she was treated with amoxicillin for sinusitis. On 9/1/2019 she had severe headache for which she received intravenous fluids at an outside emergency department and was sent home. On 9/3/19 she was found unresponsive by family and was reported to have been non-rhythmically flailing her arms. She also had severe headache, intermittent nausea, vomiting and fatigue. Later she was noted to have bilateral dilated pupils and difficulty shutting her left eye. After being transferred to an intensive care unit at an outside hospital, she decompensated following a dose of Ativan and was intubated. White blood counts were elevated to 25.2 with a neutrophilic predominance, prompting vancomycin, ceftriaxone, and acyclovir empirically. Autoimmune workup (rheumatoid factor, antinuclear antibody, ds-DNA) was negative. CSF workup for Cryptococcus, HIV, syphilis, and AFB were negative. Image studies were negative for venous thrombosis by MRV. An MRI showed diffusion restricted throughout the cerebral cortex bilaterally and edema consistent with development of infarcts, left greater than right, as well as diffuse leptomeningeal enhancement consistent with meningitis. CT angiogram showed cytotoxic cortical edema, and mild diffuse narrowing of intracranial vessels (internal carotid and middle cerebral arteries). Repeat head CT on 9/5 noted multifocal loss of gray-white differentiation. Four hours later, a brain MRI noted diffuse areas of diffusion restriction bilaterally, without a clear vascular correlate, marked cortical edema on FLAIR, and redemonstration of her pontine lesion. Her right temporal lesion evolved overnight with hemorrhagic conversion. Neurologic examination shortly thereafter was consistent with brainstem death. The decedent expired in September 2019.
