Abstract
In the present study, the progression of gait impairment in a group of patients with primary degenerative cerebellar ataxias was observed over a period of 4 years. A total of 30 patients underwent an initial gait analysis study, and thereafter only 12 were evaluated because they completed the 2- and 4-year follow-up evaluations. Time–distance parameters, trunk and joint range of motion (RoM), and variability parameters (e.g., coefficients of variation) were measured at the baseline and at each follow-up evaluation. The scale for the assessment and rating of ataxia (SARA) was used to evaluate disease severity. We found a significant increase in the SARA score at both the 2- and 4-year follow-up evaluations. Almost all the gait variables changed significantly only at the 4-year follow-up. Particularly, we found a significant decrease in the step length and in the hip, knee, and ankle joint RoM values and noted a significant increase in the trunk rotation RoM and stride-to-stride and step length variability. Furthermore, a significant difference in ankle joint RoM was found between spinocerebellar ataxia and sporadic adult-onset ataxia patients, with the value being lower in the former group of patients. Our findings suggest that patients with degenerative cerebellar ataxias exhibit gait decline after 4 years from the baseline. Moreover, patients try to maintain an effective gait by adopting different compensatory mechanisms during the course of the disease in spite of disease progression.




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References
Holmes G. The cerebellum of man. Brain. 1939;62:1–30.
Manto M. The cerebellum, cerebellar disorders, and cerebellar research-two centuries of discoveries. Cerebellum. 2008;7(4):505–16.
Fonteyn EM, Schmitz-Hubsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, et al. Prospective analysis of falls in dominant ataxias. Eur Neurol. 2013;69:53–7.
Schniepp R, Wuehr M, Schlick C, Huth S, Pradhan C, Dieterich M, et al. Increased gait variability is associated with the history of falls in patients with cerebellar ataxia. J Neurol. 2014;261:213–23.
Serrao M, Pierelli F, Ranavolo A, Draicchio F, Conte C, Don R, et al. Gait pattern in inherited cerebellar ataxias. Cerebellum. 2012;11:194–211.
Mari S, Serrao M, Casali C, Conte C, Ranavolo A, Padua L, et al. Turning strategies in patients with cerebellar ataxia. Exp Brain Res. 2012;222(1–2):65–75.
Conte C, Pierelli F, Casali C, Ranavolo A, Draicchio F, Martino G, et al. Upper body kinematics in patients with cerebellar ataxia. Cerebellum. 2014;13(6):689–97.
Martino G, Ivanenko YP, Serrao M, Ranavolo A, d’Avella A, Draicchio F, et al. Locomotor patterns in cerebellar ataxia. J Neurophysiol. 2014;112:2810–21.
Chini G, Ranavolo A, Draicchio F, Casali C, Conte C, Martino G, et al. Local stability of the trunk in patients with degenerative cerebellar ataxia during walking. Cerebellum. 2016; doi:10.1007/s12311-016-0760-6.
Bodranghien F, Bastian A, Casali C, Hallett M, Louis ED, Manto M, et al. Consensus paper: revisiting the symptoms and signs of cerebellar syndrome. Cerebellum. 2015;15 (3):369–91.
Pulst SM. Spinocerebellar ataxia type 2. 1998 Oct 23 [updated 2015 Nov 12]. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH, Stephens K, editors. Seattle (WA): University of Washington, Seattle; 1993–2016.
Schmitz-Hübsch 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;66:1717–20.
Davis RB, Ounpuu S, Tyburski D, Gage JR. A gait analysis data collection and reduction technique. Hum Mov Sci. 1991;10:575–87.
Wu G, Siegler S, Allard P, Kirtley C, Leardini A, Rosenbaum D, et al. ISB recommendation on definitions of joint coordinate system of various joints for the reporting of human joint motion—part I: ankle, hip, and spine. Int Soc Biomech J Biomech. 2002;35:543–8.
Wu G, van der Helm FC, Veeger HE, Makhsous M, Van Roy P, Anglin C, et al. ISB recommendation on definitions of joint coordinate systems of various joints for the reporting of human joint motion—part II: shoulder, elbow, wrist and hand. J Biomech. 2005;38:981–92.
Winter DA. Biomechanics of human movement. New York: Wiley; 1979.
England SA, Granata KP. The influence of gait speed on local dynamic stability of walking. Gait Posture. 2007;25:172–8.
Lord S, Howe T, Greenland J, Simpson L, Rochester L. Gait variability in older adults: a structured review of testing protocol and clinimetric properties. Gait Posture. 2011;34(4):443–50.
