原发性侧索硬化症1例报告及文献复习

Miranda Nava Gabriel
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摘要

简介:原发性侧索硬化症是一种罕见的涉及上运动神经元的疾病,可引起球脊髓痉挛。该疾病的病程是潜伏的和渐进的,通常从下肢开始,后来发展为四锥体综合征。作为一种罕见病,大多数情况下的诊断都是排斥性的,应该对患者进行广泛的临床研究,包括彻底的病史、实验室研究和相关的内阁研究。经典病例:这是一名男性患者,大约一年前开始他的临床表现是左骨盆肢体无力,随后伴有疼痛和感觉异常,随后在对侧腿和左上肢表现出相同的症状。目前增加了食指和中趾感觉迟钝,左臂中度震颤,中趾与上述手环形成对比。它在短时间内无法举起轻物体,在进行日常活动时也会在短时间里感到疲劳,这大大限制了它们的日常生活。结论:运动神经元疾病分为两组,在所研究的病例中,上运动神经元仅受影响。由于这是一种罕见病,发病率低,在得出结论之前,将考虑多种鉴别诊断,将其视为排除性诊断。尽管采取了改变疾病进程的措施,但该疾病的自然史总是会有一个严峻的结果,对生命和功能的预后很差。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary Lateral Sclerosis, Report of a Case and Bibliography Revision
Introduction: Primary lateral sclerosis is a rare disease involving the upper motor neuron, producing a bulbospinal spasticity. The course of the disease is insidious and progressive, usually starting with the lower extremities, and later becoming a tetrapyremidal syndrome. As a rare disease, diagnosis in most cases is exclusionary, and the patient should be studied extensively, clinically, including a thorough medical history, laboratorial and with the relevant cabinet studies. Classic Case: It is male patient who starts his clinical picture about a year ago with weakness in left pelvic limb, subsequently accompanied by pain and paresthesias, manifesting the same symptomatology later in the contralateral leg and upper left limb. Currently is added index and middle toe hypoesthesia, moderate tremor in left arm, with the contrast of the middle toe over the ring of said hand. It has an inability to lift light objects for short periods of time, as well as fatigue in short periods of time when performing daily activities, which greatly limits their daily life. Conclusion: Motor neurone diseases are divided into two groups, and in the case studied, the upper motor neuron is exclusively affected. As it is a rare disease, with a low incidence, multiple differential diagnoses will be considered before concluding in it, considering it a diagnosis of exclusion. The natural history of the disease will always have a grim outcome, with poor prognosis for life and function, despite the measures taken to change the course of the disease.
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