Opsoclonus-myoclonus syndrome (OMS) is an autoimmune disorder with serious neurodevelopmental morbidity and limited treatment options. We treated a toddler with moderately severe OMS with rituximab, a monoclonal anti-...Opsoclonus-myoclonus syndrome (OMS) is an autoimmune disorder with serious neurodevelopmental morbidity and limited treatment options. We treated a toddler with moderately severe OMS with rituximab, a monoclonal anti-B cell antibody. The patient’s clinical response was documented on videotape and scored with the OMS Evaluation Scale. Cerebrospinal fluid lymphocyte subsets were evaluated by flow-cytometric immunophenotyping, with a comprehensive panel of monoclonal antibodies. Eradication of cerebrospinal fluid B cells, which previously were expanded, was associated with dramatic clinical improvement. There also were secondary changes in other lymphocyte subsets that might be relevant to the clinical response and lack of serious infections. In addition to clarifying the immune response to B-cell depletion, these data reveal a promising new therapy for OMS that warrants a phase I clinical trial.展开更多
Objectives: Parents of children with opsoclonus-myoclonus syndrome (OMS) frequently describe poor sleep and rage attacks. We hypothesized that these manifestations are related and could result from underlying monoamin...Objectives: Parents of children with opsoclonus-myoclonus syndrome (OMS) frequently describe poor sleep and rage attacks. We hypothesized that these manifestations are related and could result from underlying monoaminergic dysfunction. Study design: We clinically characterized the sleep and behavioral characteristics of 51 young children with OMS; 19 of those with the most disruptive sleep patterns were treated with trazodone, a soporific serotonergic agent. Results: Sleep disturbances, including prolonged sleep latency, fragmented sleep, reduced quantity of sleep, snoring, and nonrestorative sleep, were reported in 32 children, and frequent rage attacks were reported in 25. In 59% of the poor sleepers, parents felt that the problem was severe enough to warrant treatment. Children sleeping < 10 hours/night had a higher rage frequency than those who slept more. Of the children who required trazodone, 84% were receiving corticosteroids or adrenocorticotropic hormone (corticotrophin), compared with 37% in the subgroup with normal sleep. Trazodone (3.0 ± 0.4 mg/kg/day) improved sleep and behavior in 95% of the children, significantly increasing total sleep time by 72% , decreasing the number of awakenings by 76% , and reducing rage attacks by 33% . Conclusions: Children with OMS exhibited multiple types of sleep disturbances, which contributed to rage attacks. Trazodone was effective in improving sleep and decreasing rage attacks and was well tolerated, even in toddlers.展开更多