Kang HG, Dingwell JB. Separating the effects of age and walking speed on gait variability. Gait Posture. 2008;27(4):572–7.
Crenshaw SJ, Royer TD, Richards JG, Hudson DJ. Gait variability in people with multiple sclerosis. Mult Scler. 2006;12(5):613–9.
Wright RL, Bevins JW, Pratt D, Sackley CM, Wing AM. Metronome cueing of walking reduces gait variability after a cerebellar stroke. Front Neurol. 2016;7:84.
Bender R, Lange S. Adjusting for multiple testing—when and how? J Clin Epidemiol. 2001;54(4):343–9.
Fisher RA. On the interpretation of χ 2 from contingency tables, and the calculation of P. J R Soc. 1922;85(1):87–94.
Fiher RA. Statistical methods for research workers. Oliver and Boyd. ISBN 0–05–002170-2.
Morton SM, Tseng YW, Zackowski KM, Daline JR, Bastian AJ. Longitudinal tracking of gait and balance impairments in cerebellar disease. Mov Disord. 2010;25(12):1944–52.
McAndrew Young PM, Dingwell JB. Voluntarily changing step length or step width affects dynamic stability of human walking. Gait Posture. 2012;35(3):472–7.
Schniepp R, Wuehr M, Neuhaeusser M, Kamenova M, Dimitriadis K, Klopstock T, et al. Locomotion speed determines gait variability in cerebellar ataxia and vestibular failure. MovDisord. 2012;27:125–31.
Wuehr M, Schniepp R, Schlick C, Huth S, Pradhan C, Dieterich M, et al. Sensory loss and walking speed related factors for gait alterations in patients with peripheral neuropathy. Gait Posture. 2013; doi:10.1016/j.gaitpost.2013.11.013.
Schniepp R, Schlick C, Pradhan C, Dieterich M, Brandt T, Jahn K, et al. The interrelationship between disease severity, dynamic stability, and falls in cerebellar ataxia. J Neurol. 2016;263:(7):1409–17.
Schwab A, Meijaard J. A review on bicycle dynamics and rider control. Veh Syst Dyn. 2013;51(7):1059–90.
Cain SM, Ashton-Miller JA, Perkins NC. On the skill of balancing while riding a bicycle. PLoS One. 2016;11(2):e0149340.
Saunders JB, Inman VT, Eberhart HD. The major determinants in normal and pathological gait. J Bone Joint Surg Am. 1953;35-A:543–58.
Gracovetsky S, Gracovetsky S. An hypothesis for the role of the spine in human locomotion: a challenge to current thinking. J Biomed Eng. 1985;7(3):205–16.
Van Emmerik REA, McDermott WJ, Haddad JM, Van Wegen EEH. Age-related changes in upper body adaptation to walking speed inhuman locomotion. Gait Posture. 2005;22:233–9.
Palliyath S, Hallett M, Thomas SL, Lebiedowska MK. Gait in patients with cerebellar ataxia. Mov Disord. 1998;13:958–64.
Mitoma H, Hayashi R, Yanagisawa N, Tsukagoshi H. Characteristics of parkinsonian and ataxic gaits: a study using surface electromyograms, angular displacements and floor reaction forces. J Neurol Sci. 2000;174:22–39.
Stolze H, Klebe S, Petersen G, Raethjen J, Wenzelburger R, Witt K, et al. Typical features of cerebellar ataxic gait. J Neurol Neurosurg Psychiatry. 2002;73:310–2.
Morton SM, Bastian AJ. Relative contributions of balance and voluntary leg-coordination deficits to cerebellar gait ataxia. J Neurophysiol. 2003;89(4):1844–56.
Mari S, Serrao M, Casali C, Conte C, Martino G, Ranavolo A, et al. Lower limb antagonist muscle co-activation and its relationship with gait parameters in cerebellar ataxia. Cerebellum. 2014;13:226–36.
Hausdorff JM. Stride variability: beyond length and frequency. Gait Posture. 2004;20(3):304. author reply 305
Jacobi H, Bauer P, Giunti P, Labrum R, Sweeney MG, Charles P, et al. The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study. Neurology. 2011;77(11):1035–41.
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Serrao, M., Chini, G., Casali, C. et al. Progression of Gait Ataxia in Patients with Degenerative Cerebellar Disorders: a 4-Year Follow-Up Study. Cerebellum 16, 629–637 (2017). https://doi.org/10.1007/s12311-016-0837-2
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DOI: https://doi.org/10.1007/s12311-016-0837-2