Rare Disease Registry Database for Movement Disorders in Southern Anhui Province
试验速览
- 阶段
- 不适用
- 状态
- 进行中(未招募)
- 发起方
- 入组人数
- 180
- 试验地点
- 1
- 主要终点
- Amyotrophic Lateral Sclerosis Functional Rating Scale (K-ALSFRS-R)
研究概览
简要总结
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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.
排除标准
- •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.
研究组 & 干预措施
Motor Neuron Diseases
Patient diagnosis, clinical assessment, treatment, and biological sample collection
spinocerebellar ataxia(SCAs)
Patient diagnosis, clinical assessment, treatment, and biological sample collection
hereditary muscular dystrophy
Patient diagnosis, clinical assessment, treatment, and biological sample collection
hereditary spastic paraplegia
Patient diagnosis, clinical assessment, treatment, and biological sample collection
结局指标
主要结局
Amyotrophic Lateral Sclerosis Functional Rating Scale (K-ALSFRS-R)
时间窗: through study completion, an average of 3 months
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.
Hereditary spastic paraplegia (HSP): Spastic Paraplegia Rating Scale (SPRS)
时间窗: through study completion, an average of one year
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
Scale for the Assessment and Rating of Ataxia (SARA)
时间窗: through study completion, an average of one year
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.
6-minute walk test for hereditary muscular dystrophy
时间窗: through study completion, an average of 1 year
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.
次要结局
未报告次要终点
