低频运动物理治疗理疗干预对遗传性痉挛性截瘫的影响:案例报道。
Physical therapy intervention with a low frequency of exercise for a patient with a complicated form of hereditary spastic paraplegia: a case report.
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2019-12-08 12:43
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火星译客

INTRODUCTION

介绍

Hereditary spastic paraplegia (HSP) consists of a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by progressive spasticity and weakness of the lower extremities1). According to several epidemiological surveys for HSP in Japan, the prevalence rate of HSP in Japan is estimated to be approximately 0.2 per 100,000 populations2).

遗传性痉挛性截瘫(HSP)是临床和基因异质性神经退行性疾病,以四肢进行性痉挛和无力为主要特点 。根据日本对于HSP的流行病学研究,HSP在日本的发病率大约为十万人中0.2人发病。

Clinical types of HSP are classified into pure and complicated forms3). In the complicated form4, 5), the degenerative process affects multiple parts of the nervous systems resulting in various neurological symptoms such as cognitive impairment, seizures, extrapyramidal involvement, ataxia, optic atrophy, neuropathy, and muscular atrophy.

HSP的临床分型分为单纯性和复合型。复合型中,多处神经系统退化,造成多种神经类症状,如认知损伤、癫痫、锥体束外受累、共济失调、视神经萎缩、神精病变和肌肉萎缩。

Concerning physical therapy for HSP, gait patterns6), measurement of functional evaluation7), and disease severity8) of HSP have been the major interest, and only a few studies were performed regarding physical therapy intervention for HSP. To our knowledge, there has been no published literature that described the frequency, intensity and details of physical therapy for the maintenance of the function of lower extremities and Activities of Daily Living (ADL) in HSP patients. Only Asir et al.9) proposed an original 8-week intensive rehabilitation program for pure type HSP from which the functional improvement was obtained. Their program consisted of 60–90 min exercise for each session and the frequency of exercise was 6 days per week.

大家主要对HSP患者的步态、功能障碍的评定、疾病的严重程度是感兴趣,而关于HSP的物理治疗的研究寥寥无几。在我们的认知中,还没有一篇发表的文献来介绍用于维持HSP患者下肢现有的功能和日常生活活动能力的物理治疗的频率、强度、以及其他细节。只有 Asir et al.发表了一个8周康复训练项目,对单纯性HSP的功能有一定改善。这个项目包括每周六天、每次60-90分钟为强度的运动疗法。

We had an opportunity to provide rehabilitation to a patient with complicated form of HSP. We developed an original physical therapy intervention program with low exercise frequency adapted to the limitations of the long-term care insurance system in Japan, in which frequency and a period of physical therapy are restricted to 20 min per session and 2 days per week. We observed whether our intervention program prevented functional decline of the patient.

我们有机会对复合型HSP进行此康复治疗。我们自主设计了一个低频率的物理治疗干预项目,可适应日本长期医疗保险制度的局限性,训练频率低至一周两天、每次不超过20分钟。我们由此观察我们的干预项目是否可以预防患者功能退化。

PARTICIPANT AND METHODS

参与及方法

In August 2016, a 41-year-old male diagnosed as HSP started using outpatient rehabilitation service in our Long-Term Care Health Facilities for the purpose of physical therapy. His chief compliant was difficulty in walking independently because of spastic paraplegia and blindness.

2016年8月,一位41岁的男性被诊断为HSP,开始在我们的康复门诊服务使用长期医疗护理设备来进行物理治疗。他的主要问题是由于下肢痉挛截瘫和眼盲而无法独立行走。

When he was in his late twenties, he felt difficulty in walking and decreased visual acuity. At the age of 32, he was diagnosed as HSP (causative gene was SPG 11: autosomal recessive mutation) in “A” hospital. Five years later, he underwent intrathecal baclofen therapy in “B” hospital but this therapy had no effect.

他快三十岁时,他自感不行困难、视力急剧下降。32岁在“A”医院被诊断为HSP(由于SPG11:基因常染色体隐性突变)。五年后,在“B”医院进行囊内巴多芬注射和物理治疗,但并无效果。

Spasticity and ataxia worsened progressively. He had been going outside without any walking aid or assistance until 2015. Since 2016, he used a wheel chair and needed the assistance of his friends to go outside. Additionally, he had walked for moving indoor, but there was an increase in the opportunity of creeping at home.

痉挛和共济失调发展的很严重。2015年,他可以在在室外无辅助步行。到2016年,他需要乘坐轮椅或在他朋友的帮助下才能外出。此外,他为了移动在室内活动,但是这增加家中缓慢步行的几率。

Gait analysis: The trunk was bent larger than normal in mid stance phase and in static standing position (Fig. 2). The posture at gait was wide-based because of truncal ataxia and spasticity of the lower extremities. He used Q-cane on the left hand. During the time period of mid stance phase and heel off phase of the gait, lateral bending and rotation of the trunk toward the side of the standing leg were observed with moderately larger degree than normal.

