Table 2.

Case Summaries of Patients 1 and 2

	Patient 1	Patient 2 Initial presentation	Patient 2 Relapse
Immunotherapy	Nivolumab × 24 doses	Ipilimumab/nivolumab x 4 doses then nivolumab x 4 doses	None since initial treatment
Neurologic autoimmune diagnosis	Aseptic meningitis (grade 3)	Limbic encephalitis (grade 3)	Relapsed limbic encephalitis (grade 3)
Presenting symptoms	Fever, chills, malaise, dry cough, headache, bilateral eye pain, and right ear pain	Blurry vision, headache, photophobia, and short‐term memory impairment	Confusion, paranoia, and short‐term memory impairment
Neurologic examination findings	Unremarkable	Anterior bilateral uveitis (2 mo prior) (grade 1), ataxia, Impaired delayed recall, right 6th cranial nerve palsy	Left 6th cranial nerve palsy, bilateral ptosisa, MoCA = 11/30
Brain MRI results	Punctate focus of leptomeningeal enhancement	FLAIR hyperintensities in bilateral mesial temporal lobes	Bilateral lateral rectus atrophy
Lumbar puncture results	Protein 64 mg/dL Glucose 56 mg/dL RBC 1/mm3 WBC 74/mm3 (91% lymphocytes) Opening pressure 21 mm H2O No organisms/growth Cytopathology negative	Protein 34 mg/dL Glucose 53 mg/DL RBC 0/mm3 WBC 19/mm3 (99% lymphocytes) No organisms/growth Cytopathology negative Paraneoplastic panel negative AChR negative MuSK negative	Protein 57 mg/dL Glucose 48 mg/dL RBC 0/mm3 WBC 4/mm3 NMDA receptor antibody negative bParaneoplastic, autoantibody panel negative Cytopathology negative No organisms/growth Pattern 4 oligoclonal bands
Additional findings/ assessments			EEG showed intermittent focal delta slowing in the bilateral frontal region
irAE treatment	Methylprednisolone 1 mg/kg iv x 1 then dexamethasone p.o. taper over 4‐5 wk	Methylprednisolone 1 gram iv daily x 5 then prednisone p.o. taper over 3 mo; mycophenolate unsuccessful	Methylprednisolone 1 gram iv daily x 5 then prednisone oral taper over 4 mo
irAE outcome	No sequelae	Partial improvement	Partial improvement

Abbreviations: AChR, antiacetylcholine receptor antibody; EEG, electroencephalogram; FLAIR, fluid‐attenuated inversion recovery; iv, intravenous; irAE, immune‐related adverse event; LP, lumbar puncture; MoCA, Montreal Cognitive Assessment; MuSK, muscle‐specific receptor kinase; NMDA, N‐methyl‐D‐aspartate; RBC, red blood cell; and WBC, white blood cell.

^aInitially presented with left ptosis and progressed to bilateral ptosis.

^bVGKC‐complex Ab IPA, LGI1‐IgG CBA, CASPR2‐IgG CBA, GAD65 Ab Assay, GABA‐B‐R Ab CBA, AMPA‐R Ab CBA, ANNA‐1‐3, AGNA‐1, PCA‐1 and 2, PCA‐Tr, Amphiphysin Ab, CRMP‐5‐IgG