婴儿型脊肌萎缩症的临床及电生理分析

目的 分析婴儿型脊肌萎缩症患儿的临床及电生理表现，探讨本病的电生理特点及早期诊断要点。方法回顾性分析25例婴儿型脊肌萎缩症临床资料，肌电电生理按常规方法进行，针极肌电图按汤氏正常计算，神经传导速度按本室正常计算。结果患儿大多在1岁内起病，四肢呈对称性、迟缓性瘫痪，下肢重于上肢，近端重于远端；血清CK、LDH正常。肌电图表现为3个肢体在肌肉安静时出现广泛的失神经电位，轻用力时出现长时限、高波幅的运动单位电位，大力募集时电位数减少；神经传导速度正常，肌肉复合动作电位降低；肌肉活检为典型的神经源性肌萎缩。结论本病确诊应依据临床特点、肌电电生理、肌肉活检的改变。     

儿童脊髓性肌萎缩症的临床特征及电生理分析

目的探讨儿童脊髓性肌萎缩症(SMA)的临床及电生理特点。方法收集32例SMA患儿的临床资料,其中28例患儿行肌电图、运动神经传导速度及感觉神经传导速度等神经电生理检查,24例患儿行腓肠肌活检。结果32例SMA中,SMAⅠ型15例,SMAⅡ型12例,SMAⅢ型5例,均表现为进行性肌无力、肌萎缩,肌张力低下。各型SMA患儿起病年龄及病情轻重有其相应的特点。SMAⅠ型患儿不能竖头,均有呼吸肌受累表现,其中10例有颅神经受累症状。SMAⅡ型患儿能独坐,仅1例出现矛盾呼吸及吞咽困难。SMAⅢ型患儿能独站,其中3例可行走,均无呼吸肌受累表现。肌电图呈神经源性损害,主要表现为自发电位,其出现率为87%,运动单位时限延长(幅度30%～150%)、波幅增高(幅度90%～450%),28%运动神经传导速度轻度减慢(降幅24%～40%),93%肌肉复合动作电位波幅降低(降幅56%～99%),远端潜伏期均正常。感觉神经传导速度及诱发波幅均在正常范围。肌肉活检为典型的神经源性肌萎缩。结论根据临床特点、肌电图及肌活检结果可确诊SMA,重视SMA患儿的呼吸管理,可延长患儿生存时间,提高生活质量。     

儿童不同类型格林巴利综合征神经电生理研究

目的研究不同类型格林巴利综合征（ＧＢＳ）电生理特征，探讨神经电生理检测对ＧＢＳ分型的价值。方法对５４例ＧＢＳ患儿进行前瞻性、动态的神经电生理检查，比较不同型ＧＢＳ电生理特征。结果５４例中有急性炎症性脱髓鞘型ＧＢＳ２６例（４８％），轴索型ＧＢＳ２３例（４３％），５例（９％）神经失电位不能分型。急性炎症性脱髓鞘型ＧＢＳ电生理表现为：远端潜伏期延长（１６例６２％），神经传导阻滞（１５例５８％），神经传导速度降低（１３例５０％），Ｆ波潜伏期延长（５例１９％），伴远端复合肌肉动作电位波幅降低（２３例，８９％），感觉神经电生理异常（１１例，４２％）。轴索型ＧＢＳ电生理主要表现为运动神经远端复合肌肉动作电位波幅降低。结论不同型ＧＢＳ电生理特征各异，神经电生理检测对ＧＢＳ分型有重要诊断价值     

急性感染性多发性神经根炎的心脏损害

急性感染性多发性神经根炎（AIP）是儿科常见的神经系统疾病，常以急性起病，表现为四肢对称性弛缓性瘫痪，易合并颅神经损害和呼吸肌麻痹，有关心脏损害的报道少见，现将我院1990年以来收治的心电图资料完整的82例病例分析如下。临床资料一、一般资料82例病人男45例，女37例。     

儿童慢性炎症性脱髓鞘性多发性神经病的临床特点

目的 探讨儿童慢性炎症性脱髓鞘性多发性神经病（CIDP）的临床特征及实验室改变、预后。方法 对CIDP的临床资料进行分析。并对其进行随访。结果 11例CIDP3例病前有上感史；病程为2个月-1年；男性及学龄期儿童多见；均有双下肢肌力下降，双上肢肌力下降者4例，感觉障碍者6例，肌萎缩7例，颅神经麻痹1例。均有神经电生理改变，脑脊液检查除1例外均有细胞-蛋白分离现象。结论 CIDP与AIDP/RGBS不同，表现为起病慢，达高峰时间长，肌萎缩，感觉障碍多见，很少伴发颅神经损害和呼吸肌麻痹，脑脊液细胞-蛋白分离现象多见，电生理改变明显，坚持治疗者预后较大。     

