Early or late appearance of "dropped head syndrome" in amyotrophic lateral sclerosis
Early or late appearance of "dropped head syndrome" in amyotrophic lateral sclerosis
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DOI：
10.1136/jnnp.74.5.683
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发表时间：
2003-05-01
影响因子：
11
通讯作者：
Sandhya, S
中科院分区：
文献类型：
--
作者：
Gourie-Devi, M;Nalini, A;Sandhya, S
背景：由颈部伸肌无力引起的“头部下垂综合征”在多种神经肌肉疾病中已有报道。以前发表的报告包括肌萎缩侧索硬化症(ALS)的孤立病例。在这篇报道中，我们描述了20年间9名ALS患者的情况。患者和调查：从1981年到2000年，共有683名ALS患者被诊断为ALS患者。其中9例有严重的颈部伸肌无力作为早期特征，或在疾病的后期发展。评估方案包括详细的临床病史、神经学检查、肌电图和神经传导研究。排除恶性肿瘤、淋巴组织增生性疾病、甲状腺功能障碍和胶原血管疾病。结果：低头综合征的发生率为1.3%。受影响患者的平均年龄(SD)为53.3(10.3)岁(33至65岁)，病例分布在第4至70岁之间。在6名患者中，头部下垂是早期的特征(平均发病间隔11.6个月(3至24个月))；3名患者为晚期(发病后3至8年)。在5名患者中，除了严重的伸肌无力外，还有轻度的颈部屈肌无力。9例患者均可见弥漫性上下运动神经元征象。所有患者均无呼吸困难，但均有吞咽困难和社交窘迫，均可通过简单措施纠正。结论：低头综合征是ALS的重要临床症状，多见于发病后1~2年内。这9个病例的头部下垂综合征的原因可以很容易地被确定为ALS，通过普遍的体征的存在。
Background: "Dropped head syndrome" caused by neck extensor weakness has been reported in a variety of neuromuscular disorders. Previously published reports include isolated cases with amyotrophic lateral sclerosis (ALS). In this report, nine patients with ALS and dropped head syndrome seen during a 20 year period are described.Patients and investigations: Between 1981 and 2000, 683 patients with ALS were diagnosed, based on El Escorial criteria. Nine of these had profound neck extensor weakness observed as an early feature, or developing during the later stages of the disease. The protocol for evaluation included detailed clinical history, neurological examination, electromyography, and nerve conduction studies. Investigations were undertaken to exclude malignancy, lymphoproliferative disorders, thyroid dysfunction, and collagen vascular disease.Results: The incidence of dropped head syndrome was 1.3%. The mean (SD) age of the affected patients was 53.3 (10.3) years (range 33 to 65), with an equal distribution of cases in the fourth to seventh decades. In six patients, head drop was an early feature (mean interval from onset of illness 11.6 months (range 3 to 24)); in three it was late (between three and eight years after onset). In five patients, mild neck flexor weakness was present in addition to severe extensor weakness. In all nine patients there were diffuse upper and lower motor neurone signs. None of the patients had difficulty in breathing but all had difficulty in swallowing and social embarrassment, both of which could be corrected by simple measures.Conclusions: Dropped head syndrome is an important clinical sign and usually occurs as an early feature within the first one to two years after the onset of ALS. The cause of dropped head syndrome in these nine cases could be easily established as ALS by the presence of generalised signs.