신경 및 대사 질환 환자의 움직임을 2년간 웨어러블 기기로 관찰하여 미래 임상시험을 위한 유용한 데이터 수집을 목표로 합니다.

Syde is an innovative device intended to be used in a home-based environment. It is composed of two watch-like sensors, each containing a magneto-inertial sensors that record the linear acceleration, the angular velocity, the magnetic field of the movement in all directions.The two watches can be worn as wristwatch or placed near the ankle.

Syde

This test consists of covering the greatest possible distance within 6 minutes, walking back and forth along a 25-meter hallway. The subject may stop if they need to. The evaluator will record the elapsed time after each half-lap, and the final distance that was covered at the end of the test. No help will be allowed during the test.

The 6-minute walk test (6MWT)

During this test, the subject must cover a distance of 10 meters as rapidly as possible. This test will be performed 3 times. The participant will be allowed to rest for one minute after the first and second instances of the test. No help will be allowed during the test.

The 10-meter test (10MWT)

This is a standardized test during which the subject must climb the 4 stair steps as rapidly as possible. This test will be performed 3 times. The participant will be allowed to rest for one minute after the first and second instances of the test, or more if needed.

The 4 stair-climbing test (4SC)

This is a standardized test during which the subject must rise from the floor as rapidly as possible. The test starts with the participant lying on their back. This test will be performed 3 times. The participant will be allowed to rest for one minute after the first and second instances of the test.

Test of rising from the floor (TRF)

PGIC is a patient-reported scale used to assess how a patient feels their condition has changed over time. It typically uses a 7-point scale to capture the patient's overall perception of improvement in symptoms, function, and quality of life.

Patient's Global Impression of Change (PGIC)

Dynamometric measurements of the maximum force of the following functions will be taken with the MyoTools: palmar grip (MyoGrip) and thumb-index pinch (MyoPinch). Test will be realized on the dominant side. Patients will be encouraged during the test. They will be given three trials and the best score will be entered.

Dynamometric measurements of muscle strength

The CMTES is a 28-point composite score that rates the patient's symptoms and signs. It is part of the Charcot-Marie-Tooth (CMT) neuropathy score and has been validated as a reproducible measure of disability in CMT. Higher scores indicate more severe neuropathy.

The Charcot-Marie-Tooth examination score (CMTES)

The 9-HPT is a quantitative test of upper limb function. It is the second component of the Multiple Sclerosis Functional Composite (MSFC). The patient sits at a table with a shallow container containing nine sticks and a wooden or plastic block containing nine empty holes. When a timer is started, the patient places the nine sticks one after the other as quickly as possible into the nine holes and, once they are all in the holes, removes them as quickly as possible. The total time taken to complete the task is recorded. Two consecutive attempts with the dominant hand are immediately followed by two consecutive attempts with the non-dominant hand.

9-Hole Peg Test (9-HPT)

This 8-item scale was developed to assess ataxia severity and disease progression. It evaluates gait and balance, speech, and upper and lower limb coordination. The score ranges from 0 to 40, with higher score indicating a worse clinical outcome.

The Scale for the Assessment and Rating of Ataxia (SARA)

This 74-item clinical rating scale assesses patient performance and capacity in 4 domains: motor function, cognitive function, behavioral abnormalities, and functional capacity.

The Unified Huntington's Disease Rating Scale (UHDRS)

During this standardized test, the subject is observed and timed while he/she rises from a chair, walks 3 meters, performs a 180° turn, walk back toward the chair and sits down.

The Timed up and go test (TUG)

This 28-item clinician-rated scale assesses motor and non-motor symptom severity in patients with PSP, with a score ranging from 0 (normal) to 100.

The Progressive Supranuclear Palsy Rating Scale (PSP-RS)

The MOCA was designed as a rapid screening instrument for mild cognitive dysfunction. It assesses different cognitive domains: attention and concentration, executive function, memory, language, visuoconstructional skills, conceptual thinking, calculations, and orientation. Scores on the MOCA range from 0 to 30, with higher score being better outcome.

The Montreal Cognitive Assessment (MOCA)

The QMG is a clinician-rated 13-item scale used to quantify disease severity in patients with MG. The scale grades each domain (ocular, bulbar, respiratory, and limb function), with a total score ranging from 0 to 39 (lower score indicating a better clinical outcome).

Quantitative Myasthenia Gravis (QMG)

This is an 8-item patient-reported scale assessing MG symptoms and their effects on daily activities. The score ranges from 0 to 24, with higher score indicating a worse clinical outcome.

The Myasthenia Gravis Activities of Daily Living Scale (MG-ADL)

6분 걷기 테스트(6MWT)

10m 테스트(10MWT)

4계단 오르기 테스트(4SC)

바닥에서 일어나는 테스트(TRF)

환자의 변화에 대한 전반적인 인상(PGIC)

근력의 동력학적 측정

샤르코-마리-투스 시험 점수(CMTES)

9홀 페그 테스트(9-HPT)

운동실조 평가 및 등급 척도(SARA)

통합 헌팅턴병 평가 척도(UHDRS)

시간 제한 테스트(TUG)

진행성 초핵성 마비 평가 척도(PSP-RS)

몬트리올 인지 평가(MOCA)

정량적 중증 근무력증(QMG)

중증 근무력증 일상생활활동척도(MG-ADL)

Centre Hospitalier Universitaire de Liege

Nicolas Bovy

Laurent Servais, MD

신경학적 또는 대사적 병리를 가진 성인 환자의 보행 분석 매개변수 및 상지 평가

Eligible participants that enroll will received 16 week parent based intervention for insistence on sameness

This study will examine the potential effectiveness of a virtually delivered parent-mediated intervention for insistence on sameness behaviors in autism. This includes a 16 week protocol where caregivers meet weekly for one hour with a study therapist.

신경학적 또는 대사적 병리를 가진 성인 환자의 보행 분석 매개변수 및 상지 평가

불안한 아동기 감정에 대한 지지적 양육(SPACE)과 동일성에 대한 집착(IS)