Sensory ataxia rating scale: Development and validation of a functional scale for patients with sensory neuronopathies. Issue 3 (8th July 2019)

• Record Type: Journal Article
• Authors: Martinez, Alberto R. M.; Martins, Melina P.; Martins, Carlos R.; Faber, Ingrid; de Rezende, Thiago J. R.; Nucci, Anamarli; França, Marcondes C.
• Is Part Of: Journal of the peripheral nervous system. Volume 24:Issue 3(2019)
• Page Start: 242
• Page End: 246
• Publication Date: 2019-07-08
• Subjects: clinical scale -- ganglionopathy -- rating scale -- SEARS -- sensory ataxia -- sensory neuronopathy; Nervous system -- Periodicals; Nerves, Peripheral -- Diseases -- Periodicals; Peripheral Nervous System Diseases -- Periodicals; Peripheral Nervous System -- Periodicals; 612.81
• DOI: 10.1111/jns.12330
• Languages: English
• ISSNs: 1085-9489

Abstract

Abstract: Sensory neuronopathies (SN) result from dorsal root ganglia damage and manifest with a combination of sensory deficits and proprioceptive ataxia. Characterization of the natural history and development of therapeutic trials are hampered by the lack of clinical scales that capture the whole spectrum of SN‐related manifestations. We propose and validate a rating instrument for SN. Three experienced neuromuscular specialists developed items to rate SN. The resultant instrument was later validated by the assessment of the intra‐class correlation coefficient, for inter‐rater validity in 48 SN patients, and later in a smaller subset of 16 patients to assess its intra‐rater validity. Standardized Crombach's alpha and Oblimin rotation analysis were performed to verify internal consistency and items' relationship, respectively. Evaluation of Sensory Ataxia Rating Scale (SEARS)'s external validity was performed by comparison to: scale for the assessment and rating of ataxia (SARA), Beck balance scale (BBS), and INCAT sensory sum score (ISS). A 10‐item scale with an intra‐class correlation coefficient >0.95 for intra‐ and inter‐rating measurements with a good internal consistency (standardized Cronbach's alpha of 0.83) were observed. There was a normal distribution of the scores without a floor or ceiling effect. A moderate to good correlation between SEARS and SARA, BBS, and ISS was observed. SEARS is a reliable, easy‐to‐perform and consistent instrument to rate SN. Larger cohorts and multicenter studies are needed to validate its usefulness towards possible treatment trials.