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Correlation Between the SARA and A-T NEST Clinical Severity Scores in Adults with Ataxia-Telangiectasia.

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Major, Toby 
Tiet, May Yung 
Horvath, Rita 
Hensiek, Anke E 


Ataxia-Telangiectasia (A-T) is an autosomal recessive neurodegenerative disease associated with cerebellar ataxia and extrapyramidal features. A-T has a complex and diverse phenotype with varying rates of disease progression. The development of robust natural history studies and therapeutic trials relies on the accurate recording of phenotype using relevant and validated severity of illness indexes. We compared the commonly used Scale for the Assessment and Rating of Ataxia (SARA) and the disease-specific A-T Neurological Examination Scale Toolkit (A-T NEST), in our adult A-T cohort. We found a strong correlation between A-T NEST and the established SARA score, validating the use of A-T NEST and SARA in capturing the natural history of A-T patients.


Acknowledgements: The authors would like to thank the patients for their kind cooperation in this study.


Ataxia telangiectasia, Cerebellar ataxia, Severity of illness index, Adult, Humans, Ataxia Telangiectasia, Neurodegenerative Diseases, Severity of Illness Index, Cerebellar Ataxia, Disease Progression

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Springer Science and Business Media LLC
Wellcome Trust (109915_A_15_Z)
MRC (MR/V009346/1)
National Institute for Health and Care Research (IS-BRC-1215-20014)
M.Y.T. is supported by the Addenbrookes Charitable Trust (G103290) and jointly by Action for AT, BrAshAT and AT society (grant TREAT-AT). The funders have no role in the study design, data collection and decision to submit this manuscript. R.H. is a Wellcome Trust Investigator (109915/Z/15/Z), who received support from the Medical Research Council (UK) (MR/N025431/1 and MR/V009346/1), the European Research Council (309548), the Newton Fund (UK/Turkey, MR/N027302/1), the Addenbrookes Charitable Trust (G100142), the Evelyn Trust, the Stoneygate Trust, the Lily Foundation, Action for AT and an MRC strategic award to establish an International Centre for Genomic Medicine in Neuromuscular Diseases (ICGNMD) MR/S005021/1. RH is part of the PROSPAX consortium under the frame of EJP RD, the European Joint Programme on Rare Diseases, under the EJP RD COFUND-EJP N°825575. This research was supported by the NIHR Cambridge Biomedical Research Centre (BRC-1215-20014). The views expressed are those of the authors and not necessarily those of the NIHR or the Department of Health and Social Care.