核磁共振和核磁共振波谱结合粗大运动评定量表在儿童脑性瘫痪分度诊断的应用

目的 探讨头部核磁共振、核磁共振波谱以及粗大运动评定量表对儿童脑性瘫痪的分级诊断价值.方法 40例1.0～2.0周岁的健康儿童和42例1.1 ～2.0周岁的脑性瘫痪儿童为研究对象,对比正常儿童与脑性瘫痪患儿的MRI、MRS检测结果;根据核磁共振结果对脑性瘫痪进行分度,并用核磁共振波谱检测不同核磁共振分度的脑性瘫痪患儿脑内的NAA/Cr、CHO/Cr、LAC/Cr水平并分度;考察不同核磁共振和核磁共振波谱分度脑性瘫痪患儿的粗大运动评定量表得分情况.结果 核磁共振和核磁共振波谱均能有效区分脑性瘫痪患儿脑内的典型病变,脑内的NAA、Cr、Cho能被核磁共振波谱定量检测.不同核磁共振分度的患者脑内的NAA/Cr、CHO/Cr、LAC/Cr有所差异,重度患者与中度患者间的差异具有统计学意义(P＜0.05).脑性瘫痪的核磁共振波谱分度与核磁共振分度相符率不理想,但粗大运动评定量表评分表明各不同分度之间有差异(P＜0.05).结论 核磁共振和核磁共振波谱结合粗大运动评定量表可有效完善脑性瘫痪的分度诊断,这对不同类型儿童脑性瘫痪的临床治疗具有一定的指导意义.     

小儿急性瘫痪的诊断与鉴别诊断

瘫痪是小儿神经系统疾病常见症状之一 ,临床上以随意肌运动障碍为特征 ,表现为肢体、躯干或面部肌肉活动减弱或消失 ,伴或不伴感觉功能障碍。临床根据肌力大小 ,将瘫痪分为完全性瘫痪 (0级 )、接近完全性瘫痪 (1级 )、重度瘫痪(2级 )、中度瘫痪 (3级 )、轻度瘫痪 (4级 )等。遗传因素或先天因素引起的慢性瘫痪 (如进行性肌营养不良、脊肌萎缩症、脑性瘫痪等 )已被儿科工作者熟知 ,但部分获得性急性瘫痪诊断较为困难 ,特别是近年来十分强调急性弛缓性麻痹(acuteflaccidparalysis,AFP)的报告制度 ,使急性瘫痪的鉴别诊断显得更为重要 ,因此很…     

不安运动的评估对高危新生儿运动发育的预测价值

目的 评估高危新生儿不安运动(FMs)对运动发育结局的早期预测价值.方法 随访高危新生儿39例,矫正日龄6～20周记录FMs,10～18月进行Gesell发育量表或丹佛发育筛查量表评估,结合婴儿神经系统检查确定发育结局.结果 FMs正常的33例中随访诊断运动发育迟缓1例;未出现FMs的6例中,随访诊断脑性瘫痪4例,全面发育迟缓2例.FMs评估预测脑性瘫痪敏感度为100%,特异度为94%,阳性预测值为67%,阴性预测值为100%.预测运动发育迟缓的敏感度为86%,特异度为100%,阳性预测值为100%,阴性预测值为97%.结论 FMs缺乏是早期预测脑性瘫痪良好而可靠的指标.矫正年龄10～14周单次FMs评估对运动发育也具有良好的预测价值,但对脑性瘫痪的阳性预测值较低.     

对吉兰-巴雷综合征的新认识

吉兰-巴雷综合征（Guillian-Barré syndrome,GBS）是脊髓灰质炎消灭后最常见的小儿急性迟缓性瘫痪性疾病,主要表现为快速进展的瘫痪、腱反射消失和脑脊液蛋白细胞分离。根据病理和神经电生理特点,可将其分为4种主要亚型：急性炎症性脱髓鞘性多发性神经炎（acute inflammatory demyelinating polyneuropathy,AIDP）、急性运动轴索性神经病（acute motor axonal neuropathy,AMAN）、急性运动感觉轴索性神经病（acute motorsensory axonal neuropaty,AMSAN）和Miller—Fisher综合征（MFS）。目前一致认为它是一种免疫介导的器官特异性周围神经病,体液免疫和细胞免疫不同程度地参与了各个亚型病变的发生。根据其典型临床表现及神经传导功能检测,不仅能明确GBS的诊断,还有助于临床分型。免疫调节治疗能有效地缓解神经损伤,加速患者恢复。文章着重介绍近年有关该病的分型、病因及发病机制、临床及电生理特征、鉴别诊断和治疗方面的研究进展。     

