Trial Outcomes & Findings for Novel Cognitive Treatment Targets for Epidiolex in Sturge- Weber Syndrome (NCT NCT04447846)

NCT ID: NCT04447846

Last Updated: 2023-05-25

Results Overview

Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.

Recruitment status

COMPLETED

Study phase

PHASE2

Target enrollment

11 participants

Primary outcome timeframe

Baseline, Follow-up (6 months)

Results posted on

2023-05-25

Participant Flow

After consent, subject 10 was not enrolled as they did not meet the inclusion criteria of remaining at a stable does of anti-epileptic drugs 4 weeks prior to enrollment.

Participant milestones

Participant milestones
Measure
Cannabidiol/ Epidiolex
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Overall Study
STARTED
10
Overall Study
COMPLETED
9
Overall Study
NOT COMPLETED
1

Reasons for withdrawal

Reasons for withdrawal
Measure
Cannabidiol/ Epidiolex
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Overall Study
Withdrew due to moderate side effects (fatigue)
1

Baseline Characteristics

Novel Cognitive Treatment Targets for Epidiolex in Sturge- Weber Syndrome

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Cannabidiol
n=10 Participants
Subjects took oral cannabidiol for 6 months (doses ranged from 5 mg/kg/day to 20 mg/kg/day). Neuropsychological, psychiatrics and motor assessments were administered at baseline and six months follow-up.
Age, Continuous
13.83 years
STANDARD_DEVIATION 9.67 • n=5 Participants
Sex: Female, Male
Female
6 Participants
n=5 Participants
Sex: Female, Male
Male
4 Participants
n=5 Participants
Ethnicity (NIH/OMB)
Hispanic or Latino
2 Participants
n=5 Participants
Ethnicity (NIH/OMB)
Not Hispanic or Latino
7 Participants
n=5 Participants
Ethnicity (NIH/OMB)
Unknown or Not Reported
1 Participants
n=5 Participants
Race (NIH/OMB)
American Indian or Alaska Native
0 Participants
n=5 Participants
Race (NIH/OMB)
Asian
0 Participants
n=5 Participants
Race (NIH/OMB)
Native Hawaiian or Other Pacific Islander
1 Participants
n=5 Participants
Race (NIH/OMB)
Black or African American
0 Participants
n=5 Participants
Race (NIH/OMB)
White
6 Participants
n=5 Participants
Race (NIH/OMB)
More than one race
2 Participants
n=5 Participants
Race (NIH/OMB)
Unknown or Not Reported
1 Participants
n=5 Participants
Region of Enrollment
United States
10 participants
n=5 Participants
Brain Involvement
Right-Sided Brain Involvement
6 Participants
n=5 Participants
Brain Involvement
Left-Sided Brain Involvement
2 Participants
n=5 Participants
Brain Involvement
Bilateral Brain Involvement
2 Participants
n=5 Participants
Skin Involvement
Right-Sided Skin Involvement
4 Participants
n=5 Participants
Skin Involvement
Left-Sided Skin Involvement
2 Participants
n=5 Participants
Skin Involvement
Bilateral Skin Involvement
4 Participants
n=5 Participants
Eye Involvement
Right-Sided Eye Involvement
4 Participants
n=5 Participants
Eye Involvement
Left-Sided Eye Involvement
2 Participants
n=5 Participants
Eye Involvement
Bilateral Eye Involvement
1 Participants
n=5 Participants
Eye Involvement
No Eye Involvement
3 Participants
n=5 Participants

PRIMARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 1 was unable to complete this assessment due to lack of understanding of the instructions. Subject 10 was unable to complete this assessment due to a system error, where the fully adjusted score was not obtained. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=7 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
List Sorting Working Memory Test
Baseline List Sorting Working Memory Fully Adjusted Score
37.14 T-score
Standard Deviation 10.92
List Sorting Working Memory Test
Follow-Up List Sorting Working Memory Fully Adjusted Score
39.14 T-score
Standard Deviation 13.78

