REGISTRY - an Observational Study of the European Huntington's Disease Network (EHDN)

Huntington Disease Clinical Trial

— REGISTRY  

Official title:

REGISTRY - an Observational Study of the European Huntington's Disease Network (EHDN)

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01590589
• Other study ID #: REGISTRY 3.0
• Status: Completed
• Phase: N/A
• First received: March 22, 2012
• Last updated: September 14, 2017
• Start date: June 2004
• Est. completion date: June 30, 2017

Study information

• Verified date: September 2017
• Source: European Huntington's Disease Network
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

This is a multi-centre, multi-national, prospective, observational study of Huntington's disease (HD) with a control group of volunteers to:

• obtain natural history data on many HD mutation carriers and individuals who are part of an HD family

• relate phenotypical characteristics (genetic modifiers / wet and dry biomarkers)

• expedite identification and recruitment of participants for clinical trials

• develop and validate sensitive and reliable outcome measures for detecting onset and change over the natural course of premanifest and manifest HD which may also be potential outcome measures for use in future clinical trials and clinical care

• plan for future research studies

Description:

REGISTRY integrates prospectively and systematically collected clinical research data (e.g. phenotypical clinical features, family history, demographical characteristics) with access to biological specimens (e.g. blood, urine) obtained from individuals with manifest HD, unaffected individuals known to carry the HD mutation or at risk of carrying the HD mutation, and control research participants (e.g. spouses, siblings or offspring of HD mutation carriers known not to carry the HD mutation).

REGISTRY is an open-ended study and eligible subjects are assessed at annual study visits on the phenotypical characteristics of HD regardless of whether they display clinical symptoms and signs of the disease and of individuals who are part of an HD family (irrespective of their mutation carrier status). At each study visit, general clinical, motor function, behavior, cognitive, Health Economics, Quality of Life assessments are administered. In addition, participants are given the option to consent to the donation of biosamples for the purposes of mutation (CAG repeat length) testing and for research to identify biological modifiers and markers of HD. Biological specimens and phenotypical data are made available to qualified scientists whose projects are reviewed and approved by the Scientific and Bioethical Advisory Committee (SBAC) of EHDN. Successful applicants agree to accept the EHDN policies surrounding the use of the data/materials provided and publication of results (see data sharing and publication policies of EHDN, attached). Research projects should aim to advance scientific knowledge towards establishing clinically effective treatments that delay onset and/or slow the progression of the disease.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 10000
• Est. completion date: June 30, 2017
• Est. primary completion date: June 30, 2017
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: N/A and older

Eligibility

REGISTRY-HD participants include those who are willing to participate in regular (annual) evaluations conducted by the investigators and have a diagnosis of HD, are HD mutation carriers (but who do not meet criteria for a diagnosis of HD) or persons at risk for HD (first and second degree relatives of people affected by HD), are non-HD mutation carrier relatives. Spouses of participants may take part as REGISTRY-CONTROLS.

Inclusion Criteria:

The following individuals may be eligible to participate

• Individuals, confirmed HD mutation carrier

• Manifest HD, without mutation (CAG) testing

• HD family member at-risk, without CAG testing

• HD family member, non-HD mutation carrier

• REGISTRY-CONTROL participants: companion/individual without HD history

• REGISTRY-COMPANION (any of the above).

Exclusion Criteria:

• Participants who are unable to understand the study protocol or unable to give informed consent, and have no legal representative.

• Participants with choreic movement disorder other than HD. (EHDN provides a Registry-like tool to record findings in patients affected with choreatic movement disorders other than HD under the label "Neuroacanthocytosis"; www.euro-hd.net/html/na/registry).

