- ICH GCP
- 미국 임상 시험 레지스트리
- 임상시험 NCT07838883
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.
연구 개요
연구 유형
등록 (추정된)
연락처 및 위치
연구 연락처
- 이름: Xianjin Shang, Prof.
- 전화번호: 86 151 5530 0503
- 이메일: shangxj2013@wnmc.edu.cn
연구 장소
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Anhui
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Wuhu, Anhui, 중국, 241001
- 모병
- First affiliated hospital of Wannan Medical University
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연락하다:
- Xianjin Shang, Prof.
- 전화번호: 86 15155300503
- 이메일: shangxj2013@wnmc.edu.cn
-
-
참여기준
자격 기준
공부할 수 있는 나이
- 어린이
- 성인
- 고령자
건강한 자원 봉사자를 받아들입니다
연구 대상 성별
샘플링 방법
연구 인구
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.
설명
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.
공부 계획
연구는 어떻게 설계됩니까?
디자인 세부사항
- 관찰 모델: COHORT
- 시간 관점: PROSPECTIVE
코호트 및 개입
그룹/코호트 |
|---|
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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 |
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hereditary spastic paraplegia
Patient diagnosis, clinical assessment, treatment, and biological sample collection |
연구는 무엇을 측정합니까?
주요 결과 측정
결과 측정 |
측정값 설명 |
기간 |
|---|---|---|
|
Amyotrophic Lateral Sclerosis Functional Rating Scale (K-ALSFRS-R)
기간: 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. |
through study completion, an average of 3 months
|
|
Hereditary spastic paraplegia (HSP): Spastic Paraplegia Rating Scale (SPRS)
기간: 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
|
|
Scale for the Assessment and Rating of Ataxia (SARA)
기간: 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
|
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6-minute walk test for hereditary muscular dystrophy
기간: 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
|
공동 작업자 및 조사자
간행물 및 유용한 링크
일반 간행물
- 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.
연구 기록 날짜
연구 주요 날짜
연구 시작 (실제)
기본 완료 (추정된)
연구 완료 (추정된)
연구 등록 날짜
최초 제출
QC 기준을 충족하는 최초 제출
처음 게시됨 (실제)
연구 기록 업데이트
마지막 업데이트 게시됨 (실제)
QC 기준을 충족하는 마지막 업데이트 제출
마지막으로 확인됨
추가 정보
이 연구와 관련된 용어
추가 관련 MeSH 약관
기타 연구 ID 번호
- W-MoSCA2026200
개별 참가자 데이터(IPD) 계획
개별 참가자 데이터(IPD)를 공유할 계획입니까?
약물 및 장치 정보, 연구 문서
미국 FDA 규제 의약품 연구
미국 FDA 규제 기기 제품 연구
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