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Korean Journal of Neuromuscular Disorders 2018;10(2):27-31.
Clinical and Electrophysiological Features of Sporadic Lower Motor Neuron Disease
Sung Joo Park, Joong Goo Kim, Jung Hwan Oh, Sa-Yoon Kang
Department of Neurology, Jeju National University School of Medicine, Jeju, Korea
산발하위운동신경세포질환의 임상적 및 전기생리학적 특성
박성주, 김중구, 오정환, 강사윤
제주대학교 의학전문대학원 신경과학교실
Abstract
Background
In general, motor neuron disease comprises combined upper and lower motor neuron diseases, pure lower motor neuron diseases and pure upper motor neuron diseases. The term lower motor neuron disease (LMND) may be used for all diseases in which only lower motor neuron signs are found. Methods: In this study, we investigated clinical and electrophysiological features in patients with sporadic LMND. In order to exclude patients who had amyotrophic lateral sclerosis, only patients who had had the disease for at least 4 years were selected. The patients were then classified into three groups based on the identified pattern of weakness as shown in the registry. Results: The patients with LMND were categorized into groups of generalized weakness (group 1, two patients), asymmetrical weakness of the arms (group 2, 31 patients) and asymmetrical weakness of the legs (group 3, four patients). Analysis of the results showed that the distinguished features of group 1 include, an older age at onset of the disease, more severe weakness and greater functional impairment. In addition, the results also show that the prognosis of sporadic LMND seems to be favorable and clinical abnormalities were confined to one or two limbs in most patients classified by group 2 and 3. Conclusions: The clinical phenotypes of the different subgroups described in this study may help to differentiate the several forms of LMND. Prospective studies are however needed to investigate whether specific clinical or electrophysiological variables may help to identify patients with a more benign form of LMND.


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