痉挛性斜颈和特发性头部震颤的临床研究

目的对比痉挛性斜颈(CD)患者中存在头部震颤组[HT(+)组],无头部震颤组[HT(-)组]和特发性头部震颤组(ET组)三组患者的临床特点。方法用回顾性的方法观察自1982年8月至2012年10月期间就诊于天津医科大学总医院神经内科门诊的188例CD患者,其中HT(+)组57例,HT(-)组59例和72例ET病例的临床表现,病程发展过程和家族史。分组和诊断标准依据肌张力障碍性震颤和特发性震颤的诊断标准。使用χ2和方差分析进行统计学分析。结果三组患者中,其中HT(+)组女性患者发病率高于HT(-)组(χ2=5.872,P=0.019),HT(+)组颈痛率24.6%高于仅由震颤引起的颈痛率11.3%(χ2=4.060,P=0.041),HT(+)组56.1%患者以HT作为首发症状,HT发生后4.89年出现斜颈。26.7%CD患者同时存在手部震颤,HT(+)组显著性高于HT(-)组(χ2=16.800,P=0.000),ET组69%的患者存在手颤,显著性高于HT(+)组(χ2=7.651,P=0.005)。HT(-),HT(+)和ET三组患者中,HT(-)组具有家族史阳性率最低(10.2%),与HT(+)组相比有显著性差异(χ2=9.201,P=0.002),而ET组具有震颤的家族史阳性率最高(37.5%)。结论 HT和斜颈是头颈部肌张力障碍的两个重要体征,两者可先后出现,并相互转化,而HT有时是其早期唯一的表现,不易与ET区别,在与手颤和具有震颤或其他运动障碍的家族史的患者中更易发生,支持CD与特发性震颤在发病机制上具有一定的关联。

Clinical study on cervical dystonia and essential head tremor

Objective To compare the clinical characteristics, natural history of patients with cervical dystonia（CD）with head tremor [ HT（ ＋ ）] ,without head tremor [HT（-） ] and essential head tremor（ET）. Methods We prospectively evaluated 188 consecutive patients of CD including HT（ ＋ ）57 patients, HT（ - ）59 patients and 72 patients of ET with a detailed questionnaire who came to the department of neurology, General Hospital of Tianjin Medical University. We studied clinical characteristics, progress and family history. According to diagnostic standard of dystonia tremor and essential tremor, we divided the patients into 3 groups. Chi-square and One-Way ANOVA were employed for statistical analysis. Results There was higher prevalence of HT in women in HT （ ＋ ） than in HT （ - ） （X2 = 5. 872, P = 0. 019） ,the rate of pain on the neck in HT（ - ） was 28. 8%, HT（ ＋ ） was 24. 5%, higher than the ET＇s （11.3% ,X： =4. 060,P =0. 041 ）. 56. 1% of patients in HT（ ＋ ）had tremor as one the first symptoms. CD would appear following HT after 4. 89 years. 26.7% CD patients had hand tremor,the rate of hand tremor in HT（ ＋ ）was higher than that of HT（ - ） group（x2 = 16. 800,P =0. 000） ,and 69% in ET group had hand tremor was higher than HT（ ＋ ） （X2 =7. 651 ,P =0. 005）. 10. 2% of HT（ - ）had the lowerest positive family history,there was a difference between HT（ - ）and HT（ ＋ ） （X2 =9. 201 ,P =0. 002） and 37. 5% of ET had the highest family history. Conclusion HT and CD are two important vital signs in CD, they appear alternately and chang into each other. HT sometimes is the only sign, could not differ from ET easily. It is more commonly associated with hand tremor and family history of tremor or other movement disorders. This supports a possible pathogenesis association between CD and ET.