Functional posterior rhizotomy as a surgical treatment for spastic cerebral palsy in childhood]

The author reported a case of spastic cerebral palsy in a 4-year-old boy who underwent functional posterior rhizotomy and were followed up for more than one and a half years after surgery to evaluate the degree of spasticity. The patient's preoperative ADL was highly restricted due to severe spasticity. In the surgery, the bilateral rootlets from L2 to S1 were selectively cut if an abnormal reflex activity was demonstrated by neurophysiological methods. Spasticity markedly decreased postoperatively and alleviated the family's burden for daily care. During the period of follow up, residual spasticity has subsided and the effect in controlling spasticity was long-standing. Functional posterior rhizotomy has been recognized as an established neurosurgical treatment for spastic cerebral palsy in childhood in the North America. However, the procedure is uncommon in Japan. The author outlined here the procedure and its history. Functional posterior rhizotomy is a strong armament for treating spasticity in cerebral palsy. The procedure would greatly benefit patients with spastic cerebral palsy in combination with current treatments.     

Selective peripheral neurotomy (SPN) for spasticity in childhood

Overview Excess spasticity leads to disability that is marked by impaired locomotion, handicapping deformities and, if not controlled, discomfort and pain. Selective peripheral neurotomy in the child is indicated for severe focal spasticity, when botulinum toxin injections cannot delay surgery any longer. Materials and methods Preoperative motor blocks mimicking the outcome of the surgical procedure are essential to establish the objectives of neurotomy. In the lower limb, obturator neurotomy is indicated for spasticity in the adductor muscles, hamstring neurotomy for the knee flexion and tibial neurotomy for the spastic foot. Anterior tibial neurotomy is indicated for the extensor hallucis spasticity and femoral neurotomy for spasticity in the quadriceps. In the upper limb, neurotomy of the pectoralis major and teres major nerves is indicated for spasticity of the internal rotators of the shoulder. Neurotomy of the musculocutaneous nerve is indicated for spasticity of the flexors of the elbow, and neurotomy of median and ulnar nerves are indicated for spasticity of the pronators and flexors of the wrist and fingers. Conclusion Selective peripheral neurotomy is a valuable neurosurgical procedure in well-trained surgical hands for severe focalised spasticity.     

Selective posterior rhizotomy: experience of 30 cases

The increasing interest in selective posterior rhizotomy for reduction of spasticity in children with cerebral palsy and other neurological disorders comes from the selectivity that this procedure has achieved with intra-operative electromyographic monitoring. Thirty patients were operated on between April 1989 and October 1991. Spasticity was of cerebral origin in 27 cases and secondary to spinal cord lesion in 3 others. A reduction in the abnormally high muscle tone was observed in all cases, mainly in the lower extremities, but also, to a lesser degree, in the upper extremities. All patients showed functional improvements that depended on the individual preoperative condition.Even severely disabled patients with quadriplegia and intellectual impairment, whose spasticity interfered with their daily care, had a significantly improved quality of life after rhizotomy. These patients became much looser, with better swallowing and less drooling, and were much more easily managed by their caretakers. Preliminary results with follow-up from 1 to 30 months indicates that selective posterior rhizotomy is a safe procedure which contributes to significant functional improvement in spastic patients.The clinical aspect of this paper was presented at the XIII Congress of the European Society for Paediatric Neurosurgery, Berlin 1992, and the surgical aspect and results were presented at the XX Annual Meeting of the International Society for Pediatric Neurosurgery, Prague 1992     