肠道病毒71型感染手足口病合并急性弛缓性麻痹临床分析

目的探讨肠道病毒71型感染手足口病合并急性弛缓性麻痹的临床特点。方法对10例肠道病毒71型感染手足口病合并急性弛缓性麻痹的患儿进行临床观察,并作病原学、头颅和脊髓磁共振成像、神经电生理及脑脊液检查。结果 10例中8例为2岁以下儿童,瘫痪前期均伴发热和皮疹,单侧肢体瘫痪占70%,1周左右患肢运动功能开始恢复,轻症多于1～3个月完全恢复。磁共振成像及神经电生理检查结果与临床症状具有高度一致性,提供了神经受累的定位证据。所有病例随访4～12周,7例(70%)肌力恢复至Ⅴ级。结论急性弛缓性麻痹是肠道病毒71型感染手足口病的严重并发症,磁共振成像及神经电生理检查对评估病情及预后有重要价值。     

肉毒杆菌中毒导致呼吸衰竭二例

肉毒杆菌外毒素其嗜神经毒性极强,0.1~1.0μg的肉毒毒素就可导致人中毒死亡。病死率可达25％~44％,合并呼吸肌麻痹病死率更高,可达30％。我院2015年7月收治2例肉毒杆菌中毒患儿,经救治病情好转出院。     

超声定位A型肉毒毒素肌肉注射治疗痉挛型脑性瘫痪30例疗效观察

目的探讨不同定位术A型肉毒毒素肌肉注射治疗痉挛型脑性瘫痪患儿的临床疗效。方法选取痉挛型脑瘫患儿,按就诊次序编号,单号编入意愿分为超声定位A型肉毒毒素痉挛肌靶点注射术组(观察组),双号编入神经电生理刺激仪定位神经肌肉接头处阻滞术组(对照组),两组各30例患儿。两组患儿均给予一般康复训练和治疗,如针灸、推拿、功能训练,疗程3个月。治疗前后进行GMFM量表评分、关节活动度比较;操作时程比较;好转率比较。结果两组患儿经不同探测定位方式注射A型肉毒毒素后疗效比较,观察组好转率明显高于对照组,差异有统计学意义(P<0.05);在GMFM评分比较中,注射后3个月时两组对比结果:观察组评分明显提高,与对照组比较差异有统计学意义(P<0.05);两组操作时程在45min以内结果比较差异有统计学意义(P<0.05)。结论超声定位A型肉毒毒素肌肉注射治疗方法优于神经电生理刺激仪定位神经肌肉接头处阻滞术。     

急性多发性神经根炎

急性多发性神经根炎的发病率,近年来有增加的趋势,成为比较常见的神经系统疾病之一。其发病的季节性较强,6～8月发病较多。临床特点是对称性周围性的肢体麻痹,可有手套、袜套式的感觉障碍,重型病例常有呼吸肌与颅神经的麻痹。     

Miller Fisher综合征的肌电图及诱发电位表现

应用肌电图、体感和脑干听力诱发电位对８例ＭｉｌｌｅｒＦｉｓｈｅｒ综合征患儿进行系列检查。发现７例患儿中４例出现复合肌肉动作电位波幅减低伴感觉神经动作电位不能引出，其中２例尚有运动神经传导速度减慢；２例仅有复合肌肉动作电位波幅减低；１例感觉神经动作电位波幅位于低限；３例体感诱发电位和２例脑干听力诱发电位示周围神经性损害。提示本病主要损害周围神经（以感觉神经为重）的轴索并伴有髓鞘脱失。     

Reversible electrophysiological abnormalities in hypokalemic periodic paralysis

Compound muscle action potential (CMAP) amplitude declines during a paralytic attack in patients with hypokalemic periodic paralysis (HPP). However, serial motor nerve conduction studies in HPP have not been commonly reported. We report a 9 year old girl with HPP, who had severely reduced CMAPs in all motor nerves at presentation during the episode of quadriparesis. However, the amplitude of CMAPs increased and reached normal levels as the serum potassium concentration and motor power returned to normal state.     

