Cui Fang, Sun Liuqing, Qiao Jie, Xiong Jianmei, Zhao Yangang, Li Jianyong, Li Mao, Chen Siyu, Huang Xusheng
a Department of Neurology , Hainan Branch of Chinese PLA General Hospital , Sanya , Hainan Province , China.
b Department of Neurology , Chinese PLA General Hospital , Beijing , China.
Neurol Res. 2018 Dec;40(12):1088-1093. doi: 10.1080/01616412.2018.1522412.
Hereditary spastic paraplegias (HSP) is a heterogeneous group of inherited neurologic disorders with diversified clinical manifestations. The purpose of this study was to summarize the clinical manifestations of HSP by analyzing the clinical data of 56 HSP patients.
A total of 56 HSP patients treated in our hospital from January 2014 to March 2016 were included. Demographic and clinical characteristics of patients were collected. The severity of HSP was assessed by disease severity score.
The patients included 40 males and 16 females. The mean onset age was 17.86 ± 12.56 years (range: 1-47). The mean disease duration was 13.46 ± 12.82 years (range: 1-63). There were 29 pure (51.8%) forms and 27 complicated (48.2%) HSP. The common manifestations included increased deep tendon reflexes in the lower extremities (94.6%), positive Babinski sign (94.6%), increased muscle tone of lower extremities (91.1%), scissors gait (83.9%), ankle clonus (69.6%), reduced muscle strength in the lower extremities (48.2%) and skeletal deformities (37.5%). Reduced cognitive function was the most common manifestation (55.6%) of the complicated HSP patients. The mean disease severity score was significantly higher in males than in females (2.75 ± 0.55 vs. 2.18 ± 1.13, P = 0.013). Patients with a disease duration >30 years had a significantly higher disease severity score than those with disease duration of 1-10 and 21-30 years.
We reported the clinical features of HSP from 56 patients in our hospital. Our findings should be helpful for better understanding of clinical features of HSP.
遗传性痉挛性截瘫（HSP）是一组临床表现多样的遗传性神经系统疾病。本研究旨在通过分析56例HSP患者的临床资料，总结其临床表现。
纳入2014年1月至2016年3月在我院治疗的56例HSP患者。收集患者的人口统计学和临床特征。采用疾病严重程度评分评估HSP的严重程度。
患者中男性40例，女性16例。平均发病年龄为17.86±12.56岁（范围：1 - 47岁）。平均病程为13.46±12.82年（范围：1 - 63年）。其中29例为单纯型（51.8%）HSP，27例为复杂型（48.2%）HSP。常见表现包括下肢腱反射亢进（94.6%）、巴宾斯基征阳性（94.6%）、下肢肌张力增高（91.1%）、剪刀步态（83.9%）、踝阵挛（69.6%）、下肢肌力减弱（48.2%）和骨骼畸形（37.5%）。认知功能减退是复杂型HSP患者最常见的表现（55.6%）。男性的平均疾病严重程度评分显著高于女性（2.75±0.55 vs. 2.18±1.13，P = 0.013）。病程>30年的患者疾病严重程度评分显著高于病程为1 - 10年和21 - 30年的患者。
我们报告了我院56例HSP患者的临床特征。我们的研究结果有助于更好地了解HSP的临床特征。
