Massa R, Scalise A, Iani C, Palmieri M G, Bernardi G
Clinica Neurologica, Universita di Roma Tor Vergata, Rome, Italy.
Electroencephalogr Clin Neurophysiol. 1998 Dec;109(6):523-6. doi: 10.1016/s1388-2457(98)00029-7.
We report the case of a young man from the south of India, initially presenting the typical signs of benign monomelic amyotrophy (BMA) in the left upper limb. After several years, the involvement of other limbs and the appearance of bulbar signs suggested the possible diagnosis of the Madras pattern of motor neuron disease (MMND). Serial motor evoked potential (MEP) recordings allowed detection of the onset of a focal involvement of upper motor neurons (UMN) controlling innervation in the originally amyotrophic limb. Therefore, serial MEP recordings can be useful for the early detection of sub-clinical UMN damage in motor neuron disease presenting with pure lower motor neuron (LMN) signs.
我们报告了一名来自印度南部的年轻男子的病例，该男子最初表现出左上肢良性单肢肌萎缩（BMA）的典型症状。几年后，其他肢体受累以及延髓症状的出现提示可能诊断为马德拉斯型运动神经元病（MMND）。连续运动诱发电位（MEP）记录能够检测到控制最初肌萎缩肢体神经支配的上运动神经元（UMN）局灶性受累的起始情况。因此，连续MEP记录对于早期检测以单纯下运动神经元（LMN）症状为表现的运动神经元病中的亚临床UMN损伤可能有用。
