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.

Background Intracranial dural arterio-venous fistulas are pathological anastomoses between arteries and veins located within dural sheets and whose clinical manifestations depend on location and hemodynamic features. They can sometimes display perimedullary venous drainage (Cognard type V fistulas—CVFs) and present as a progressive myelopathy. Our review aims at describing CVFs’ variety of clinical presentation, investigating a possible association between diagnostic delay and outcome and assessing whether there is a correlation between clinical and/or radiological signs and clinical outcomes. Methods We conducted a systematic search on Pubmed, looking for articles describing patients with CVFs complicated with myelopathy. Results A total of 72 articles for an overall of 100 patients were selected. The mean age was 56.20 ± 14.07, 72% of patients were man, and 58% received an initial misdiagnosis. CVFs showed a progressive onset in 65% of cases, beginning with motor symptoms in 79% of cases. As for the MRI, 81% presented spinal flow voids. The median time from symptoms’ onset to diagnosis was 5 months with longer delays for patients experiencing worse outcomes. Finally, 67.1% of patients showed poor outcomes while the remaining 32.9% obtained a partial-to-full recovery. Conclusions We confirmed CVFs’ broad clinical spectrum of presentation and found that the outcome is not associated with the severity of the clinical picture at onset, but it has a negative correlation with the length of diagnostic delay. We furthermore underlined the importance of cervico-dorsal perimedullary T1/T2 flow voids as a reliable MRI parameter to orient the diagnosis and distinguish CVFs from most of their mimics.

No abstract