Gourie-Devi M, Suresh T G
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
J Neurol Neurosurg Psychiatry. 1988 Jun;51(6):773-7. doi: 10.1136/jnnp.51.6.773.
This paper presents the clinical features in 12 patients with the Madras pattern of motor neuron disease (MMND) seen over a period of 10 years. Ten of the patients were from other parts of South India, outside Madras. Young age at onset, sporadic occurrence, sensorineural deafness, bulbar palsy, diffuse atrophy with weakness of limbs and progressive but benign course were the striking features. Electromyography revealed chronic partial denervation. MMND formed 3.7% of all forms of motor neuron disease. Although isolated cases have been seen elsewhere in India, this is the first report of a large number of patients of MMND seen outside Madras (Tamil Nadu). Recognition of this clinical syndrome is of importance for prognostication and as well for search of possible aetiological factors.
本文介绍了在10年期间所见到的12例具有马德拉斯型运动神经元病（MMND）患者的临床特征。其中10例患者来自马德拉斯（金奈）以外的印度南部其他地区。发病年龄较轻、散发性、感音神经性耳聋、延髓麻痹、肢体弥漫性萎缩伴无力以及病程呈进行性但为良性，这些都是显著特征。肌电图显示慢性部分失神经。MMND在所有运动神经元病类型中占3.7%。尽管在印度其他地方也见过个别病例，但这是马德拉斯（泰米尔纳德邦）以外地区大量MMND患者的首次报告。认识这种临床综合征对于预后判断以及寻找可能的病因学因素都很重要。
