Treadmill Training in Fragile X-associated Tremor/Ataxia Syndrome

A test (not a scale) to assess balance and postural sway. Subjects will be asked to stand still with their hands across their chests and their feet positioned a set distance apart (scaled to their height) with a wearable sensor that records movements applied to the lumbar trunk. This sensor will record parameters including mean total sway area, path length, jerk, and sway distance in the mediolateral and anteroposterior directions.

A test to examine fatigue over time and that will also allow more accurate calculation of gait variability indices by increasing the number of steps. The subject will walk 25 meters, turn and walk back, and repeat this until 2 minutes has elapsed.

This is a shortened version of the Balance Evaluation systems Test (BESTest). It aims to target and identify 6 different balance control systems and has been used as a performance-based balance measure in many interventions and clinical trials. 1) The original BESTest 4 level (0-3) scoring was revised to 3 levels (0-2) due to redundancy. 2) Total Score = 28 points per test directions. 3) Two items have right and left assessment in which the lower score is used within the total score (directions specify which to use). 4) For research, many studies specify use of both left and right data, thus calculating data based on 32 (vs 28 points).

A 16-item self-report questionnaire in which patients rate their balance confidence for performing activities. Items are rated on a rating scale that ranges from 0 - 100. Score of zero represents no confidence, a score of 100 represents complete confidence. Overall score is calculated by adding item scores and then dividing by the total number of items.

A screening instrument designed to help health professionals detect mild cognitive dysfunction. Subjects will answer questions to measure different areas of cognition including attention and concentration, executive functions, memory, language, visuo-constructional skills, conceptual thinking, calculations, and orientation. MoCA scores range between 0 and 30. A score of 26 or over is considered to be normal.

A test for evaluating information processing speed, and the subject uses a reference key to pair specific numbers with given abstract geometric figures in 90 seconds. Scores are the correct number of pairs made in 90 seconds, with greater scores indicating greater cognitive abilities.

A phonemic test for verbal fluency and verbal memory. The COWAT uses the three letter set of C, F, and L to assess phonemic fluency. Subjects will be given 1 min to name as many words as possible beginning with one of the letters.

A neuro-psychological test for attention and response inhibition the assess the ability to inhibit cognitive interference while processing a specific stimulus.

One of the oldest and most widely used test of short-term verbal and working memory. On each trial subjects will be presented with a series of digits appearing one at a time on a computer screen. Subjects will be tested in 2 variants; forward-span and backward-span. In the forward-span, the subject will be asked to recall the digits in the order they appeared. In the backward-span, subjects will be asked to recall the digits in the reverse order they appeared.

Neuro-QoL (Quality of Life in Neurological Disorders) is a measurement system that evaluates and monitors the physical, mental, and social effects experienced by adults and children living with neurological conditions. It is a self-report of health related quality of life in 17 domains and sub-domains for adults and 11 for children with neurological disorders. Raw scores are converted based on consistent metric (T-distribution) with data from the US general population with a t-score mean of 50 and standard deviation of 10. Adult Banks include 19-45 items. Items are scored on a 5-point scale that uses different language depending on assessment. the questions range from least (1) to most (5) based on frequency of behavior, amount of difficulty, or degree of agreement.

The CES-D is a brief self-report measure that assesses symptoms of depression in the general population. It is a 20-item, self-report measure designed to be used in the general population that assess current symptoms of depression (i.e. this week). The standard cut-off score suggesting depression >16.

The PSQI is an effective instrument used to measure the quality and patterns of sleep in adults. it differentiates "poor" from "good" sleep quality by measuring seven different areas over the last month; 19 self-reported items belongs to one of seven subcategories: subjective sleep quality, sleep latency, sleep duration, habitual sleep efficiency, sleep disturbances, use of sleeping medication, and daytime dysfunction. Five additional questions rated by the respondent's roommate or bed partner are included for clinical purposes and are not scored.

The Borg Rating of Perceived Exertion Scale measures physical activity intensity felt by the participant based on the physical sensations a participant experiences during physical activity, including increased heart rate, increased respiration or breathing rate, increased sweating, and muscle fatigue.It is a 20-point scale with verbal descriptors to standardize perceived exertion across tasks and individuals. a score of 0 = nothing at all to 10 = very hard.

The daily steps counts monitored by Fitbit wristband will be used as the measure of physical activity

The BAI is a self-report measure of anxiety that assesses anxiety symptoms and symptom severity. The BAI can discriminate anxiety from depression. 4-point Likert scale response for anxiety symptoms are severity within a week; the total score is calculated by calculating the sum of 21 items. Minimum score of 0 and a maximum score of 63. Score Interpretation: 0-7 = minimal anxiety; 8-15 = mild anxiety; 16-25 = moderate anxiety; 26-63 = severe anxiety