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芬兰的肌萎缩侧索硬化症。II:临床特征。

Amyotrophic lateral sclerosis in Finland. II: Clinical characteristics.

作者信息

Jokelainen M

出版信息

Acta Neurol Scand. 1977 Sep;56(3):194-204. doi: 10.1111/j.1600-0404.1977.tb01425.x.

DOI:10.1111/j.1600-0404.1977.tb01425.x
PMID:906794
Abstract

The clinical characteristics of ALS in Finland were investigated. The study was based on the hospital records of 255 patients. No correlation was found between the disease and other previous illnesses. Muscular weakness was the most frequent complaint of the patients as their first symptom (149/255, i.e. 58 per cent). The duration of the disease was shortest (1.9 years) in male cases with the first symptoms in the bulbar area, and it was longest (3.4 years) in females with a spinal onset of the symptoms. The duration of the disease was in inverse relation to the age when it was contracted. Progressive muscular atrophy with fasciculations was considered the most important clinical sign of ALS. It was most frequently observed in the upper extremities including shoulders. Sensory disturbances were absent, sphincter abnormalities were reported in only three cases, bed sores did not exist, and there were no reports of impotence in the male patients. Two families with two familial cases in each were found, altogether two men and two women. Their disease did not differ from that of the rest of the series. The ALS patients soon became too disabled to work; 56 per cent bacame permanently disabled in less than 1 year's time, and only 9 per cent retained their working capacity for more than 2 years. The average length of stay at hospital was 32 days for ALS and 37 days for MS patients, but the later group was hospitalized 2-3 times more frequently than the former. Gastrostomy or tracheostomy and artificial respiration did not lenghten the lives of the patients.

摘要

对芬兰肌萎缩侧索硬化症(ALS)的临床特征进行了调查研究。该研究基于255例患者的医院记录。未发现该疾病与其他既往疾病之间存在关联。肌无力是患者最常见的首发症状(149/255,即58%)。首发症状出现在延髓区域的男性患者病程最短(1.9年),首发症状出现在脊髓的女性患者病程最长(3.4年)。病程与发病时的年龄呈负相关。伴有肌束震颤的进行性肌肉萎缩被认为是ALS最重要的临床体征。最常出现在包括肩部在内的上肢。无感觉障碍,仅3例报告有括约肌异常,无褥疮,男性患者无阳痿报告。发现两个家族,每个家族有两例家族性病例,共两名男性和两名女性。他们的疾病与该系列其他患者的疾病并无差异。ALS患者很快就因残疾而无法工作;56%的患者在不到1年的时间内永久致残,只有9%的患者保留工作能力超过2年。ALS患者的平均住院时间为32天,多发性硬化症(MS)患者为37天,但MS患者的住院频率比ALS患者高2至3倍。胃造口术、气管造口术和人工呼吸并未延长患者的生命。

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