A Clinical Study Of 40 Cases With Paraneoplastic Neurological Syndromes

ObjectiveTo explore the clinical features and prognosis of paraneoplastic neurological syndromes.MethodCollected the clinical and follow-up data of 40 patients who were diagnosed with definite PNS(diagnosis with 2004 Graus diagnostic criteria)in the Department of Neurology of the Second Affiliated Hospital of Zhejiang University between February 2014 and October 2019.Analyzed the clinical features and prognosisResultThe median age of onset was 61 years(in the range of 29-81 years),and the male to female ratio was 1.86:1.Myasthenia and paresthesia is common first symptom.There were 25 patients presenting with classical syndromes with limbic encephalitis and subacute cerebellar degeneration being common,and 15 patients presenting with non-classical syndrome,with subacute/chronic sensorimotor neuropathy being the most common.Tumor occurrence rate in patients with classical syndromes was higher than those with non-classical syndromes(P=0.033).Patients with central nervous system PNS are more common in male(P=0.027),and often present with classic syndromes.While patients with peripheral nervous system PNS are more likely present with non-classical syndromes(P<0.0005).Of the 40 patients,33 had PNS-related antibodies,of which 30 were well-characteristic antibodies.Anti-Hu antibodies is the most common.Among the 40 patients,there were 22 patients diagnosed with tumor,of which lung cancer was the most common,and the pathological type was mainly small cell cancer.Of the 37 patients with complete follow-up data,21 had good short-term prognosis,especially among patients receiving immunotherapy or tumor therapy(P=0.028),but the overall long-term prognosis was poor.ConclusionPNS mainly occurs in middle-aged and elderly people,myasthenia and paresthesia is common first symptom,limbic encephalitis,subacute cerebellar degeneration and subacute/chronic sensorimotor neuropathy being the common syndromes.Anti-Hu antibody is the most common and small cell lung cancer is the most common associated tumor.Neurological symptoms mostly occur before tumors are found.Compared with the involvement of the central nervous system,there is no obvious clinical manifestation when the peripheral nervous system is involved,so other causes should be thoroughly investigated when su specting the PNS diagnosis.Early administration of immunotherapy or tumor therapy can improve the short-term prognosis and the quality of life,but the overall long-term prognosis of patients with PNS is poor.