Chitapure Tajuddin, Jethwani Divya, Zubair Ahmed Syed, Panigrahy Chinmoyee
Assistant Professor, MGM School of Physiotherapy, Aurangabad, a constituent unit of MGMIHS, Navi Mumbai, IND.
Associate Professor, Department of Physiotherapy, Tilak Maharashtra Vidyapeeth Jayantrao Tilak College of Physiotherapy, Pune, IND.
Cureus. 2021 Aug 15;13(8):e17201. doi: 10.7759/cureus.17201. eCollection 2021 Aug.
Charcot-Marie-Tooth (CMT) disease is the most inherited form of peripheral neuropathy. This condition is also known as hereditary motor and sensory neuropathy (HMSN), which is a slowly progressive neuropathy affecting peripheral nerves and causes sensory loss, weakness and muscle wasting. This primarily involves distal muscles of feet, lower legs, hands and forearm. CMT is the most frequently inherited peripheral neuropathy known to affect 1 in 2500 individuals. There are four types: CMT1, CMT2, CMT3 and CMT4, depending upon the involvement of specific gene deficit, inheritance pattern, age of onset and whether the primary defect results in an abnormality of the myelin or axon of the nerve. Diagnosis of CMT is done based on physical examination, genetic testing, and electromyography (EMG) and nerve conduction velocity (NCV) test results. Symptoms of weakness progress from distal to proximal, i.e., it begins from feet and ankles. CMT is a known length dependent neuropathy which results in foot drop and later weakness of hands and forearm becomes evident. Due to the involvement of sensory nerve fibres, sensations like heat, touch, pain and most prominently vibration is also present distally. Long standing effects of CMT leads to the development of deformity and/or contractures which may involve forefoot, hindfoot, toes and/or long finger flexors. As physiotherapy is a means to maintain and/or gain maximum possible functional independence, consistency of the treatment becomes the most important factor. This case report intends to show that consistency in performing physiotherapeutic exercises helps in gaining maximum possible functional independence. This case report is a discussion of a 25-year-old male patient referred to the physiotherapy department with the polyneuropathy type symptoms with his history and investigation reports being consistent with that of Charcot-Marie-Tooth disease.
夏科-马里-图思(CMT)病是周围神经病变最常见的遗传形式。这种疾病也被称为遗传性运动和感觉神经病变(HMSN),是一种缓慢进展的神经病变,会影响周围神经,导致感觉丧失、虚弱和肌肉萎缩。这主要累及足部、小腿、手部和前臂的远端肌肉。CMT是最常见的遗传性周围神经病变,每2500人中就有1人受其影响。根据特定基因缺陷的累及情况、遗传模式、发病年龄以及主要缺陷是否导致神经髓鞘或轴突异常,CMT可分为四种类型:CMT1、CMT2、CMT3和CMT4。CMT的诊断基于体格检查、基因检测以及肌电图(EMG)和神经传导速度(NCV)测试结果。虚弱症状从远端向近端发展,即从足部和脚踝开始。CMT是一种已知的长度依赖性神经病变,会导致足下垂,随后手部和前臂的虚弱也会变得明显。由于感觉神经纤维受累,足部也会出现热、触觉、疼痛等感觉,最明显的是振动觉。CMT的长期影响会导致畸形和/或挛缩的发展,可能累及前足、后足、脚趾和/或手指长屈肌。由于物理治疗是维持和/或获得最大可能功能独立性的一种手段,治疗的连贯性成为最重要的因素。本病例报告旨在表明,进行物理治疗锻炼的连贯性有助于获得最大可能的功能独立性。本病例报告讨论的是一名25岁男性患者,他因多发性神经病变症状被转诊至理疗科,其病史和检查报告与夏科-马里-图思病相符。