Somatosensory eye blink reflex in peripheral facial palsy

To investigate the association between somatosensory blink reflex (SBR) and peripheral facial palsy (PFP) severity and trigeminal blink reflex (BR) changes in cases with PFP and subsequent postparalytic facial syndrome development (PFS). One hundred and twenty subjects with peripheral facial palsy and post-facial syndrome and 44 age and gender matched healthy volunteers were enrolled to this study. Blink reflexes and somatosensory blink reflex were studied in all. The association between R1 and R2 responses of the BR and SBR positivity was investigated. SBR was elicited in 36.3% of normal subjects, in 18.3% of PFP and in 65.3% of PFS patients. In the paralytic side, the frequency of SBR positivity was significantly lower in PFP group compared to controls and SBR was most frequently observed in patients with PFS. Compared to PFP and control groups, SBR positivity on the non-paralytic side significantly revealed a higher rate in PFS patients. SBR positivity of patients in whom R1 or R2 were absent, was significantly lower than those subjects with prolonged or normal R1 or R2 responses. PFP and successive PFS are good models for the sensory motor gate mechanisms and/or excitability enhancement of brainstem neurons responsible for SBR.     

Influence of age and gender on the jaw-stretch and blink reflexes

The aim of this study is to investigate the influence of age and gender on jaw-stretch and blink reflexes (BR). Thirty “young” (26.5±0.7 years) and thirty “old” (47.8±1.8 years) healthy adults were included. Short-latency stretch reflex responses were evoked in the masseter and temporalis muscles by fast jaw-stretches, and BR in orbicularis oculi muscle were evoked by painful electrical pulses (0.5 ms duration), delivered by a concentric electrode placed on the left lower forehead close to the supraorbital foramen. For the jaw-stretch reflex, the pre-stimulus EMG activity in the old subjects was significantly lower than that of the young subjects in the right and left masseter and temporalis muscles (P<0.006), whereas there was no difference in the results between males and females. The normalized peak-to-peak amplitude of the EMG in the left masseter and left and right temporalis muscles was significantly lower in the old subjects compared with the young subjects (P<0.02). Females had significantly higher normalized peak-to-peak EMG amplitudes compared with males in the right masseter and left temporalis muscles (P<0.05). The old subjects had significantly lower root mean square (RMS) (P=0.01) and average (P<0.02) BR values in the right and left orbicularis oculi muscles, and lower area under the curve (AUC) (P=0.02) values in the left orbicularis oculi muscle compared with the young subjects. Female subjects had significantly lower AUC (P=0.02) in the left orbicularis oculi muscle compared with males. The old subjects had significantly later offset (P<0.003) and longer duration (P<0.001) in the left orbicularis oculi compared with the young subjects. The results of the present study demonstrated a significant effect of both age and gender on stretch and BR and suggested that these variables should be taken into consideration in the interpretation of brainstem reflexes in basic and clinical studies.     

Symmetry of neurological signs in Pakistani patients with probable inherited spastic cerebral palsy

Seven Pakistani families are described with children manifesting a spastic cerebral palsy-like syndrome. One feature common to all affected patients was symmetry of neurological signs. Only one of these families had been offered genetic counselling and fewer than half had undergone clinical investigations. The importance of symmetry of neurological signs is reaffirmed as an indicator of a probable genetic aetiology in children with spasticity. Many families at risk of recurrence are not offered appropriate genetic advice, and some children with potentially diagnosable conditions are given a label of cerebral palsy without investigation of possible underlying inherited disorders.     

Fat distribution in children with cerebral palsy

Triceps and subscapular skinfolds were measured on 95 North American children with cerebral palsy. Triceps fat was far more depleted than subscapular fat in comparison with population standards for sex, age and race. This truncal distribution of fat may be related to the high prevalence of under nutrition found in the sample.     

Fat distribution in children with cerebral palsy

Triceps and subscapular skinfolds were measured on 95 North American children with cerebral palsy. Triceps fat was far more depleted than subscapular fat in comparison with population standards for sex, age and race. This truncal distribution of fat may be related to the high prevalence of under nutrition found in the sample.     

Facilitation and inhibition of the human startle blink reflexes by stimulus anticipation

The cutaneous eyeblink has 2 electromyographic components, 1 unilateral and early (R1) and 1 bilateral and late (R2), which are served by different neural pathways. These 2 reactions were measured when the eliciting stimulus was expected or relatively surprising. Forewarning was varied in 3 ways: Subjects received notice that the stimulus was about to occur on some trials (Experiment 1); delivered the stimulus to themselves on some trials (Experiments 2 & 3); or experienced a series of trials in which a tone was paired with the eliciting stimulus, followed by tone-alone trials interspersed with test trials (Experiment 4). In each case, forewarning enhanced R1 amplitudes while depressing R2 but reduced the latency of both components. This mixed pattern of effects reveals that the preparatory state provoked by forewarning focuses excitatory and inhibitory processes simultaneously on different reflex pathways: inhibition central and excitation peripheral.     

腰椎椎板原位指骨钢板回植内固定治疗痉挛性脑性瘫痪

背景：选择性脊神经后根部分切断已广泛应用于痉挛性脑性瘫痪的治疗,以往采用的方法是在脊髓圆锥水平将L1或L1+T12全椎板切除,对解剖结构的破坏较大,远期有导致脊柱畸形的可能。 目的：观察应用选择性脊神经后根部分切断后椎板原位回植指骨钢板内固定治疗痉挛性脑性瘫痪的临床效果。 方法：2008-01/2011-01采用后路选择性脊神经后根部分切断术治疗29例痉挛性脑性瘫痪患者,使用摆锯将相应节段椎板完整截取后再行原位回植,以指骨钢板螺钉进行内固定。观察治疗前后双下肢肌张力变化,通过复查X射线片、CT,观察回植椎板融合情况、椎管形态以及腰椎稳定性等情况。 结果与结论：29例患者均获得随访,治疗后大部分患者双下肢肌张力较治疗前平均降低Ⅱ~Ⅲ级,末次随访时回植椎板均获得骨性融合,未发现明显椎管狭窄、脊柱失稳等相关并发症。提示选择性脊神经后根部分切断治疗痉挛性脑性瘫痪的效果确切,此方案能充分暴露椎管,术后能重建椎管完整性,同时也能有效预防选择性脊神经后根部分切断后瘢痕粘连、脊柱失稳等并发症。关键词：椎板原位回植;脑性瘫痪;内固定;选择性脊神经后根部分切断;脊柱稳定性 缩略语注释：SPR：selective posterior rhizotomy,选择性脊神经后根部分切断 doi:10.3969/j.issn.1673-8225.2012.17.013     

Prestimulation effects on blink and cardiac reflexes of 15-month human infants

Reduction of reflex startle by brief changes in prestimulation is a robust phenomenon in adults of several species. Although the phenomenon does not require structures above midbrain, it has a long and uneven developmental course. This study of human infants assessed prestimulus effects at 15 months, within a period which has failed, in past work, to show the usual inhibitory modulation. Magnitude and onset latency of the startle blink and concurrent changes in heart rate were measured under four conditions: 2 single-stimulus conditions, 25-msec, 84-dB, 1000-Hz tone or 50-msec, 109-dB white noise; 2 paired-stimulus conditions, noise bursts preceded by tone at lead times of 125 msec or 225 msec. Compared to noise-alone, paired conditions elicited insignificant increases in blink size, significant shortening of blink onset latency, and significant attenuation of heart rate responses. The findings add to growing evidence of a dissociation between modulating effects on blink magnitude and latency and of a dissociation between modulating effects on somatic and autonomic reflexes.     

大脑性瘫痪患者的体感诱发电位和运动诱发电位分析

目的；观察大脑性瘫痪（脑瘫，CP）患者体感诱发电位（SEP）和运动诱发电位（MEP）的表现。方法：对15例CP患者作四肢SEP和磁刺激MEP测试，并与对照组进行比较。结果：14例CP患者SEP中有3例异常，异常率21％，15例CP患者中MEP有14例异常，异常率93％，与临床表现相一致。结论：SEP和MEP可检测神经感觉和运动通路功能状况，对CP的诊断可提供客观依据。     

Somatosensory evoked blink response in hemifacial spasm and peripheral facial palsy

Somatosensory blink response (SBR) is produced by electrical stimulation of peripheral nerves or skin areas remote from the face. We investigated the presence of SBR in cases with hemifacial spasm (HFS) and peripheral facial palsy (PFP). Fifty-seven cases of HFS, 54 cases of PFP and 39 normal subjects were included in the study. A routine blink reflex study was performed in all subjects. Supramaximal stimulation of the median nerve was given ipsilateral to the either spasm or paralytic side for SBR. Recordings were made at the orbicularis oculi (o.oc) bilaterally and ipsilaterally at the orbicularis oris (o.or) muscles. SBR was elicited in 12 of 39 control subject. Twenty four of 48 HFS cases were SBR positive. Twelve of them had o.or response. An SBR was elicited at the ipsilateral o.oc in 35 of 46 patients with PFP with synkinesia. Twenty-six patients had an SBR at the ipsilateral o.or. In the 13 patients with PFP without synkinesia only 3 people had an SBR. SBR positivity was seen more often in PFP with synkinesia than in cases with HFS. SBR if positive spreads to the lower part of the face in most of cases with HFS and PFP with synkinesia.     

Biomechanical assessment of patellar tendon advancement in patients with cerebral palsy and crouch gait

BackgroundPatellar height is a valuable measure to evaluate the effect of patellar tendon advancement (PTA) on knee function. In the literature, there is no validated procedure to measure the patellar height. In this study we aimed to (1) determine the patella position through musculoskeletal modeling, (2) investigate the effects of two surgical procedures applied for PTA, and (3) assess the effect of PTA in combination with single-event multilevel surgery (SEMLS) on the knee kinematics of patients with cerebral palsy (CP) and crouch gait.MethodThree-dimensional gait and X-ray data of children with CP and crouch gait were retrospectively analyzed if they had received a SEMLS in combination with PTA (PTA group, n = 18) or without PTA (NoPTA group, n = 18). A computational musculoskeletal model was used to quantify patella position, knee extension moment arm, and knee kinematics pre- and postoperatively.ResultsPatellar height significantly decreased in the PTA group (P = 0.004), while there was no difference in the NoPTA group (P > 0.05). The bony procedure for PTA provided a better Insall–Salvati ratio than the soft tissue procedure. The peak knee extension moment arm significantly increased in the PTA group (P = 0.008). In terms of postoperative knee joint kinematics, the PTA group was closer to typically developed children than the NoPTA group.ConclusionMusculoskeletal modeling was found to be an effective tool for the determination of the patellar height. PTA improved the patella position, knee extension moment arm, and knee kinematics and was an effective procedure for the surgical management of crouch gait in patients with CP.     

Functional recovery in cerebral palsy may be potentiated by administration of selective serotonin reuptake inhibitors

Cerebral palsy is a disorder of movement and posture caused by a non-progressive lesion to the brain. The incidence of cerebral palsy is over 2 per 1000 live births in Europe. Management of cerebral palsy is primarily supportive - none of the current treatments offered attempt to correct the primary problem of a brain lesion.Neurological problems may be treated by upregulating cerebral plasticity. Evidence suggests that this is the mechanism of action of selective serotonin reuptake inhibitors (SSRIs) in the treatment of depression. Encouraging evidence of motor improvements in stroke patients treated with SSRIs suggest the possibility of similar improvements in cerebral palsy. Patients with less severe cerebral palsy show more evidence of plasticity than patients with more severe forms.Evidence should initially come from animal models, and thereafter case reports and case series in selected cases, before progression to large scale trials. SSRIs would have to be used in conjunction with cooling, which prevents secondary damage. Due consideration is needed to prevent harmful side-effects.     

Eye hand coordination in children with cerebral palsy

Reaching to grasp an object of interest requires complex sensorimotor coordination involving eye, head, hand and trunk. While numerous studies have demonstrated deficits in each of these systems individually, little is known about how children with cerebral palsy (CP) coordinate multiple motor systems for functional tasks. Here we used kinematics, remote eye tracking and a trunk support device to examine the functional coupling of the eye, head and hand and the extent to which it was constrained by trunk postural control in 10 children with CP (6–16 years). Eye movements in children with CP were similar to typically developing (TD) peers, while hand movements were significantly slower. Postural support influenced initiation of hand movements in the youngest children (TD & CP) and execution of hand movements in children with CP differentially depending on diagnosis. Across all diagnostic categories, the most robust distinction between TD children and children with CP was in their ability to isolate eye, head and hand movements. Results of this study suggest that deficits in motor coordination for accurate reaching in children with CP may reflect coupled eye, head, and hand movements. We have previously suggested that coupled activation of effectors may be the default output for the CNS during early development.     

Electromyographic analysis of reach in individuals with cerebral palsy

Electromyographic analysis in both the time domain (root mean square EMG) and the frequency domain (mean power frequency EMG) of the biceps, triceps, wrist extensors and wrist flexors were analysed in six young cerebral palsied adults and six normal individuals. The subjects sat in a Rifton positioning chair. Each subject's right arm was positioned with the shoulder adducted, the elbow at 90 degrees and the hand resting on the arm rest. The subject then reached the right arm forward to grasp a dowel which was placed at shoulder level in front of the subject. There was no significant difference between the time it took the two groups to do the required movement. The RMS analysis indicated the muscle activation was variable among subjects, with evidence of concontraction of the antagonist muscles for the disabled group. The frequency analysis indicated that the disabled group had significantly lower mean power for the biceps and the wrist extensor muscles compared to the normal group. Neurological differences or fibre type abnormalities may account for these differences.     

10例以脑发育不良为主要特征的脑瘫患儿的遗传学病因分析

目的探讨脑瘫患儿的遗传学病因。方法对表型存在差异的1对双胞胎脑瘫患儿进行全基因组测序,对另外8例脑瘫患儿进行全外显子组测序,用自定义的过滤流程对所发现的基因变异进行筛选,探讨可能与脑瘫相关的生物学通路和基因。结果共发现3个与脑瘫相关的生物学通路,分别为轴突导向通路(axon guidance)、化学突触传播(transmission across chemical synapses)和突触处蛋白相互作用(protein-protein interactions at synapses),同时发现25个脑瘫易感基因。结论对脑瘫相关的分子机制进行了初步探讨,可能为脑瘫的药物开发提供新的线索。     

Morphofunctional characteristics of skeletal muscle in rats with cerebral palsy

Knowledge of skeletal muscle adaptations is important to understand the functional deficits in cerebral palsy (CP). This study aimed to investigate the morphofunctional characteristics of skeletal muscle in a CP animal model. Initially, pregnant Wistar rats were injected intraperitoneally with saline or lipopolysaccharide over the last five days of pregnancy. The control group (n = 8) consisted of male pups born to females injected with saline. The CP group (n = 8) consisted of male pups born to females injected with lipopolysaccharide, which were submitted to perinatal anoxia [day of birth, postnatal day 0 (P0)] and sensorimotor restriction (P1‐P30). The open‐field test was undertaken on P29 and P45. On P48, the animals were weighed, and the plantaris muscle was collected and its weight and length were measured. Transverse sections were stained with haematoxylin‐eosin, NADH‐TR, Masson's trichrome and non‐specific esterase reaction for analysis. and transmission electron microscopy was performed. In the CP group, reductions were observed in mobility time, number of crossings and rearing frequency, body weight, muscle weight and length, and nucleus‐to‐fibre and capillary‐to‐fibre ratios. There was a statistically significant increase in the percentage area of the muscle section occupied by collagen; reduction in the area and increase in the number of type I muscle fibres; increase in myofibrillar disorganization and Z‐line disorganization and dissolution; and reduction in the area and largest and smallest diameters of neuromuscular junctions. Thus this animal model of CP produced morphofunctional alterations in skeletal muscle, that were associated with evidence of motor deficits as demonstrated by the open‐field test.