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 10 did not complete this assessment due to a technical issue where the fully adjusted score was not calculated. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on cognitive function was collected using the Picture Vocabulary subtest from the NIH Toolbox. Single words are presented via an audio file, paired simultaneously with 4 screen images of objects, actions, and/or depictions of concepts. The task is to pick the picture that matches the spoken word. Performance on the task was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=8 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Picture Vocabulary Test
Baseline Mean of the Fully Adjusted Picture Vocabulary Score
42.88 T-score
Standard Deviation 8.86
Picture Vocabulary Test
Follow-up Mean of the Fully Adjusted Picture Vocabulary Score
42.38 T-score
Standard Deviation 9.43

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Change in seizure frequency by seizure score at pre-treatment baseline and after six months. The seizure score is taken from the Sturge-Weber Neuroscore on a scale of 0 to 4 where 0=none, 1=1+. but controlled, 2=Breakthrough, 3=monthly, 4=Weekly+. Higher scores indicate worse outcome.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Seizure Frequency
Baseline Seizure Score from Sturge-Weber Neuroscore
1.11 score on a scale
Standard Deviation 0.33
Seizure Frequency
Follow-up Seizure Score from Sturge-Weber Neuroscore
1.00 score on a scale
Standard Deviation 0.00

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on migraine severity will be collected using patient responses to questions on a standard six-point scale. Data will be collected on the frequency of an event (e.g. feelings of frustration, performance of daily activities) which will be transformed into a score from 0 to 100 where higher scores indicate a less migraine severity and better outcomes. Data will be collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=8 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Migraine Severity
Baseline Mean Migraine-Quality of Life Score
96.83 score on a scale
Standard Deviation 7.62
Migraine Severity
Follow-up Mean Migraine-Quality of Life Score
97.50 score on a scale
Standard Deviation 4.69

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on motor function was collected using a Modified House Classification. Data was collected on the ability to complete a task with the subject's non-dominant hand which was transformed into a score from 1 to 8 and 0 to 32 where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Modified House Classification Scores
Baseline Mean Highest Category in Which All Items were Successfully Completed
5.11 score on a scale
Standard Deviation 0.93
Modified House Classification Scores
Follow-up Mean Highest Category in Which All Items were Successfully Completed
5.44 score on a scale
Standard Deviation 1.33
Modified House Classification Scores
Baseline Mean Total Number of Items Successfully Completed
26.67 score on a scale
Standard Deviation 4.12
Modified House Classification Scores
Follow-up Mean Total Number of Items Successfully Completed
27.44 score on a scale
Standard Deviation 4.75

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on motor function was collected using the Erhardt Developmental Prehension assessment. Data was collected on the ability to complete a task with each hand, which was scored as age equivalence of task performance (in months). Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Erhardt Developmental Prehension Assessment Scores
Baseline Mean Dominant Hand Total Score
54.22 Months
Standard Deviation 2.64
Erhardt Developmental Prehension Assessment Scores
Follow-up Mean Dominant Hand Total Score
53.56 Months
Standard Deviation 4.04
Erhardt Developmental Prehension Assessment Scores
Baseline Mean Non-Dominant Hand Total Score
51.22 Months
Standard Deviation 9.48
Erhardt Developmental Prehension Assessment Scores
Follow-up Mean Non-Dominant Hand Total Score
53.78 Months
Standard Deviation 4.15