Related Conditions & MeSH terms

• Huntington Disease
• Huntington's Disease

Locations

Country	Name	City	State
Austria	Universitätsklinik für Psychiatrie, Neuropsychiatrische Ambulanz	Graz
Austria	Universitätsklinik Innsbruck, Neurologie	Innsbruck
Belgium	UCL-St Luc, Centre de génétique humaine	Brussels
Belgium	Institute of Pathology and Genetics (IPG)	Gosselies
Belgium	Universitair Ziekenhuis Gasthuisberg, Dienst Neurologie	Leuven
Czechia	Fakultní nemocnice Olomouc, Neurologická klinika	Olomouc
Czechia	Centrum extrapyramidových onemocnení, Neurologická klinika, 1. LFUK	Praha 2
Denmark	University Hospital of Copenhagen - Rigshospitalet, Dept. of Neurology	Copenhagen Ø
Finland	The Family Federation of Finland Väestöliitto, Department of Medical Genetics	Helsinki
Finland	Helsinki University Hospital, Dept. of Neurology	HUS
Finland	Kuopio University Hospital, Neurology	Kuopio
Finland	Ålands Hälso- och Sjukvård, Långvårdsenheten	Mariehamn
Finland	Oulu University Hospital (OUH), Dept. of Neurology	Oulu
Finland	Suomen Terveystalo	Tampere
Finland	The Finnish Parkinson Association, Suvituuli	Turku
Finland	Turku University Hospital, Dept. of Neurology	Turku
France	CHU d'Amiens - Hôpital Nord, Service de Neurologie	Amiens cedex
France	CHU d'Angers, Service de Neurologie Charcot	Angers cedex 9
France	Hôpital Pellegrin, Service de Génétique Médicale (ESF 3e étage)	Bordeaux cedex
France	HU Clermont-Ferrand - Hôpital Gabriel Montpied, Service de Neurologie	Clermont-Ferrand
France	Hôpital Henri Mondor, Service de Neurologie	Créteil cedex
France	Clinique Neurologique Hôpital Roger Salengro CHRU, Neurologie et Pathologie du Mouvement	Lille cedex
France	CHU La Timone, Service de Neurologie (9e étage)	Marseille cedex 5
France	Hôpital La Pitié Salpétrière, Génétique & Inserm U679	Paris
France	CHU Rouen Charles Nicolle, Service de Neurologie	Rouen cedex
France	Hôpital Civil, Service de Neurologie	Strasbourg cedex
France	Hôpital Purpan, INSERM U825, Pôle Neurosciences	Toulouse cedex 03
Germany	Universitätsklinikum Aachen, Neurologische Klinik	Aachen
Germany	Charité - Universitätsmedizin Berlin, Klinik und Poliklinik für Neurologie, Klinik für Psychiatrie und Psychotherapie	Berlin
Germany	Ruhruniversität Bochum, Neurologie	Bochum
Germany	Reha-Zentrum Dinslaken im Gesundheitszentrum Lang, Tagesklinik	Dinslaken
Germany	Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Klinik und Poliklinik für Neurologie	Dresden
Germany	Universtität Erlangen, Abteilung für Molekulare Neurologie	Erlangen
Germany	Universitätsklinikum Freiburg, Neurologie	Freiburg
Germany	Universitätsklinikum Hamburg-Eppendorf, Neurologische Abteilung	Hamburg
Germany	Neurologische Klinik mit Klinischer Neurophysiologie, Medizinische Hochschule Hannover	Hannover
Germany	Psychatrium Gruppe AöR, Haus 2/1	Heiligenhafen
Germany	Schwerpunktpraxis Huntington, Neurologie-Psychiatrie	Itzehoe
Germany	Praxis	Marburg
Germany	Universität Marburg, Neurologie	Marburg
Germany	Technische Universität München, Abteilung Neurologie	München
Germany	Universitätsklinikum Münster, Klinik und Poliklinik für Neurologie	Münster
Germany	Isar-Amper-Klinikum - Klinik Taufkirchen (Vils)	Taufkirchen
Germany	University Hospital of Ulm, Dept. of Neurology	Ulm
Italy	University of Bari, Dept. of Neurological and Psychiatric Sciences	Bari
