老年重症肌无力诊治分析

目的 探讨老年重症肌无力(MG)的临床特点.方法 回顾性分析2014-01—2020-01在郑州大学第二附属医院接受治疗的89例老年MG患者的临床资料.结果 老年MG患者男女比例为1:1.02.以OssermanⅠ型起病者44例,男24例(54.5％),女20例(45.5％);以OssermanⅡ型起病者43例,男18...     

重症肌无力的分子遗传学研究进展

重症肌无力(MG)是一种以神经-肌肉接头传递功能障碍为主要病理特征的获得性自身免疫疾病.临床主要表现为部分或全身骨骼肌无力和易疲劳,活动后症状加重,经休息和胆碱酯酶抑制剂治疗后症状减轻.该病起病隐匿,缓解与复发交替,多数病例迁延数年至数十年,致残致死率高,严重威胁患者健康.流行病学研究发现,父母双亲均为MG患者的家族中,其家族成员MG患病率高达4％,而在一般人群中小于0.01％.这种显著的差异表明遗传因素与MG易患性有密切联系.     

眼肌型和全身型重症肌无力的差异

重症肌无力(myasthenia gravis,MG)是由乙酰胆碱受体抗体(acetylcholine receptor antibody,AChRAb)介导的、细胞免疫依赖的、补体参与的自身免疫性疾病,其主要临床症状为横纹肌收缩无力,多数患者起病表现为眼肌型MG(ocular MG,OMG),以后逐渐进展为全身型MG(generalizedMG,GMG),严重者累及呼吸肌,发生肌无力危象而死亡[1]。     

碘造影剂诱发重症肌无力危象一例

<正>重症肌无力(myasthenia gravis,MG)是一种累及神经-肌肉接头突触后膜,引起神经-肌肉接头传递障碍,出现骨骼肌收缩无力的获得性自身免疫性疾病,主要临床表现为骨骼肌无力、易疲劳,活动后加重,休息和应用胆碱酯酶抑制剂后症状明显缓解、减轻[1]。重症肌无力危象(myasthenic crisis,MC)是MG在起病或治疗过程中突然出现的、严重威胁患者生命的并发症,主要由于呼吸肌严重受累,表现呼吸无力或衰竭、低氧血症,需气管插管     

重症肌无力诊断与治疗最新进展

<正>重症肌无力(myasthenia gravis,MG)是一种神经肌肉接头传递障碍疾病,以骨骼肌无力、晨轻暮重、症状有波动为主要临床特点。近年来老年MG的发病率逐年增加,新发现的一些抗体(anti-MusK和anti-LRP4)能诊断原抗体检测难以诊断的MG,新的电生理诊断方法(单纤维肌电图和双神经刺激试验)能发现常规肌电图不能发现的神经肌肉接头处突触传递障碍,新涌现的治疗药物(单抗隆抗体和粒白细胞集     

重症肌无力患者的护理体会

重症肌无力（MG）是一种累及神经肌肉接头突触后膜上乙酰胆碱受体（AchR）的自身免疫性疾病.临床症状：主要表现为部分或全身骨骼肌无力,易疲劳,活动后症状加重,经休息和胆碱酯酶抑制剂（ChEI）治疗后症状减轻[1].发病率为8～20/10万,患病率为50/10万,我国南方发病率较高.其中MG患者中65%～80%有胸腺增生,10%～20%伴发胸腺瘤.重症肌无力患者的治疗：包括药物治疗、放射治疗和手术治疗.其中药物治疗,口服肾上腺皮质激素和胆碱酯酶抑制剂是现今国际公认有效的常规保守疗法.手术治疗,胸腺切除加前纵隔脂肪清扫术是目前认为治疗MG最根本的方法.     

重视重症肌无力的临床诊断

重症肌无力（myasthenia gravis，MG）是神经肌肉接头（neuromuscular junction，NMJ）的自身免疫病，表现为波动性肌无力症状，亦可有较恒定的体征。MG可累及全身骨骼肌，亦可累及平滑肌和心肌，受累肌群的不同使症状丰富多样。其首发症状缺乏特异性，患者肌群运用情况不同也使发现症状的背景不同，给诊断带来困难。MG是可治性疾病，早期诊断和治疗有助于控制发展。应重视MG的临床诊断。我院3100例随访较完善的MG资料总结与国内另一MG中心的大宗报道相近，现结合相关数据和研究进展探讨MG的临床诊断。     

难治性重症肌无力的处理

大约10%的重症肌无力(MG)属于难治性,明确难治性MG有助于及时换用有效的免疫治疗,控制病情.目前难治性MG还缺乏比较统一的定义.判断难治性MG需要经过一定的流程,并进行鉴别诊断和病理生理诊断.判断为难治性MG后,在调整病理生理状况的基础上选择更强的免疫抑制剂治疗才能有效控制病情并取得较好的长期疗效.     