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on motor function was collected using the Pediatric Evaluation of Disability Inventory Computer Adapted Test. Data was collected on the subject's ability to complete tasks involved in daily activities, mobility, and social/ cognitive activities. The data was transformed into scaled scores ranging from 20 to 80, where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Baseline Mean Score for Daily Activities
58.67 score on a scale
Standard Deviation 7.76
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Follow-up Mean Score for Daily Activities
60.00 score on a scale
Standard Deviation 6.50
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Baseline Mean Score for Mobility
67.11 score on a scale
Standard Deviation 4.76
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Follow-Up Mean Score for Mobility
68.56 score on a scale
Standard Deviation 4.07
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Baseline Mean Score for Responsibility
52.44 score on a scale
Standard Deviation 11.67
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Follow-Up Mean Score for Responsibility
53.89 score on a scale
Standard Deviation 11.10
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Baseline Mean Score for Social Cognitive
66.89 score on a scale
Standard Deviation 5.35
Pediatric Evaluation of Disability Inventory Computer Adapted Test
Follow-up Mean Score for Social Cognitive
67.22 score on a scale
Standard Deviation 4.63

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to persistent side effect and was not included in analysis.

Data on motor function was collected using the ABILHAND questionnaire, a measure of manual ability for adults with upper limb impairments. Data was collected on the subject's ability to complete daily activities that involve the upper limbs. Score was collected as a patient measure with scores ranging from -10 to 10. Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=8 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
ABILHAND Questionnaire
Baseline Mean of the ABILHAND
1.81 score on a scale
Standard Deviation 1.98
ABILHAND Questionnaire
Follow-up Mean of the ABILHAND
2.88 score on a scale
Standard Deviation 1.39

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subjects 1 and 10 did not complete this questionnaire as they were too young. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on cognitive function was collected using selected subtests, from either the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or the Wechsler Adult Intelligence Scale (WAIS-IV). For the WISC-V and WAIS-IV subtests selected, scaled scores were derived from the normative data which account for age and demographic information. The selected WISC-V/WAIS-IV subtests were as follows: Digit Span, Symbol Search, Coding and Processing Speed; the first three subtests were scored from 0-10, the fourth subtest from 0-100, and for all subtests higher score is better. The selected subtests, from the WISC-V and the WAIS-IV, were combined to increase statistical power. Statistically rare changes in individual test scores were determined using a reliable change index methodology based upon normative information for these assessments.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=7 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Baseline Mean Digit Span Score
5.71 score on a scale
Standard Deviation 2.81
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Follow-up Mean Digit Span Score
6.43 score on a scale
Standard Deviation 2.07
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Baseline Mean Symbol Search Score
6.00 score on a scale
Standard Deviation 2.71
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Follow-up Mean Symbol Search Score
5.57 score on a scale
Standard Deviation 2.07
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Baseline Mean Coding Score
5.57 score on a scale
Standard Deviation 2.88
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Follow-up Mean Coding Score
4.71 score on a scale
Standard Deviation 2.22
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Baseline Mean Processing Speed Score
77.14 score on a scale
Standard Deviation 13.93
Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
Follow-up Mean Processing Speed Score
73.43 score on a scale
Standard Deviation 11.19

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subjects below the age of 18 completed the pediatric version of this questionnaire and were included in analysis.

Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event (e.g. forgetting schoolwork) was collected and transformed into a t-score from 0 to 100 where higher t-scores indicate worse anger, worse anxiety, worse pain, better social relationships, worse stigma, worse depression, better cognitive function, and worse fatigue. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=6 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Anxiety
41.07 T-score
Standard Deviation 5.74
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Anger
53.02 T-score
Standard Deviation 3.09
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Anger
49.67 T-score
Standard Deviation 7.00
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Anxiety
45.20 T-score
Standard Deviation 5.79
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Pain
40.20 T-score
Standard Deviation 4.16
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Pain
43.50 T-score
Standard Deviation 5.89
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Social Relations
43.28 T-score
Standard Deviation 15.10
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Social Relations
54.33 T-score
Standard Deviation 6.96
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Stigma
42.45 T-score
Standard Deviation 6.42
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Stigma
44.52 T-score
Standard Deviation 4.88
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Depression
44.07 T-score
Standard Deviation 6.29
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Depression
44.62 T-score
Standard Deviation 8.58
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Cognitive Function
49.27 T-score
Standard Deviation 3.36
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Cognitive Function
51.33 T-score
Standard Deviation 6.44
Pediatric Neurological Quality of Life (Neuro-QoL)
Baseline Mean Pediatric Score for Fatigue
46.38 T-score
Standard Deviation 9.44
Pediatric Neurological Quality of Life (Neuro-QoL)
Follow-up Mean Pediatric Score for Fatigue
45.97 T-score
Standard Deviation 8.14

