A Scale for the Assessment and Rating of Ataxia

Česká verzia

Authors: J. Schwabová ¹; F. Zahálka ²; V. Komárek ¹; T. Malý ²; P. Hráský ²; T. Gryc ²; A. Zumrová ¹
Authors place of work: Klinika dětské neurologie UK 2. LF a FN v Motole, Praha ¹; Laboratoř sportovní motoriky FTVS UK v Praze ²
Published in the journal: Cesk Slov Neurol N 2010; 73/106(6): 689-693
Category: Original Paper

Summary

The goal of this study was to test the validity of the Scale for Assessment and Rating of Ataxia (SARA), published in 2006, using patients with autosomal dominant spino-cereberal ataxia (AD SCA) and Friedreich’s ataxia (FRDA). The data obtained was compared with that acquired from the International Cooperative Ataxia Rating Scale (ICARS) and correlated with selected posturographic parameters (PP). A total of 30 patients – 17 AD SCA and 13 FRDA – were examined. Time-monitored ICARS and SARA scales were employed. Posturographic examination on a tensometric platform followed, as per standard protocol. The results were statistically processed with tests for non-parametric correlation (Kendall’s tau-b). ICARS and SARA exhibited high correlation in both groups and with AD SCA (p <0.001); mid-level correlation was evident with FRDA (p <0.05); ICARS with PP and standing and walking – part of ICARS – correlated closely with PP in both groups (p <0.01); SARA correlated at mid-level with PP in both groups and with AD SCA (p <0.05). FRDA patients did not correlate. The validity of the SARA scale, including its lower time demands, was demonstrated to be in agreement with the literature. Correlations between the scales and selected PP were established, as well as the possibility of using posturography when reviewing the validity of these scales with ataxia patients. Surprisingly, the results of the SARA scale did not correlate with PP in FRDA (p >0.05) patients and the correlation between ICARS and SARA in FRDA patients was lower (τb = 0.545; p <0.05). The reason for this may lie in the lower number of FRDA patients tested, but SARA may just be less sensitive to the more distinctive symptoms in the dorsal column.

Keywords:
scale for the assessment and rating of ataxia – International Cooperative Ataxia Rating Scale – autosomal dominant spinocerebelar ataxia – ataxia – Friedreich’s ataxia – posturography

Zdroje

1. Storey E, Tuck K, Hester R, Hughes A, Churchyard A. Inter-rater reliability of the International Cooperative Ataxia Rating Scale (ICARS). Mov Disord 2004; 19(2): 190–192.

2. Cano SJ, Hobart JC, Hart PE, Korlipara LV, Schapira AH, Cooper JM. International Cooperative Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich’s ataxia? Mov Disord 2005; 20(12): 1585–1591.

3. Fahey MC, Corben L, Collins V, Churchyard AJ, Delatycki MB. How is disease progress in Friedreich’s ataxia best measured? A study of four rating scales. J Neurol Neurosurg Psychiatry 2007; 78(4): 411–413.

4. Pulst SM. Ataxia rating scales in the balance. Nat Clin Pract Neurol 2007; 3(3): 119.

5. Schmitz-Hubsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R et al. Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients. Mov Disord 2006; 21(5): 699–704.

6. Manto M, Marmolino D. Cerebellar ataxias. Curr Opin Neurol 2009; 22(4): 419–429.

7. Grewal RP, Tayag E, Figueroa KP, Zu L, Durazo A, Nunez C et al. Clinical and genetic analysis of a distinct autosomal dominant spinocerebellar ataxia. Neurology 1998; 51(5): 1423–1426.

8. Pandolfo M, Pastore A. The pathogenesis of Friedreich ataxia and the structure and function of frataxin. J Neurol 2009; 256 (Suppl 1): 9–17.

9. Trouillas P, Takayanagi T, Hallett M, Currier RD, Subramony SH, Wessel K et al. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci 1997; 145(2): 205–211.

10. Yabe I, Matsushima M, Soma H, Basri R, Sasaki H. Usefulness of the Scale for Assessment and Rating of Ataxia (SARA). J Neurol Sci 2008; 266(1–2): 164–166.

11. Schmitz-Hubsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C et al. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006; 66(11): 1717–1720.

12. Weyer A, Abele M, Schmitz-Hubsch T, Schoch B, Frings M, Timmann D et al. Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord 2007; 22(11): 1633–1637.

13. Yabe I, Sasaki H, Yamashita I, Takei A, Tashiro K. Clinical trial of acetazolamide in SCA6, with assessment using the Ataxia Rating Scale and body stabilometry. Acta Neurol Scand 2001; 104(1): 44–47.

14. Manabe Y, Honda E, Shiro Y, Sakai K, Kohira I, Kashihara K et al. Fractal dimension analysis of static stabilometry in Parkinson’s disease and spinocerebellar ataxia. Neurol Res 2001; 23(4): 397–404.

15. Mauritz KH, Dichgans J, Hufschmidt A. Quantitative analysis of stance in late cortical cerebellar atrophy of the anterior lobe and other forms of cerebellar ataxia. Brain 1979; 102(3): 461–482.

16. Diener HC, Dichgans J. Pathophysiology of cerebellar ataxia. Mov Disord 1992; 7(2): 95–109.

17. Diener HC, Dichgans J, Bacher M, Guschlbauer B. Characteristic alterations of long-loop “reflexes” in patients with Friedreich‘s disease and late atrophy of the cerebellar anterior lobe. J Neurol Neurosurg Psychiatry 1984; 47(7): 679–685.

18. Horak FB, Diener HC. Cerebellar control of postural scaling and central set in stance. J Neurophysiol 1994; 72(2): 479–493.

19. Timmann D, Horak FB. Prediction and set-dependent scaling of early postural responses in cerebellar patients. Brain 1997; 120(Pt 2): 327–337.

20. Bares M, Lungu OV, Husárova I, Gescheidt T. Predictive motor timing performance dissociates between early diseases of the cerebellum and Parkinson‘s disease. Cerebellum 2010; 9(1): 124–135.

21. Bares M, Lungu O, Liu T, Waechter T, Gomez CM, Ashe J. Impaired predictive motor timing in patients with cerebellar disorders. Exp Brain Res 2007; 180(2): 355–365.

22. Ivry RB, Spencer RM, Zelaznik HN, Diedrichsen J. The cerebellum and event timing. Ann N Y Acad Sci 2002; 978: 302–317.

23. O’Reilly JX, Mesulam MM, Nobre AC. The cerebellum predicts the timing of perceptual events. J Neurosci 2008; 28(9): 2252–2260.

24. Buhusi CV, Meck WH. What makes us tick? Functional and neural mechanisms of interval timing. Nat Rev Neurosci 2005; 6(10): 755–765.

25. Kapteyn TS, Bles W, Njiokiktjien CJ, Kodde L, Massen CH, Mol JM. Standardization in platform stabilometry being a part of posturography. Agressologie 1983; 24(7): 321–326.