# Exploring the Potential of Scales to Assess Different Types of Ataxia: Meta-review

**Authors:** Sonia Racero-Ríos, Rita-Pilar Romero-Galisteo, Manuel González-Sánchez

PMC · DOI: 10.1007/s12311-026-01969-5 · Cerebellum (London, England) · 2026-03-13

## TL;DR

This study reviews and compares tools used to assess different types of ataxia, highlighting their strengths and limitations in measuring symptoms and disease severity.

## Contribution

The paper provides a comprehensive meta-review of assessment scales for ataxia, identifying the most widely used and effective tools.

## Key findings

- Scales like ICARS and SARA are the most widely used for assessing ataxia severity.
- Internal consistency and reliability of the scales are generally good to excellent.
- Validity and sensitivity of the scales show less consistent results.

## Abstract

Ataxia is a coordination disorder that encompasses more than 300 neurological diseases and more than 200 types of hereditary ataxia. Depending on the pathology with which it is associated, the symptomatology present is different: balance and gait disturbance, dysdiadochokinesia, dysarthria, nystagmus, etc. Due to this heterogeneity, the assessment of ataxia is a complex task that is further hindered by the broad variety of existing tools, both specific to ataxia and related to a particular pathology, such as those that assess coordination and gait components. Analyse, organize and compare the structural and psychometric characteristics of the tools used for the assessment of different types of ataxia. A meta-review registered in PROSPERO was conducted following the PRISMA Checklist System. Six database were used and two blinded researchers selected the papers according the criteria. Five reviews were included in this study. The main psychometric properties of the 16 scales found to assess ataxia symptoms in different pathologies were analysed. Internal consistency and reliability obtained good-to-excellent values for the total score of the different scales and parameters studied. However, validity and sensitivity showed less homogeneous values. The scales that globally assess the severity of the disease stand out, with a great variety of scales, including the International Cooperative Ataxia Assessment Scale (ICARS) and the Scale for the Assessment and Rating of Ataxias (SARA) as the most widely used.

## Linked entities

- **Diseases:** ataxia (MONDO:0000437)

## Full-text entities

- **Genes:** ATXN3 (ataxin 3) [NCBI Gene 4287] {aka AT3, ATX3, JOS, MJD, MJD1, SCA3}, CTBP1 (C-terminal binding protein 1) [NCBI Gene 1487] {aka BARS, HADDTS}, SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}, ABCB6 (ATP binding cassette subfamily B member 6 (LAN blood group)) [NCBI Gene 10058] {aka ABC, LAN, MTABC3, PRP, umat}, BBS2 (Bardet-Biedl syndrome 2) [NCBI Gene 583] {aka BBS, RP74}, FXN (frataxin) [NCBI Gene 2395] {aka CyaY, FA, FARR, FRDA, X25}, ATXN2 (ataxin 2) [NCBI Gene 6311] {aka ATX2, SCA2, TNRC13}
- **Diseases:** cerebellar vascular accident (MESH:D020521), SCA (MESH:C565772), sensory neuropathy (MESH:D009477), sensory impairment (MESH:D012678), Friedreich's (MESH:D005621), Cerebellar Cortical Atrophy (MESH:D002526), NPC (MESH:D052556), loss of reflexes (MESH:C000721448), OPCA (MESH:D020754), appendicular ataxia (MESH:D001259), musculoskeletal decline (MESH:D009140), nystagmus (MESH:D009759), Sporadic Adult Onset Ataxia (MESH:C564020), infections (MESH:D007239), balance impairment (MESH:D060825), proprioceptive impairment (MESH:D020886), ALS (MESH:D008113), alcohol intoxication (MESH:D000435), cerebral palsy (MESH:D002547), recessive diseases (MESH:D004194), autosomal recessive spastic ataxia (OMIM:613672), Dentatorubral-Pallidoluysian Atrophy (MESH:D020191), cerebellar degeneration (MESH:D013132), PMM2 (MESH:C535739), falls (MESH:C537863), ataxic symptoms (MESH:D012816), Olivopontocerebellar Atrophy (MESH:D009849), MS (MESH:D009103), balance and gait disturbance (MESH:D020233), MSA-c (MESH:C537381), visual and speech impairment (MESH:D014786), CCA (MESH:C536211), oculomotor dysfunction (MESH:D015840), paralysis (MESH:D010243), Hauser Deambulation (MESH:C537371), DI (MESH:C564703), movement impairment (MESH:D009069), abnormal saccades (MESH:C537423), immunological diseases (MESH:D007154), ataxia telangiectasia (MESH:D001260), deficit in anteroposterior balance (MESH:D009461), Machado-Joseph Disease (MESH:D017827), central coordination deficits (MESH:D019957), MDC (MESH:D009402), SRM (MESH:D018746), Multiple System Atrophy (MESH:D019578), Parkinson's disease (MESH:D010300), psychiatric disorders (MESH:D001523), frailty (MESH:D000073496), Autosomal Recessive Ataxia (MESH:C565188), Cerebellar Ataxia (MESH:D002524), neurological diseases (MESH:D020271), gait instability (MESH:D043171), genetic diseases (MESH:D030342), balance disturbance (MESH:D014832), intention tremor (MESH:D014202), Ataxic gait (MESH:D020234), dysarthria (MESH:D004401), Ataxia with Oculomotor Ataxia Type 2 (MESH:C537308)
- **Chemicals:** 25FWT (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## References

5 references — full list in the complete paper: https://tomesphere.com/paper/PMC12987783/full.md

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Source: https://tomesphere.com/paper/PMC12987783