Table 1

Characteristics of patients with CNS-ICI

VariableAll patients
(N=19)
Main clinical syndrome
Limbic encephalitis
(n=8)
Meningoencephalitis
(n=4)
Cerebellar syndrome
(n=4)
Atypical syndrome
(n=3)
Age, median (range), years69 (32–80)63 (37–80)59 (46–72)66 (32–72)72 (70–76)
Sex, male, n (%)14 (74)6 (75)3 (75)4 (100)1 (25)
Additional clinical featuresPeripheral nervous system involvement (3 patients)Diencephalitis (3 patients)Peripheral nervous system involvement (2 patients)Cranial nerves involvement (1 patient)Isolated confusion (2 patients), polyradiculoneuritis and parkinsonism (1)
Brain MRI
Normal73031
Temporomesial hyperintensities55000
Other abnormalities*70412
Cerebrospinal fluid
Normal42001
Pleocytosis (cells/mm3)112, usually mild (range: 0–10)4, usually marked (range: 20–210)3, usually moderate (range: 11–32)2
Proteinorrachia (g/L)125, usually mild (range: 0.3–0.9)3, usually marked (range: 0.9–2.5)4, usually moderate (0.5–1.1)0
Oligoclonal bands51 out of 4 tested2 out of 2 tested2 out of 2 testedNA
Immunological background
Cancer8 NSCLC, 3 melanoma, 2 bladder, 2 kidney, 4 other cancers4 NSCLC, 1 pleural mesothelioma, 1 kidney, 1 melanoma,
1 liposarcoma+GIST
2 NSCLC, 1 kidney, 1 melanoma1 NSCLC, 1 SCLC, 1 bladder, 1 Hodgkin’s lymphoma1 NSCLC+rectal cancer, 1 bladder, 1 melanoma
Central nervous system antibodiesMa2 in 7, GFAP in 2, Hu in 1, CASPR2 in 1, atypical staining in 5Ma2 in 7, atypical neuropil staining in 1GFAP in 2, atypical neuropil staining in 1Hu in 1, atypical neuropil staining in 2CASPR2 in 1, atypical staining in 1
ICI treatmentNivolumab 8, pembrolizumab 6, nivolumab+ipilimumab 3, atezolizumab 1, durvalumab+ipilimumab 1Pembrolizumab 4, nivolumab 3, nivolumab+ipilimumab 1Nivolumab 2, pembrolizumab 1, nivolumab+ipilimumab 1Nivolumab 2, atezolizumab 1, durvalumab+ipilimumab 1Nivolumab 1, pembrolizumab 1, nivolumab+ipilimumab 1
Other immune-related adverse events7 in 5 patients1 vitiligo+thrombocytopaenia1 hypophysitis1 inflammatory arthritis1 colitis+thyroiditis, 1 colitis
CTCAE grade, median (range)3 (3–5)3.5 (3–5)4 (3–5)3 (3)3 (3–5)
Follow-up period, median (range), months5 (1–23)4.5 (1–13)14 (3–15)4.5 (2–23)2.5 (1–19)
Outcome6 died, 1 worsened despite treatment, 4 stabilised, 8 improved or recovered4 died, 1 worsened despite treatment, 2 stabilised and 1 improved1 died, 1 stabilised, 2 improved3 markedly improved, 1 stabilised1 died, 1 improved, and 1 recovered
  • *Excluding brain metastasis.

  • CASPR2, contactin-associated protein-like 2; CNS-ICI, central nervous system immune-related complications; CTCAE, Common Terminology Criteria for Adverse Events; GFAP, glial fibrillar acidic protein; GIST, gastrointestinal stromal tumour; ICI, immune checkpoint inhibitor; NA, not available; NSCLC, non-small cell lung cancer; SCLC, small cell lung cancer.