Yilmaz Oznur, Alemdaroğlu Ipek, Karaduman Ayşe, Haliloğlu Göknur, Topaloğlu Haluk
Department of Physical Therapy and Rehabilitation, Faculty of Health Sciences, Hacettepe University, Ankara, Turkey.
Turk J Pediatr. 2011 Jul-Aug;53(4):471-6.
Monomelic amyotrophy (MMA) is a benign motor neuron disease characterized by neurogenic amyotrophy, which usually affects one of the upper or lower extremities. Progression is slow and symptoms are clinically stable. Symptoms are seen in the second or third decades of life. In this study, we present a seven-year-old girl who was diagnosed and directed to the Physiotherapy Department at the age of 5 years and had unilateral proximal upper limb involvement. Family history of the case was recorded. Neurologic evaluation was performed. Range of joint motion, muscle shortness and strength, posture, extremity lengths, gait, timed performance, arm function, and motor and mental maturation were assessed. The physiotherapy program was designed progressively as strengthening and resistive exercises. Motor and mental developmental milestones were normal. There was no limitation in active or passive motion of all joints. She had more flexible joints, scapula alata, asymmetry between shoulder levels, and weakness on proximal muscles of the right upper extremity. In the follow-up assessment at eight months, there was no asymmetry between shoulder levels and scapular symmetry began to improve. Female gender and involvement restricted to one proximal upper limb are rare in the literature. This patient demonstrates the positive effects of physical therapy with early diagnosis of MMA. The rapid recovery of muscle weakness shows the importance of strengthening and resistive exercises applied to specific muscles in the treatment.
单肢肌萎缩症（MMA）是一种以神经源性肌萎缩为特征的良性运动神经元疾病，通常影响上肢或下肢中的一侧。病情进展缓慢，临床症状稳定。症状出现在人生的第二个或第三个十年。在本研究中，我们报告一名7岁女孩，她在5岁时被诊断出并转诊至理疗科，有单侧上肢近端受累情况。记录了该病例的家族史。进行了神经学评估。评估了关节活动范围、肌肉挛缩和力量、姿势、肢体长度、步态、定时表现、手臂功能以及运动和智力发育情况。理疗计划逐步设计为强化和抗阻运动。运动和智力发育里程碑正常。所有关节的主动或被动活动均无受限。她的关节更灵活，有翼状肩胛、肩部不对称以及右上肢近端肌肉无力。在8个月时的随访评估中，肩部不对称情况消失，肩胛对称性开始改善。文献中女性且仅单侧上肢近端受累的情况罕见。该患者展示了MMA早期诊断后物理治疗的积极效果。肌肉无力的快速恢复表明治疗中针对特定肌肉进行强化和抗阻运动的重要性。
