Trial Outcomes & Findings for Riluzole in Fragile X Syndrome (NCT NCT00895752)

NCT ID: NCT00895752

Last Updated: 2017-04-18

Results Overview

The Clinical Global Impression - Improvement scale (CGI-I) is a 7 point scale that requires the clinician to assess how much the patient's illness has improved or worsened relative to a baseline state at the beginning of the intervention. and rated as: 1, very much improved; 2, much improved; 3, minimally improved; 4, no change; 5, minimally worse; 6, much worse; or 7, very much worse

Recruitment status

COMPLETED

Study phase

PHASE4

Target enrollment

6 participants

Primary outcome timeframe

Obtained at Week 6

Results posted on

2017-04-18

Participant Flow

Participant milestones

Participant milestones
Measure
Riluzole
6 subjects received open label riluzole, maximum dose of 50mg bid.
Overall Study
STARTED
6
Overall Study
COMPLETED
6
Overall Study
NOT COMPLETED
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Riluzole in Fragile X Syndrome

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
Age, Categorical
<=18 years
0 Participants
n=5 Participants
Age, Categorical
Between 18 and 65 years
6 Participants
n=5 Participants
Age, Categorical
>=65 years
0 Participants
n=5 Participants
Age, Continuous
22.5 years
n=5 Participants
Sex: Female, Male
Female
0 Participants
n=5 Participants
Sex: Female, Male
Male
6 Participants
n=5 Participants
Region of Enrollment
United States
6 participants
n=5 Participants
Aberrant Behavior Checklist
ABC Irritability
15.2 units on a scale
STANDARD_DEVIATION 11.4 • n=5 Participants
Aberrant Behavior Checklist
ABC Social Withdrawal
15.8 units on a scale
STANDARD_DEVIATION 11.0 • n=5 Participants
Aberrant Behavior Checklist
ABC Stereotypy
8.3 units on a scale
STANDARD_DEVIATION 7.8 • n=5 Participants
Aberrant Behavior Checklist
ABC Hyperactivity
19.5 units on a scale
STANDARD_DEVIATION 8.3 • n=5 Participants
Aberrant Behavior Checklist
ABC Inappropriate Speech
8.5 units on a scale
STANDARD_DEVIATION 2.7 • n=5 Participants
ADHD rating scale
24.3 units on a scale
STANDARD_DEVIATION 8.4 • n=5 Participants
Clinical Global Improvement-Severity
4.3 units on a scale
STANDARD_DEVIATION 0.5 • n=5 Participants
Peabody Picture Vocabulary Test
116.8 units on a scale
STANDARD_DEVIATION 40.2 • n=5 Participants
Social Reciprocity Scale
114.7 units on a scale
STANDARD_DEVIATION 23.6 • n=5 Participants
Children's Yale-Brown Obsessive Compulsive Scale modified for pervasive developmental disorders
11.2 units on a scale
STANDARD_DEVIATION 3.2 • n=5 Participants

PRIMARY outcome

Timeframe: Obtained at Week 6

The Clinical Global Impression - Improvement scale (CGI-I) is a 7 point scale that requires the clinician to assess how much the patient's illness has improved or worsened relative to a baseline state at the beginning of the intervention. and rated as: 1, very much improved; 2, much improved; 3, minimally improved; 4, no change; 5, minimally worse; 6, much worse; or 7, very much worse

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
Clinical Global Impression-Improvement (CGI-I)
3.5 units on a scale
Interval 2.0 to 4.0

PRIMARY outcome

Timeframe: Obtained at Baseline and Week 6

The CY-BOCS PDD has been utilized in a largescale clinical treatment study of repetitive behavior in idiopathic ASDs. CYBOCS-PDD scores range from 0 to 20 and measure repetitive/compulsive behavior and not obsessions. Higher score indicate worse outcome.

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
Children's Yale-Brown Obsessive Compulsive Scale (CY-BOCS)
Baseline
11.2 Units on a Scale
Standard Deviation 3.2
Children's Yale-Brown Obsessive Compulsive Scale (CY-BOCS)
Six Weeks
9.7 Units on a Scale
Standard Deviation 3.3

SECONDARY outcome

Timeframe: Week 6

The Aberrant Behavior Checklist (ABC) is a 58-item measure of maladaptive behaviors and is used as a measure of drug effects. The ABC has 5 subscales: Social Withdrawal (16 items) ranging from 0 (not at all) to 48 (severe), Irritability (15 items) ranging from 0 (not at all) to 45 (severe), Inappropriate Speech (4 items) ranging from 0 (not at all) to 12 (severe), Hyperactivity (16 items) ranging from 0 (not at all) to 48 (severe), and Stereotypy (7 items) ranging from 0 (not at all) to 21 (severe). Items are rated from 0 (not at all) to 3 (severe).

