As the use of
cancer immunotherapy with
immune checkpoint inhibitors (ICIs) is expanding rapidly for the treatment of many
tumor types, it is crucial that both neurologists and oncologists become familiar with the diagnosis and treatment of neurological immune-related adverse events (n-irAEs). These are rare complications, developing in their severe forms in only 1-3% of the patients, but are highly relevant due to their mortality and morbidity burden. The diagnosis of n-irAEs is-however-challenging, as many alternative diagnoses need to be considered in the complex scenario of a patient with advanced
cancer developing neurological problems. A tailored diagnostic approach is advisable according to the presentation, clinical history, and known specificities of n-irAEs. Several patterns characterized by distinct clinical, immunological, and prognostic characteristics are beginning to emerge. For example,
myasthenia gravis is more likely to develop after anti-
programmed cell death protein 1 (PD-1) or anti-programmed cell death
ligand 1 (PD-L1) treatment, while
meningitis appears more frequently after anti-cytotoxic T lymphocyte antigen-4 (CTLA-4)
therapy. Also,
peripheral neuropathy and
Guillain-Barré syndrome seem to be more common in patients with an underlying
melanoma.
Central nervous system disorders (CNS) are less frequent and are more often associated with
lung cancer, and some of them (especially those with
limbic encephalitis and positive onconeural
antibodies) have a poor prognosis. Herein, we provide an update of the recent advances in the diagnosis and treatment of neurological toxicities related to ICI use, focusing on the exclusion of alternative diagnoses, diagnostic specificities, and treatment of n-irAEs.