Clinic of Neurology, University Clinical Centre Nis, Nis, Serbia.
Medical Faculty, University of Nis, Nis, Serbia.
Medicine (Baltimore). 2023 Sep 29;102(39):e35435. doi: 10.1097/MD.0000000000035435.
Monomelic amyotrophy is a rare form of motor neuron disease in which the neurogenic atrophy is restricted to 1 limb, mostly the distal part of the arm. The disease most often occurs in Asia, especially in Japan and India, while in European countries, this disease is rarely recognized. Registration and publication of new cases of this disease aims to increase the awareness of clinicians about the existence of this disease in European countries, and with the aim of easier recognition and faster diagnosis of this essentially benign disorder.
Five patients with signs of atrophy of the muscles of 1 leg were examined at our Institution.
The criteria for selecting patients were as follows: clinical evidence of wasting and weakness confined to the 1 lower limb; progressive course, or initial progression followed by stationary course; absence of any definite sensory loss or central nervous system involved; no evidence of compression lesion of the spinal cord.
The clinical characteristics of our patients were similar to those previously described in the literature. The characteristic clinical features were sporadic occurrence, predominance in males with an initially progressive course for 2 to 5 years followed by a stationary state. There was no family history of neuromuscular disease.
The electromyographic finding was consistent with a chronic neuropathic disorder. Magnetic resonance imaging of the lumbosacral spine excluded intraspinal pathologies and root compression in all cases.
Monomelic amyotrophy of the lower limb is a rare disease that should be considered in cases of slow progressive unilateral amyotrophy of a single leg, especially in younger and middle-aged men, not only in Asia but also in the Western Balkans and Europe.
单一肢带型肌萎缩症是一种罕见的运动神经元疾病，其神经源性萎缩仅限于 1 个肢体，主要是手臂的远端部分。这种疾病最常发生在亚洲，特别是在日本和印度，而在欧洲国家，这种疾病很少被认识到。登记和发表这种疾病的新病例旨在提高欧洲国家临床医生对这种疾病存在的认识，并旨在更容易识别和更快诊断这种本质上良性的疾病。
我们机构检查了 5 名腿部肌肉萎缩迹象的患者。
选择患者的标准如下：仅 1 下肢存在萎缩和无力的临床证据；进行性病程，或初始进展后稳定；无任何明确的感觉丧失或中枢神经系统受累；无脊髓压迫性病变的证据。
我们患者的临床特征与文献中先前描述的相似。特征性的临床特征是散发性发病，男性居多，最初进展 2 至 5 年，然后稳定。无家族性神经肌肉疾病史。
肌电图检查结果与慢性神经病变一致。腰骶部磁共振成像排除了所有病例的椎管内病变和神经根压迫。
下肢单肢型肌萎缩症是一种罕见疾病，在单侧进行性单肢肌无力缓慢进展的情况下应考虑这种疾病，特别是在亚洲，也应在西巴尔干半岛和欧洲考虑这种疾病。
