儿童抗接触相关蛋白2抗体自身免疫性脑炎1例并文献复习
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摘要
目的探讨抗接触相关蛋白2(Caspr2)抗体自身免疫性脑炎的临床特点。方法回顾1例Caspr2抗体脑炎患儿的临床资料,并复习相关文献。结果女性患儿,5岁,表现为近期记忆力下降、易动怒、认知功能受损、幻觉、睡眠紊乱等;血清Caspr2抗体阳性,除外其他疾病后诊断Caspr2抗体脑炎。文献检索到资料完整的Caspr2抗体脑炎50例,男43例、女7例,起病年龄最小为本例患儿5岁,另有1例8岁,其余均为成人起病;边缘性脑炎32例(62. 75%),莫旺综合征19例(37. 25%);最常见的临床症状是记忆受损,癫痫发作,精神症状如焦虑、淡漠、易激惹、幻觉或妄想、周围神经高兴奋性、小脑共济失调及自主神经紊乱等;痊愈或好转44例(86.27%),合并肿瘤8例(15.69%),复发8例(15.69%)。结论 Caspr 2抗体脑炎比较少见尤其是儿童罕见,如患者有边缘性脑炎症状,且合并周围神经高兴奋性、共济失调、自主神经症状等需考虑Caspr2抗体脑炎可能。
Objective To explore the clinical features of anti-Caspr2 antibody-associated encephalitis. Method The clinical data of anti-Caspr2 antibody-associated encephalitis in a child were retrospectively reviewed. Results A 5-year-old girl manifested as recent memory loss, irritability, cognitive impairment, hallucination, sleep disorders, and so on. The child had positive serum Caspr2 antibody, and was diagnosed with Caspr2 antibody encephalitis after exclusion of other diseases. The literature search retrieved 50 cases of Caspr2 antibody encephalitis with complete data, including 43 male cases and 7 female cases. The minimum onset age of the disease was 5 years in this case. In addition, 1 patient developed the disease at the age of 8, while the rest developed in adulthood. There were 32 cases(62.75%) of marginal encephalitis and 19 cases(37.25%) of morvan's syndrome. The most common clinical symptoms were impaired memory, epileptic seizures, and psychiatric symptoms such as anxiety, apathy, irritability, hallucinations or delusions, peripheral nerve hyperexcitability(PNH), cerebellar ataxia and autonomic nervous disorders. Forty-four cases(86.27%) recovered or improved, 8 cases(15.69%) were complicated with tumors and 8 cases(15.69%) relapsed. Conclusion Caspr2 antibody encephalitis is relatively rare, especially in children. If the patient has marginal encephalitis symptoms, combining with PNH, ataxia and autonomic nerve symptoms, the possibility of Caspr2 antibody encephalitis should be considered.
Objective To explore the clinical features of anti-Caspr2 antibody-associated encephalitis. Method The clinical data of anti-Caspr2 antibody-associated encephalitis in a child were retrospectively reviewed. Results A 5-year-old girl manifested as recent memory loss, irritability, cognitive impairment, hallucination, sleep disorders, and so on. The child had positive serum Caspr2 antibody, and was diagnosed with Caspr2 antibody encephalitis after exclusion of other diseases. The literature search retrieved 50 cases of Caspr2 antibody encephalitis with complete data, including 43 male cases and 7 female cases. The minimum onset age of the disease was 5 years in this case. In addition, 1 patient developed the disease at the age of 8, while the rest developed in adulthood. There were 32 cases(62.75%) of marginal encephalitis and 19 cases(37.25%) of morvan's syndrome. The most common clinical symptoms were impaired memory, epileptic seizures, and psychiatric symptoms such as anxiety, apathy, irritability, hallucinations or delusions, peripheral nerve hyperexcitability(PNH), cerebellar ataxia and autonomic nervous disorders. Forty-four cases(86.27%) recovered or improved, 8 cases(15.69%) were complicated with tumors and 8 cases(15.69%) relapsed. Conclusion Caspr2 antibody encephalitis is relatively rare, especially in children. If the patient has marginal encephalitis symptoms, combining with PNH, ataxia and autonomic nerve symptoms, the possibility of Caspr2 antibody encephalitis should be considered.
引文
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[6]Lancaster E,Huijbers MG,Bar V,et al.Investigations of caspr2,an autoantigen of encephalitis and neuromyotonia[J].Ann Neurol,2011,69(2):303-311.
