Couch J R
Adv Neurol. 1976;14:245-58.
The occurence of extranuchal dystonia, facial spasm, parkinsonian symptoms (facial masking, bradykinesia, rigidity), tremor and family history of tremor was tabulated in a group of 30 patients with IST. The incidence of extranuchal dystonia increased as severity of IST increased. There was a strong trend for severity of extranuchal dystonia to increase as severity of torticollis increased, which was significant (p less than 0.001). There was a similar trend for severity of facial spasm to increase with increasing severity of torticollis (p less than 0.025). Parkinsonian features were seen in 10 of 30 patients, and in three the diagnosis of Parkinson's disease could be entertained. Tremor was seen in 26 of 30 patients being mild in 12, moderate in 11, and severe in three. A family history of tremor was present in 16 of 28 cases for whom history was available (12 primary, four secondary relations). The results are most consistent with the hypothesis that IST is a variant of DMD with tremor as an integral part of the disease and tremor represents a forme of the disease in family members.
在一组30例特发性斜颈(IST)患者中,对颈部以外的肌张力障碍、面部痉挛、帕金森症状(面具脸、运动迟缓、肌强直)、震颤以及震颤家族史进行了列表统计。随着IST严重程度的增加,颈部以外肌张力障碍的发生率也增加。随着斜颈严重程度的增加,颈部以外肌张力障碍的严重程度有明显升高趋势,差异有统计学意义(p<0.001)。随着斜颈严重程度的增加,面部痉挛的严重程度也有类似趋势(p<0.025)。30例患者中有10例出现帕金森特征,其中3例可考虑诊断为帕金森病。30例患者中有26例出现震颤,其中12例为轻度,11例为中度,3例为重度。在有家族史记录的28例患者中,16例有震颤家族史(12例一级亲属,4例二级亲属)。这些结果最符合以下假设:IST是肌张力障碍的一种变异型,震颤是该疾病的一个组成部分,并且震颤在家族成员中代表该疾病的一种形式。