周期性肢体运动（periodic legs movement of sleep，PLMS）又称夜间肌阵挛综合征。其特征为睡眠中反复发生的一侧或双侧下肢腿和脚的周期性运动，即睡眠中反复发生的下肢肌肉痉挛。该运动主要发生在非快速眼动睡眠中，发作时表现为足...周期性肢体运动（periodic legs movement of sleep，PLMS）又称夜间肌阵挛综合征。其特征为睡眠中反复发生的一侧或双侧下肢腿和脚的周期性运动，即睡眠中反复发生的下肢肌肉痉挛。该运动主要发生在非快速眼动睡眠中，发作时表现为足部背屈、大拇趾伸直，在膝部和髋部常有腿的屈曲，踝、膝、髋的三步屈曲运动平均持续时间为1．5—2．5s。上肢也能发生类似的运动，但非常少见。关于PLMS对脑卒中偏瘫患者康复预后的影响，国内相关文献较少，尚未见系统的研究报道。本研究对我院2年来收治的22例脑卒中偏瘫伴PLMS患者给予抗PLMS药物辅助其康复治疗，并与进行常规康复治疗的患者和不伴PLMS的脑卒中偏瘫患者进行比较，现报道如下。展开更多
目的总结儿童眼球阵挛-肌阵挛-共济失调综合征(OMAS)患儿的临床及预后特征。方法对2015年6月至2023年6月于北京儿童医院神经内科住院的46例OMAS患儿进行回顾性病例分析。并在2023年6至8月对患儿进行集中的互联网诊疗或电话访视。收集患...目的总结儿童眼球阵挛-肌阵挛-共济失调综合征(OMAS)患儿的临床及预后特征。方法对2015年6月至2023年6月于北京儿童医院神经内科住院的46例OMAS患儿进行回顾性病例分析。并在2023年6至8月对患儿进行集中的互联网诊疗或电话访视。收集患儿住院和门诊随访期间的病例资料,包括临床表现、辅助检查、治疗及预后情况等。根据患儿是否合并肿瘤分为两组,组间比较采用χ^(2)检验或Mann-Whitney U检验。使用单因素Logistic回归分析OMAS复发和预后相关因素。结果46例患儿中男25例,起病年龄1.5(1.2,2.4)岁。26例(57%)患儿病程中确诊神经母细胞瘤,无患儿高危。36例(78%)患儿随访≥6个月,这36例患儿均使用糖皮质激素、丙种球蛋白和(或)促肾上腺皮质激素作为一线治疗,其中9例(25%)使用二线治疗≥3个月(包括利妥昔单抗和环磷酰胺),17例(47%)使用神经母细胞瘤相关的化疗;随访4.2(2.2,5.5)年,10例(28%)患儿出现OMAS复发。36例患儿末次随访时Mitchell and Pike OMS评分量表评分为0.5(0,2.0)分,7例(19%)认知轻度落后于同龄儿,6例(17%)重度落后。仅1例患儿在随访中出现肿瘤复发。未合并肿瘤组起病前疫苗接种或感染病史较合并肿瘤组更多见[55%(11/20)比23%(6/26),χ^(2)=4.95,P=0.026],首发症状肌阵挛出现更频繁[40%(8/20)比4%(1/26),χ^(2)=7.23,P=0.007]。Logistic单因素回归分析发现,合并肿瘤组比未合并肿瘤组更少出现复发[OR=0.19(0.04~0.93),P=0.041],病程6个月内使用二线治疗或化疗预后更好[OR=11.64(1.27~106.72),P=0.030]。结论儿童OMAS多于幼儿期起病,约半数合并神经母细胞瘤。合并OMAS的神经母细胞瘤通常危险分级低,预后好。对比合并和未合并肿瘤的OMAS患儿,后者起病前感染或接种疫苗诱因更常见,首发症状肌阵挛更多见。早期加用二线治疗与OMAS更好的预后相关。展开更多
文摘Opsoclonus-myoclonus syndrome (OMS) is an autoimmune disorder with serious neurodevelopmental morbidity and limited treatment options. We treated a toddler with moderately severe OMS with rituximab, a monoclonal anti-B cell antibody. The patient’s clinical response was documented on videotape and scored with the OMS Evaluation Scale. Cerebrospinal fluid lymphocyte subsets were evaluated by flow-cytometric immunophenotyping, with a comprehensive panel of monoclonal antibodies. Eradication of cerebrospinal fluid B cells, which previously were expanded, was associated with dramatic clinical improvement. There also were secondary changes in other lymphocyte subsets that might be relevant to the clinical response and lack of serious infections. In addition to clarifying the immune response to B-cell depletion, these data reveal a promising new therapy for OMS that warrants a phase I clinical trial.
