Ataxia is a neurological disorder affecting coordination, caused by damage to the cerebellum, brainstem, or related pathways. It can be hereditary (e.g., Friedreich's ataxia) or acquired (e.g., multiple sclerosis, stroke). Though rare, ataxia significantly impacts quality of life and independence. Treatments are limited and mainly focus on multidisciplinary rehabilitation. Accurate assessment is essential, yet current tools like Scale for the Assessment and Rating of Ataxia (SARA) have limitations. This study aims to validate a new scale, named the Ataxia Trunk, Lower And upper extremity Scale (ATLAS), through Rasch analysis, to develop a shorter, reliable version. It will assess internal consistency, construct validity, and inter-rater reliability.
For the valitdity part, statistics will be used (1) to see if the different items of the scale are indeed different and complementary to each other, and (2) to compare the results of this scale with other scales already known and valid (SARA, Trunk Impairment Scale (TIS) and Functional Impairment Measurement(FIM)). Secondly, the investigators would like to know whether ATLAS is reliable. In this particular case, the reliability being assessed is inter-rater reliability, i.e. whether all raters give the same score on the items performed by the patient. To carry out such a study, 64 people will be needed to achieve these goals. Each person will complete the 20 items of the ATLAS scale, those of a trunk motor capacity assessment (TIS), and will evaluate his or her functional independence (FIM).
Be adults
Be able to provide written informed consent
Be able to understand and follow simple physical instructions
Have a diagnosis of genetic or hereditary ataxia, or a cerebellar or pontine stroke (either hemorrhagic or ischemic), confirmed by a neurologist
Muscle group strength < 3 on the Medical Research Council (MRC) scale
Spasticity > 2 in any muscle on the Modified Ashworth Scale
Pain > 5 on the Visual Analogue Scale (VAS) in any part of the body
The target population of the study consists of individuals with motor impairments of cerebellar origin. These individuals are expected to exhibit typical symptoms of cerebellar dysfunction and, as such, will be found among those with hereditary or genetic ataxia, as well as individuals who have experienced a pontine or cerebellar stroke.
Other
Cross-sectional
New Scale to evaluate ataxia motor symptoms
Other interventionThe Ataxia Trunk, Lower And upper extremity Scale (ATLAS) is a new scale. It differs from other Ataxia Scale by focusing on the assessment of coordination abilities of specific body segments: the trunk, lower limbs, and upper limbs.
Ataxia Trunk, Lower And upper extremity Scale scores (ATLAS)
The Ataxia Trunk, Lower And upper extremity Scale (ATLAS) includes 20 items: 14 related to the trunk, 3 to the lower limbs (LL), and 3 to the upper limbs (UL). Each item is rated using an ordinal scale ranging from 2 to 4. A maximum total score of 64 indicates severe ataxia, while a score of 0 reflects the absence of ataxia. The maximum subscore for the trunk is 28, for the lower limbs 18, and for the upper limbs 18, with each side assessed separately.
Functional Independence Measure (FIM)
The Functional Independence Measure (FIM) assesses six functional domains using 18 items: Self-care, Sphincter control, Mobility/Transfers, Locomotion, Communication, and Social cognition. These items are grouped into two domains: Motor (13 items) and Cognitive (5 items). Each item is rated on a 7-point Likert scale, where 1 indicates complete dependence requiring total assistance, and 7 indicates complete independence. Assessment can be conducted through interview or direct observation of performance. The total score ranges from 18 to 126 points, with 18 representing complete dependence and 126 representing full independence. Subscores for the Motor and Cognitive domains can also be calculated.
Trunk Impairment Scale 2.0 (TIS 2.0)
Trunk Impairment Scale 2.0 is a shortened form of the Trunk Impairment Scale. The scale consists of 7 tests, each rated using an ordinal scale with 2 or 3 levels (0 to 2), based on observational criteria. It is divided into two categories: Dynamic Sitting Balance (10 points) and Coordination (6 points). Higher scores indicate better functional abilities.
Scale for the Assessment and Rating of Ataxia (SARA)
The Scale for the Assessment and Rating of Ataxia includes 8 items assessing different aspects of ataxia, such as gait, balance, upper and lower limb coordination, and speech articulation. Each item is rated on an ordinal scale, resulting in a total score ranging from 0 (no ataxia) to 40 (severe ataxia).
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