Patients with neuromyelitis optica spectrum disorder (NMOSD) are at an increased risk of pregnancy complications. Lack of NMOSD treatment during pregnancy is a risk factor for relapse. Here, we report two cases of pregnant women with anti-aquaporin-4 antibody-positive (AQP4+) NMOSD treated with eculizumab during pregnancy. Patient 1 was diagnosed with AQP4+ NMOSD 2 mo after giving birth to her first child. She was treated with tacrolimus and prednisolone, before switching to prednisolone monotherapy. Following concerns of teratogenicity associated with immunosuppressive therapy and oral steroid use, she began eculizumab treatment prior to a second pregnancy. When she became pregnant, eculizumab treatment was briefly paused while safety data were reviewed with her neurologist. A total of three doses were missed. Patient 2 was diagnosed with AQP4+ NMOSD and began prednisolone treatment. Following a relapse, tacrolimus was added to her treatment regimen. Prior to pregnancy, she began eculizumab treatment alongside prednisolone and tacrolimus and maintained this regimen throughout her pregnancy. No relapses or meningococcal infections occurred after eculizumab initiation in either patient, and both gave birth without complications to healthy babies. Patient 2 continues to receive eculizumab while breastfeeding. We present two cases of pregnant women with AQP4+ NMOSD treated with eculizumab. Both women gave birth to healthy babies, have had no relapses since initiating eculizumab, and continued their treatment after birth. These cases are further evidence of the successful use of eculizumab during pregnancy.
We report a rare case of paraneoplastic neurological syndrome with dual seropositivity of anti-aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in a 40 year-old woman with metastatic triple-negative breast cancer. She received multiple lines of anti-neoplastic treatment, including immunotherapy with pembrolizumab, as well as cytotoxic chemotherapy. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis followed by left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid analysis during both episodes showed normal glucose and protein, and elevated white blood cell count. Cytology was negative for malignancy. Cerebrospinal fluid was positive for neuromyelitis optica immunoglobulin G antibody anti-aquaporin-4, and autoimmune myelopathy panel was positive for myelin oligodendrocyte glycoprotein antibody. The patient had significant clinical and radiographic improvement after completion of five cycles of plasmapheresis followed by intravenous immunoglobulin. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every 6 months and daily low-dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case shows that these antibodies can occur concurrently and cause clinical features, such as both neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody disease, in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or cerebrospinal fluid profile negative for leptomeningeal carcinomatosis.
Hideyuki Takeuchi is an editorial board member of Clinical and Experimental Neuroimmunology.
Myasthenia gravis (MG) is a disease that causes muscle weakness and fatigue due to an abnormality in the neuromuscular junction, which prevents the transmission of information. In the 1970s, it became clear that MG was caused by an autoimmune mechanism, and treatments began to be developed. Most studies on the pathophysiology and treatment of MG have been conducted in adults. However, even in terms of acetylcholine receptor antibodies, which are related to MG pathophysiology, adults and children differ significantly. Moreover, epidemiological studies around the world have revealed significant differences in the prevalence of MG between East Asia, Europe, and the United States. Treatment methods also differ slightly across countries, depending on the differences in pathophysiology and medical practices that have been developed in each region of the world. Japanese pediatric neurologists have revealed a pathophysiology unique to children and differences in treatment methods for childhood MG. However, few studies have systematically reported these findings. Therefore, this article reviews the current basic concepts related to the pathophysiology and treatment of childhood MG in Japan.
Clinical and Experimental NeuroimmunologyEarly View LETTER TO THE EDITOR All differential diagnoses of cerebellar ataxia should be ruled out before SARS-CoV-2 is blamed as the cause Josef Finsterer, Corresponding Author Josef Finsterer fifigs1@yahoo.de orcid.org/0000-0003-2839-7305 Neurology & Neurophysiology Center, Vienna, Austria Correspondence Josef Finsterer, Neurology & Neurophysiology Center, Postfach 20, 1180, Vienna, Austria. Email: fifigs1@yahoo.deSearch for more papers by this author Josef Finsterer, Corresponding Author Josef Finsterer fifigs1@yahoo.de orcid.org/0000-0003-2839-7305 Neurology & Neurophysiology Center, Vienna, Austria Correspondence Josef Finsterer, Neurology & Neurophysiology Center, Postfach 20, 1180, Vienna, Austria. Email: fifigs1@yahoo.deSearch for more papers by this author First published: 08 December 2022 https://doi.org/10.1111/cen3.12737Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation
