Stiff muscle fibers in calf muscles of patients with cerebral palsy lead to high passive muscle stiffness

Cerebral palsy (CP), caused by an injury to the developing brain, can lead to alterations in muscle function. Subsequently, increased muscle stiffness and decreased joint range of motion are often seen in patients with CP. We examined mechanical and biochemical properties of the gastrocnemius and soleus muscles, which are involved in equinus muscle contracture. Passive mechanical testing of single muscle fibers from gastrocnemius and soleus muscle of patients with CP undergoing surgery for equinus deformity showed a significant increase in fiber stiffness (p < 0.01). Bundles of fibers that included their surrounding connective tissues showed no stiffness difference (p = 0.28).). When in vivo sarcomere lengths were measured and fiber and bundle stiffness compared at these lengths, both fibers and bundles of patients with CP were predicted to be much stiffer in vivo compared to typically developing (TD) individuals. Interestingly, differences in fiber and bundle stiffness were not explained by typical biochemical measures such as titin molecular weight (a giant protein thought to impact fiber stiffness) or collagen content (a proxy for extracellular matrix amount). We suggest that the passive mechanical properties of fibers and bundles are thus poorly understood. © 2014 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 32:1667–1674, 2014.     

Local and distant effects of isolated calf muscle lengthening in children with cerebral palsy and equinus gait

Purpose The purpose was to assess the local and distant effects of isolated calf muscle lengthening in ambulant children with cerebral palsy. Methods The study included fifteen ambulant children with cerebral palsy (nine with diplegia and six with hemiplegia), average age 8.8 years, Gross Motor Function Classification System (GMFCS) level I and II. None of the children had previously undergone orthopaedic surgery, apart from one child who had tendo-achilles lengthening (TAL) nine years earlier. All the children underwent pre and post-operative clinical examination and three-dimensional gait analysis (gait analysis). Twenty calf muscle lengthenings were performed, ten TAL and ten gastrocnemius recessions (GR). Results Post-operative ankle kinematics showed significant improvements in all parameters. Ankle power during push-off increased, but only significantly after TAL. Only one limb (5%) was over-corrected. Four limbs (20%) were under-corrected and one of these limbs remained in mild equinus position in stance. There was one recurrent equinus (5%) during the follow-up period of three years (range: 13–55 months). Distant effects on joints and segments were more marked in diplegia than in hemiplegia. Ten of 17 kinematic parameters distant from the ankle joint improved significant post-operatively when the preoperative values were 1SD below or above the mean of the normal material. There was no significant deterioration in any of the measured parameters. Conclusion The improvement in ankle kinematics and kinetics supported the experience of other studies. The distant effects, which have previously not been evaluated in three planes, showed improvement in several kinematic parameters indicating that additional surgery in selected patients could be abandoned or delayed.     

Janeway gastrostomy in children with cerebral palsy

Brain damaged children frequently are unable to eat by mouth due to incoordination of tongue and pharyngeal swallowing muscles. The commonly utilized Stamm gastrostomy has several drawbacks relative to the indwelling catheter. A permanent gastrostomy consisting of a mucosal-lined tube flap from the stomach wall, as described by Janeway, is easily constructed using the GIA stapler. This provides a simple, tubeless, and safe way to feed cerebral palsied children unable to eat by mouth.     

Overstretching of sarcomeres may not cause cerebral palsy muscle contracture.

To answer the question whether the muscle contracture in patients with cerebral palsy is caused by overstretching of in-series sarcomeres we studied the active and passive force-length relationship of the flexor carpi ulnaris muscle (FCU) in relation to its operating length range in 14 such patients with a flexion deformity of the wrist. Force-length relationship was measured intra-operatively using electrical stimulation, a force transducer, and a data-acquisition system. Muscle length was measured in maximally flexed and maximally extended position of the wrist. The spastic FCU was found to exert over 80% of its maximum active force at maximal extension of the wrist and this indicates abundant overlap of the sarcomeres. At maximal wrist extension, FCU passive force corresponded with only 0.7-18% of maximum active force. Both findings imply that the FCU sarcomeres are not overstretched when the wrist is extended. We conclude that the overstretching of in-series sarcomeres appears not to be the cause of contracture of the spastic FCU.     