Italy	Università di Bologna, Dip. di Scienze Neurologiche - Clinica Neurologica	Bologna
Italy	Azienda Ospedaliera Spedali Civili di Brescia, Clinica Neurologica	Brescia
Italy	University of Florence, Dip. di Scienze Neurologiche e Psichiatriche	Florence
Italy	Università di Genova, Dept. of Neurosciences	Genova
Italy	Fondazione IRCCS Istituto Neruologico Carlo Besta, SOSD Genetics of Neurodegenerative and Metabolic Diseases	Milano
Italy	Fondazione IRCCS Istituto Neurologico Carlo Besta, I Neurologia	Milano
Italy	Università di Napoli Federico II, Dip. Scienze Neurologiche Edificio 17	Napoli
Italy	Azienda Ospedaliera Universitarie Pisana, Dip. di Neuroscienze - UO Neurologia	Pisa
Italy	IRCCS Neuromed, U.O. Neurogenetica	Pozzilli (IS)
Italy	Azienda Ospedaliera S. Andrea, UOC Neurologia e Centro Neurologico Terapie Sperimentali CENTERS	Rome
Italy	Università Cattolica del Sacro Cuore "A.Gemelli", Istituto di Neurologia / CNR, Istituto di Farmacologia Traslazionale	Rome
Netherlands	Medisch Spectrum Twente, Dept. of Neurology	Enschede
Netherlands	UMC Groningen, Neurology	Groningen
Netherlands	Leiden University Medical Centre (LUMC), Neurology K5-Q	Leiden
Netherlands	MUMC, Dept. Neurology	Maastricht
Norway	NKS Olaviken Alderpsykiatriske sykehus, Poliklinikk og Huntington Klinikk	Bergen
Norway	Rikshospitalet, Dept. of Medical Genetics	Oslo
Norway	Ulleval University Hospital, Medical Genetics	Oslo
Norway	St. Olavs Hospital HF, Neurology	Trondheim
Poland	Specialistic Hospital in Gdansk, Dept. of Neurology	Gdansk
Poland	Medical University of Silesia, Dept. of Neurology	Katowice
Poland	Krakowska Akademia Neurologii	Krakow
Poland	Poznan University of Medical Sciences, Dept. of Social Medicine	Poznan
Poland	Institute of Psychiatry and Neurology, First Dept. of Neurology	Warsaw
Poland	Medical University of Warsaw, Dept. of Neurology	Warsaw
Portugal	Hospital de Coimbra, Neurology Dept.	Coimbra
Portugal	Hospital de Santa Maria, Centro de Estudos Egas Moniz, Neurology Dept.	Lisbon
Portugal	Hospital dos Capuchos, Centro Hospitalar Lisboa Central, Neurology Dept.	Lisbon
Portugal	Hospital Fernando Fonseca, Neurology Dept.	Lisbon
Portugal	Hospital de São João, Neurology Dept.	Porto
Portugal	Hospital Geral de Santo António, Neurology Dept.	Porto
Portugal	Centro Hospitalar de Trás-os-Montes e Alto Douro, Neurology Dept.	Vila Real
Russian Federation	Huntington's Disease Society of Russia	Moscow
Spain	Complejo Hospitalario Universitario de Albacete, Neurología	Albacete
Spain	Hospital Infanta Cristina, Neurología	Badajoz
Spain	Hospital Bellvitge, Neurología	Barcelona
Spain	Hospital Clínic i Provincial, Neurología	Barcelona
Spain	Hospital Vall d'Hebrón, Neurología, 3ª planta, consultas externas	Barcelona
Spain	Hospital General Yagüe, Neurology	Burgos
Spain	Hospital Puerta del Mar, Neurología	Cádiz
Spain	Centro Ramón y Cajal, Neurología	Madrid
Spain	Fundación Jiménez Díaz, Neurology	Madrid
Spain	Hospital Clínico de Madrid, Servicio de Neurología	Madrid
Spain	Hospital de Fuenlabrada, Neurology	Madrid
Spain	Hospital Virgen de la Arrixaca, Neurology	Murcia
Spain	Hospital Central de Oviedo, Neurología	Oviedo
Spain	Hospital Son Dureta, Neurology	Palma de Mallorca
Spain	Hospital Virgen del Camino, Medical Genetics	Pamplona
Spain	Hospital Donostia. Universidad del Pais Vasco, Neurociencias	San Sebastián
Spain	Hospital General de Segovia, Neurología	Segovia