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
Aberrant Behavior Checklist
Irritablity
8.3 units on a scale
Standard Deviation 7.7
Aberrant Behavior Checklist
Social Withdrawal
9.7 units on a scale
Standard Deviation 8.0
Aberrant Behavior Checklist
Stereotypy
5.3 units on a scale
Standard Deviation 6.4
Aberrant Behavior Checklist
Hyperactivity
15.0 units on a scale
Standard Deviation 9.2
Aberrant Behavior Checklist
Inappropriate Speech
6.2 units on a scale
Standard Deviation 4.0

SECONDARY outcome

Timeframe: Week 6

The ADHD Rating Scale is an 18-item scale directly derived from DSM-IV criteria for Attention Deficit Hyperactivity Disorder. The ADHD Rating Scale-IV is completed by the parent and scored by a clinician. The scale consists of 2 subscales: inattention (9 items) and hyperactivity-impulsivity (9 items). If 3 or more items are skipped, the clinician should use extreme caution in interpreting the scale. The total score can range from 0 to 54, with a higher score indicating greater severity

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
The ADHD Rating Scale
19.0 units on a scale
Standard Deviation 9.7

SECONDARY outcome

Timeframe: Week 6

The Clinical Global Impression - Severity scale (CGI-S) is a 7-point scale that requires the clinician to rate the severity of the patient's illness at the time of assessment, relative to the clinician's past experience with patients who have the same diagnosis. Considering total clinical experience, a patient is assessed on severity of mental illness at the time of rating 1, normal, not at all ill; 2, borderline mentally ill; 3, mildly ill; 4, moderately ill; 5, markedly ill; 6, severely ill; or 7, extremely ill

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
The Clinical Global Impression - Severity Scale
4.2 units on a scale
Standard Deviation 0.4

SECONDARY outcome

Timeframe: Week 6

The Peabody Picture Vocabulary Test is one of the most commonly used assessment tests that measure verbal ability in standard American English vocabulary. This test has been nationally standardized using examinees from various age groups, from children to adults. Thus, the raw scores are equated to mental age, using the norms obtained from standardization. The total standard scores range from 40 (worse receptive vocabulary) to 160 (better receptive vocabulary). The scores can also be converted to percentile rank.

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
The Peabody Picture Vocabulary Test
123.2 units on a scale
Standard Deviation 36.6

SECONDARY outcome

Timeframe: Week 6

The 65-item SRS is a standardized measure of the core symptoms of autism. Each item is scored on a 4-point Likert scale. The score of each individual item is summed to create a total raw score. A total scores results are as follows: 0-62: Within normal limits 63-79: Mild range of impairment 80-108: Moderate range of impairment 109-149: Severe range of impairment.

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
The Social Reciprocity Scale
103.5 units on a scale
Standard Deviation 24.4

SECONDARY outcome

Timeframe: Screen and Week 6

ERK activations times, as defined as the time in minutes for ERK phosphorylation to reach the half maximal level.

Outcome measures

Outcome measures
Measure
Riluzole
n=6 Participants
Six subjects received 6-weeks of open-label riluzole with maximum dose of 50mg bid.
Extra-cellular Signal-relatedness Kinase (ERK)
2.99 minutes
Standard Deviation 0.3

Adverse Events

Riluzole

Serious events: 0 serious events
Other events: 3 other events
Deaths: 0 deaths

Serious adverse events

Adverse event data not reported

Other adverse events

Other adverse events
Measure
Riluzole
n=6 participants at risk
Six week open-label treatment with riluzole, maximum dose of 50 mg twice a day. Riluzole: Six week open-label treatment with riluzole, maximum dose of 50 mg twice a day.
General disorders
Tiredness
33.3%
2/6 • Number of events 3 • Six Weeks
General disorders
Irritability
50.0%
3/6 • Number of events 3 • Six Weeks
Psychiatric disorders
Worsening of Inattention
16.7%
1/6 • Number of events 1 • Six Weeks
Metabolism and nutrition disorders
Appetite Reduction
16.7%
1/6 • Number of events 1 • Six Weeks

Additional Information

Craig Erickson MD

Cincinnati Childrens Hospital

Phone: 5136366265

Results disclosure agreements

  • Principal investigator is a sponsor employee
  • Publication restrictions are in place