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on executive function was collected using the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Data on the frequency of an event (e.g. becomes upset too easily) was collected and transformed into a t-score. The following BRIEF-2 subtests were evaluated: Behavioral Regulation Index, Emotional Regulation Index, Cognitive Regulation Index, and Global Executive Composite. For each BRIEF-2 subtest, t-scores range from 0 to 100; a score of 50 indicates the population mean with a standard deviation of 10. For all BASC-3 subtests, higher scores are worse outcome.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Baseline Mean for Behavioral Regulation Index
55.11 T-score
Standard Deviation 11.66
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Follow-Up Mean for Behavioral Regulation Index
52.44 T-score
Standard Deviation 13.60
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Baseline Mean for Emotional Regulation Index
60.22 T-score
Standard Deviation 10.24
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Follow-up Mean for Emotional Regulation Index
51.22 T-score
Standard Deviation 12.29
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Baseline Mean for Cognitive Regulation Index
54.11 T-score
Standard Deviation 7.06
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Follow-up Mean for Cognitive Regulation Index
54.22 T-score
Standard Deviation 10.59
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Baseline Mean for Global Executive Composite
56.78 T-score
Standard Deviation 8.17
Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
Follow-up Mean for Global Executive Composite
53.33 T-score
Standard Deviation 11.57

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on social function was collected using the Social Responsiveness Scale-Second Edition (SRS-2). Data on a child's ability to engage in emotionally appropriate reciprocal social interactions in naturalistic settings was collected and transformed into a t-score from 0 to 100 where higher scores indicate greater impairment in social function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Social Responsiveness Scale, Second Edition (SRS-2)
Baseline Mean Total Score
54.22 T-score
Standard Deviation 7.71
Social Responsiveness Scale, Second Edition (SRS-2)
Follow-up Mean Total Score
49.22 T-score
Standard Deviation 7.09

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on behavioral function was collected using the Behavioral Assessment System for Children, Third Edition (BASC-3). Data on the frequency of a behavior (e.g. avoids eye contact) was collected and transformed into a t-score. Subscales for the BASC-3 scored were: External Problems Composite, the Internal Problems Composite, the Behavioral Symptoms Index, and the Adaptive Skills Composite. For the first three subscales, lower t-score indicates better outcome; for the fourth, a lower t-score indicates worse outcome. T-scores for all the BASC-3 subscales range from 0 to 100, and a t-score of 50 indicates the population mean with a standard deviation of 10.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Behavioral Assessment System for Children, Third Edition (BASC-3)
Baseline Mean External Problems Composite
51.22 T-score
Standard Deviation 6.10
Behavioral Assessment System for Children, Third Edition (BASC-3)
Follow-up Mean External Problems Composite
50.11 T-score
Standard Deviation 6.31
Behavioral Assessment System for Children, Third Edition (BASC-3)
Baseline Mean Internal Problems Composite
52.22 T-score
Standard Deviation 5.81
Behavioral Assessment System for Children, Third Edition (BASC-3)
Follow-Up Mean Internal Problems Composite
46.22 T-score
Standard Deviation 7.17
Behavioral Assessment System for Children, Third Edition (BASC-3)
Baseline Mean Behavioral Symptoms Index
54.22 T-score
Standard Deviation 4.21
Behavioral Assessment System for Children, Third Edition (BASC-3)
Follow-up Mean Behavioral Symptoms Index
50.11 T-score
Standard Deviation 8.19
Behavioral Assessment System for Children, Third Edition (BASC-3)
Baseline Mean Adaptive Skills Composite
45.00 T-score
Standard Deviation 8.29
Behavioral Assessment System for Children, Third Edition (BASC-3)
Follow-Up Mean Adaptive Skills Composite
48.44 T-score
Standard Deviation 7.63

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.