Urodynamic findings in children with cerebral palsy

AIM: More than one-third of children with cerebral palsy are expected to present with dysfunctional voiding symptoms. The voiding dysfunction symptoms of the cerebral palsy patients in the present study were documented. METHODS: Of the study group, 16 were girls and 20 were boys (mean age: 8.2 years). Children with cerebral palsy were evaluated with urodynamics consisting of flow rate, filling and voiding cystometry, and electromyography findings of the external urethral sphincter to determine lower urinary tract functions. Treatment protocols were based on the urodynamic findings. Anticholinergic agents to reduce uninhibited contractions and to increase bladder capacity were used as a treatment. Clean intermittent catheterization and behavioral modification were used for incomplete emptying. RESULTS: Of the children, 24 (66.6%) were found to have dysfunctional voiding symptoms. Daytime urinary incontinence (47.2%) and difficulty urinating (44.4%) were the most common symptoms. Urodynamic findings showed that neurogenic detrusor overactivity (involuntary contractions during bladder filling) with a low bladder capacity was present in 17 (47.2%) children, whereas detrusor-sphincter dyssynergia was present in four patients (11%). The mean bladder capacity of patients with a neurogenic bladder was 52.2% of the expected capacity. CONCLUSIONS: The present study concluded that voiding dysfunction was seen in more than half of the children with cerebral palsy, which is a similar result to other published studies. We propose that a rational plan of management of these patients depends on the evaluation of the lower urinary tract dysfunction with urodynamic studies. These children benefit from earlier referral for assessment and treatment.     

Postoperative neuroleptic malignant syndrome that occurred repeatedly in a patient with cerebral palsy

A wide variety of neuroleptic agents are associated with neuroleptic malignant syndrome (NMS). However, the association between general anesthesia and NMS is uncertain. We report a case of a patient with cerebral palsy, who showed signs of NMS only after repeated general anesthesia. The patient received general anesthesia three times in a period of 9 months. The first anesthetic passed uneventfully. NMS symptoms were observed only after the second and third anesthetics. The NMS was effectively treated with IV dantrolene and the patient recovered on both occasions. Inhalational anesthetics, muscle relaxants and fentanyl were suspected as possible triggering factors for NMS. After examining the three anesthesia records and previous publications, we surmized that a nondepolarizing muscle relaxant was associated with NMS in this patient.     

Surgical correction of equinus deformity in children with cerebral palsy: a systematic review

Purpose  

Equinus is the most common deformity in cerebral palsy. However, despite the large volume of published studies, there are poor levels of evidence to support surgical intervention. This study was undertaken to examine the current evidence base for the surgical management of equinus deformity in cerebral palsy.     

Correlation between motor function and lower urinary tract dysfunction in patients with infantile cerebral palsy

AIMS: The aim of this study was to evaluate urodynamic findings in patients with infantile cerebral palsy (CP) and to correlate the findings with impaired motor function. METHODS: We conducted a videourodynamic investigation on a highly select group of 29 patients (3-53 years). Motor function was assessed in each patient by the Gross Motor Function Classification System for CP (GMFCS). With this system, motor function is divided into five levels: patients in Level I have the most independent motor function and patients in Level V the least. The patients were divided into Group 1 (23 symptomatic patients with recurrent urinary tract infection or urinary incontinence) and Group 2 (6 asymptomatic patients). RESULTS: In Group 1, 21 patients (91%) had reduced compliance (0.6-16.4 ml/cmH(2)O) and 16 patients (70%) had increased DLPP (>40 cmH(2)O). Detrusor overactivity and pelvic floor overactivity were found in all 23 patients. In Group 2, two patients (33%) had reduced compliance (0.7 and 5.8 ml/cmH(2)O) and four (67%) had increased DLPP (>40 cm H(2)O). Detrusor overactivity and pelvic floor overactivity were observed in five patients (83%). Symptomatic patients showed higher GMFCS levels than asymptomatic patients. In the group of asymptomatic patients, there was no one classified as Levels IV or V, while there were no symptomatic patients classified as Level I. CONCLUSIONS: We conclude that urinary symptoms and pathological urodynamic findings increase along with the degree of motor function impairment shown by the GMFCS. Pathologic urodynamic findings can be found in both symptomatic and in asymptomatic patients.     