手足口病合并急性弛缓性麻痹患儿的脊髓磁共振特点及临床随访研究

目的 研究8例手足口病(hand-foot-and-mouth disease,HFMD)并发急性弛缓性麻痹(AFP)患儿的脊髓MRI特点并进行临床随访.方法 观察8例于足口病并发急性弛缓性麻痹患儿临床特点,于起病2周行脊髓和头颅MRI检查,早期进行针对性功能锻炼、药物治疗,并儿随访3个月.结果 8例手足口病并发急性弛缓性麻痹病例中,2例四肢瘫痪,1例双下肢瘫痪,5例单侧下肢瘫痪.大部分患儿表现为轻瘫,以下肢麻痹为主.病程2周行脊髓MRI,示患儿脊髓病变集中于颈2～7和(或)胸12～腰1,表现为脊髓前角T1长T2信号.脊髓前角病变严重程度与临床表现、预后相关.下肢瘫痪恢复较快,四肢瘫痪者,恢复缓慢;并发脑炎者,恢复相对较慢.结论 本组手足口病患儿合并急性弛缓性麻痹病例约占0.83%.AFP病变涉及颈2～7和(或)胸12～腰1脊髓前角区,引起所支配的上肢和(或)下肢肌群的急性弛缓性麻痹.随访结果显示,手足口病伴发AFP可能是可逆性病变,单侧下肢瘫痪恢复快,四肢瘫痪恢复慢.MRI可准确的显示脊髓病,变,对全面评估病情及预后有重要价值.     

小儿偏瘫(讲座)

瘫痪是骨骼肌有意识的收缩力的减弱或消失。瘫痪程度可分为0～5度,即完全瘫痪、接近于完全瘫痪、重度瘫痪、轻度瘫痪、接近正常以及肌肉完全正常。瘫痪可分为上运动神经元瘫痪(中枢性瘫痪)和下运动神经元瘫痪(周围性瘫痪),前者为大脑皮质运动细胞及其锥体束受损所致,其特点是肌张力增高(尤其是上肢屈肌、旋前肌及下肢伸肌张力增高)、腱反     

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目的探讨粗大运动功能分级系统(GMFCS)在痉挛型脑性瘫痪患儿中的再测信度。方法收集2005年2月至2008年2月北京儿童医院治疗的痉挛型脑性瘫痪患儿83例,予以运动疗法(PT)、作业疗法(OT)、水疗(HT)、针灸及按摩治疗,在治疗前后对其采用GMFCS进行粗大运动功能分级并评估。结果 83例中1例治疗前后粗大运动功能级别发生变化,82例并未发生变化。GMFCS对于痉挛型脑性瘫痪患儿具有良好的再测信度(P<0.01),在<2岁阶段的再测信度低于其他年龄组(P<0.01)。结论 GMFCS是目前惟一针对脑性瘫痪患儿粗大运动水平进行标准化评价的工具,具良好再测信度,但在<2岁的再测信度低于其他年龄组。应进一步进行多中心合作研究,将全部类型、全部级别的脑性瘫痪患儿纳入研究以证实GMFCS在脑性瘫痪康复中作用及意义     

脑性瘫痪合并视听觉障碍的临床康复研究进展

脑性瘫痪是一组以运动障碍和姿势异常为核心表现的发育障碍综合征,可伴有言语障碍、智力障碍、视听觉障碍和行为障碍等.因其高发病率与致残率,脑性瘫痪现已成为全世界需共同攻克的难题.目前国内的研究主要是针对运动障碍、言语障碍和智力障碍,而对感知觉障碍缺乏足够的重视.感知觉障碍中发病率最高的是视听觉障碍,且后者对儿童运动、认知、语言、交流、心理等方面发育均存在影响.该文重点综述脑性瘫痪合并视听觉障碍的发病率、表现形式、对脑性瘫痪儿童发育的影响以及康复治疗进展,以提高视听觉障碍的重视度,并为促进该类儿童的全面康复奠定基础.     

婴儿脊髓性进行性肌萎缩症2例

婴儿脊髓性进行性肌萎缩症在临床较为少见,患病率为0.1～1.0/10万.本病主要病变脊髓前角运动神经细胞退行性变,导致患者肢体肌肉瘫痪,重者因呼吸肌瘫痪而死亡,少数存活者由于下肢肌肉软弱而行走不便.     

急性运动轴索神经病

急性运动轴索神经病大连医科大学附属第二医院（１１６０２３）夏经在我国北方，Ｇｕｉｌｌａｉｎ－Ｂａｒｒｅ综合征（ＧＢＳ）发病率颇高，而在北美西欧则较罕见。这种中国的急性弛缓性瘫痪（弛瘫，ＡＦＰ），与当前西方国家所诊断的ＧＢＳ究竟有何不同，近１０年来引起...     

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.     

Poliomyelitis-like paralysis during recovery from acute bronchial asthma: possible etiology and risk factors.