免疫吸附治疗重症肌无力术后肌无力危象

重症肌无力(myasthenia gravis,MG)是累及神经一肌肉接头突触后膜上乙酰胆碱受体(AchR).主要由AchR抗体(AchR-Ab)介导的的自身免疫性疾病[1].目前胸腺切除已经成为治疗MG的重要治疗方法.肌无力危象是胸腺切除术后最严重的并发症,病情危急,死亡率高田.南华大学附属第二医院心胸外科自2001年至2006年应用葡萄球菌蛋白A(staphylococcal protein A,SPA)免疫吸附治疗MG术后肌无力危象患者11例,取得了满意的疗效,现总结报道如下.     

重症肌无力患者汉密尔顿抑郁量表评分分析

目的探讨重症肌无力(myasthenia gravis,MG)患者汉密尔顿抑郁量表(Hamilton depression rating scale,HDRS)评分情况及其影响因素分析。方法横断面研究2013-07—2015-03作者医院就诊的188例MG患者的临床资料和HDRS评分情况,并根据HDRS评分将其分为抑郁组和非抑郁组,分析两组MG患者的临床特点及其与HDRS评分间的关系。结果所纳入MG患者男女比例为1.02∶1,眼肌型重症肌无力(ocular myasthenia gravis,OMG)和全身型重症肌无力(generalized myasthenia gravis,GMG)的比例为1.2∶1,以OMG起病和以GMG起病患者的比例为6.2∶1,病程中位数为2年,四分位数间距为1.8年,平均量化重症肌无力评分(quantitative myasthenia gravis,QMG)为(6.7±2.3)分,平均HDRS评分为(8.7±3.4)分,并发抑郁者65例,未并发抑郁者123例。影响HDRS评分和抑郁发生的相关因素包括性别(P0.01)、MG类型(P0.01)、QMG得分(P0.01)和美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型(P0.01)、有无甲状腺功能亢进(P0.05)。结论影响MG患者HDRS评分和抑郁发生的相关因素包括性别、MG类型、QMG评分和MGFA分型、有无甲状腺功能亢进,充分认识其抑郁发生情况有利于更好地治疗MG。     

Phobic and anxiety disorders in Hispanic and Caucasian youth

Knowledge about phobic and anxiety disorders in Hispanic youth is sparse. This study compared 99 Hispanic and 143 Caucasian youth, aged 6–17, on various sociodemographic and clinical characteristics. Results indicated that Hispanics and Caucasians were remarkably similar on the majority of indices examined, including age at intake, gender, patterns of primary diagnoses, clinician interference rating, percentage with school refusal, and percentage with more than one diagnosis. Hispanic children were more likely to present with a primary diagnosis of separation anxiety disorder and were more likely to come from families with lower incomes compared to Caucasians. Hispanic parents also rated their children as more fearful than did Caucasian parents. The study's findings are viewed as an initial step in extending the existing literature on childhood anxiety disorders and Hispanic mental health.     

Childhood depression: an overview.

A review of the psychiatric literature on childhood depression indicates confusion in the basic concept of depressive illness in childhood. There is no general agreement on criteria for childhood depression. The diagnosis of depression in childhood is being based on clinical impression, arbitrarily selected criteria, or on a favorable response to treatment with antidepressants. There is no distinction between primary and secondary depression. The diagnosis of "masked depression" is given to children with a wide range of different problems, bassed on the hypothesis that depression in childhood can be manifested by clinical symptomatology quite distinct from that found in adults. However, recent studies do not support this hypothesis. Most studies on childhood depression include a wide range of children in spite of the fact that from the data available in current literature it seems justified to distinguish between the different age groups of children, mainly the prepubertal and the adolescent groups.     

Pseudotumor cerebri as an important differential diagnosis of papilledema in children

INTRODUCTION: Primary pseudotumor cerebri (PTC) in childhood is a rare but important differential diagnosis in children presenting with papilledema. It is defined as elevated cerebrospinal fluid (CSF) pressure of more than 20 cm H(2)O, normal CSF composition, and exclusion of underlying structural or systemic causes. Visual loss is a serious complication, which requires careful monitoring and management. PATIENTS AND METHODS: We conducted a retrospective chart review of 12 patients with primary PTC. The mean age at presentation was 8212 years, and there was a male-to-female ratio of 7:5. The aim of this study was to investigate the clinical features of primary PTC in children, and to highlight the different treatment options in normalizing intracranial pressure in these patients. RESULTS: In the majority of cases, children presented with headache. Four patients had no obvious symptoms and papilledema was found on routine eye examination. Obesity was uncommon and there was no distinct sex predilection. Acetazolamide was our drug of choice for the initial treatment. Furosemide and prednisone were used as second-line agents. Treatment was gradually decreased after resolution of the papilledema with exception of the two youngest children, who remained symptomatic. One child underwent ventricular-peritoneal shunting. DISCUSSION: The treatment goals of PTC are the relief of symptoms, and preservation of visual function. Acetazolamide is an effective first-line method of lowering raised intracranial pressure. In our study group especially the young children were difficult to treat. This might indicate an age-related difference in the etiology of PTC. When medical treatment remains ineffective and visual function deteriorates, surgical treatment should be considered.     

