Rare Disease Registry Database for Movement Disorders in Southern Anhui Province (W-MoSCA)
According to the World Health Organization (WHO) definition, a rare disease is a disease affecting fewer than 6.5 people per 10,000; in China, it generally refers to diseases with a prevalence of less than 1/10,000 (i.e., with a total of no more than 140,000 patients). Such diseases are mostly hereditary or congenital in origin. Patients with rare diseases often present with complex symptoms, most of which are neurological. Although a large number of rare diseases have been identified, the small number of patients with each disease and the diversity of symptoms make early diagnosis difficult and render large-sample clinical trials challenging. Consequently, most rare diseases lack curative treatments; available therapies are of limited efficacy or prohibitive cost, ultimately leading to severe disability or death and imposing a substantial socioeconomic burden. This study establishes a registry for motor neuron diseases (MND), spinocerebellar ataxias (SCAs), hereditary muscular dystrophy (HMD), and hereditary spastic paraplegia (HSP). Owing to the low incidence, complex clinical diagnosis, unclear pathogenic mechanisms, and strong genetic heterogeneity, epidemiological data on rare motor neuron diseases are limited, further increasing the difficulty of biomarker screening and targeted therapeutic development. Therefore, there is an urgent need to establish a comprehensive registry and to obtain reliable evidence on risk factors and early diagnosis through research. This study aims to establish a registry of rare neurological diseases in the southern Anhui Province and to build a high-quality biobank of human biological resources.
研究概览
地位
研究类型
注册 (估计的)
联系人和位置
学习联系方式
- 姓名:Xianjin Shang, Prof.
- 电话号码:86 151 5530 0503
- 邮箱:shangxj2013@wnmc.edu.cn
学习地点
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Anhui
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Wuhu、Anhui、中国、241001
- 招聘中
- First affiliated hospital of Wannan Medical University
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接触:
- Xianjin Shang, Prof.
- 电话号码:86 15155300503
- 邮箱:shangxj2013@wnmc.edu.cn
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-
参与标准
资格标准
适合学习的年龄
- 孩子
- 成人
- 年长者
接受健康志愿者
有资格学习的性别
取样方法
研究人群
These rare movement-related neurological diseases are all characterized primarily by gait abnormalities; although muscle weakness, ataxia, poor muscle endurance, and spastic paraplegia are their typical presentations, respectively, they share overlapping motor neurological symptoms and similar early clinical manifestations.
描述
Inclusion Criteria:
- Adults aged 19 years or older who are capable of giving informed consent.
- Definitive diagnosis of one of the following diseases: (1) amyotrophic lateral sclerosis (diagnosed according to the revised El Escorial criteria, Awaji criteria, or Gold Coast criteria); (2) spinocerebellar ataxia; (3) muscular dystrophy; (4) hereditary spastic paraplegia.
- Others: primary lateral sclerosis, progressive muscular atrophy, ALS-FTD syndrome, progressive bulbar palsy, benign focal muscular atrophy, and other motor neuron diseases.
Exclusion Criteria:
- Patients with concomitant systemic diseases;
- Vulnerable research subjects: minors and patients with cognitive impairment;
- Participants from whom clinical information and human biological samples cannot be collected;
- Other patients judged by the investigators to be unsuitable for participation in the study.
学习计划
研究是如何设计的?
设计细节
- 观测模型:COHORT
- 时间观点:PROSPECTIVE
队列和干预
团体/队列 |
|---|
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Motor Neuron Diseases
Patient diagnosis, clinical assessment, treatment, and biological sample collection |
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spinocerebellar ataxia(SCAs)
Patient diagnosis, clinical assessment, treatment, and biological sample collection |
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hereditary muscular dystrophy
Patient diagnosis, clinical assessment, treatment, and biological sample collection |
|
hereditary spastic paraplegia
Patient diagnosis, clinical assessment, treatment, and biological sample collection |
研究衡量的是什么?
主要结果指标
结果测量 |
措施说明 |
大体时间 |
|---|---|---|
|
Amyotrophic Lateral Sclerosis Functional Rating Scale (K-ALSFRS-R)
大体时间:through study completion, an average of 3 months
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Total score: 0 to 48 points 48 points: completely normal function 0 points: complete loss of function in the corresponding dimension. The lower the score, the more severe the functional disability. |
through study completion, an average of 3 months
|
|
Hereditary spastic paraplegia (HSP): Spastic Paraplegia Rating Scale (SPRS)
大体时间:through study completion, an average of one year
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Including 13 items, each item is scored from 0 to 4 (0 = normal, 4 = most serious impairment) Total score: 0 to 52 points; the higher the score, the more serious the condition |
through study completion, an average of one year
|
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Scale for the Assessment and Rating of Ataxia (SARA)
大体时间:through study completion, an average of one year
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Including 8 items, each item has a different maximum score. Total score: 0 to 40 points; the higher the score, the more severe the ataxia. 0 points: no ataxia. 40 points: the most severe ataxia. |
through study completion, an average of one year
|
|
6-minute walk test for hereditary muscular dystrophy
大体时间:through study completion, an average of 1 year
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The subjects walked continuously for 6 minutes as fast and safely as possible in a flat hard corridor; they can use the patient's daily walkers, crutches, and orthopedic braces; they are allowed to stop and rest in the middle (timer does not stop), and the total walking meters PMC is recorded at the end of 6 minutes. |
through study completion, an average of 1 year
|
合作者和调查者
出版物和有用的链接
一般刊物
- Cao Y, Zheng H, Zhu Z, Yao L, Tian W, Cao L. Clinical and Genetic Spectrum in a Large Cohort of Hereditary Spastic Paraplegia. Mov Disord. 2024 Apr;39(4):651-662. doi: 10.1002/mds.29728. Epub 2024 Jan 31.
- Tong YR, Geng C, Guan YZ, Zhao YH, Ren HT, Yao FX, Ling C, Wang DC, Chen L, Cui LY, Zhang SY, Dai Y. A Comprehensive Analysis of 2013 Dystrophinopathies in China: A Report From National Rare Disease Center. Front Neurol. 2020 Sep 30;11:572006. doi: 10.3389/fneur.2020.572006. eCollection 2020.
- Xu L, Chen L, Wang S, Feng J, Liu L, Liu G, Wang J, Zhan S, Gao P, Fan D. Incidence and prevalence of amyotrophic lateral sclerosis in urban China: a national population-based study. J Neurol Neurosurg Psychiatry. 2020 May;91(5):520-525. doi: 10.1136/jnnp-2019-322317. Epub 2020 Mar 5.
研究记录日期
研究主要日期
学习开始 (实际的)
初级完成 (估计的)
研究完成 (估计的)
研究注册日期
首次提交
首先提交符合 QC 标准的
首次发布 (实际的)
研究记录更新
最后更新发布 (实际的)
上次提交的符合 QC 标准的更新
最后验证
更多信息
与本研究相关的术语
其他相关的 MeSH 术语
其他研究编号
- W-MoSCA2026200
计划个人参与者数据 (IPD)
计划共享个人参与者数据 (IPD)?
药物和器械信息、研究文件
研究美国 FDA 监管的药品
研究美国 FDA 监管的设备产品
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