不同发病年龄的特发性震颤临床表型比较

Comparison of clinical phenotypes of essential tremor of different ages at onset

目的探讨不同发病年龄的特发性震颤(essential tremor,ET)与特发性震颤叠加综合征(essential tremor plus,ET-Plus)患者的临床表型及预后差异。方法收集ET及ET-Plus患者79例,以发病年龄<55岁为早发型(EAO),发病年龄≥55岁为晚发型(LAO)。收集患者病史,对两组患者一般临床特征、头部磁敏感加权成像(SWI)、药物敏感性、震颤特征及其他神经系统症状、焦虑抑郁的发生情况进行对比分析。结果EAO组男性患者比例高于LAO组(62.22%比35.29%,P<0.05),HAMD量表及HAMA量表评估总分高于LAO组〔分别5.00(6.00,8.00)比4.00(5.00,7.25),6.00(7.00,10.00)比2.00(3.00,14.00),均P<0.05〕。LAO组震颤进展率显著快于EAO组〔19.32(11.28,24.38)比13.69(9.35,18.28),P<0.05〕,串联步态受损和轻度运动迟缓发生率均显著高于EAO组〔分别50.00%比22.22%,44.12%比11.11%,均P<0.05〕。两组间比较,震颤家族史、头部SWI成像提示铁沉积的发生率、饮酒试验阳性率,单独应用指南推荐一线、二线、一线+二线、与其他药物联用的药物应答良好发生率及各类药物应用均疗效欠佳发生率,静止性震颤发生率及震颤评定量表(Fahn-Tolosa-Marin Tremor Rating Scale,TRS)评分,震颤在下肢、头部、下颌、口唇及声音、中轴部位等部位分布,以及轻中度以上抑郁、肯定有焦虑的患者发生率差异均无统计学意义(均P>0.05)。结论ET及ET-Plus患者多数对药物反应良好,LAO组震颤进展率显著快于EAO组。LAO组容易合并其他神经系统症状,向其他变性疾病转化,青年起病的患者焦虑、抑郁评分更高。震颤进展以及新症状与体征的动态,可能有助于疾病的治疗与预后判断。

Objective To investigate the clinical phenotype and prognosis of essential tremor(ET)and essential tremor plus syndrome(ET-Plus)patients with different ages at onset(AAO).Methods 79 patients diagnosed with ET or ET-Plus were collected.Those whose AAO<55 years old were defined as early-onset patients(EAO),while those whose AAO≥55 years old were defined as late-onset patients(LAO).We collected these patients’medical histories,compared and analyzed the general clinical characteristics,head SWI,drug sensitivity,tremor characteristics,other neurological symptoms,and the occurrence of anxiety and depression between two groups.Results The proportion of male patients in the EAO group was significantly higher than those in the LAO group(62.22%vs.35.29%,P<0.05).No significant difference was found between the two groups in the incidence of iron deposition in head SWI imaging,drinking test,family history of tremor(P>0.05).No significant difference was found between the two groups in the effective rates of first-line drugs,second-line drugs,first-line+second-line drugs,combined with other drugs and the incidence of poor treatment effects of various drugs(P>0.05).No significant difference was found between the two groups in the incidence of resting tremor and the Fahn-Tolosa-Marin Tremor Rating Scale(TRS)score(P>0.05).The progression rate of tremor in the LAO group was significantly faster than that in the EAO group〔19.32(11.28,24.38)vs.13.69(9.35,18.28),P<0.05〕.There was no significant difference in the distribution of anatomical parts among the lower limbs,head,face,lip,voice,and central axis between the two groups(P>0.05).The incidences of tandem gait impairment and mild brakykinesia in the LAO group were significantly higher than those in the EAO group(50.00%vs.22.22%,44.12%vs.11.11%P<0.05).The total scores of the HAMD scale and HAMA scale of the EAO group were significantly higher than those of the LAO group〔5.00(6.00,8.00)vs.4.00(5.00,7.25),6.00(7.00,10.00)vs.2.00(3.00,14.00)both P<0.05〕.There was no significant difference in the incidence of mild to moderate depression and anxiety between the two groups.(both P>0.05).Conclusions Most patients with ET or ET-Plusreacted well to medication.Patients in the LAO group were prone to accompany other neurological symptoms and signs,they mighe more likely transform into other degenerative diseases.Patients in the EAO group had higher anxiety and depression scores.Paying attention to the progress of tremor and the dynamic changes of new symptoms and signs may benefit the treatment and prognosis of the disease.