文摘Objectives: Parents of children with opsoclonus-myoclonus syndrome (OMS) frequently describe poor sleep and rage attacks. We hypothesized that these manifestations are related and could result from underlying monoaminergic dysfunction. Study design: We clinically characterized the sleep and behavioral characteristics of 51 young children with OMS; 19 of those with the most disruptive sleep patterns were treated with trazodone, a soporific serotonergic agent. Results: Sleep disturbances, including prolonged sleep latency, fragmented sleep, reduced quantity of sleep, snoring, and nonrestorative sleep, were reported in 32 children, and frequent rage attacks were reported in 25. In 59% of the poor sleepers, parents felt that the problem was severe enough to warrant treatment. Children sleeping < 10 hours/night had a higher rage frequency than those who slept more. Of the children who required trazodone, 84% were receiving corticosteroids or adrenocorticotropic hormone (corticotrophin), compared with 37% in the subgroup with normal sleep. Trazodone (3.0 ± 0.4 mg/kg/day) improved sleep and behavior in 95% of the children, significantly increasing total sleep time by 72% , decreasing the number of awakenings by 76% , and reducing rage attacks by 33% . Conclusions: Children with OMS exhibited multiple types of sleep disturbances, which contributed to rage attacks. Trazodone was effective in improving sleep and decreasing rage attacks and was well tolerated, even in toddlers.
文摘周期性肢体运动（periodic legs movement of sleep，PLMS）又称夜间肌阵挛综合征。其特征为睡眠中反复发生的一侧或双侧下肢腿和脚的周期性运动，即睡眠中反复发生的下肢肌肉痉挛。该运动主要发生在非快速眼动睡眠中，发作时表现为足部背屈、大拇趾伸直，在膝部和髋部常有腿的屈曲，踝、膝、髋的三步屈曲运动平均持续时间为1．5—2．5s。上肢也能发生类似的运动，但非常少见。关于PLMS对脑卒中偏瘫患者康复预后的影响，国内相关文献较少，尚未见系统的研究报道。本研究对我院2年来收治的22例脑卒中偏瘫伴PLMS患者给予抗PLMS药物辅助其康复治疗，并与进行常规康复治疗的患者和不伴PLMS的脑卒中偏瘫患者进行比较，现报道如下。
文摘目的总结儿童眼球阵挛-肌阵挛-共济失调综合征(OMAS)患儿的临床及预后特征。方法对2015年6月至2023年6月于北京儿童医院神经内科住院的46例OMAS患儿进行回顾性病例分析。并在2023年6至8月对患儿进行集中的互联网诊疗或电话访视。收集患儿住院和门诊随访期间的病例资料,包括临床表现、辅助检查、治疗及预后情况等。根据患儿是否合并肿瘤分为两组,组间比较采用χ^(2)检验或Mann-Whitney U检验。使用单因素Logistic回归分析OMAS复发和预后相关因素。结果46例患儿中男25例,起病年龄1.5(1.2,2.4)岁。26例(57%)患儿病程中确诊神经母细胞瘤,无患儿高危。36例(78%)患儿随访≥6个月,这36例患儿均使用糖皮质激素、丙种球蛋白和(或)促肾上腺皮质激素作为一线治疗,其中9例(25%)使用二线治疗≥3个月(包括利妥昔单抗和环磷酰胺),17例(47%)使用神经母细胞瘤相关的化疗;随访4.2(2.2,5.5)年,10例(28%)患儿出现OMAS复发。36例患儿末次随访时Mitchell and Pike OMS评分量表评分为0.5(0,2.0)分,7例(19%)认知轻度落后于同龄儿,6例(17%)重度落后。仅1例患儿在随访中出现肿瘤复发。未合并肿瘤组起病前疫苗接种或感染病史较合并肿瘤组更多见[55%(11/20)比23%(6/26),χ^(2)=4.95,P=0.026],首发症状肌阵挛出现更频繁[40%(8/20)比4%(1/26),χ^(2)=7.23,P=0.007]。Logistic单因素回归分析发现,合并肿瘤组比未合并肿瘤组更少出现复发[OR=0.19(0.04~0.93),P=0.041],病程6个月内使用二线治疗或化疗预后更好[OR=11.64(1.27~106.72),P=0.030]。结论儿童OMAS多于幼儿期起病,约半数合并神经母细胞瘤。合并OMAS的神经母细胞瘤通常危险分级低,预后好。对比合并和未合并肿瘤的OMAS患儿,后者起病前感染或接种疫苗诱因更常见,首发症状肌阵挛更多见。早期加用二线治疗与OMAS更好的预后相关。