[7]Joubert B,Gobert F,Thomas L,et al.Autoimmune episodic ataxia in patients with anti-CASPR2 antibodyassociated encephalitis[J].Neurol Neuroimmunol Neuroinflamm,2017,4(4):e371.
[8]GaliéE,Renna R,Plantone D,et al.Paraneoplastic Morvan's syndrome following surgical treatment of recurrent thymoma:a case report[J].Oncol Lett,2016,12(4):2716-2719.
[9]G?vert F,Witt K,Erro R,et al.Orthostatic myoclonus associated with Caspr2 antibodies[J].Neurology,2016,86(14):1353-1355.
[10]Demirbas S,Aykan MB,Zengin H,et al.Morvan syndrome:a rare cause of syndrome of inappropriate antidiuretic hormone secretion[J].Clujul Med,2017,90(3):353-355.
[11]何妮,田婧,张萍,等.以Morvan综合征为表现的Caspr2抗体阳性自免脑炎1例报告[J].中风与神经疾病杂志,2017,34(7):648.
[12]Lee CH,Lin JJ,Lin KJ,et al.Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma[J].J Neurol Sci,2014,341(1-2):36-40.
[13]Joubert B,Saint-Martin M,Noraz N,et al.Characterization of a subtype of autoimmune encephalitis with anti-contactinassociated protein-like 2 antibodies in the cerebrospinal fluid,prominent limbic symptoms,and seizures[J].JAMA Neurol,2016,73(9):1115-1124.
[14]Sundal C,Vedeler C,Miletic H,et al.Morvan syndrome with Caspr2 antibodies.Clinical and autopsy report[J].J Neurol Sci,2017,372:453-455.
[15]Sharma AK,Kaur M,Paul M.Morvan's syndrome with anti contactin associated protein like 2-voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion[J].J Neurosci Rural Pract,2016,7(4):577-579.
[16]Sunwoo JS,Lee ST,Byun JI,et al.Clinical manifestations of patients with CASPR2 antibodies[J].J Neuroimmunol,2015,281:17-22.
[17]Vale TC,Pedroso JL,Dutra LA,et al.Morvan syndrome as a paraneoplastic disorder of thymoma with anti-CASPR2antibodies[J].Lancet,2017,389(10076):1367-1368.
[18]Kim SY,Choi SA,Ryu HW,et al.Screening autoimmune anti-neuronal antibodies in pediatric patients with suspected autoimmune encephalitis[J].J Epilepsy Res,2014,4(2):55-61.
[19]陈彬,牛松涛,张佳明,等.以周围神经病首发的接触蛋白相关蛋白-2抗体阳性患者一例[J].中华神经科杂志,2016,49(8):644-646.
[20]郝红琳,李胜德,孙鹤阳,等.莫旺综合征睡眠障碍临床分析[J].中华神经科杂志,2017,50(8):590-593.
[21]Buckley C,Oger J,Clover L,et al.Potassium channel antibodies in two patients with reversible limbic encephalitis[J].Ann Neurol,2001,50(1):73-78.
[22]Irani SR,Alexander S,Waters P,et al.Antibodies to Kv1potassium channel-complex proteins leucine-rich,glioma inactivated 1 protein and contactin-associated protein-2in limbic encephalitis,Morvan's syndrome and acquired neuromyotonia[J].Brain,2010,133(9):2734-2748.
[23]van Sonderen A,Ari?o H,Petit-Pedrol M,et al.The clinical spectrum of Caspr2 antibody-associated disease[J].Neurology,2016,87(5):521-528.
[24]Irani SR,Pettingill P,Kleopa KA,et al.Morvan syndrome:clinical and serological observations in 29 cases[J].Ann Neurol,2012,72(2):241-255.
[25]中华医学会神经病学分会.中国自身免疫性脑炎诊治专家共识[J].中华神经科杂志,2017,50(2):91-98.
[26]Dahm L,Ott C,Steiner J,et al.Seroprevalence of autoantibodies against brain antigens in health and disease[J].Ann Neurol,2014,76(1):82-94.
[2]Ueno S,Hirano M,Sakamoto H,et al.Eyelid tremor in a patient with anti-caspr2 antibody-related encephalitis[J].Case Rep Neurol,2014,6(2):222-225.
[3]K?rtvelyessy P,Bauer J,Stoppel CM,et al.Complementassociated neuronal loss in a patient with CASPR2 antibody-associated encephalitis[J].Neurol Neuroimmunol Neuroinflamm,2015,2(2):e75.
[4]Schr?der O,Schriewer E,Golombeck KS,et al.Impaired autonomic responses to emotional stimuli in autoimmune limbic encephalitis[J].Front Neurol,2015,6:250.