Motor evoked potentials and compound muscle action potentials as prognostic tools for neonates with spina bifida

MEPs and CMAPs as prognostic tools for spina bifida.AimThe aim of this prospective study was to determine the prognostic value of neurophysiological investigations compared to clinical neurological examination in infants with spina bifida.MethodsThirty-six neonates born with spina bifida between 2002 and 2007 were evaluated and followed for 2 years. Lumbar motor evoked potentials (MEPs) and compound muscle action potentials (CMAPs) were obtained at the median age of 2 days old before surgical closure of the spinal anomaly. MEPs were recorded from the quadriceps femoris, tibialis anterior, and gastrocnemius muscles and CMAPs from the latter two muscles. Areas under the curve and latencies of the MEPs and CMAPs were measured. Clinical neurological outcome at the age of 2 years was described using Muscle Function Classes (MFCs) and ambulation status.ResultsThe areas under the curve of MEPs and CMAPs in the legs were associated with lower neonatal levels of motor and sensory impairment. Better muscle function class of the lower limbs at 2 years of age was associated with larger MEP and CMAP areas of the gastrocnemius and tibialis anterior muscles at neonatal age.DiscussionMEPs and CMAPs of the gastrocnemius and tibialis anterior muscles are of prognostic value for clinical neurological outcome in neonates born with spina bifida.     

Erb 点刺激在儿童吉兰-巴雷综合征早期诊断中的作用

目的 探讨Erb 点刺激在儿童吉兰-巴雷综合征(GBS)早期诊断中的作用。方法 选取2013年10 月至2014 年12 月诊断为GBS 的32 例患儿进行神经电生理检查,同期选取30 例健康儿童为对照组,检测并分析两组正中神经和尺神经的腕部、肘部、Erb 点刺激复合肌肉动作电位(CMAP)及末端运动传导潜伏期(DML),正中神经F 波潜伏期,以及胫神经H 反射潜伏期。结果 病例组F 波和H 反射潜伏期均明显长于对照组(P<0.05)。32 例患儿中,24 例Erb 点波幅异常(75%),22 例Erb 点潜伏期异常(69%),20 例Erb 点传导阻滞(62%),病例组尺神经、正中神经 Erb 点CMAP 均小于对照组,而DML 均长于对照组(P<0.05);两组腕部和肘部的CMAP 及DML 差异无统计学意义(P>0.05)。结论 Erb 点刺激在GBS 早期诊断中有一定价值,可作为一项常规检查应用于GBS 的早期诊断。     

Asian paralysis syndrome

We report 20 children admitted to the paediatric ward of a public general hospital for acute flaccid paralysis, which was bilaterally symmetrical in all cases and was associated with bulbar involvement in eight of them. Recovery was partial. Nerve conduction studies showed motor axonal neuropathy. This new disease, variously termed as non-inflammatory neuropathy/Chinese paralysis syndrome must be differentiated from Guillain-Barré syndrome (GBS) and poliomyelitis. Both GBS and Asian paralysis syndrome have bilaterally symmetrical flaccid paralysis but GBS tends to have sensory involvement, full recovery occurs in 90% of cases and nerve conduction shows demyelinating neuropathy. Asian paralysis syndrome and poliomyelitis are pure motor lesions without sensory changes and partial recovery, but poliomyelitis differs in that paralysis is asymmetrical and unequal, muscle spasm is always present in the initial stage and there are prodromal symptoms. Nerve conduction studies show anterior horn cell disease. This new entity, common in Asian populations, assumes public health importance when it mimics poliomyelitis in a country that has tried to eliminate poliomyelitis by universal immunization. To the best of our knowledge, this is the first report of Asian paralysis syndrome in children in our area.     

Maturation of corticospinal tracts assessed by electromagnetic stimulation of the motor cortex.

The motor cortex can be excited in adults using electromagnetic stimulation, and the latency to the evoked muscle action potential allows an assessment of the integrity of corticospinal tracts. We applied this technique in children to describe the maturation of corticospinal tracts. The latency from cortical stimulation to the onset of the evoked muscle action potentials and the subject''s height were recorded. The subject''s height was divided by the latency to the onset of the evoked muscle action potential to provide an index of the conduction velocity within descending motor pathways (VI). It is possible to evoke muscle action potentials after electromagnetic stimulation of the motor cortex in children including preterm babies and there is a stepwise increase in the sensitivity to stimulation between 8 and 11 years of age. In addition there is a progressive increase in VI with age; adult values are attained at about 11 years. The successful application of this technique in children suggests that electromagnetic stimulation of the motor cortex has the potential to allow detection of abnormality in motor pathways in newborn babies and young children.     

