Abstract

BackgroundMultiple system atrophy (MSA) is a rare progressive neurodegenerative disorder for which brief yet sensitive scale is required in order for use in clinical trials and general screening. We previously compared several scales for the assessment of MSA symptoms and devised an eight-item pilot scale with large standardized response mean [handwriting, finger taps, transfers, standing with feet together, turning trunk, turning 360°, gait, body sway]. The aim of the present study is to investigate the validity and reliability of a simple pilot scale for assessment of multiple system atrophy symptoms.MethodsThirty-two patients with MSA (15 male/17 female; 20 cerebellar subtype [MSA-C]/12 parkinsonian subtype [MSA-P]) were prospectively registered between January 1, 2014 and February 28, 2015. Patients were evaluated by two independent raters using the Unified MSA Rating Scale (UMSARS), Scale for Assessment and Rating of Ataxia (SARA), and the pilot scale. Correlations between UMSARS, SARA, pilot scale scores, intraclass correlation coefficients (ICCs), and Cronbach’s alpha coefficients were calculated.ResultsPilot scale scores significantly correlated with scores for UMSARS Parts I, II, and IV as well as with SARA scores. Intra-rater and inter-rater ICCs and Cronbach’s alpha coefficients remained high (> 0.94) for all measures.ConclusionThe results of the present study indicate the validity and reliability of the eight-item pilot scale, particularly for the assessment of symptoms in patients with early state multiple system atrophy.

Highlights

  • Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder for which brief yet sensitive scale is required in order for use in clinical trials and general screening

  • Our prior study revealed that the Unified MSA Rating Scale (UMSARS) Part II, Part IV

  • Further analyses revealed that our eight-item semi-quantitative pilot scale (Table 1) exhibited an SRM larger than those observed for the UMSARS Part II/Part IV, Scale for Assessment and Rating of Ataxia (SARA), and Berg Balance Scale (BBS) [4], suggesting that the pilot scale was most effective in detecting rapid changes in symptoms of MSA

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Summary

Introduction

Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder for which brief yet sensitive scale is required in order for use in clinical trials and general screening. We previously compared several scales for the assessment of MSA symptoms and devised an eight-item pilot scale with large standardized response mean [handwriting, finger taps, transfers, standing with feet together, turning trunk, turning 360°, gait, body sway]. We subsequently devised a simple pilot scale comprised of eight items representative of those exhibiting the largest standardized response means (handwriting, Matsushima et al Cerebellum & Ataxias (2017) 4:11 finger taps, transfers, standing with feet together, turning trunk, turning 360°, gait, and body sway) [4]. Further analyses revealed that our eight-item semi-quantitative (total score = 36 points) pilot scale (Table 1) exhibited an SRM larger than those observed for the UMSARS Part II/Part IV, SARA, and BBS [4], suggesting that the pilot scale was most effective in detecting rapid changes in symptoms of MSA.

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