The surgical management of spasticity

Neurosurgery is only considered for severe spasticity following the failure of noninvasive management (adequate medical and physical therapy). The patients are carefully selected, based on rigorous multidisciplinary clinical assessment. In this we evaluate the contribution of the spasticity to the disability and any residual voluntary motor function. The goals for each patient are: (a) improvement of function and autonomy; (b) control of pain; and (c) prevention of orthopaedic disorders. To achieve these objectives, the surgical procedure must be selective and reduce the excessive hypertonia without suppressing useful muscle tone and limb functions. The surgical procedures are: (1) Classical neuro-ablative techniques (peripheral neurotomies, dorsal rhizotomies) and their modern modifications using microsurgery and intra-operative neural stimulation (dorsal root entry zone: DREZotomy). These techniques are destructive and irreversible, with the reduced muscle tone reflecting the nerve topography. It is mainly indicated when patients have localized spasticity without useful mobility. (2) Conservative techniques based on a neurophysiological control mechanism. These procedures are totally reversible. The methods involve chronic neurostimulation of the spinal cord or the cerebellum. There are only a few patients for whom this is indicated. Conversely, chronic intrathecal administration of baclofen, using an implantable pump, is well established in the treatment of diffuse spasticity of spinal origin. From reports in the literature, we critically review the respective indications in terms of function, clinical progression and the topographic extent of the spasticity.     

Residual spasticity after selective posterior rhizotomy

The technique used in performing selective posterior rhizotomies to treat spastic cerebral palsy remains controversial. One hundred nine children who had undergone selective posterior rhizotomies were studied 6 months after their surgery. Their residual spasticity was correlated to the number of roots and whether or not abnormally responding roots were left, in order to validate the surgical technique used to treat spastic cerebral palsy at most neurosurgical centers in North America. The children were divided into three groups (group A: children who had their L2-S1 roots tested and selectively lesioned,n = 15; group B: children who had their L2-S2 roots tested and selectively lesioned,n = 62; group C: children who had their L2-S2 roots tested and whose lesioning was directed both by the response to the stimulation and mapping of the S1–S3 dorsal roots for afferent pudendal nerve activity,n = 32). Clinically significant residual spasticity was present in the gastrocnemius in 33% of the group A children, 11% of the group B children, and 6% of the group C children. We found that there was no significant increase in residual spasticity in the group C children when abnormally responding roots were not cut in order to preserve pudendal nerve activity. This study shows that the inclusion of the S2 roots decreases the amount of residual spasticity (P<0.01). It also shows that leaving abnormally responding S2 roots to preserve pudendal nerve activity does not affect the incidence of postoperative spasticity (P>0.1).     

Two cases of improvement of smooth pursuit eye movements after selective posterior rhizotomy

Objective  Selective posterior rhizotomy (SPR) represents a standard neurosurgical approach in the treatment of spasticity in children with cerebral palsy (CP). Beside the reduction of spasticity in lower limbs, SPR may have suprasegmental effects, considerably above the surgery site. In this communication, we report on the improvement of smooth pursuit eye movements (SPEM) in two children after SPR. Material and methods  Four children with CP underwent SPR. Eye movements were registered by infrared video-oculography before and after the surgery. Results  The analysis of SPEM showed the improvement of the correlation coefficient of the eye response to the stimulus after SPR in two subjects. Improvement of SPEM performance was largely due to suppression of spontaneous fixation nystagmus. Conclusion  SPR may lead to the improvement of SPEM in children with CP. The influence of SPEM improvement on quality of life in a group of severely disabled nonambulant children with CP remains to be assessed.     

存在瘤内血栓的颅内大型动脉瘤的手术治疗

目的探讨伴有瘤内血栓的颅内大型或巨大型动脉瘤的诊断和手术治疗。方法本组瘤内有陈旧性血栓的颅内大型动脉瘤62例,其中行动脉瘤瘤颈夹闭或塑型夹闭47例,动脉瘤孤立术13例,动脉瘤部分切除后缝合瘤壁重建载瘤动脉1例,另1例双侧椎动脉汇合处巨大梭形动脉瘤在深低温、停循环技术辅助下行载瘤动脉重建手术。结果根据GOS评分,病人恢复良好51例(82.3%),中残4例(6.5%),重残2例(3.2%),死亡5例(8.1%)。中、重残病例随访一年以上,中残的4例中3例恢复术前生活能力;重残的2例中,1例因大面积脑梗死、偏瘫仅有部分恢复,另1例深低温、停循环辅助手术病例,手术后2d内意识清楚,但术后13个月因肝功能衰竭死亡。结论颅内血栓性大型或巨大型动脉瘤手术处理困难,致残率及死亡率高于同类型无血栓的颅内动脉瘤。载瘤动脉暂时性阻断、动脉瘤切开血栓清除均有助于瘤颈夹闭。     

