High-Tech Rehabilitation Pathway for Chronic Adult Neuromuscular Diseases - Fit4MedRob-Chronic MND Project

The Fatigue Severity Scale questionnaire contains nine statements that rate the severity of the fatigue symptoms (from 1 to 7, where 1 means complete disagreement with the statement and 7 means complete agreement).

Timed Up and Go Test (TUG) is a simple test used to assess mobility, balance and locomotor performance. It uses the time that a person need to rise from a chair, walk three meters, turn around 180 degrees, walk back to the chair and sit down while turning 180 degrees. The shorter the time taken to perform the test, the better the patient's mobility.

The Two-Minute Walk Test (2MWT) is a simple and quick assessment used to measure a person's walking endurance and functional mobility. It is based on the measurement of the distance that a person can walk in two minutes. The individual is instructed to walk as far as possible in two minutes, typically along a marked course. Assistive devices can be used if needed but should be kept consistent across tests. The total distance walked in meters is recorded.

The SF-12 is a self-reported questionnaire designed to measure health-related quality of life. It is a shortened version of the SF-36, created to reduce the burden on respondents while still providing reliable and valid results. The SF-12 covers eight health domains: Physical functioning; Role-physical (limitations due to physical health problems); Bodily pain; General health; Vitality (energy and fatigue); Social functioning; Role-emotional (limitations due to emotional problems); Mental health (psychological distress and well-being). The survey produces two summary scores: Physical Component Summary (PCS) and Mental Component Summary (MCS). These scores are norm-based, with a mean of 50 and a standard deviation of 10. Scores above 50 indicate better-than-average health related quality of life, while scores below 50 suggest below-average health.

To assess the patient perception of the technology assisted rehabilitation using the Technology Assisted Rehabilitation Patient Perception Questionnaire (TARPP-Q). The TARPP-Q consists of 10 questions with multiple choice answers, totalling 29 items. The Questionnaire assesses patients' personal experiences and perceptions of technology assisted rehabilitation, including aspects like usability, positive attitude, hindrance perception, and distress.

The Charcot-Marie-Tooth Neuropathy Score (CMTNS) is a clinical scale used to assess the severity of Charcot-Marie-Tooth neuropathy. This scale is used both to monitor disease progression and to assess the effectiveness of potential experimental treatments. Consists of 9 items, grouped into 3 major categories: Patient-Reported Symptoms, Clinical Examination and Electrophysiological data. Each item is scored from 0 to 4, where 0 means no impairment and 4 very severe impairment, with a total score ranges from 0 to 36.

The Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R) is a scale that includes 12 questions, related to bulbar, motor and respiratory function. This scale is commonly used to evaluate the severity and progression of Amyotrophic Lateral Sclerosis. Each item can have a score from 0 to 4, with a maximum total score of 48, where a score of 0 would indicate no function while a score of 4 would indicate normal function.

Will be measured these biomarkers in order to assess the levels changes during the treatment. Serum level will be measured in nanogram/milliliter

Will be measured this biomarker in order to assess the levels changes during the treatment. Plasma level will be measured in picogram/milliliter.

Sarcopenia level will be measured through two different analysis. One of this analysis is the assessment of Appendicular Skeletal Muscle Mass (ASMM). ASMM is measured using bioelectrical impedance analysis (BIA) devices, which assess body composition by analyzing the electrical resistance of body tissues. A low ASMM is a key indicator of sarcopenia, the age-related loss of muscle mass. ASMM is measured in kilograms.

The other analysis used to assess the level of sarcopenia will be the measurement of Temporal Muscle Thickness (TMT) using ultrasound imaging. Craniofacial muscle mass has been identified as a reliable indicator for detecting sarcopenia, in addition to thoracolumbar skeletal muscle mass. Ultrasound imaging is performed on the temporal muscle to measure parameters such as muscle thickness (measurement of the musclular depth from the skin surface) and cross-sectional area (area of the muscle in a specific plane, indicating muscle mass).