Pearls & Oy-sters: Anti-GABA A Receptor Encephalitis Presenting as Fulminant Hemicerebellitis in a 5-Year-Old Girl.
Kellie E Krohn, Rebecca Luke, Adrian Lacy et al.
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Case in brief
A previously healthy 5-year-old girl developed acute neurologic decline after several days of fever and malaise. CT showed severe right cerebellar inflammation and edema with fourth-ventricle effacement, prompting emergency posterior fossa decompression. CSF had mild pleocytosis; MRI showed nonenhancing right cerebellar lesions and evolving supratentorial cortical involvement. She developed seizures and progressive neuroinflammation despite empiric antimicrobials and initial immunotherapy. Expanded antibody testing confirmed anti-GABA A receptor encephalitis. After further IL-6-directed treatment and intrathecal dexamethasone, she stabilized, with radiographic improvement and meaningful neurologic recovery.
What made this case unusual
The encephalitis presented as fulminant unilateral cerebellitis with life-threatening posterior fossa edema and evolving cortical involvement, before expanded antibody testing identified anti-GABA A receptor encephalitis.
Diagnostic / clinical pearl
In severe cerebellitis with evolving neurologic features, consider autoimmune encephalitis and include expanded antibody testing alongside other diagnostic evaluation; deterioration may require urgent management of posterior fossa mass effect.