Investigation of raising burden of children with autism, physical disability and mental disability in China

The family economic burden of raising autistic children, physical disabled children and mental disabled children were evaluated in China. 227 parents of children with autism, children with physical disability, children with mental disability and normal children were interviewed for children's costs, family income and economic assistance, etc. The medical cost and caring cost of disabled children were significantly more than those of normal children, and the education cost, clothes cost and amusement cost of disabled children were significantly less than those of normal children. Family income was only predicted by parents’ education level. Families of disabled children received more economic assistance than families of normal children except families of autistic children. More children the family had, less economic assistance the family acquired. Compared with normal children, the raising burden of children with disabilities were as follows: children with autism (19582.4 RMB per year), children with physical disability (16410.1 RMB per year), children with mental disability (6391.0 RMB per year). Families of autistic children, physical disabled children and mental disabled children have heavier raising burden than families of normal children, they need more help from many aspects.     

内镜下经侧裂岛叶入路治疗高血压基底核血肿

目的探讨内镜下经外侧裂岛叶入路治疗高血压脑出血的方法及疗效。方法回顾性分析67例采用经侧裂岛叶入路内镜下手术治疗的高血压基底核脑出血病人的临床资料。结果术后血肿清除率〉90％以上59例,血肿清除率达80％～90％8例。无再次出血病例。随访6个月,以GOS分级进行评定：恢复良好37例,轻度残疾23例,重度残疾3例,植物生存3例,死亡1例。结论内镜下经侧裂岛叶入路具有视野开阔、损伤小、并发症少等优点,是治疗高血压基底核出血安全、有效的治疗方法。     

高血压性脑干出血的个体化手术治疗

目的 探讨高血压性脑干出血的显微手术治疗的手术指征、手术技巧、手术方法和预后。方法 回顾性分析2011年 1月至2018年12月收治的54例高血压性脑干出血的临床资料。根据出血量及出血部位采用个体化手术治疗。结果 术后3个月,按GOS评分:脑室穿刺治疗的30例中,8例恢复良好,12例中残或重残,4例植物状态生存,6例死亡;开颅手术治疗的20例中,3例恢复良好,5例中残或重残,5例植物状态生存,7例死亡;脑干穿刺治疗的4例中,中残2例,重残1例,植物状态生存1例。结论 根据出血量及出血部位采用不同的方法治疗高血压性脑干出血,效果良好。     

The surgical treatment of cervical myelopathy due to spondylosis and disc degeneration.

A personal prospective study of the surgical treatment of cervical myelopathy due to spondylosis and disc protrusion is presented. One hundred and thirty-seven patients with severe disability have undergone surgery according to a protocol involving both the anterior approach of Cloward and decompressive laminectomy. Fifty-two percent of patients have returned to full employment, 39% to light employment and 9% remained disabled though improved from their pre-operative status. The pathenogenesis, natural history and treatment are discussed and reasons advanced to suggest that the disability resulting from severe forms of the condition may be alleviated in the majority of cases.     

Psychiatric disorders in children with intellectual disability

We investigated psychiatric disorders in intellectually disabled children; the prevalence rate and types of psychiatric disorders, and the association of psychiatric disorders with sex, residence type, level of disability, and epilepsy were examined. The population comprised 155 intellectually disabled children. Medical information was obtained from case files, which indicated that a psychiatric disorder had previously been diagnosed for 11% of patients. A careful re-examination of the case files revealed a psychiatric disorder for 33%. The criteria of a psychiatric ICD-10 diagnosis were fulfilled by 23%, and a further 10% had an unspecified mental disorder. The most common diagnoses were pervasive developmental disorders and hyperkinetic disorders. The former were less common among children with mild intellectual disability. In conclusion, about one-third of children with intellectual disability have a psychiatric disorder which needs to be taken into account in treatment.     