A poliomyelitis-like paralytic disease during recovery from an attack of bronchial asthma is described in two young children. They presented at the age of 13 and 22 months, respectively, with acute flaccid paralysis of one or both lower limbs and preserved sensation. Cerebrospinal fluid examinations revealed mild protein elevation in both and pleocytosis in the second infant. Enteroviruses were isolated in a nasal swab and stools of the second patient. Acute onset of flaccid paralysis with absent motor action potential and normal sensory responses, detected by electrophysiologic studies, are highly suggestive of motor anterior horn cell disease in these infants. A multifactorial setup of immune suppression, stress, and neurotoxic drugs during an acute bronchial asthma attack triggered by a viral disease may render the patient vulnerable to viral invasion of the anterior horn cell with enteroviruses other than poliovirus. The overall experience of 22 patients with this serious complication is reviewed.     

Surgical treatment of focal inhibitory motor seizures

The authors describe a rare case of a pediatric patient with focal inhibitory motor seizures (ictal paralysis) with the seizure focus in the primary motor cortex. Seizures were characterized by ictal paralysis of the contralateral limbs without loss of consciousness. The seizure focus and progression were examined using long-term video electroencephalography (EEG) monitoring and MR imaging. Invasive recordings of the ictal EEG revealed spike rhythms arising from the primary motor cortex, suggesting that seizures associated with paralysis can arise from hyperactivity in either positive or negative motor areas. Resection of the cortical area related to ictal onset resulted in a seizure-free status for the last 3 years.     

Guillian Barré Syndrome — Recent advances

Guillian Barré Syndrome (GBS) is an acquired disease of the peripheral nerves that is characterized clinically by rapidly progressing paralysis, areflexia, and albumino-cytological dissociation. It affects both genders, involves people of all ages, is reported worldwide, and in the post-polio era, it is the most common cause of an acute generalized paralysis. The clinical features are distinct and a history and an examination generally lead to a high suspicion of the diagnosis that can then be confirmed by supportive laboratory tests and electrodiagnostic studies. This review discusses the recent advances in understanding of the different variants of GBS such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and the Fisher syndrome. The clinical, electrodiagnostic criteria, immunopathogenesis, and management of GBS and its variants are discussed.     

GB Syndrome with Herpes simplex infection

G.B. Syndrome is an acute flaccid lower motor neuron (LMN) paralysis. The diagnosis is based on clinical presentation, course of illness with supportive investigations. This article reports an uncommon case of GB Syndrome caused by HSV infection in a 2yr-9mth-old child and have analysed the natural history, etiopathology, treatment and prognosis of the disease.     

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。     

Progressive bulbur paralysis(Fazio-Londe disease)

Progressive bulbar paralysis of childhood is characterised by progressive paralysis of muscles innervated by cranial nerves. The authors report a case of progressive bulbar paralysis of childhood in a 12-year-old child. Child was admitted with the complaints of drooping of eyelids, difficulty in swallowing and hoarse voice. She had involvement of III, VII, IX, X, XI and XII cranial nerves and the corticospinal tracts. Electromyography revealed spontaneous activity in the form of fasciculations, giant motor unit potential and discrete recruitment of motor neurons suggestive of denervation pattern. Hearing assessment was normal. Muscle biopsy was also suggestive of neurogenic atrophy.     

我国脊髓性肌萎缩症多学科管理和诊治模式

脊髓性肌萎缩症(SMA)主要累及运动神经元导致肌无力及肌萎缩,在疾病不同阶段可出现多系统(骨骼、呼吸、消化等)合并症。随着SMA的疾病修正治疗药物在国内的应用及研究的开展,SMA多学科管理及诊治模式迎来了新的挑战。该文从疾病修正药物治疗、康复管理、骨骼管理、营养管理、呼吸管理等方面进行了介绍。     

8例散发儿童肉毒中毒的临床特点及神经电生理分析北大核心CSCD

目的探讨儿童肉毒中毒的临床特点和神经电生理结果,旨在提高临床医师对该病的认识。方法回顾性分析2015年8月—2022年10月湖南省儿童医院神经内科确诊的8例肉毒中毒儿童的临床资料。结果8例患儿均有对称性颅神经麻痹和四肢弛缓性麻痹,呈下行性;7例出现呼吸肌麻痹。电生理检查结果显示,复合肌肉动作电位波幅降低5例;高频重复神经电刺激复合肌肉动作电位波幅递增超过40.0%6例;运动单位动作电位呈短时程、低波幅及多位相4例。结论儿童肉毒中毒的主要临床特征为从颅神经开始的对称性、下行性弛缓性麻痹,可出现呼吸肌麻痹;其电生理异常有复合肌肉动作电位波幅降低,高频重复神经电刺激复合肌肉动作电位波幅递增,运动单位动作电位呈短时程、低波幅、多位相。     

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.