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Abstract Background Immunotherapy is nowadays considered a mainstay of cancer treatment, dramatically affecting the disease-free survival rate in several aggressive malignancies. Unfortunately, cancer immunotherapy can also trigger life-threatening autoimmune neurological complications named “neurological adverse effects” (NAEs). NAEs can affect both the central nervous system (CNS), as in ipilimumab-related aseptic meningitis, and the peripheral nervous system (PNS), as in nivolumab-induced myasthenia gravis. Current evidence The incidence of NAEs is highly variable, ranging from 2 to 4% using checkpoint inhibitors to 50% using blinatumomab. Looking at these numbers, it appears clear that neurologists will soon be called more and more frequently to decide upon the best therapeutic strategy for a patient receiving immunotherapy and experiencing a NAE. Most of them can be treated or reverted withholding the offending drug and adding IVIg, plasmapheresis, or steroids to the therapy. Sometimes, however, for oncological reasons, immunotherapy cannot be stopped so the neurologist needs to know what countermeasures have proven most effective. Moreover, patients with a pre-existing autoimmune neurological disease (AID), such as myasthenia gravis or multiple sclerosis, might need immunotherapy during their life, risking a severe worsening of their symptoms. In that setting, the neurologist needs to properly counsel patients about the risk of a therapy-related relapse. Conclusion In this article, we describe the most frequently reported NAEs and aim to give neurologists a practical overview on how to deal with them.

Cerebral amyloid angiopathy (CAA) has been associated with a variety of neurodegenerative disorders, included prion diseases and Alzheimer's disease; its pathophysiology is still largely unknown. We report the case of an 80-year-old man with rapidly progressive dementia and neuroimaging features consistent with CAA carrying two genetic defects in the PRNP and SORL1 genes.Neurological examination, brain magnetic resonance imaging (MRI), electroencephalographic-electromyographic (EEG-EMG) polygraphy, and analysis of 14-3-3 and tau proteins, Aβ40, and Aβ42 in the cerebrospinal fluid (CSF) were performed. The patient underwent a detailed genetic study by next generation sequencing analysis.The patient presented with progressive cognitive dysfunction, generalized myoclonus, and ataxia. Approximately 9 months after symptom onset, he was bed-bound, almost mute, and akinetic. Brain MRI was consistent with CAA. CSF analysis showed high levels of t-tau and p-tau, decreased Aβ42, decreased Aβ42/Aβ40 ratio, and absence of 14.3.3 protein. EEG-EMG polygraphy demonstrated diffuse slowing, frontal theta activity, and generalized spike-waves related to upper limb myoclonus induced by intermittent photic stimulation. Genetic tests revealed the presence of the E270K variant in the SORL1 gene and the presence of a single octapeptide repeat insertion in the coding region of the PRNP gene.The specific pathogenic contribution of the two DNA variations is difficult to determine without neuropathology; among the possible explanations, we discuss the possibility of their link with CAA. Vascular and degenerative pathways actually interact in a synergistic way, and genetic studies may lead to more insight into pathophysiological mechanisms.