Patterns of antidepressant use among children and adolescents

OBJECTIVE: The purpose of this study was to identify patterns of new antidepressant use among children and adolescents and to determine whether the duration of treatment was sufficient. METHODS: A retrospective 12-month analysis was conducted of claims data for a cohort of nine- to 18-year-old new users of antidepressants in an Ohio Medicaid population. Treatment duration was categorized into five time intervals reflecting initial treatment through various continuation periods. RESULTS: A total of 554 children and adolescents started an antidepressant regimen during a three-month period. These children were mostly Caucasians (78 percent), and their average age was 13 years. Boys and girls were equally represented. The use of antidepressants increased with age among girls but declined among boys. The distribution of antidepressants dispensed was selective serotonin reuptake inhibitors, 47 percent; tricyclic antidepressants, 27 percent; and other antidepressants, 23 percent. The specific agent used varied by primary psychiatric diagnosis. The proportion of children who completed treatment was 94 percent for the four-week treatment period, 23.5 percent for the six-month period, and 12.6 percent for the whole year. CONCLUSIONS: Antidepressants are used in the treatment of children and adolescents who have a wide array of mental health problems. As with adults, continuation of treatment among children and adolescents declines dramatically after an initial period. In addition to studies of the clinical efficacy of antidepressant use among children and adolescents, future research is needed to assess adherence to practice guidelines and health outcomes in childhood and adolescent mental health.     

Accuracy of adult recall of childhood attention deficit hyperactivity disorder

OBJECTIVE: Although reports of childhood status are necessary for making a diagnosis of adult attention deficit hyperactivity disorder (ADHD), systematic investigation of the accuracy of retrospective self-reports has been limited. This study examined accuracy of adult recall of childhood ADHD. METHOD: Participants were from a controlled, prospective 16-year follow-up of children with ADHD. At a mean age of 25 years, 176 probands (85% of the 207 subjects in the initial cohort) and 168 non-ADHD comparison subjects were interviewed by clinicians who were unaware of the subjects' childhood status. Subjects were asked about specific childhood ADHD behaviors, and the diagnosis of childhood ADHD was retrospectively established. RESULTS: Seventy-eight percent of the probands and 11% of the comparison subjects were identified as having childhood ADHD. Six symptoms demonstrated high discriminating power in differentiating the subject groups: distractibility, concentration difficulties, complaints of inattention, acting before thinking, being on the go, and fidgeting/squirming. When findings were adjusted for the prevalence of ADHD in the general population, the power of prediction was low. Positive predictive value was 0.27, i.e., of all adults retrospectively given a diagnosis of childhood ADHD, only 27% would be correctly identified. As expected, positive predictive value increased with increases in the estimated prevalence of ADHD. CONCLUSIONS: Retrospective diagnoses of childhood ADHD made on the basis of self-reports will in most cases be invalid in settings such as epidemiological surveys and primary care facilities. Greater accuracy can be expected in settings in which childhood ADHD is frequent. The results stress the importance of obtaining contemporaneous information on childhood symptoms in establishing a childhood history of ADHD. Future directions and implications for DSM-V are discussed.     

Diagnostic pitfalls in paediatric ischaemic stroke

Diagnosing ischaemic stroke and determining its cause is difficult in children. Both are important for selection of treatment and prediction of outcome. This study explored the diagnostic changes that lead to a delay in the correct diagnosis of paediatric stroke. Case histories of 45 children with ischaemic stroke (31 males, 14 females; median age 6y; age range 2mo-16y) were retrospectively reviewed. The initial clinical diagnosis, based on the interpretation of presenting symptoms, was compared with the final aetiological stroke diagnosis after completion and review of diagnostic work-up. The type of diagnostic change, consequent time delay until correct diagnosis, reasons for change of diagnosis, and alterations to management were evaluated. Twenty-four diagnostic changes were identified; 19 in 'primary stroke diagnosis' (symptoms initially not attributed to stroke), and five in 'aetiological diagnosis' (incorrect initial determination of type or cause of stroke). The median interval between initial and final diagnosis was 7 days (3h-2y). The change in diagnosis led to therapeutic alterations in 17 patients. Risk factors for childhood stroke differ from those in adults. Stroke is frequently not recognized as the cause of the child's symptoms, and the correct determination of stroke aetiology takes time. We recommend that children with stroke be evaluated in a centre with expertize, using standardized diagnostic protocols and careful follow-up.     

