Efficacy Of Oral Trehalose In Spinocerebellar Ataxia 3

NCT ID: NCT04399265

Last Updated: 2022-08-16

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Clinical Phase

NA

Total Enrollment

20 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-02-24

Study Completion Date

2023-06-30

Brief Summary

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This study evaluates the effectiveness of oral trehalose in alleviating the neuropathological and motor behaviour deficits among patients with SCA3. A total of 40 participants with SCA3 will be recruited, with 20 participants to be administered with trehalose while another 20 participants to be administered with a maltose placebo.

Detailed Description

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Spinocerebellar ataxia 3 (SCA3) is a rare form of inherited neurodegenerative disease involving progressive degeneration of spinocerebellar tract. SCA3 is characterised by increasingly worsening cerebellar function leading to gait abnormalities and poor coordination, dysarthria, and abnormal eye movements. Non-ataxia features include pyramidal and extrapyramidal manifestations, sensorimotor, neuropsychological and psychiatric symptoms. This is attributed to the role of cerebellum in motor, cognitive and affective processing (i.e. cerebellar cognitive and affective syndrome; CCAS), as well as its extensive connection with cerebral structures.

Trehalose is an omnipotent disaccharide molecule found in lower and higher life forms except in vertebrates. It has an amorphous (i.e. non-reducing) property, which is shown in its high hydrophilicity, chemical stability and strong resistance to denaturation / breakdown by heat, acid or enzyme. It is also shown to help refold partially denatured protein, thereby stabilizes protein aggregates, including those of polyglutamine, in vivo as well as in vitro. This has provided an avenue in which trehalose as a therapeutic agent for neurodegenerative disorders with pathological changes of protein aggregates.

In this study, a double-blinded randomised controlled trial (RCT) will be employed. A total of 40 patients with SCA3 will be randomly allocated to oral trehalose group and a placebo group (20 participants for each arm). With regards to clinical outcomes, motor and cognitive performances will be assessed to infer the efficacy of trehalose. Likewise, structural, resting-state fMRI (i.e. functional connectivity), and MR spectroscopy (i.e. metabolism), will be used as imaging biomarkers in this study.

Conditions

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Spinocerebellar Ataxia Type 3

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

This study will employ a double-blind randomised controlled trial (RCT) with two arms. Patients with SCA3 will be randomly allocated to oral trehalose group and a maltose placebo group. A total of 20 participants will be allocated to each group. There is a possibility of including other types of SCA at later stage of the study.
Primary Study Purpose

TREATMENT

Blinding Strategy

DOUBLE

Participants Outcome Assessors
Patients with SCA 3 will be randomised into the active treatment group and placebo group to which they are blinded from their group allocation. Likewise, the outcome assessor will be blinded from the patient's group status. A partial unblinding will be made with Group A vs. Group B (rather than the actual grouping) for preliminary analysis on Visit 1 and Visit 2.

Study Groups

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Trehalose

Trehalose powder form to be dissolved in water, to be consumed by mouth, every day for 3 months.

Group Type EXPERIMENTAL

Trehalose

Intervention Type DIETARY_SUPPLEMENT

A disaccharide sugar usually used as food preservative, but for this study it is given at a higher dose

Maltose placebo

Isocaloric maltose powder form to be dissolved in water, to be consumed by mouth, every day for 3 months.

Group Type PLACEBO_COMPARATOR

Maltose

Intervention Type DIETARY_SUPPLEMENT

Maltose power

Interventions

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Trehalose

A disaccharide sugar usually used as food preservative, but for this study it is given at a higher dose

Intervention Type DIETARY_SUPPLEMENT

Maltose

Maltose power

Intervention Type DIETARY_SUPPLEMENT

Eligibility Criteria

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Inclusion Criteria

* Age between 18 and 85 years old.
* Genetically confirmed SCA 3 diagnosis.
* Presence of progressive cerebellar syndrome (i.e. symptomatic) and genetic confirmation of SCA 3 in one of the immediate family members with similar clinical syndrome.
* Able to read, speak, and understand English or Malay.

Exclusion Criteria

* Diagnosis of Diabetes Mellitus Type 1 or Type 2
* Presence of any concomitant neurological condition that might interfere with clinical measures used in this study.
* Presence of contraindication or hypersensitivity to trehalose.
* Use of stimulant / medication, caffeine, and tobacco within less than 24 hours washout period prior to assessment and / or scan.
* Presence of severe visual and / or auditory perceptual deficits.
* MRI contra-indications: claustrophobia, pregnancy, electronic implants (e.g. pacemaker) in the body, aneurysm clip, and current or past employment as machinists, welders or metal workers.
Minimum Eligible Age

18 Years

Maximum Eligible Age

85 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Radboud University Medical Center

OTHER

Sponsor Role collaborator

National University of Malaysia

OTHER

Sponsor Role lead

Responsible Party

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Dr Norlinah Mohamed Ibrahim

Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Norlinah Mohamed Ibrahim, MRCP

Role: PRINCIPAL_INVESTIGATOR

National University of Malaysia

Locations

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UKM Medical Centre

Cheras, Kuala Lumpur, Malaysia

Site Status RECRUITING

Countries

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Malaysia

Central Contacts

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Kah Hui Yap, Master

Role: CONTACT

+60165536721

Norlinah Mohamed Ibrahim, MRCP

Role: CONTACT

+60122145306

Facility Contacts

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Kah Hui Yap, Master

Role: primary

0165536721

Norlinah Mohamed Ibrahim, MRCP

Role: backup

+60122145306

Other Identifiers

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DIP-2019-007

Identifier Type: -

Identifier Source: org_study_id

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