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Monomelic Amyotrophy

Monomelic Amyotrophy  单肢肌肉萎缩


Monomelic amyotrophy (also known as MMA, Hirayama’s disease, Sobue diease or Juvenile nonprogressive amyotrophy) is an untreatable, focal, motor neuron disease that primarily affects young (15 – 25 year-old) males in India and Japan. MMA is marked by insidious onset of muscular atrophy, which stabilizes at a plateau after two to five years from which it neither improves nor worsens. There is no pain or sensory loss associated with MMA. Unlike other lower motor neuron diseases, MMA is not believed to be hereditary and fasciculations (involuntary muscle twitches) are rare.

Monomelic单肌萎缩 (又称MMA甲基丙烯酸甲酯平山郁夫症祖父江症少年无动态肌肉萎缩)是不治之症,这种聚焦的运动神经细胞疾病主要是影响印度和日本的青年男性(15 – 25岁)。甲基丙烯酸甲酯(MMA)的特点是隐匿性的肌肉萎缩发难,在二至五年后到了某一个高点就稳定下来,不再提高也不恶化。与甲基丙烯酸甲酯(MMA)相关的病症没有疼痛或丧失感觉,不像其他的较轻微运动神经元疾病,MMA不是遗传的,肌束颤动(不自主的肌肉抽搐)是罕见的。

EMG tests reveal loss of the nerve supply, or denervation, in the affected limb without conduction block (nerve blockage restricted to a small segment of the nerve). Increased sweating, coldness and cyanosis have been reported for a few patients, indicating involvement of the sympathetic nervous system.


While MMA will cause weakness and/or wasting in only one limb, EMG and NCV tests often show signs of reinnervation in the unaffected limbs.



Contents  目录

·      Treatment 治疗处理

·      Prognosis 病症诊断

·      Epidemiology 流行病学

·      External links 外部链接


Treatment  治疗处理

There is no cure for MMA. Treatment consists of muscle strengthening exercises and training in hand coordination.



Prognosis  病症诊断

The symptoms of MMA usually progress slowly for one to two years before reaching a plateau, and then remain stable for many years. Disability is generally slight. Rarely, the weakness progresses to the opposite limb. There is also a slowly progressive variant of MMA known as O’Sullivan-McLeod syndrome, which only affects the small muscles of the hand and forearm and has a slowly progressive course.



Epidemiology  流行病学

MMA occurs in males between the ages of 15 and 25. Onset and progression are slow. MMA is seen most frequently in Asia, particularly in Japan and India; it is much less common in North America.



External links  外部链接

monomelic_amyotrophy at NINDS。NINDS的单肢肌肉萎缩

MND Association。MND协会

Overview at Washington University  华盛顿大学的概况


Categories  分类

  • Motor neurone disease  运动神经元疾病

—— END ——


Source > Wikipedia at en.wikipedia.org/wiki/Monomelic_amyotrophy

Translated by > BlogHost

Word Count > approx. 350 words in English


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