Spain	Hospital Universitario Virgen del Rocío, Servicio de Neurología	Sevilla
Spain	Hospital Virgen Macarena, Neurología	Sevilla
Spain	Hospital Universitario Mutua de Terrassa, Neurologia - Investigación	Terrassa - Barcelona
Spain	Hospital Virgen de la Salud, Neurology	Toledo
Spain	Hospital La Fe, Consultas Externas de Neurología	Valencia
Spain	Hospital Clinico Universario, Department of Neurology	Zaragoza
Sweden	Sahlgren University Hospital, Dept. of Clinical Genetics	Göteborg
Sweden	Skåne Universitetssjukhus, Neurologiska kliniken	Lund
Sweden	Karolinska University Hospital - Huddinge Division, Neurology	Stockholm
Sweden	Neuroenheten Utsikten	Stockholm
Sweden	Norrlands Universitet Sjukhus, Dept. of Neurology	Umeå
Sweden	Uppsala University Hospital, Neurology	Uppsala
Switzerland	Neurologische Klinik des Inselspitals, Praxis	Bern
Switzerland	CHUV - Centre Hospitalier Universitaire Vaudois, Département de Neurologie	Lausanne
Switzerland	University Hospital Zurich, Dept. of Neurology	Zurich
United Kingdom	Clinical Genetics Centre, Ground Floor, Ashgrove House	Aberdeen
United Kingdom	The Barberry Centre, Dept. of Psychiatry	Birmingham
United Kingdom	Blandford Hospital	Blandford Forum
United Kingdom	Frenchay Hospital, Neurology Dept.	Bristol
United Kingdom	Cambridge Centre for Brain Repair, Forvie Site	Cambridge
United Kingdom	Cardiff University, Life Sciences Building	Cardiff
United Kingdom	Ninewells Hospital and Medical School, Human Genetics Unit	Dundee
United Kingdom	Molecular Medicine Centre, Western General Hospital, Dept. of Clinical Genetics	Edinburgh
United Kingdom	Royal Devon and Exeter Foundation Trust Hospital, Department of Neurology	Exeter
United Kingdom	Queen Margaret Hospital	Fife
United Kingdom	Scottish Huntington Association, Units 105/106	Glasgow
United Kingdom	Gloucestershire Royal Hospital, Neurology Dept.	Gloucester
United Kingdom	Chapel Allerton Hospital, Yorkshire Regional Genetics Service	Hull
United Kingdom	Chapel Allerton Hospital	Leeds
United Kingdom	Leicestershire Partnership NHS Trust, EMD South School, OSL house	Leicester
United Kingdom	The Walton Centre for Neurology and Neurosurgery	Liverpool
United Kingdom	Guy's Hospital, Dept. of Neurology	London
United Kingdom	National Hospital for Neurology and Neurosurgery, Dept. for Neurology	London
United Kingdom	St George's Hospital Medical School, South West Thames Regional Genetics Unit	London
United Kingdom	The Royal Hospital for Neuro-disability, Research and Development	London
United Kingdom	University of Manchester, Genetic Medicine, St. Mary's Hospital	Manchester
United Kingdom	Institute of Human Genetics, International Centre for Life	Newcastle upon Tyne
United Kingdom	Churchill Hospital, Dept. of Clinical Genetics	Oxford
United Kingdom	Derriford Hospital, Dept. of Clinical Neuropsychology	Plymouth
United Kingdom	Poole Hospital Foundation Trust, Brain Injury Service	Poole
United Kingdom	Lancashire Teaching Hospitals NHS Foundation Trust, Neurosciences Directorate, Royal Preston Hospital	Preston
United Kingdom	Sheffield Children's Hospital, Dept. of Clinical Genetics	Sheffield
United Kingdom	Southampton General Hospital and University of Southampton, Neurology / Wellcome Trust Clinical Research Facility	Southampton
United Kingdom	Bucknall Hospital, Neuropsychiatry Service	Stoke-on-Trent
United Kingdom	Victoria Centre	Swindon

