Beta Testing of a New Assessment in Huntington's Disease (HD)

Conditions

Huntington Disease

Keywords

beta testing, assessment battery, HD

Brief summary

Huntington's disease (HD) is an inherited neurodegenerative disease for which there are no existing disease-modifying treatments. Repair-HD is an EU FP7 consortium that aims to establish all the preclinical requirements for transplantation of stem cell-derived neurons in HD in order to replace those lost to the disease process. These requirements include the generation of new clinical assessments for detailed monitoring of patients with HD who have undergone cell replacement therapy. This protocol describes the beta testing of a new clinical assessment battery: Core Assessment Protocol for Intrastriatal Transplantation in HD version 2 (CAPIT-HD beta / CAPIT-HD2). CAPIT-HD beta represents a substantial revision of a previous CAPIT-HD battery published over 20 years ago, which is in need of updating in order to accommodate knowledge from clinical transplant studies over this time and to take advantage of technological advances in patient assessment. HD is a complex disorder in which there is relentless deterioration of motor, cognitive and behavioural functions, usually from mid-life onwards. The original CAPIT battery aimed to capture elements of change in all three domains, but was based predominantly on subjective semi-quantitative assessment tools that have poor inter-rater reliability. Moreover, a number of deficits, such as impairments in social cognition, were not recognised when the original CAPIT-HD battery was constructed, so we have developed novel assessments of these deficits, some of which are included in CAPIT-HD beta. The beta testing will take place in established HD clinical centres in Cardiff, Manchester, Paris, and Munster by teams of researchers who are experienced in leading clinic research in HD. Patients with early to moderate HD will be assessed at baseline, and at one and twelve months later, to assess the reliability and sensitivity of the CAPIT-HD beta battery. Arrangements for data storage and analysis are in place.

Lucie Chenain, Katia Youssov, Chloé Clavel, Anne-Catherine Bachoud-Lévi

Scientific Reports2026-08-06

10.1038/s41598-026-66000-7

xHD-Vox, an Automated Speech Model for Estimating Motor and Cognitive Scores in Huntington Disease: Development and Longitudinal Validation.

Le Ludec T, Gil-Salcedo A, Titeux H, Louiset R, Le Moine Veillon C, Massart R, Bachoud-Lévi AC.

2026-07-08

10.2196/83838

Specific contribution of cognitive and motor impairments with functional capacity and dependence in Huntington's disease.

Gil-Salcedo A, Lunven M, Jacquemot C, Massart R, Bachoud-Levi AC.

2025-02-22

10.1007/s00415-025-12982-9

The joint memory effect: challenging the selfish stigma in Huntington’s disease?

Romain Dalléry, Nicolas Fraisse, Laurent Cléret de Langavant, Katia Youssov, Graça Morgado et al. (15 authors)

Brain Communications2024-12-092 citations

10.1093/braincomms/fcae440

Graph methods to infer spatial disturbances: Application to Huntington's Disease's speech.

Chenain L, Riad R, Fraisse N, Jubin C, Morgado G, Youssov K, Lunven M, Bachoud-Levi AC.

2024-05-171 citations

10.1016/j.cortex.2024.04.014

Predicting clinical scores in Huntington's disease: a lightweight speech test.

Riad R, Lunven M, Titeux H, Cao XN, Hamet Bagnou J, Lemoine L, Montillot J, Sliwinski A, Youssov K, Cleret de Langavant L, Dupoux E, Bachoud-Lévi AC.

2022-05-149 citations

10.1007/s00415-022-11148-1

Interventions

OTHERCAPIT-HD beta

New assessment protocol for assessment of complex therapies in Huntington's disease for both groups

Study design

Allocation

NON_RANDOMIZED

Intervention model

PARALLEL

Primary purpose

OTHER

Masking

NONE

Eligibility

Sex/Gender

ALL

Age

18 Years to No maximum

Healthy volunteers

Yes

Inclusion criteria

1. Patients Inclusion criteria * Must be confirmed to carry the HD gene through genetic testing (CAG ≥ 36) * Must be 18 years or above * Stage I or II disease (TFC staging)

Exclusion criteria

* The inability to approve consent * Any comorbid condition that has the potential to confound the results of the study 2. Controls Inclusion criteria - Must be 18 years or above

Design outcomes

Primary

Measure	Time frame	Description
All dysfunction or disorder of huntington patient measured by means of a new battery assessments	1 year	The goal is to validate a battery of assessments for application in a wide range of complex therapies for Huntington's disease (HD)

Secondary

Measure	Time frame	Description
Difference on motor score between patient and healthy volontary measured by motor tests of the revised Core Assessment Protocol	1 year	The goal is to validate the discriminative ability of the revised Core Assessment Protocol for Intracranial transplantation in HD for the motor
Difference on cognitive score between patient and healthy volontary measured by cognitive tests of the revised Core Assessment Protocol	1 year	The goal is to validate the discriminative ability of the revised Core Assessment Protocol for Intracranial transplantation in HD for the cognitive
Difference on psychiatric score between patient and healthy volontary measured by psychiatric evaluation of the revised Core Assessment Protocol	1 year	The goal is to validate the discriminative ability of the revised Core Assessment Protocol for Intracranial transplantation in HD for the psychiatric
Difference on functional scale between patient and healthy volontary measured by functional evaluation of the revised Core Assessment Protocol	1 year	The goal is to validate the discriminative ability of the revised Core Assessment Protocol for Intracranial transplantation in HD for the functional domains of impairment in HD
Number of new assessment battery performed correctly	1 year	—

Countries

France

Contacts

Primary ContactAnne-Catherine BACHOUD-LEVI, MD, PhD

anne-catherine.bachoud-levi@aphp.fr(0)1.49.81.43.01

Outcome results

None listed