Clinical Analysis Of 19 Children With Paraneoplastic Neurological Syndrome

Objective: By retrospectively analyzing the clinical characteristics of children with paraneoplastic neurological syndrome,raise awareness of this syndrome,reduce misdiagnosis and wrong treatment,and improve prognosis.Methods:Retrospective analysis of clinical information of 19 children with PNS that admitted to the Children’s Hospital of Chongqing Medical University from January 2010 to December 2019,and follow up all children.Results:1.In our study,15 patients(78.95%)were female and 4 children(21.05%)were male.11 cases(57.89%)were 1-3 years old(including 3years old),5 cases(26.32%)were 3 years old to 6 years old(including 6years old),3 cases(15.79%)were over 6 years old.The primary tumors in19 children were neuroblastic tumors(12/19,63.16%),acute lymphoblastic leukemia(4/19,21.05%),teratomas(2/19,10.53%),T Cellular lymphoblastoma(1/19,5.26%).2.The clinical manifestations of PNS in children are diverse,and 14patients(73.68%)have neurological symptoms as the initial manifestation.The main clinical manifestations are ataxia(10/19),weakness of the lower limbs(6/19),convulsions(4/19),nystagmus(2/19),headache(1/19),Character changes(1/19),disturbance of consciousness(2/19),sleep disorder(1/19),articulation disorder(1/19),peripheral facial paralysis(1/19).4 patients(21.05%)had been misdiagnosed.2 cases(10.53%)of classical syndromes in children with PNS: ocular-myoclonus syndrome and paraneoplastic cerebellar ataxia,.3.15 PNS related to solid tumors,CT scans of the chest or abdomen revealed tumor-occupying lesions;4 PNS related to acute lymphoblastic leukemia were confirmed by bone marrow examination.13 patients had no obvious abnormalities on the head MRI;11 patients The child had a complete cerebrospinal fluid examination.There were not any abnormalities in 8 cases and abnormalities in 3 cases.The abnormalities were: slightly elevated CSF protein,slightly elevated leukocytes with positive anti-glutamate receptor(NMDA type)antibody.Statistical analysis found that the primary tumors were neuroblastoma-derived tumors,acute lymphoblastic leukemia,teratomas,and T-cell lymphoblastoma-related CT scans of the chest and abdomen(p <0.001)The difference in tumor detection rate between abdominal color Doppler ultrasound(p = 0.042)and bone marrow aspiration(p = 0.001)was statistically significant.4.In 17 patients with PNS who were successfully followed up,after primary tumor treatment,the neurological symptoms of 13 children were relieved;2 patients(11.76%)had leftover neurological dysfunction and 1patient was neuroblastoma-related Of the children with OMS,1 patient with acute lymphoblastic leukemia-related PNS;2 patients(11.76%)died with neuroblastoma-derived tumors.Through statistical analysis,the prognosis of 10 PNS patients with neuroblastic tumors successfully followed up was correlated with tumor stage(p = 0.016).Conclusion:In our study,PNS is more prevalent in 1-3 year-old children,and there are more female patients.The primary tumors causing paraneoplastic syndrome of children’s nervous system in this group are neuroblastoma-derived tumors,acute lymphoblastic leukemia,teratomas,and T-cell lymphoblastomas.The abnormalities of the nervous system are mostly the first manifestations,and the clinical manifestations are diverse.Children with ataxia and lower limb weakness / lame should be alert to the possibility of PNS.Active treatment of primary tumors in children with PNS,most of the children’s neurological symptoms can be relieved spontaneously,a small number of children can be left with neurological dysfunction or even death.