Sponsors (1)

Lead Sponsor	Collaborator
European Huntington's Disease Network

Countries where clinical trial is conducted

Austria,  Belgium,  Czechia,  Denmark,  Finland,  France,  Germany,  Italy,  Netherlands,  Norway,  Poland,  Portugal,  Russian Federation,  Spain,  Sweden,  Switzerland,  United Kingdom, 

References & Publications (12)

Aziz NA, Jurgens CK, Landwehrmeyer GB; EHDN Registry Study Group, van Roon-Mom WM, van Ommen GJ, Stijnen T, Roos RA. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease. Neurology. 2009 Oct 20;73(16):1280-5. do — View Citation

Busse M, Al-Madfai DH, Kenkre J, Landwehrmeyer GB, Bentivoglio A, Rosser A; European Huntington's Disease Network. Utilisation of Healthcare and Associated Services in Huntington's disease: a data mining study. PLoS Curr. 2011 Jan 21;3:RRN1206. doi: 10.13 — View Citation

Henley SM, Ridgway GR, Scahill RI, Klöppel S, Tabrizi SJ, Fox NC, Kassubek J; EHDN Imaging Working Group. Pitfalls in the use of voxel-based morphometry as a biomarker: examples from huntington disease. AJNR Am J Neuroradiol. 2010 Apr;31(4):711-9. doi: 10 — View Citation

Ho AK, Hocaoglu MB; European Huntington's Disease Network Quality of Life Working Group. Impact of Huntington's across the entire disease spectrum: the phases and stages of disease from the patient perspective. Clin Genet. 2011 Sep;80(3):235-9. doi: 10.11 — View Citation

Lee JM, Ramos EM, Lee JH, Gillis T, Mysore JS, Hayden MR, Warby SC, Morrison P, Nance M, Ross CA, Margolis RL, Squitieri F, Orobello S, Di Donato S, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa — View Citation

López-Sendón JL, Royuela A, Trigo P, Orth M, Lange H, Reilmann R, Keylock J, Rickards H, Piacentini S, Squitieri F, Landwehrmeyer B, Witjes-Ane MN, Jurgens CK, Roos RA, Abraira V, de Yébenes JG; European HD Network. What is the impact of education on Hunt — View Citation

Orth M, Handley OJ, Schwenke C, Dunnett SB, Craufurd D, Ho AK, Wild E, Tabrizi SJ, Landwehrmeyer GB; Investigators of the European Huntington's Disease Network. Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY. Version — View Citation

Orth M; European Huntington's Disease Network, Handley OJ, Schwenke C, Dunnett S, Wild EJ, Tabrizi SJ, Landwehrmeyer GB. Observing Huntington's disease: the European Huntington's Disease Network's REGISTRY. J Neurol Neurosurg Psychiatry. 2011 Dec;82(12):1 — View Citation

Quarrell OW, Handley O, O'Donovan K, Dumoulin C, Ramos-Arroyo M, Biunno I, Bauer P, Kline M, Landwehrmeyer GB; European Huntington’s Disease Network. Discrepancies in reporting the CAG repeat lengths for Huntington's disease. Eur J Hum Genet. 2012 Jan;20( — View Citation

Rickards H, De Souza J, Crooks J, van Walsem MR, van Duijn E, Landwehrmeyer B, Squitieri F, Simpson SA; European Huntington’s Disease Network. Discriminant analysis of Beck Depression Inventory and Hamilton Rating Scale for Depression in Huntington's dise — View Citation

Rickards H, De Souza J, van Walsem M, van Duijn E, Simpson SA, Squitieri F, Landwehrmeyer B; European Huntington's Disease Network. Factor analysis of behavioural symptoms in Huntington's disease. J Neurol Neurosurg Psychiatry. 2011 Apr;82(4):411-2. doi: — View Citation

Saft C, Epplen JT, Wieczorek S, Landwehrmeyer GB, Roos RA, de Yebenes JG, Dose M, Tabrizi SJ, Craufurd D; REGISTRY Investigators of the European Huntington's Disease Network, Arning L. NMDA receptor gene variations as modifiers in Huntington disease: a re — View Citation

* Note: There are 12 references in all — Click here to view all references

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Phenotypical characteristics of HD	The goal of the project is to collect longitudinal data on the phenotypical characteristics of HD gene mutation carriers.	13 years

External Links

•   EHDN