中日友好医院脑性瘫痪手术病例患病相关因素分析

目的探讨脑性瘫痪患病相关因素。方法回顾性分析卫生部中日友好医院神经外科及脑瘫治疗中心5年来手术治疗的1605例脑瘫患儿病因方面的资料，采用多元Logistic回归分析统计学方法对脑瘫的患病相关因素进行分析。结果早产低出生体重、难产窒息缺氧是各型脑瘫的主要患病相关因素；新生儿黄疸是手足徐动型和混合型脑瘫的重要患病相关因素。结论提高对脑瘫患病相关因素的认识有利于早期预防脑瘫的发生、早期诊断、早期治疗。     

Stair-shaped Achilles tendon lengthening in continuity — A new method to treat equinus deformity in patients with spastic cerebral palsy

Background

Equinus of the ankle is a common deformity in spastic cerebral palsy. Achilles tendon lengthening is one of the effective options for the treatment of equinus deformity.

脑瘫患者选择性脊神经后根切断术后康复训练效果观察

目的总结脑瘫患者选择性脊神经后根切断术(SPR)术后康复训练的效果。方法对随机选择的20例实施SPR手术的痉挛性脑性瘫痪患者执行预先拟订的康复训练方法并分别在术前、术后及康复后进行肌力、肌张力和关节活动度的测定,观察坐、立、跪、走的姿势。结果手术后康复训练与否肌力改变差异显著(P<0.05)。手术前、手术后与康复治疗后三者之间关节活动度改变在统计学上均有显著差异(P<0.05)。随访资料完整的18例显示坐位、直跪、站立及行走姿势均有明显改善。结论SPR可解除痉挛、降低部分肌张力,改善运动功能,而术后正确的康复训练是改善运动功能不可缺少的重要环节。     

Modulation of ryanodine-induced contractures in human skeletal muscle pretreated with dantrolene

Dantrolene seems to be the causal therapy in malignant hyperthermia (MH) crisis but the complex mechanisms of MH and dantrolene therapy are still not fully understood. The influence of dantrolene on ryanodine-induced contractures has been reported in animal studies only. In the present study 20 patients from] 7 families were tested for MH using the protocol of the European Malignant Hyperthermia Group. In addition ryanodine-induced contractures were evaluated following bolus application of 10.0 μmol · 1^-1 ryanodine. After pretreatment with 1 μimol · 1^-1 dantrolene ryanodine-provoked contractures developed significantly later in MHS (15.8±1.8 min) and MHN (46.0±4.2 min) muscle specimens than after ryanodine alone (MHS 4.8±0.7 min), (MHN 13.7±0.9 min). They were no longer observed in either group after pretreatment with 5 μimol · 1^-1 dantrolene. We conclude that dantrolene is able to attenuate ryanodine-induced contractures dose-dependendy, and therefore it is speculated that dantrolene could specifically act at the ryanodine receptor binding site.     

Oculomotor nerve palsy in patients with cerebral aneurysms

Twenty-six patients with oculomotor nerve palsy due to cerebral aneurysms were examined. There were six males and twenty females with a mean age of 55 years. 25 of the 26 aneurysms were located at the junction of the internal carotid and the posterior communicating artery and one was at the junction of the basilar artery and the superior cerebellar artery. Twelve patients had associated subarachnoid hemorrhage (SAH); the other 14 did not. The initial symptoms in many patients were ptosis and double vision. Twenty-one of the patients had total oculomotor nerve palsy, one had a sparing of medial rectus muscle; three patients had only ptosis and anisocoria, and one had oculomotor nerve palsy with pupillary sparing. All aneurysms, including giant aneurysms, were clipped under a microscope, and six oculomotor nerves were found to be decompressed at surgery. The follow-up periods were from six months to three years. Nine patients had a complete recovery of oculomotor function; thirteen had an incomplete recovery; and four remained unchanged. The mean interval between the onset of palsy and the time of surgery was 24 days in complete recovery cases, 42 days in incomplete recovery cases, and 119 days in unchanged cases. The recovery of oculomotor function started with the levator palpebrae muscle and followed by the medial rectus muscle. The recovery of pupillary function was, however, not consistent. Of the factors influencing recovery from oculomotor nerve palsy, the interval between the onset of palsy and the time of surgery was most important. Therefore, aneurysms with oculomotor nerve palsy should be operated on as early as possible, regardless of the presence or absence of SAH.     

The psoas muscle as cause of low back pain in infantile cerebral palsy

Psoas muscle spasticity is hypothesised as a rare cause of low back pain in patients with infantile cerebral palsy. The authors describe a new manoeuvre for the study of psoas tenderness and ultrasound (US)-guided transabdominal botulinum toxin injection technique. A possible causal relationship between psoas tension and low back pain was found incidentally in two examined cases. In subsequent patients, botulinum toxin was injected and, in cases of disappearance of symptoms, the psoas tendon was sectioned at the pelvic brim with definitive disappearance of pain. The relationship between psoas tension and low back pain in patients with infantile cerebral palsy seems likely, given the result in the four patients.     