Acute flaccid myelitis caused by enterovirus D68: Case definitions for use in clinical practice

Acute flaccid myelitis (AFM) was increasingly detected in recent years, coinciding with upsurges of enterovirus D68 (EV-D68) infections. We reviewed the evidence for a causal relationship between both. Based on reported cases, we provide case definitions for AFM caused by EV-D68 infections to enable a standard procedure for affected patients. Current case definitions are focussing on epidemiological aspects but clinical case definitions are still missing. We propose the following case definitions to be used in clinical practice in order to mirror clinical realities and facilitate a common systematic approach in case management: A possible case is defined as a person presenting with either acute myelitis/paralysis or Guillain-Barré Syndrome (GBS), particularly during periods of EV-D68 circulation. A probable case is defined as a person presenting with symptoms of either acute myelitis/paralysis or GBS and at least one of the following criteria: i) MRI abnormality representing with T2 hyperintensity in spinal cord grey matter with or without hyperintensity at dorsal brain stem, ii) investigations showing an axonal neuropathy including reduced compound motor action potentials with normal conduction velocities and absence of conduction blocks compatible with anterior horn cell disease or iii) detection of enteroviruses in a respiratory specimen obtained from the lower respiratory tract during periods of EV-D68 circulation. A confirmed case is defined as a person presenting with acute flaccid myelitis/paralysis, MRI abnormality and detection of enterovirus-D68-specific nucleic acids in a respiratory specimen using a validated PCR assay targeting the VP1 gene with subsequent sequencing and typing.     

Przebieg kliniczny i odległe następstwa zespołu Guillaina-Barrégo u dzieci

IntroductionGuillain-Barré syndrome (GBS) is an autoimmune disorder of peripheral nervous system causing progressive weakness and areflexia. The process leads to acute flaccid paralysis of the two or four limbs and cranial nerves dysfunction. Epidemiologic studies have reported an annual incidence of 1:100000 children under 15.AimThe aim of study was to analyse the clinical course and long-term evaluation of GBS in children.Material and methodsEighteen children with classic form of Guillain-Barré syndrome were enrolled. In the first part of study we retrospectively reviewed the clinical course of disorder. In the second part we analysed the children's recovery, basing on the inquiry studies.ResultsIn the analysed group all children developed the paralysis of lower limbs, 38.9% – sphincter and bladder dysfunction, 72% – tetraparesis, 22.2% – respiratory tract insufficiency. Upper respiratory tract infections were the most common preceding GBS. Under performed treatment all children started to walk in first 6 months after GBS but they learned to run and climb the stairs more than one year. The efficiency of upper limbs was coming back faster than the lower limbs, 83% children could dress themselves and carry the heavier objects in half of the year. More than the half of group suffered from the subjective symptoms: limbs pains, hyperesthesia, and fatigability. These signs disappeared in the year after GBS. We reported the late effects of GBS, which were detected in analysed group after 12 month from GBS. There were: slowly running, fatigability, inability of dressing, climbing the stairs, hyperesthesia, tingling hands.     

Of war and sausages: A case‐directed review of infant botulism

Within a 3‐month period, two infants presented non‐specifically to hospital and rapidly progressed developing flaccid paralysis. Both children were diagnosed with infant botulism. We briefly review these two cases and discuss the diagnostic and management issues involved with this rare childhood disease.     

Two cases of type A infant botulism in Grenoble, France: no honey for infants

We report two severe cases of infant botulism diagnosed at Grenoble University Hospital, France, respectively in 2006 and 2009. Both cases were characterized by a delay in diagnosis, severe neurological manifestations and extended period of hospitalization in intensive care unit, but a complete recovery. Infant botulism is a rare but life-threatening disease. It primarily affects infants, and the main risk factor is honey ingestion. Diagnosis should be systematically evoked by pediatricians in infants suffering from constipation, fatigue, muscle weakness, difficult feeding and altered cry, but before the onset of generalized flaccid paralysis, so as to administer specific treatment (BabyBIG?, a human derived botulinum antitoxin) at an early stage of the disease when it is most effective. In conclusion, parents should be aware of the role of honey as a source of spores of Clostridium botulinum and therefore infant botulism in the first year of life.