A Single Center Pilot Study to Assess the Intra-observer Reliability of Measuring Muscle Strength Using a Hand Held Dynamometer in Children and Adults With Neurofibromatosis Type 1 (NF1) and Type 2 (NF2)

NCT ID: NCT03672838

Last Updated: 2021-03-18

Basic Information

• Recruitment Status: COMPLETED
• Clinical Phase: NA
• Total Enrollment: 33 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2018-09-24
• Study Completion Date: 2019-12-02

Brief Summary

Patients with clinically confirmed neurofibromatosis type 1 (NF1) or neurofibromatosis type 2 (NF2) or a known neurofibromatosis (NF) mutation aged 5 years and above will be eligible to participate and will be recruited from the neurofibromatosis clinic. Given the need for identifying measures that can reliably and sensitively measure focal muscle weakness and allow for measuring muscle strength as a functional outcome in therapeutic clinical trials in NF, this pilot study will assess the reliability of measuring muscle strength in NF1 and NF2 using a hand-held dynamometer.

Conditions

Neurofibromatosis 1 and 2 (NF1 and NF2)

Study Design

• Allocation Method: NON_RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: OTHER
• Blinding Strategy: NONE

Study Groups

Cohort 1

Patients with NF1

Group Type: ACTIVE_COMPARATOR

hand-held dynamometer

Intervention Type: BEHAVIORAL

Strength of one weak muscle group and of the biceps of the dominant arm will be measured using a hand-held dynamometer. An average of three readings will be used as one observation. Three observations per patient will be obtained on one day with a minimum of 15 minute rest period between each observation.

Cohort 2

Patients with NF2

Group Type: ACTIVE_COMPARATOR

hand-held dynamometer

Intervention Type: BEHAVIORAL

Strength of one weak muscle group and of the biceps of the dominant arm will be measured using a hand-held dynamometer. An average of three readings will be used as one observation. Three observations per patient will be obtained on one day with a minimum of 15 minute rest period between each observation.

Interventions

hand-held dynamometer

Strength of one weak muscle group and of the biceps of the dominant arm will be measured using a hand-held dynamometer. An average of three readings will be used as one observation. Three observations per patient will be obtained on one day with a minimum of 15 minute rest period between each observation.

Intervention Type: BEHAVIORAL

Eligibility Criteria

Inclusion Criteria

• For cohort 1:

• Patients must have clinically confirmed NF1 using the NIH consensus criteria or a known NF1 mutation
• Ages 5 years and above
• < 5/5 strength on Manual Muscle Testing (MMT) testing in at least one muscle group tested on the day of enrollment
• Able to follow instructions and cooperate with exam to assess strength
• For Cohort 2

• Patients must have clinically (NIH or Manchester criteria) or genetically confirmed diagnosis of NF2
• Ages 5 years and above
• < 5/5 strength on MMT testing in at least one muscle group tested on the day of enrollment
• Able to follow instructions and cooperate with exam to assess strength

Exclusion Criteria

• No orthopedic procedure or other major surgery that could influence extremity strength in past 6 months
• No tibial dysplasia

• Minimum Eligible Age: 5 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

NYU Langone Health

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Kaleb H Yohay, MD

Role: PRINCIPAL_INVESTIGATOR

New York School of Medicine

Locations

New York School of Medicine

New York, New York, United States

Countries

United States

Other Identifiers

18-00831

Identifier Type: -

Identifier Source: org_study_id