Psychiatric disorders in children with intellectual disability

We investigated psychiatric disorders in intellectually disabled children; the prevalence rate and types of psychiatric disorders, and the association of psychiatric disorders with sex, residence type, level of disability, and epilepsy were examined. The population comprised 155 intellectually disabled children. Medical information was obtained from case files, which indicated that a psychiatric disorder had previously been diagnosed for 11% of patients. A careful re-examination of the case files revealed a psychiatric disorder for 33%. The criteria of a psychiatric ICD-10 diagnosis were fulfilled by 23%, and a further 10% had an unspecified mental disorder. The most common diagnoses were pervasive developmental disorders and hyperkinetic disorders. The former were less common among children with mild intellectual disability. In conclusion, about one-third of children with intellectual disability have a psychiatric disorder which needs to be taken into account in treatment.     

Evaluation of social functioning ability of disabled children

Since 1999, we have been trying to establish a reliable method for evaluating social functioning ability (SFA) of physically handicapped children, in cooperation with the Project of Comprehensive Study of Disability, Social Health, and Welfare supported by the Ministry of Health, Labor and Welfare, Japan. In this study, we investigated the reliability and accuracy of the 4th edition of "The Evaluation of SFA". This evaluation consists of 40 items in 15 areas. We examined 45 disabled patients (26 males and 19 females) aged 12-27 years (mean age, 16 years). The reliability was checked by agreement of evaluations of the same patient carried out by two examiners. The results showed good reliability (kappa value of 0.42). According to the results of questionnaires, 97% of the examiners regarded this evaluation to be useful. Although the score of SFA correlated with intelligent quotient/development quotient, the score did not correlate with motor disability level. We hope that this evaluation will be widely used to upgrade SFA of disabled children.     

胫神经部分切断治疗脑瘫性踝痉挛

目的 探讨胫神经部分切断治疗脑瘫患儿踝痉挛的效果。方法 回顾分析近1年37例病儿53只足踝痉挛采用胫神经部分切断术的疗效。结果 全部病人术后有不同程度踝痉挛缓解,平均随访8.6个月,缓解率为94.34％(50/53)。术后发生小腿、足部感觉障碍2例,随访期间均见好转。结论 胫神经部分切断术是治疗脑瘫患儿踝痉挛安全有效的显微外科手术方法。     

Physiologic and pharmacologic approaches to spasticity

The term spasticity is used to describe many relatively unrelated syndromes and, because they share few common pathophysiologic mechanisms, it is not possible to define the physiology or pharmacology of spasticity. In patients with spastic paresis, it is the latter negative symptom (rather than the spasticity) that accounts for almost all the functional disability. Clinical neurophysiologic techniques are useful for categorization of patients with clinically identical syndromes into subgroups which respond to different therapies. Fusimotor or spindle primary afferent hyperactivity have not been demonstrated in spastic patients; reduction in central inhibitory mechanisms probably accounts for spastic hyper-reflexia. Increased passive muscle stiffness may also be clinically significant. Therapies for spasticity include elimination of causative or enhancing factors, frequent muscle stretching, surgical approaches and chemotherapy. The latter includes dantrolene (which weakens muscles), baclofen (particularly useful for reduction of flexor spasms and flexor dystonia in patients with spinal lesions) and diazepam.     

Selective dorsal rhizotomies in the treatment of spasticity related to cerebral palsy

Rationale Selective dorsal rhizotomy (SDR) is a surgical technique developed over the past decades to manage patients diagnosed with cerebral palsy suffering from spastic diplegia. It involves selectively lesioning sensory rootlets in an effort to maintain a balance between elimination of spasticity and preservation of function. Several recent long-term outcome studies have been published. In addition, shorter follow-up randomized controlled studies have compared the outcome of patients having undergone physiotherapy alone with those that received physiotherapy after selective dorsal rhizotomy. Materials and methods In this account, we will discuss the rationale and outcome after SDR. The outcome is addressed in terms of the gross motor function measurement scale (GMFM), degree of elimination of spasticity, strength enhancement, range of motion, fine motor skills, activity of daily living, spastic hip, necessity for postoperative orthopedic procedures, bladder and sphincteric function, and finally possible early or late complications associated with the procedure. Conclusion We conclude that SDR is a safe procedure, which offers durable and significant functional gains to properly selected children with spasticity related to cerebral palsy.     

