AQP4‐IgG positive paraneoplastic NMOSD: A case report and review. Issue 10 (14th September 2021)

• Record Type: Journal Article
• Authors: Ding, Manqiu; Lang, Yue; Cui, Li
• Is Part Of: Brain and behavior. Volume 11:Issue 10(2021)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2021-09-14
• Subjects: AQP4 -- NMOSD -- paraneoplastic; Neurology -- Periodicals; Neurosciences -- Periodicals; Psychology -- Periodicals; Psychiatry -- Periodicals; 616.8005
• DOI: 10.1002/brb3.2282
• Languages: English
• ISSNs: 2162-3279

Abstract

Abstract: Introduction: Neuromyelitis optica spectrum disorder (NMOSD; also known as Devic syndrome) is a clinical syndrome of central nervous system characterized by immune mediated attacks of acute optic neuritis and myelitis. Paraneoplastic neurological syndrome is a group of nervous system disorders resulting from the remote immune effects of malignant neoplasm. NMOSD occurs mostly in young people, and tumor is not a common cause, especially recurrent tumor. Methods: We reported a case of a 59‐year‐old man who developed anti‐aquaporin‐4 IgG positive longitudinally extensive myelitis. We also summarized and analyzed previously reported cases of paraneoplastic NMOSD. Results: Among these 43 patients, 88.4% patients are female. The largest number of patients is between 60 and 69 years old. Breast cancer and lung cancer are the most common types. The most common lesions were located in the cervicothoracic region with patchy gadolinium enhancement. The existing treatment can only delay rather than stop the progress of the disease. Conclusion: It is necessary to perform tumor screening in patients with NMOSD, especially patients over 50 years. Abstract : Wereported a rare case of AQP4‐IgG positive paraneoplastic NMOSD with recurrent rectal cancer. We summarized the previously reported cases of paraneoplastic NMOSD from demographics, clinical features, neuroimaging findings, laboratory examination, therapy, outcome, and other aspects and highlighted the necessity to perform tumor screening in patients with NMOSD, especially patients over 50 years. We analyzed the pathogenesis of AQP4‐IgG positive paraneoplastic NMOSD and discussed the phenomenon of paraneoplastic NMOSD with intractable hyponatremia.