Checkpoint inhibitor-related immune-mediated encephalitis

From IDWiki

Background

Clinical Manifestations

  • Onset variable, days to weeks to months
  • Symptoms are most frequently cognitive (confusion, agitation, altered mental status, memory impairment) and neuropsychiatric (behaviour changes, hallucinations, emotional lability)
  • Can have headache, nausea/vomiting, fevers, confusion, gait impairment, dysautonomia, and seizures
  • May be concurrent with other organ involvement, such as dermatitis, hepatitis, hypophysitis, and thyroiditis

Investigations

  • MRI findings can include:
    • Within normal limits
    • Bilateral temporal or multiple T2/FLAIR lesions
    • Leptomeningitial or dural enhancement, particularly in patients with CSF pleocytosis
    • Can have an appearance typical for limbic encephalitis or autoimmune encephalitis
  • CSF findings:
    • Within normal limits about a quarter
    • CSF pleocytosis is common, most commonly with lymphocyte predominance (occasionally neutrophilic)
    • Elevated protein
    • Negative flow cytometry and cytopathology
    • May have one or more detectable autoantibodies, including anti-Hu, anti-Ma2, anti-GAD65, anti-GFAP, and anti-NMDAR

Management

  • Discontinue the causative medication
  • Methylprednisolone 500-1000 mg IV daily for 3 to 5 days followed by tapering corticosteroid
  • Sometimes adjunctive IVIg is tried
  • Recovery can take several weeks

Further Reading