步态分析:在站立中期和静态站立时,他的躯干弯曲的比正常人要大。他的步态是受躯体的共济失调和下肢的痉挛影响的。他左手使用Q型手杖,在支撑相中期及足跟触底的阶段,有观察到患者躯干向支撑侧侧屈及旋转,并且幅度比一般人要大。

Home visit evaluation: He lived with a sister and father. His sister stayed mainly at home as a main caregiver, assisting preparation of meals, housework, and hospital visit. The ADL evaluated by the modified Barthel index was 65/100. Deduction items of the modified Barthel index were feeding, transfer ability, toilet, gait, stairs climbing, and dressing (Table1). He mainly walked with Q-cane, but occasionally he crawled. He took a bath by himself once a week with great difficulty. Most of the time he lied on the bed. He scarcely went out except when he used outpatient service. His demand for the physical therapy was to prevent inability to walk alone. On the other hand, his family’s hope for the physical therapy was to maintain the ability of taking a bath alone.

家访评估:他同妹妹及父亲一起居住。他的妹妹绝大部分时间呆在家中照顾他、做饭、做家务、陪他去医院。该患者的ADL评估Barthel指数为65/100。在改良的Barthel指数中进食、转移、如厕、步行、上下楼梯以及穿衣等项目指数中均有减分(表格1)。他大部分时间步行需要借助Q型手杖,但是他偶尔也能自行缓慢行走。他每周可以自己洗一次澡,但十分困难。他绝大部分时间是躺在床上的。除了去门诊看病以外他几乎不出门。对于物理治疗,一方面患者希望自己不要丧失自助步行的能力,另一方面对家人来说,他们希望可以维持患者独立洗澡的功能。

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Table 2 shows our original intervention program. The purpose of our intervention program was to maintain lower limb function and to keep the ability to take a bath alone. As we had to provide a rehabilitation program adapted to the limitations of long-term care insurance system in Japan, we set the frequency of rehabilitation substantially low compared with a conventional rehabilitation program. Practically, he underwent 40 min intervention for 2 days a week for 1 month. Then he continued 20 min intervention and 20 min personal practice (total 40 min practice) for 2 months. With reference to the degree of intensity, this program was planned according to the guidelines presented by American College of Sports Medicine (ACSM)10), except that the degree of intensity was increased gradually. Physical condition was evaluated 3 months after the intervention.

表二描述的是我们最初的干预手法。干预治疗的目的是为了维持患者下肢功能以及独立洗澡的能力。我们制定的康复计划是根据日本上期医保系统的限制进行了调整,我对训练频率也进行了调整,比传统法康复要低一些。实际情况为,在第一个月内患者每周要进行两次康复训练,每次训练时间为40分钟。之后的两个月内进行20分钟康复训练以及20分钟的自主训练(共40分钟)。关于紧张度的治疗师根据美国运动医学院(ACSM)指南所制定的,除非患者肌肉紧张度不断增长,在干预治疗后,每隔三个月对患者的身体情况进行评估。

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Additionally, we used two original techniques in order to control the hip abduction11). First, by holding the elbows of the patient straighten, we prevented forward tilting of the trunk, hip flexion, and excessive activities of upper limbs during exercise. Second, we selected kneeling position in order to reduce the activity of hip abductor, thereby learning exact position of the trunk more easily. In the trunk control exercise (Fig. 3), the therapist supported his elbows to minimize the support of upper extremities and maximize the activities of trunk muscles and hip adductor.

另外,我们使用了两种传统疗法来控制哈尊和的髋外展动作。第一种方法:让患者肘关节伸直,在训练过程中我们要防止患者出现躯干倾斜、屈髋以及上肢的过度运动的情况。第二种方法,我们通过跪位练习来减少髋内收的动作,从而让患者更容易了解躯干的正确位置。在躯干控制训练中(表三),治疗师要支撑患者的肘关节,从而减少患者上支援短的支撑力,进而激活躯干肌肉以及髋内收肌。

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Written informed consent was obtained from the patient regarding the publication of a case report with masked photographs. Additionally, this study has obtained approval from the research ethics committee of Aomori University of Health and Welfare (Approval number: 1731).

该案例的发布已经获得了患者的书面知情同意,并对照片进行了打码。另外本研究已经获得了青森卫生复立大学的实验伦理审批(批准文号:31)。

RESULTS

结果

The patient underwent our original intervention program for 24 sessions (2 sessions a week × 4 weeks × 3 months). The number of repetition of squatting exercise was gradually increased to 100 times per session in the last half of the intervention.

患者进行了24个阶段的原始干预疗法(每周两阶段×4周×三个月)。在干预治疗的后半段时期,深蹲练习的数量也逐渐增加到每阶段100个。

At the beginning of the intervention, grip force was 28.0/29.3 kgf and quadriceps strength was 23.8/24.3 kgf. Three months after the intervention, grip force was 32.0/31.7 kgf and quadriceps strength was 26.3/28.0 kgf, indicating that the muscle strength was maintained throughout the intervention period. In addition, all scores of each item of Barthel index showed no change. He was still able to take a bath once a week and no accident happened in the bath at home. The posture at standing and gait showed no remarkable change.