1090例脑性瘫痪外科手术治疗统计分析

目的分析我国脑性瘫痪（cerebralpalsy,CP）的病因、手术年龄分布、临床特征和外科治疗方法,为了解我国CP发病情况、病情特点、外科治疗状况等提供大样本的临床参考资料。方法回顾性分析1990年9月至2007年12月行外科手术治疗的CP患者1090例,采用SPSSl2软件对资料进行统计学分析。结果1090例脑瘫患者中,男760例,女330例;手术时年龄3～41岁,其中3～5岁167例,6～10岁356例,11—15岁304例,16～25岁218例,26岁以上45例;CP病因中早产占29％、难产窒息占23．9％,明显高于出生后的其他原因,有16％不确定发病原因;CP分型中双下肢痉挛型最多占41％,其次是偏瘫痉挛型25．5％和四肢瘫痉挛型12．1％;20．09％合并流涎,4．59％合并癫痫;术前徒手跛行53％,扶物站立行走31．5％,不能站立行走者15．5％;51．2％智力低于正常,重度智力障碍者占4．1％;语言表达能力低于正常者占53．2％,完全失语占2．8％。1090例cP共实施手术2885术次,其中下肢矫形手术为2535术次,上肢矫形手术59术次,颈总动脉外膜交感神经网剥脱切除术269术次,选择性脊神经后根切断术（selective posterior rhizotomy,SPR）22术次。结论早产、难产和窒息为痉挛型CP发生的最常见原因。我国存在大量未能在学龄前实施矫形手术的CP患者,甚至到成年期仍未接受正确的手术治疗,致使下肢畸形发展到严重程度,说明对CP进行综合外科治疗的专科医师十分缺乏。CP是一个综合征,矫形手术主要适用于痉挛型。根据患者的年龄、类型和病情特点,选择相应的手术方法;病情复杂者,优化组合不同的手术方法,术后配合合理的康复,以提高外科治疗效果。     

The impact of asymmetry on the radiographical outcomes following hip reconstruction in patients with cerebral palsy

Purpose:The purpose of this study was to evaluate the impact of asymmetric hip dysplasia on the outcome of hip reconstruction in patients with cerebral palsy according to preoperative migration percentage (MP).Methods:This study was institutional review board-approved for retrospective cohort review. From 2008 to 2018, 65 patients met inclusion criteria: Gross Motor Function Scale Classification (GMFSC) III to V with spastic hips (MP > 30%) who underwent bilateral hip reconstruction, with a follow-up > 24 months. Main exclusion criteria: children with associated syndromes or chromosomal disorders. The cohort was subdivided into three groups according to preoperative MP difference between hips: Group A > 50%, group B 20% to 50% and Group C < 20%. Subsequently, the groups were analyzed individually and then compared. The asymmetry of extended abduction of the hip was also evaluated and separated into three groups: no asymmetry (< 20° difference), mild asymmetry (20° to 50° difference) and severe (> 50° difference).Results:In total, 65 patients underwent bilateral bony reconstructive surgery (130 hips). Mean age at surgery was 10.1 years (sd 3.6; 3.6 to 18.4). Mean age at follow-up was 14.7 years (sd 3.8; 8 to 21). Preoperative GMFSC distribution was grade III (four, 6%), IV (15, 23%) and V (46, 71%). In all, 21 symmetric hips (< 20% MP difference) had a preoperative MP difference of 9% and a follow-up MP difference of 18% (p > 0.05); 32 had a preoperative MP difference of 34% and a follow-up MP difference of 16% (p < 0.0001); 12 had a preoperative MP difference of 80% and a follow-up difference of 6% (p < 0.0001). According to pre- and postoperative abduction values, the mean high hip abduction preoperatively was 34° (sd 17°), whereas low hip abduction was 23° (sd 17°).Conclusion:Hips with asymmetrical dysplasia and/or abduction undergoing bilateral reconstructive surgery focused on symmetric abduction, and corrected dysplasia in patients with cerebral palsy has improved symmetry in hip abduction and MP. Obtaining this goal immediately postoperatively is maintained to medium-term follow-up.Level of Evidence:IV