Adductor spasticity in children with cerebral palsy and treatment with botulinum toxin type A: the parents' view of functional outcome

Botulinum toxin type A (BTX-A) has been used successfully to manage spasticity in children with cerebral palsy. Little has been done to evaluate treatment outcome and satisfaction from the patients' and parents' points of view. The aim of this study was to investigate the parents' perceptions of the benefits of BTX-A on movement disorders in children with cerebral palsy. Twenty-six children with adductor spasticity were enrolled into an open-label, prospective study. Patients received intramuscular injections of BTX-A, and assessments of joint mobility (passive range of motion), degree of spasticity (Modified Ashworth Scale) and functional benefit (Gross Motor Function Measure) were made before and 12–18 weeks after treatment. Parents' assessment of treatment outcomes were evaluated using a standardised questionnaire. BTX-A was shown to be effective in reducing muscular hyperactivity and functional limitations. Parents' satisfaction with the treatment outcome was high. For non-ambulatory patients, the reported benefits included facilitation of daily care, ease of positioning and reduction of pain. For patients who were disabled to a lesser extent, improvements in gait and posture included sitting with improved comfort, standing for longer periods of time and/or walking longer distances. The parents' responses supported the impressions of the therapists, demonstrating that BTX-A produced beneficial effects on daily activities, according to both objective measures and parents' observation.     

Cordectomy as final treatment option for diffuse intramedullary malignant glioma using 5-ALA fluorescence-guided resection

Background

We present a case of an anaplastic astrocytoma (WHO-grade III, AA III) in a 27-year-old woman treated by spinal cordectomy. The patient was pretreated by surgery, radiation therapy and temozolomide chemotherapy and repeat surgery at recurrence. Later on, she developed paraplegia and a diffuse severe pain syndrome. MRI demonstrated intramedullar invasion from T12 to T9. To assess tumor invasion intraoperatively, we used tumor fluorescence derived from 5-aminolevulinic acid (5-ALA).

Patients course

The spinal cord was amputated caudally to the root entry zones of the T10 sensory roots. Additional cordectomy was performed because of tumor infiltration at the cut end to T9 as identified by intraoperative tumor fluorescence, and as verified histologically. The final transected level was between T8 and T9, and the cut end did not reveal any tumor invasion intraoperatively by tumor fluorescence and postoperatively by MRI and with regard to the pathological result. After surgery, the patient was unchanged concerning spasticity, motor and sensory function, and showed complete relief of pain. She refused additional adjuvant therapy. The patient is free of recurrence 15 months after surgery.

Conclusion

Our observation suggests 5-ALA fluorescence-guided resections to be useful in the context of malignant spinal cord gliomas. Furthermore, our particular case indicates that palliative spinal cordectomy with a wide margin and intraoperative resection using fluorescence guidance may be a final option for patients with recurrent spinal malignant glioma presenting with complete deficit below the lesion.     

Epilepsy surgery in people with intellectual disability – English Version

In patients with refractory epilepsy, evaluation of epilepsy surgery should be part of the diagnostic work-up. Epilepsy and refractory epilepsy are common in people with intellectual disability. Therefore, epilepsy surgery with its chances of success and its risks has to be discussed. The presurgical diagnostic procedures may have to be adapted to the patientʼs cognitive and emotional abilities. Whether people with intellectual disabilities experience postoperative seizure freedom less frequently, has yet to be determined. Patients with genetic epileptic encephalopathies (particularly channelopathies) as well as non-lesional patients usually are not good surgical candidates. Neurocognitive functions usually do not suffer after epilepsy surgery in patients with intellectual disability. Even nonresective epilepsy surgery may be carried out with good outcome both in children and adult patients. Therefore, epilepsy surgery is an important tool in epilepsy care for people with intellectual disability.