NCT00640003

Brief Summary

This research is being done to find out if Baclofen, a medicine that is often used for the treatment of abnormal stiffness, might also be useful to treat some of the neurologic problems caused by ataxia telangiectasia (A-T). The investigators also want to find out if there are better ways to measure the problems of ataxia and abnormal eye movement for future studies of medication in ataxia telangiectasia.

Trial Health

87
On Track

Trial Health Score

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

Enrollment
10

participants targeted

Target at below P25 for early_phase_1

Timeline
Completed

Started Apr 2007

Longer than P75 for early_phase_1

Geographic Reach
1 country

2 active sites

Status
completed

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

Trial Relationships

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Study Timeline

Key milestones and dates

Study Start

First participant enrolled

April 1, 2007

Completed
12 months until next milestone

First Submitted

Initial submission to the registry

March 14, 2008

Completed
6 days until next milestone

First Posted

Study publicly available on registry

March 20, 2008

Completed
2.9 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

February 1, 2011

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

February 1, 2011

Completed
Last Updated

December 20, 2017

Status Verified

December 1, 2017

Enrollment Period

3.8 years

First QC Date

March 14, 2008

Last Update Submit

December 18, 2017

Conditions

Keywords

ATAtaxiaTelangiectasia

Outcome Measures

Primary Outcomes (1)

  • Improvement in the decay constant for velocity storage as assessed by quantitative video examination of eye movement during and after rotation.

    3 months

Secondary Outcomes (1)

  • Other outcome measures include quantitative measurement of tremor, postural stability and a standard neurologic examination.

    3 months

Study Arms (2)

1

EXPERIMENTAL
Drug: BaclofenDrug: Placebo

2

EXPERIMENTAL
Drug: BaclofenDrug: Placebo

Interventions

Drug given by orally via capsules. Escalation steps in 4-day intervals. Step 1: 5mg QAM. Step 2: 5mg BID. Step 3: 5mg TID. Step 4: 10mg QAM, 5mg BID. Step 5: 10mg BID, 5mg QMid-day. Step 6: 10mg TID. Step 6 will last up to 2 weeks. De-escalation will start from highest dose step and work backwards in 2-day intervals. Complete drug administration of escalating, staying constant at 10mg TID and de-escalating will be done with both drug and placebo.

Also known as: Butanoic Acid
12

placebo drug

12

Eligibility Criteria

Age12 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Teenagers over 12 years old and young adults with A-T may join if they have been evaluated previously in the A-T Clinical Center at Johns Hopkins Hospital and have a measurable abnormality of eye movement.
  • Patients who are presently taking Baclofen will be eligible for the study if they are presently receiving the medication under the direction of Dr. Crawford in the ATCC, and are willing to withdraw from the medication for a period of one month prior to the initial screening visit.
  • Female patients who are sexually active will be given a standard serum HCG pregnancy test.
  • Those who are sexually active will be counseled about necessary precautions against pregnancy during the duration of the trial.

You may not qualify if:

  • A positive pregnancy test.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (2)

Johns Hopkins Hospital

Baltimore, Maryland, 21108, United States

Location

Johns Hopkins Hospital

Baltimore, Maryland, 21287, United States

Location

MeSH Terms

Conditions

Ataxia TelangiectasiaAtaxiaTelangiectasis

Interventions

BaclofenButyric Acid

Condition Hierarchy (Ancestors)

Spinocerebellar AtaxiasCerebellar AtaxiaCerebellar DiseasesBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesNeurocutaneous SyndromesDyskinesiasNeurologic ManifestationsVascular DiseasesCardiovascular DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesPrimary Immunodeficiency DiseasesDNA Repair-Deficiency DisordersMetabolic DiseasesNutritional and Metabolic DiseasesImmunologic Deficiency SyndromesImmune System DiseasesSigns and SymptomsPathological Conditions, Signs and Symptoms

Intervention Hierarchy (Ancestors)

gamma-Aminobutyric AcidAminobutyratesButyratesAcids, AcyclicCarboxylic AcidsOrganic ChemicalsFatty Acids, VolatileFatty AcidsLipids

Study Officials

  • Thomas O Crawford, M.D.

    Johns Hopkins School of Medicine

    PRINCIPAL INVESTIGATOR

Study Design

Study Type
interventional
Phase
early phase 1
Allocation
RANDOMIZED
Masking
TRIPLE
Who Masked
PARTICIPANT, CARE PROVIDER, INVESTIGATOR
Purpose
TREATMENT
Intervention Model
CROSSOVER
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

March 14, 2008

First Posted

March 20, 2008

Study Start

April 1, 2007

Primary Completion

February 1, 2011

Study Completion

February 1, 2011

Last Updated

December 20, 2017

Record last verified: 2017-12

Locations