NCT04319796

Brief Summary

The recent implementation of European Reference Networks for Rare Diseases (ERNs) is an unprecedented move to improve the care of patients suffering from rare health disorders by transnational collaboration. ERN-RND, the ERN for Rare Neurological Diseases, oversees more than 35,000 patients in 31 specialist centers in 13 countries. The ERN-RND registry aims to gather information on patient cohorts in the multiple specialist centers and to provide an overview on patient numbers principally accessible for translational studies.

Trial Health

35
At Risk

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Trial has exceeded expected completion date
Enrollment
5,000

participants targeted

Target at P75+ for all trials

Timeline
Completed

Started Oct 2021

Longer than P75 for all trials

Status
unknown

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

March 20, 2020

Completed
4 days until next milestone

First Posted

Study publicly available on registry

March 24, 2020

Completed
1.5 years until next milestone

Study Start

First participant enrolled

October 1, 2021

Completed
4.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

November 1, 2025

Completed
1 month until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2025

Completed
Last Updated

September 29, 2021

Status Verified

September 1, 2021

Enrollment Period

4.1 years

First QC Date

March 20, 2020

Last Update Submit

September 28, 2021

Conditions

Keywords

Rare DiseasesAtaxiaHereditary Spastic Spinal ParalysisNeurodegeneration with Brain Iron AccumulationLeukodystrophyDystoniaAtypical ParkinsonismHuntington's DiseaseChorea HuntingtonEuropean Reference Network on Rare Neurological DiseasesFrontotemporal Dementia

Outcome Measures

Primary Outcomes (1)

  • Representative cohorts of RND patients

    The register study aims to compile representative cohorts of RND patients and provide demographic data for the planning of translational studies. As all of the index diseases are rare the primary goal is to reach as large cohorts as possible.

    Day 1

Study Arms (6)

Ataxia & HSP

Patients suffering of Ataxia or HSP or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by these Rare Neurological Disease (RND).

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Leukodystrophies

Patients suffering of Leukodystrophies or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by this RND.

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Frontotemporal Dementia

Patients suffering of Frontotemporal Dementia or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by this RND.

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Dystonia, Paroxysmal Disorders and Neurodegeneration with

Patients suffering of Dystonia, Paroxysmal Disorders and Neurodegeneration with Brain Iron Accumulation (NBIA) or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by these RND.

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Atypical Parkinsonism

Patients suffering of Atypical Parkinsonism or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by this RND.

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Huntington's Disease & Choreas

Patients suffering of Huntington's Disease or Choreas or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by these RND.

Other: Data set as defined by the ERN Research Workgroup of the European Commission

Interventions

The ERN-RND registry will be restricted to the minimum data set as defined by the ERN Research Workgroup of the European Commission. This includes the following data: * Name of specialist center * Pseudonym: * Date of birth: For confidentiality reasons the ERN-RND registry will restrict this information to the Year of birth * Sex * Date of death: For confidentiality reasons the ERN-RND registry will restrict this information to the Year of death * Age of onset * Orphacode for specific rare disease: https://www.orpha.net/consor/cgi-bin/Disease\_Search.php?lng=DE * Online Mendelian Inheritance in Man (OMIM) code as defined for genetic diseases: https://www.omim.org/ * Human Phenotype Ontology (HPO) terms for key features: https://hpo.jax.org/app/ * Agreement to be contacted for research purposes: Yes/No * Biological samples (Yes / No) * Link to a biobank (Link / No) * Classification of disability (Disease group specific score)

Ataxia & HSPAtypical ParkinsonismDystonia, Paroxysmal Disorders and Neurodegeneration withFrontotemporal DementiaHuntington's Disease & ChoreasLeukodystrophies

Eligibility Criteria

Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Patients suffering of one of the rare neurological diseases indicated according to the inclusion criteria or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by a RND. Patients visiting in Tübingen, Germany the specialist center Department of Neurology and/ or the Department of Neuropediatric Diseases at the University Hospital.

You may qualify if:

  • Patients suffering of one of the rare neurological diseases indicated below or probands who are at risk to develop such a disease since they are first degree relatives of patients affected by a RND including:
  • Ataxia and HSP
  • Leukodystrophies
  • Frontotemporal Dementia
  • Dystonia, Paroxysmal Disorders and Neurodegeneration with Brain Iron Accumulation (NBIA)
  • Atypical Parkinsonism
  • Huntington's Disease \& Choreas

You may not qualify if:

  • Missing informed consent of the patient and/ or their parents

Contact the study team to confirm eligibility.

Sponsors & Collaborators

MeSH Terms

Conditions

Rare DiseasesAtaxiaPantothenate Kinase-Associated NeurodegenerationDystoniaHuntington DiseaseChoreaFrontotemporal Dementia

Condition Hierarchy (Ancestors)

Disease AttributesPathologic ProcessesPathological Conditions, Signs and SymptomsDyskinesiasNeurologic ManifestationsNervous System DiseasesSigns and SymptomsBasal Ganglia DiseasesBrain DiseasesCentral Nervous System DiseasesNeuroaxonal DystrophiesMovement DisordersHeredodegenerative Disorders, Nervous SystemNeurodegenerative DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesDementiaCognition DisordersNeurocognitive DisordersMental DisordersFrontotemporal Lobar DegenerationTDP-43 ProteinopathiesProteostasis DeficienciesMetabolic DiseasesNutritional and Metabolic Diseases

Study Officials

  • Ludger Schöls, Prof. Dr.

    University Hospital Tübingen

    STUDY DIRECTOR

Central Study Contacts

Ludger Schöls, Prof. Dr.

CONTACT

Ingeborg Krägeloh-Mann, Prof. Dr.

CONTACT

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Target Duration
25 Years
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Clinical Professor

Study Record Dates

First Submitted

March 20, 2020

First Posted

March 24, 2020

Study Start

October 1, 2021

Primary Completion

November 1, 2025

Study Completion

December 1, 2025

Last Updated

September 29, 2021

Record last verified: 2021-09

Data Sharing

IPD Sharing
Will share

The full pseudonymised dataset will be exported once a year as an Excel file that is made accessible in an encrypted form to the coordinators of each specialist center who contribute to the ERN-RND registry. To this end the encrypted dataset will be provided in a specific folder of the Hertie Institute for Clinical Brain Research Tübingen, Germany (HIH) cloud for a limited amount of time (one week). Coordinators of all ERN-RND health care providers will get access to this folder to download the encrypted file. In addition, they will receive a password that enables decryption of the file. Each coordinator needs to confirm a priori with its local institutional review board the issues of data storage. This is part of the local project plan, patient information and consent that is premise for data entry. This procedure enables each center who contributes data to consider studies of their special interest within the ERN.

Shared Documents
ANALYTIC CODE
Time Frame
Data will become available after analysis and unlimited.
Access Criteria
Authorized users within the participating organizations.