The role of plasmapheresis in childhood Guillain-Barré syndrome

Plasmapheresis has been advocated in the treatment of childhood Guillain-Barré syndrome under the assumption that the results of adult series can be extrapolated to children. To test this assumption, we retrospectively evaluated the medical charts of all children who were admitted to The Children's Hospital of Philadelphia between January 1984 and March 1989, with the diagnosis of Guillain-Barré syndrome. Of the 30 patients identified, 7 were excluded because they had mild disease. Of the remaining 23, 9 underwent plasmapheresis and 14 served as historic control subjects. The two groups were similar with respect to age, presenting symptoms, findings on initial physical examination, and antecedent illnesses at the time of diagnosis. The mean time to recover to Grade 2 (independent ambulation) was significantly shorter in the plasmapheresis-treated group, 24.0 +/- 25.4 days, compared to 60.2 +/- 43.6 days in control subjects (mean +/- 1 SD). Our results indicate that plasmapheresis diminishes morbidity in childhood Guillain-Barré syndrome by shortening the interval until recovery of independent ambulation.     

Childhood anxiety disorders: parent-child (dis)agreement using a structured interview for the DSM-IV

OBJECTIVE: To evaluate parent-child agreement for diagnosis of childhood anxiety disorders using a DSM-IV-based structured interview in an anxiety-clinic-referred sample and to explore the role of age and gender on agreement. METHOD: Forty-five children (ages 7-14 years) and their parents completed independent structured diagnostic interviews conducted by separate diagnosticians. Agreement between parent- and child-report on childhood anxiety disorder diagnoses was determined. RESULTS: Levels of parent-child agreement were poor for the major childhood anxiety disorders presenting either as principal diagnosis or presence anywhere in the clinical picture. No significant differences were found for age or gender. However, somewhat greater agreement was found in older children for the principal diagnosis of specific phobia, and in females for the principal diagnosis of social phobia. Agreement was found to be greater between parents and children when each reported at least one principal anxiety diagnosis. CONCLUSIONS: When separate diagnosticians are used, there is limited agreement between parents and children for the DSM-IV anxiety disorders. Appropriately integrating the discrepant reports in reaching a diagnosis remains a methodological issue. Implications for treatment and future research are discussed.     

Targeted Behavioral Therapy for childhood generalized anxiety disorder: A time-series analysis of changes in anxiety and sleep

This study examined the efficacy of Targeted Behavioral Therapy (TBT), a newly developed intervention targeting features of childhood generalized anxiety disorder (GAD). Using a time-series design, 4 children (7–12 years) with primary GAD were treated with TBT, which includes sleep improvement strategies, systematic desensitization for reducing intolerance of uncertainty, and in vivo exposures for anxiety. Diagnostic interviews and questionnaires were administered at baseline, post-treatment and 3 months follow-up. Anxiety symptoms and sleep characteristics/problems were rated weekly during a 4-week baseline and 14-weeks of treatment. Two children remitted at post-treatment and no child had a GAD diagnosis at follow-up. Child but not parent report revealed improvements in both worry and sleep. Despite improvements from pre- to post-assessment, considerable symptom fluctuation observed during the baseline period preclude conclusion that symptom changes are specifically attributable to the course of treatment. Overall, preliminary support is provided for the efficacy of TBT for childhood GAD.     

Myasthenia gravis in childhood and infancy. Usefulness of electrophysiologic studies

The diagnostic yield of electrodiagnostic tests was evaluated in 21 children with myasthenia gravis (MG). Twelve children had juvenile autoimmune MG, four had neonatal MG, four had congenital MG, and one had MG with arthrogryposis. Repetitive stimulation (RS) of the ulnar nerve was performed in every patient, sometimes with sensitization by ischemia. When the RS test was negative, the spinal or facial nerve was tested. In the group with neonatal MG, the RS test disclosed a significant decrement in 75% of the patients. Among the 17 other children, the overall percentage of positive RS tests was 88%. An ulnar nerve RS test was positive in 41% of them. Sensitization of ulnar nerve RS by ischemia improved the diagnostic yield to 66%. A spinal or facial nerve RS test was positive in seven of 10 patients. These results confirmed the diagnostic value of RS, with sensitization by ischemia, when necessary, in childhood MG whenever a careful technique is provided.