Clinical Features Of Paraneoplastic Neurological Syndrome In Patients With Anti-amphiphysin Antibody Positivity

BackgroundParaneoplastic neurological syndrome(PNS)is a tumor-related neurological disease,not caused by direct tumor invasion,but by a tumor that triggers an immune response,causing a long-range effect,causing acute or subacute damage to the nervous system.So there is a series of neurological syndromes.PNS is relatively rare and shows a variety of clinical manifestations.Common classical manifestations include encephalomyelitis,limbic encephalitis,subacute cerebellar degeneration,opsoclonic-myoclonus,sensory neuronal neuropathy,and dermatomyositis.Most PNS patients show specific anti-neuron autoantibodies,such as anti-Hu,anti-Yo,anti-Ri,anti-CV2,anti-amphiphysin and anti-Ma2,suggesting that there may be some occult tumors when paraneoplastic neurological symptoms occur.The presence of these specific autoantibodies provides a powerful means of detecting early diagnosis of PNS and is essential for the timely detection and eradication of cancer in the host.The amphiphysin antibody is a characteristic antibody in PNS.Since the discovery of this antibody by Lichte et al.in 1992,many case reports and clinical case analyses have described the clinical features of PNS related to the amphiphysin antibody.The typical clinical manifestations are stiff-person syndrome and encephalomyelitis.However,a literature review showed inconsistencies in the results reported in most studies,with common clinical manifestations differing across studies,different studies presenting contradicting and conflicting results,and The number of research cases is small and not representative.Although amphiphysin antibody-positive PNS is more confusing and difficult to diagnose and treat,amphiphysin antibodies are extremely important for the diagnosis of PNS,and more in-depth studies are needed.ObjectiveTo investigate the clinical features,treatment methods,and prognosis of amphiphysin antibody-positive PNS.Methods1.Serum anti-amphiphysin positive patients were screened from suspected PNS patients in several hospitals in Henan Province from 2012 to 2017.The clinical data of the patients were sorted out,and the patients who met the diagnostic criteria of PNS were clinically observed and followed up.2.The disease severity before and after treatment was assessed using a modified Rankin score(mRS).3.Kendall’s tau-b was used to evaluate the relationship between antibody levels and disease severity in the study subjects.4.The kruskal-wallis test was used to study the relationship between the decline of mRS scores in each group.Results1.36 patients with positive amphiphysin antibodies met the diagnostic criteria for PNS.2.Nervous system syndromes included peripheral neuropathy(12 cases;33.3%),encephalomyelitis(9 cases;25%)and other neurological syndromes in 15 cases.3.Amphiphysin antibody levels and disease severity were not significantly correlated(Kendall’s tau-b =-0.014,P = 0.924).4.After treatment,the mRS scores of the tumor resection combined with immunotherapy group were significantly lower than those of the symptomatic treatment group,and the difference was statistically significant(P<0.05).Conclusion1.The most common anti-amphiphysin related neurological syndrome is peripheral neuropathy and encephalomyelitis.2.There is no significant correlation between the intensity of amphiphysin antibodies and the severity of neurological symptoms.3.Tumor resection and immunotherapy can improve the patient’s symptoms.