IDIOPATHIC CERVICAL DYSTONIA - CLINICAL CHARACTERISTICS
IDIOPATHIC CERVICAL DYSTONIA - CLINICAL CHARACTERISTICS
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DOI：
10.1002/mds.870060206
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发表时间：
1991-01-01
影响因子：
8.6
通讯作者：
FAHN, S
中科院分区：
文献类型：
--
作者：
CHAN, J;BRIN, MF;FAHN, S
我们回顾了266例特发性颈部肌张力障碍（通常称为痉挛性斜颈）患者的详细临床特征。 平均发病年龄（41岁）、男女比例（1.9：1）、发病年龄集中在30 - 59岁之间（70%）、肌张力障碍家族史（12%）和缓解（9.8%）与既往研究中发现的结果相似。 与以往单一的大型临床研究相反，没有发现右利手或明显的甲状腺疾病，75%的患者出现疼痛，并对残疾评分有贡献（p < 0.01），这是该综合征与所有其他局灶性肌张力障碍的区别。 疼痛还与持续（与间歇性）头部转动、头部转动的严重程度和痉挛的存在密切相关。 83%的患者在头部无支撑的情况下坐着时有75%的时间头部偏斜（休息时头部持续偏斜）。 在97%的转头者中，81%的人也有各种组合的头部倾斜。 23%的手震颤患者发病年龄较大（平均46岁对41岁; p < 0.05）。 有肌张力障碍家族史（平均36岁）、出现症状前不久有创伤（平均36岁）、头部转动方向发生变化（平均36岁）的患者发病年龄较早（p < 0.05）。30岁），缓解（平均33岁）。 62%的患者出现了头部的急动或强迫性短暂痉挛，这些患者符合“痉挛性斜颈”的逻辑。 因此，由于38%的患者的颈部肌张力障碍不是痉挛性的，我们认为“痉挛性斜颈”一词并不是一个完全合适的名称。 我们建议将特发性颈肌张力障碍或特发性肌张力障碍性斜颈，最好是前者，原因在文中解释。
We reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female-to-male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right-handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p < 0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty-three percent of patients had deviation of the head of 75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p < 0.05). An earlier age at onset (p < 0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation "spasmodic torticollis" could logically apply. Therefore, because the cervical dystonia in 38% of patients is not spasmodic, we propose that the term "spasmodic torticollis" is not a completely appropriate designation for this condition. We suggest labeling the condition either idiopathic cervical dystonia or idiopathic dystonic torticollis, preferably the former for reasons explained in the text.