[Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9–10):309–321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.]

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

[Peripheral neuropathy is caused by structural or functional damage of nervous system. The pathophysiology is not well known. Its clinical features are established but there is a need to standardize CIPN assessment, also considering that health care providers and patients frequently have a different perception of CIPN severity. Neurotoxicity caused by traditional chemotherapy is widely recognized in patients with cancer. The adverse effects of newer therapeutics, such as targeting and immunotherapeutic agents, need more information for the proper management. This review addresses the main neurotoxicities of cancer treatments with a focus on the newer therapeutics. Recognition of these patterns of toxicity is important because drug discontinuation or dose adjustment might prevent further neurological injury. Treatment is symptomatic. For prevention or treatment there is need for further basic research outcomes.]

[The use of biological therapeutic agents increases the risk of certain infectious diseases. The greatest hazards are the reactivation of tuberculosis or hepatitis B infection and the development of sepsis. The degree of risk is comparable to that experienced with the use of routine DMARD therapy: in case of an adequate choice of treatment the risk is equal to or slightly higher than that of conventional treatment. However, this hazard is definitely smaller than that associated with the use of corticosteroids at doses necessary to reach the required immunosuppressive effect. This article provides a brief summary of potential infectious illnesses on the basis of the literature.]

[INTRODUCTION - Inactivated influenza and H1N1 vaccination is recommended yearly for patients with inflammatory bowel disease receiving immunosuppressive therapy; however, immunomodulator and biological therapy might impair the immune response to the vaccination. In our study, we assessed whether immunity can develop in response to H1N1 influenza vaccination in patients receiving immunomodulator and/or biological therapy. We also assessed the occurrence of side effects after the immunisation in these patients. PATIENTS AND METHODS - In our prospective study, blood samples were obtained from 24 patients (12 Crohn’sdisease, 12 ulcerative colitis) one month after immunisation against influenza A/California/ 07/2009 (H1N1) virus. At the time of vaccination, all patients have been receiving immunomodulator and/or biological therapy for at least three month. Antiviral antibodies were detected by using microneutralisation assay. The safety of the vaccination was assessed by questionnaires. RESULTS - Every patient developed complete immunity against influenza A (H1N1) virus, independently from the type of immunosuppressive therapy. Regarding side effects, local symptoms occurred in six patients and systemic symptoms in another six patients. Mild diarrhea occurred in five patients. Moderate exacerbation of the disease was observed in 2 patients with Crohn’s disease and in one patient with ulcerative colitis. CONCLUSIONS - According to our results, immunocompromised patients with IBD can be safely advised to receive the vaccination. In our study, all patients developed adequate immunity according to microneutralisation titers.]