Gourie-Devi M, Nalini A
Department of Neurology, National Institute of Mental Health and Neurosciences, 506 029, Bangalore, India.
J Neurol Sci. 2003 May 15;209(1-2):13-7. doi: 10.1016/s0022-510x(02)00458-6.
Madras motor neuron disease (MMND) has the characteristic features of onset in the young, atrophy and weakness of the limbs, multiple cranial nerve palsies particularly the seventh, ninth to twelfth and sensorineural hearing loss with unique geographic distribution to southern part of India. During a period of 28 years (1974-2001), 7 (13%) among 54 patients of MMND seen at a tertiary referral center at Bangalore, India, had the additional features of optic atrophy in all and cerebellar involvement in three of them. There were three males and four females, the mean age at onset was 11.7 years, with a mean duration of illness of 6.4 years. All except one patient were ambulant and independent in the activities of daily living. Family history of MMND was present in more than a quarter (28.6%) of patients. Compared to MMND, these patients had onset of illness at a younger age and family history was more frequently observed, however, these differences were not statistically significant. Bulbar palsy was an invariable feature, being present in all patients compared to 38.3% of MMND and the difference was statistically significant (p=0.003). This clinical profile may be considered to be a variant of Madras motor neuron disease (MMNDV).
马德拉斯运动神经元病（MMND）具有发病于年轻人、肢体萎缩和无力、多组颅神经麻痹尤其是第七、第九至第十二对颅神经麻痹以及感音神经性听力损失等特征，且在印度南部有独特的地理分布。在28年（1974 - 2001年）期间，在印度班加罗尔一家三级转诊中心诊治的54例MMND患者中，有7例（13%）除具备上述特征外，全部伴有视神经萎缩，其中3例还累及小脑。患者中男性3例，女性4例，平均发病年龄为11.7岁，平均病程为6.4年。除1例患者外，所有患者均可独立行走且日常生活活动自理。超过四分之一（28.6%）的患者有MMND家族史。与MMND相比，这些患者发病年龄更小，家族史更常见，但这些差异无统计学意义。球麻痹是所有患者的一个恒定特征，而MMND患者中球麻痹的比例为38.3%，两者差异有统计学意义（p = 0.003）。这种临床特征可被视为马德拉斯运动神经元病的一种变异型（MMNDV）。