[5]耿张燕,李艳敏,王铭维.接触蛋白相关蛋白-2抗体阳性的自免脑炎1例报告[J].中风与神经疾病杂志,2018,35(1):63-64.
[6]Lancaster E,Huijbers MG,Bar V,et al.Investigations of caspr2,an autoantigen of encephalitis and neuromyotonia[J].Ann Neurol,2011,69(2):303-311.
[7]Joubert B,Gobert F,Thomas L,et al.Autoimmune episodic ataxia in patients with anti-CASPR2 antibodyassociated encephalitis[J].Neurol Neuroimmunol Neuroinflamm,2017,4(4):e371.
[8]GaliéE,Renna R,Plantone D,et al.Paraneoplastic Morvan's syndrome following surgical treatment of recurrent thymoma:a case report[J].Oncol Lett,2016,12(4):2716-2719.
[9]G?vert F,Witt K,Erro R,et al.Orthostatic myoclonus associated with Caspr2 antibodies[J].Neurology,2016,86(14):1353-1355.
[10]Demirbas S,Aykan MB,Zengin H,et al.Morvan syndrome:a rare cause of syndrome of inappropriate antidiuretic hormone secretion[J].Clujul Med,2017,90(3):353-355.
[11]何妮,田婧,张萍,等.以Morvan综合征为表现的Caspr2抗体阳性自免脑炎1例报告[J].中风与神经疾病杂志,2017,34(7):648.
[12]Lee CH,Lin JJ,Lin KJ,et al.Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma[J].J Neurol Sci,2014,341(1-2):36-40.
[13]Joubert B,Saint-Martin M,Noraz N,et al.Characterization of a subtype of autoimmune encephalitis with anti-contactinassociated protein-like 2 antibodies in the cerebrospinal fluid,prominent limbic symptoms,and seizures[J].JAMA Neurol,2016,73(9):1115-1124.
[14]Sundal C,Vedeler C,Miletic H,et al.Morvan syndrome with Caspr2 antibodies.Clinical and autopsy report[J].J Neurol Sci,2017,372:453-455.
[15]Sharma AK,Kaur M,Paul M.Morvan's syndrome with anti contactin associated protein like 2-voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion[J].J Neurosci Rural Pract,2016,7(4):577-579.
[16]Sunwoo JS,Lee ST,Byun JI,et al.Clinical manifestations of patients with CASPR2 antibodies[J].J Neuroimmunol,2015,281:17-22.
[17]Vale TC,Pedroso JL,Dutra LA,et al.Morvan syndrome as a paraneoplastic disorder of thymoma with anti-CASPR2antibodies[J].Lancet,2017,389(10076):1367-1368.
[18]Kim SY,Choi SA,Ryu HW,et al.Screening autoimmune anti-neuronal antibodies in pediatric patients with suspected autoimmune encephalitis[J].J Epilepsy Res,2014,4(2):55-61.
[19]陈彬,牛松涛,张佳明,等.以周围神经病首发的接触蛋白相关蛋白-2抗体阳性患者一例[J].中华神经科杂志,2016,49(8):644-646.
[20]郝红琳,李胜德,孙鹤阳,等.莫旺综合征睡眠障碍临床分析[J].中华神经科杂志,2017,50(8):590-593.
[21]Buckley C,Oger J,Clover L,et al.Potassium channel antibodies in two patients with reversible limbic encephalitis[J].Ann Neurol,2001,50(1):73-78.
[22]Irani SR,Alexander S,Waters P,et al.Antibodies to Kv1potassium channel-complex proteins leucine-rich,glioma inactivated 1 protein and contactin-associated protein-2in limbic encephalitis,Morvan's syndrome and acquired neuromyotonia[J].Brain,2010,133(9):2734-2748.
[23]van Sonderen A,Ari?o H,Petit-Pedrol M,et al.The clinical spectrum of Caspr2 antibody-associated disease[J].Neurology,2016,87(5):521-528.
[24]Irani SR,Pettingill P,Kleopa KA,et al.Morvan syndrome:clinical and serological observations in 29 cases[J].Ann Neurol,2012,72(2):241-255.
[25]中华医学会神经病学分会.中国自身免疫性脑炎诊治专家共识[J].中华神经科杂志,2017,50(2):91-98.
[26]Dahm L,Ott C,Steiner J,et al.Seroprevalence of autoantibodies against brain antigens in health and disease[J].Ann Neurol,2014,76(1):82-94.