Data on anxiety was collected using the Screen for Child Anxiety Related Disorders (SCARED). Data on the truthfulness of a statement (e.g. I am nervous) was collected and transformed into a score from 0 to 82 where higher scores indicate greater anxiety. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=8 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Screen for Child Anxiety Related Disorders (SCARED)
Baseline Mean of Total Score
14.86 score on a scale
Standard Deviation 14.49
Screen for Child Anxiety Related Disorders (SCARED)
Follow-up Mean of Total Score
11.57 score on a scale
Standard Deviation 11.16

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up appointment due to a persistent side effect and was not included in analysis.

Data on quality of life was collected using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Data on the frequency of an event (e.g. had trouble concentrating on a task) was collected and transformed into a score from 0 to 100 where higher scores reflect better quality of life. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=8 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)
Baseline Mean Total Score
61.17 score on a scale
Standard Deviation 18.14
Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)
Follow-up Mean Total Score
71.39 score on a scale
Standard Deviation 10.75

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Safety of Epidiolex was measured by the number of adverse events and serious adverse events that result from study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=10 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Safety of Epidiolex
Number of Adverse Events that Resulted from the Study Drug
24 Events
Safety of Epidiolex
Number of Serious Adverse Events that Resulted from the Study Drug
0 Events

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up visit and not included in analysis.

Data on neurological function was collected using the Neuroscore. Data on frequency of seizures, extent of hemiparesis, assessment of visual field cut, and degree of cognitive functioning was transformed into a score from 0 to 15 where higher scores indicate worse neurologic function. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Neuroscore
Baseline Mean Seizure Score
1.11 score on a scale
Standard Deviation 0.33
Neuroscore
Follow-Up Mean Seizure Score
1.00 score on a scale
Standard Deviation 0.00
Neuroscore
Baseline Mean Hemiparesis Score
1.44 score on a scale
Standard Deviation 1.13
Neuroscore
Follow-Up Mean Hemiparesis Score
1.00 score on a scale
Standard Deviation 0.87
Neuroscore
Baseline Mean Visual Field Cut Score
0.11 score on a scale
Standard Deviation 0.33
Neuroscore
Follow-Up Mean Visual Field Cut Score
0.00 score on a scale
Standard Deviation 0.00
Neuroscore
Baseline Mean Cognitive Function Score
2.33 score on a scale
Standard Deviation 0.50
Neuroscore
Follow-up Mean Cognitive Function Score
1.44 score on a scale
Standard Deviation 0.53
Neuroscore
Baseline Mean Composite Score
5.00 score on a scale
Standard Deviation 1.32
Neuroscore
Follow-up Mean Composite Score
3.44 score on a scale
Standard Deviation 0.88

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subject 9 was withdrawn prior to the 6 month follow-up appointment and was not included in analysis.

Data on facial port-wine birthmarks was collected using the Port-wine Birthmark Score. Data on percent of face covered, thickness of birthmark, and darkness of birthmark color was collected and transformed into a score from 0 to 43 where higher scores indicate greater severity and greater surface area involved. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=9 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Port-wine Birthmark Score
Baseline Mean Total Score
8.56 score on a scale
Standard Deviation 3.09
Port-wine Birthmark Score
Follow-up Mean Total Score
8.11 score on a scale
Standard Deviation 3.18

SECONDARY outcome

Timeframe: Baseline, Follow-up (6 months)

Population: Subjects above the age of 18 completed the pediatric version of this questionnaire and were included in analysis.

Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event was collected and transformed into a t-score from 0 to 100. Higher t-scores indicate better communication, better ability to participate in social activity, worse anxiety, worse depression, worse emotional and behavioral dyscontrol, worse fatigue, better positive affect, worse sleep disturbance, better lower and upper extremity functions, worse stigma, better satisfaction with social roles, and better cognitive function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.

