- ICH GCP
- US Clinical Trials Registry
- Klinisk forsøg 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.
Studieoversigt
Status
Undersøgelsestype
Tilmelding (Anslået)
Kontakter og lokationer
Studiekontakt
- Navn: Xianjin Shang, Prof.
- Telefonnummer: 86 151 5530 0503
- E-mail: shangxj2013@wnmc.edu.cn
Studiesteder
-
-
Anhui
-
Wuhu, Anhui, Kina, 241001
- Rekruttering
- First affiliated hospital of Wannan Medical University
-
Kontakt:
- Xianjin Shang, Prof.
- Telefonnummer: 86 15155300503
- E-mail: shangxj2013@wnmc.edu.cn
-
-
Deltagelseskriterier
Berettigelseskriterier
Aldre berettiget til at studere
- Barn
- Voksen
- Ældre voksen
Tager imod sunde frivillige
Køn, der er berettiget til at studere
Prøveudtagningsmetode
Studiebefolkning
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.
Beskrivelse
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.
Studieplan
Hvordan er undersøgelsen tilrettelagt?
Design detaljer
- Observationsmodeller: COHORT
- Tidsperspektiver: PROSPECTIVE
Kohorter og interventioner
Gruppe / kohorte |
|---|
|
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 |
Hvad måler undersøgelsen?
Primære resultatmål
Resultatmål |
Foranstaltningsbeskrivelse |
Tidsramme |
|---|---|---|
|
Amyotrophic Lateral Sclerosis Functional Rating Scale (K-ALSFRS-R)
Tidsramme: 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)
Tidsramme: 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 |
through study completion, an average of one year
|
|
Scale for the Assessment and Rating of Ataxia (SARA)
Tidsramme: 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. |
through study completion, an average of one year
|
|
6-minute walk test for hereditary muscular dystrophy
Tidsramme: 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. |
through study completion, an average of 1 year
|
Samarbejdspartnere og efterforskere
Publikationer og nyttige links
Generelle publikationer
- 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.
Datoer for undersøgelser
Studer store datoer
Studiestart (Faktiske)
Primær færdiggørelse (Anslået)
Studieafslutning (Anslået)
Datoer for studieregistrering
Først indsendt
Først indsendt, der opfyldte QC-kriterier
Først opslået (Faktiske)
Opdateringer af undersøgelsesjournaler
Sidste opdatering sendt (Faktiske)
Sidste opdatering indsendt, der opfyldte kvalitetskontrolkriterier
Sidst verificeret
Mere information
Begreber relateret til denne undersøgelse
Yderligere relevante MeSH-vilkår
- Neurologiske manifestationer
- Hjernesygdomme
- Sygdomme i centralnervesystemet
- Sygdomme i nervesystemet
- Neuromuskulære sygdomme
- Genetiske sygdomme, medfødte
- Sygdomme i det perifere nervesystem
- Neurodegenerative sygdomme
- Medfødte abnormiteter
- Heredodegenerative lidelser, nervesystem
- Rygmarvssygdomme
- Misdannelser i nervesystemet
- Dyskinesier
- Polyneuropatier
- Cerebellære sygdomme
- Cerebellar ataksi
- Spinocerebellare degenerationer
- Arvelig sensorisk og motorisk neuropati
- Ataksi
- Medfødte, arvelige og neonatale sygdomme og abnormiteter
- Motor neuron sygdom
- Spinocerebellære ataksier
- Spastisk paraplegi, arvelig
Andre undersøgelses-id-numre
- W-MoSCA2026200
Plan for individuelle deltagerdata (IPD)
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