在干预治疗前期,患者握力为28.0/29.3 kgf,股四头肌肌力为23.8/24.4 kgf。在治疗后三个月,患者的握力增长到32.0/31.7 kgf, 股四头肌肌力为26.3/28.0 kgf,这说明训练有效的维持了患者的肌力。另外,Barthel指数中的项目分数并没有改变,他依然可以每周独立洗澡,并且在家中洗澡时没有意外发生。站立位以及步态并没有明显的改善。

DISCUSSION

讨论

We succeeded in maintaining the function of lower extremities and ADL abilities of the patient with complicated form of HSP by applying our original intervention program adapted to the limitations of Japanese long-term care insurance system in which time and frequency of rehabilitation is restricted.

我们利用适用日本长期医保系统的干预方式(限制了康复治疗的时间和频率),成功的保留了完全性HSP患者下肢功能以及日常生活活动能力。

Asir et al.9) reported functional improvement of the patients with HSP by providing their original program in which frequency of the exercise was 6 sessions a week. In contrast to their program, we set the frequency of rehabilitation to 2 days a week, which amounted less than half of Asir’s frequency. Without any exercise, functional decline should be predicted in a patient with HSP. However, our low exercise frequency program resulted in the maintenance of muscle strength, ADL abilities, and posture. These results suggest that low exercise frequency probably be enough for the maintenance of physical function in patients with HSP.

Asir et al.报告指出在经过每周6阶段的传统干预后,HSP患者的功能得到改善。至于对照组,我们设立了一周两次的康复训练——是Asir治疗频率的一半。若HSP患者没有进行任何治疗,该患者的功能肯定会下降。然而,我们低频率的训练结果显示有效的维持了患者的肌力、ADL以及患者的姿势。这些结果指出对于HSP患者维持现有的功能,低频率的物理治疗室完全足够的。

In order to keep the muscle strength, we considered the frequency and intensity of the training. ACSM’s guideline indicates that strength training is needed at least 2 days per week to reinforce the muscle strength10). In addition, the muscle performance at approximately 20–30% one repetition maximum level10) is needed to maintain the muscle strength. The muscle activities of that level can be obtained during squatting exercise12). Therefore we provided squatting exercise to the patient and succeeded in maintaining the muscle strength. As the patient had decreased visual acuity, we provided verbal instruction about trunk position and knee flexion angle. This verbal instruction might have also made a positive contribution to the maintenance of the muscle strength.

为了维持患者的肌力,我们要考虑训练的频率和强度。ACSM直男显示,想要保持肌力,要进行至少每周两次的力量训练。此外,肌力训练的维持量需要每次增加最大肌力的20%-30%。此水平的肌力训练可以通过深蹲练习来实现。所以我们为患者提供了深蹲练习,并且成功了维持了患者的肌力。当患者的视敏度下降时,我们会给予患者关于躯干姿势以及膝关节屈度的口头指示。这种口头提示对维持肌力有一定的积极影响。

In order to maintain the posture at standing and gait, we paid attention to the activity of gluteus medius muscle and the position of hip joint while body weight was loaded. Spasticity of the hip adductor is one of the key features in HSP patients7). In addition, he bent the trunk lager than normal in mid stance phase and in static standing position. The gluteus medius originates mainly from the iliac crest and ends at the greater trochanter13) and works most effectively when the hip joint is at an extended position. In order to increase the activity of gluteus medius, we prevented the trunk tilting forward and prevented hip flexion by holding his elbow straighten. Consequently, the posture at standing and gait remained unchanged. Our method probably prevented the worsening of the posture. In addition, kneeling exercise also contributed to the improvement of weight control abilities of trunk and hip joint. However, kneeling exercise may have reduced the opportunity for unified control exercise using ankle, knee, and hip.

为了保持患者站立位以及行走时的姿势,我们将重点关注身体负重时臀中肌以及髋关节的位置。髋内收肌痉挛是HSP患者的典型表现。此外,在支撑相中期以及静态站立时,患者躯干的侧屈程度以比一般人大。臀中肌起始于髂骨终止于股骨大转子,在髋关节神战中起着重要的作用。为了激活臀中肌,我们防止躯干前倾,并且自爱患者肘伸位时预防屈髋。结果患者站立以及步行时的姿势并没有明显改善。我们的治疗可能只是预防患者的错误姿势。另外,跪位练习改善了躯干和髋关节的负重控制。然而,跪位练习减少了踝关节、膝关节以及髋关节的统一联合运动。

In addition, the motion exercise we performed in accordance with the environment of his bathroom at home was effective in the maintenance of ADL abilities.

此外,基于患者家中洗澡环境的运动训练对于ADL的维持有很好的效果。

Finally, by applying our original intervention program adapted to the limitations of long-term care insurance system in Japan, we were able to maintain the functions of lower extremities and ADL ability in a patient with HSP for at least 3 months. The present results seems to have the beneficial effect of low exercise frequency in the physical therapy of patients with HSP at outpatient rehabilitation service.

最终,通过利用适用于日本长期医保系统限制的传统干预,我们成功的维持了HSP患者至少三个月内下肢的功能以及ADL。现阶段的结果显示,门诊的低频率物理治疗对HSP患者是很有利的。

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