Outcome measures

Outcome measures
Measure
Cannabidiol/ Epidiolex
n=3 Participants
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Anxiety
46.77 T-score
Standard Deviation 13.33
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Anxiety
46.87 T-score
Standard Deviation 6.45
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Depression
42.70 T-score
Standard Deviation 10.05
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Depression
44.23 T-score
Standard Deviation 9.53
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Fatigue
45.23 T-score
Standard Deviation 9.53
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Fatigue
42.80 T-score
Standard Deviation 4.60
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Upper Extremity Function
48.97 T-score
Standard Deviation 8.37
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Upper Extremity Function
53.80 T-score
Standard Deviation 0.00
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Lower Extremity Function
58.60 T-score
Standard Deviation 0.00
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Lower Extremity Function
58.60 T-score
Standard Deviation 0.00
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Cognitive Function
47.03 T-score
Standard Deviation 15.52
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Cognitive Function
46.73 T-score
Standard Deviation 5.01
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Emotional and Behavioral Dyscontrol
51.10 T-score
Standard Deviation 14.97
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Emotional and Behavioral Dyscontrol
41.37 T-score
Standard Deviation 9.98
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Well-being
54.30 T-score
Standard Deviation 4.91
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Well-being
55.70 T-score
Standard Deviation 6.59
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Sleep Disturbances
47.03 T-score
Standard Deviation 7.49
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Sleep Disturbances
43.93 T-score
Standard Deviation 4.90
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Participation in Social Activities
53.00 T-score
Standard Deviation 7.41
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Participation in Social Activities
51.03 T-score
Standard Deviation 8.01
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Satisfaction in Social Activities
47.70 T-score
Standard Deviation 3.28
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Satisfaction in Social Activities
49.23 T-score
Standard Deviation 2.54
Adult Neurological Quality of Life (Neuro-QoL)
Baseline Mean Adult Score for Stigma
53.90 T-score
Standard Deviation 8.80
Adult Neurological Quality of Life (Neuro-QoL)
Follow-Up Mean Adult Score for Stigma
56.47 T-score
Standard Deviation 6.25

Adverse Events

Cannabidiol/ Epidiolex

Serious events: 0 serious events
Other events: 7 other events
Deaths: 0 deaths

Serious adverse events

Adverse event data not reported

Other adverse events

Other adverse events
Measure
Cannabidiol/ Epidiolex
n=10 participants at risk
All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome. Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
Skin and subcutaneous tissue disorders
Darkening of port-wine birthmark
10.0%
1/10 • Number of events 1 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Metabolism and nutrition disorders
Decreased appetite
10.0%
1/10 • Number of events 3 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Gastrointestinal disorders
Diarrhea
20.0%
2/10 • Number of events 2 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Ear and labyrinth disorders
Dizziness
20.0%
2/10 • Number of events 3 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Metabolism and nutrition disorders
Fatigue
20.0%
2/10 • Number of events 2 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Nervous system disorders
Headache
20.0%
2/10 • Number of events 3 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Hepatobiliary disorders
Increased alanine aminotransferase
20.0%
2/10 • Number of events 2 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Hepatobiliary disorders
Increased aspartate aminotransferase
20.0%
2/10 • Number of events 2 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Psychiatric disorders
Irritable mood
10.0%
1/10 • Number of events 1 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Psychiatric disorders
Jitteriness
10.0%
1/10 • Number of events 1 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Skin and subcutaneous tissue disorders
Mouth sores
10.0%
1/10 • Number of events 1 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.
Gastrointestinal disorders
Nausea
20.0%
2/10 • Number of events 3 • 6 months
Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects. Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.

Additional Information

Dr. Anne Comi

Kenndey Krieger Institute

Phone: 443-923-9127

Results disclosure agreements

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