BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CAS...BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.展开更多
Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic ...Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic diseases,and they may affect any part of the nervous system.The simultaneous involvement of different areas of the nervous system by the paraneoplastic process is not unusual.Until date,this is the first report of concurrent development of paraneoplastic cerebellar degeneration （PCD） and paraneoplastic limbic encephalitis （PLE） associated with the advanced ovarian cancer and anti-Yo antibodies following hepatitis B （HB） vaccination.The cause of most PNS is believed to be an immune response against neuronal proteins expressed by the tumor.展开更多
Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception...Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception, mood and behavioral changes, sleep disturbances, and seizures. Despite the growing number of cases being reported, it still poses a diagnostic challenge. We encountered a patient with a myriad of neuropsychiatric symptoms who exhibited a highly variable response to therapy. A 36-year-old male presented with memory impairment, excessive sleepiness, and slurred speech. Brain magnetic resonance imaging revealed hyperintensities in the temporal lobes and hypothalamus, all suggestive of limbic encephalitis. He was found to have a mixed germ cell testicular teratoma. Screening for commonly associated antibodies did not yield positive results, which emphasizes that sero-negative PLE can be missed in patients with malignancies. In reporting this case, we urge neurologists to consider PLE as part of the differential diagnosis in similar ambiguous clinical scenarios.展开更多
Objective To probe into the pathogenic mechanisms of paraneoplastic limbic encephali- tis( PL E) in patients with small cell lung carcinoma( SCLC) .Methods The indirect im- munoperoxidase method and Western blotanalys...Objective To probe into the pathogenic mechanisms of paraneoplastic limbic encephali- tis( PL E) in patients with small cell lung carcinoma( SCLC) .Methods The indirect im- munoperoxidase method and Western blotanalysis were used for detecting anti- Hu antibodies in1 6 PLE patients associated with SCL C.Autopsy and pathological study were performed on two cases.Results Eight patients( 5 0 % ) had anti- Hu antibodies( anti- Hu+) whereas eight patients ( 5 0 % ) no detectable antineuronal antibodies ( anti- Hu- ) .The clinical and laboratory features of PLE and time to diagnosis of SCLC were similar in the anti- Hu+and anti- Hu- groups.Involvement of other areas of the nervous system in seven( 87.5 % ) patients of the anti- Hu+group but in only one ( 1 2 .5 ) % of the anti- Hu- group ( P=0 .0 1 2 ) . Conclusions The presence of this characteristic neurological disorder strongly suggested that with an ac- companying SCLC,existence of anti- Hu autoantibody was in favour of an autoimmune mech- anism participating in PLE.展开更多
目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例...目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例)和强阳性组(16例),根据CSF抗体情况将患者分为CSF抗体阳性组(22例)和阴性组(87例),根据病程将患者分为病程≤3个月组(76例)和病程>3个月组(42例)。分析各组间年龄以及CSF白细胞计数、总蛋白、IgG指数、24 h IgG鞘内合成率、寡克隆区带(CSF-OCB)和白蛋白商(QAlb)间的差异。结果(1)抗体强阳性组CSF-OCB阳性率和QAlb异常率高于阳性组和弱阳性组(P<0.05)。(2)CSF抗体阳性组CSF白细胞、总蛋白、IgG指数、24 h IgG鞘内合成率和QAlb异常率均高于CSF抗体阴性组(P<0.05)。(3)病程≤3个月组CSF白细胞高于病程>3个月组(P<0.01)。结论副肿瘤特征性抗体阳性患者存在中枢神经系统炎性反应,其发病与体液免疫相关,病程早期CSF白细胞计数增高明显,研究CSF相关指标有助于全面了解患者免疫状况。展开更多
文摘BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.
文摘Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic diseases,and they may affect any part of the nervous system.The simultaneous involvement of different areas of the nervous system by the paraneoplastic process is not unusual.Until date,this is the first report of concurrent development of paraneoplastic cerebellar degeneration （PCD） and paraneoplastic limbic encephalitis （PLE） associated with the advanced ovarian cancer and anti-Yo antibodies following hepatitis B （HB） vaccination.The cause of most PNS is believed to be an immune response against neuronal proteins expressed by the tumor.
文摘Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception, mood and behavioral changes, sleep disturbances, and seizures. Despite the growing number of cases being reported, it still poses a diagnostic challenge. We encountered a patient with a myriad of neuropsychiatric symptoms who exhibited a highly variable response to therapy. A 36-year-old male presented with memory impairment, excessive sleepiness, and slurred speech. Brain magnetic resonance imaging revealed hyperintensities in the temporal lobes and hypothalamus, all suggestive of limbic encephalitis. He was found to have a mixed germ cell testicular teratoma. Screening for commonly associated antibodies did not yield positive results, which emphasizes that sero-negative PLE can be missed in patients with malignancies. In reporting this case, we urge neurologists to consider PLE as part of the differential diagnosis in similar ambiguous clinical scenarios.
文摘Objective To probe into the pathogenic mechanisms of paraneoplastic limbic encephali- tis( PL E) in patients with small cell lung carcinoma( SCLC) .Methods The indirect im- munoperoxidase method and Western blotanalysis were used for detecting anti- Hu antibodies in1 6 PLE patients associated with SCL C.Autopsy and pathological study were performed on two cases.Results Eight patients( 5 0 % ) had anti- Hu antibodies( anti- Hu+) whereas eight patients ( 5 0 % ) no detectable antineuronal antibodies ( anti- Hu- ) .The clinical and laboratory features of PLE and time to diagnosis of SCLC were similar in the anti- Hu+and anti- Hu- groups.Involvement of other areas of the nervous system in seven( 87.5 % ) patients of the anti- Hu+group but in only one ( 1 2 .5 ) % of the anti- Hu- group ( P=0 .0 1 2 ) . Conclusions The presence of this characteristic neurological disorder strongly suggested that with an ac- companying SCLC,existence of anti- Hu autoantibody was in favour of an autoimmune mech- anism participating in PLE.
文摘目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例)和强阳性组(16例),根据CSF抗体情况将患者分为CSF抗体阳性组(22例)和阴性组(87例),根据病程将患者分为病程≤3个月组(76例)和病程>3个月组(42例)。分析各组间年龄以及CSF白细胞计数、总蛋白、IgG指数、24 h IgG鞘内合成率、寡克隆区带(CSF-OCB)和白蛋白商(QAlb)间的差异。结果(1)抗体强阳性组CSF-OCB阳性率和QAlb异常率高于阳性组和弱阳性组(P<0.05)。(2)CSF抗体阳性组CSF白细胞、总蛋白、IgG指数、24 h IgG鞘内合成率和QAlb异常率均高于CSF抗体阴性组(P<0.05)。(3)病程≤3个月组CSF白细胞高于病程>3个月组(P<0.01)。结论副肿瘤特征性抗体阳性患者存在中枢神经系统炎性反应,其发病与体液免疫相关,病程早期CSF白细胞计数增高明显,研究CSF相关指标有助于全面了解患者免疫状况。
