| Objective: Paraneoplastic neurological syndrome(PNS)are rare complications of cancer caused by an immune cross-reaction between antigens expressed by tumor cells and neurons,about 70% of PNS patients can appear when they lack the clinical manifestations of primary tumors.This study retrospectively analyzed the clinical data of 26 patients with PNS,summarized the clinical characteristics of PNS in the Han population in Hebei region,in order to deepen clinicians’ understanding of PNS,provide a basis for early diagnosis and treatment of tumors,and lay a foundation for further research on PNS.Methods: In this study,clinical data of 26 patients with PNS were collected and analyzed retrospectively,which including general information,clinical manifestation,tumor screening,laboratory tests,cerebrospinal fluid examination,neural electrophysiological examination,imaging examination,treatment method,the modified Rankin scale score before and after the treatment,and follow-up of patients survival.Results: Among the 26 patients with PNS,57.7% were males and 42.3% were females.The median age of onset was 61 years old.The most common clinical syndromes were limbic encephalitis(6 cases,23.1%)and subacute cerebellar degeneration(6 cases,23.1%).The tumor incidence rate was 96.2%(25/26),and small cell lung cancer(SCLC)was 48%(12/25).11 patients(44%,11/25)had neurological symptoms prior to tumor diagnosis,in 14 cases(56%,14/24),neurological symptoms appeared after the tumor was diagnosed,and PNS occurred on average 4 months after tumor diagnosis.The positive rate of antibody was 77.8%(14/18),and the highest positive rate of Hu was 50%(7/14).The positive rate of tumor markers was 73.1%,and the tumor markers with the highest positive rate were NSE,PROGRP,and CYFRA,respectively.There was no statistically significant difference in tumor incidence between the positive group and the negative group(P > 0.05).D-dimer was higher than normal in 12 cases.Cerebrospinal fluid(CSF)correlation test was performed in 3 patients with limbic encephalitis,2 patients with CSF increased cell number and protein content,1 patient with CSF routine and biochemical abnormalities,and 1 patient with positive serum and CSF anti-NMDAR antibody.The dynamic electroencephalogram of a patient with limbic encephalitis showed that diffuse slow waves were observed in each guide during the waking period,and no obvious epileptic discharge was observed during the waking and sleeping periods.Cranial MRI in 6 patients with limbic encephalitis showed abnormal signals in 2 patients and no abnormal signals in the remaining 4 patients.Electromyogram in 3 patients with peripheral neuropathy indicated peripheral nerve damage,electromyogram in 3 patients with dermatomyositis showed myogenic damage,and in 1 patient with LEMS,the compound muscle action potential amplitude increased by more than 100% during high-frequency(50Hz)stimulation.The difference of m RS score before and after treatment was significant(P=0.017).After treatment,the clinical symptoms of the patients were significantly relieved,with an effective rate of 69.2%(18/26).There was no statistical difference in the effective rate between the positive and negative groups(P > 0.05),and the median survival time of PNS patients was 3.25 years.Conclusions:1.PNS is subacute or chronic,and the most common clinical symptoms are limbic encephalitis and subacute cerebellar degeneration.2.SCLC was the tumor type with the highest incidence in PNS patients,and Hu antibody was the antibody type with the highest detection rate.3.Whether the tumor markers are abnormal or not,systemic tumor screening should be conducted.For the patients whose tumor is not found in the initial screening,regular screening and close follow-up should be conducted.If there is an unknown reason for D-dimer increase,tumor screening should also be conducted to avoid missed diagnosis.4.Antitumor therapy,or immunotherapy,symptomatic support therapy can improve the patient’s clinical symptoms,prolong the survival period. |
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