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Published in: Journal of Neurology 7/2016

01-07-2016 | Original Communication

The interrelationship between disease severity, dynamic stability, and falls in cerebellar ataxia

Authors: Roman Schniepp, Cornelia Schlick, Cauchy Pradhan, Marianne Dieterich, Thomas Brandt, Klaus Jahn, Max Wuehr

Published in: Journal of Neurology | Issue 7/2016

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Abstract

Cerebellar ataxia (CA) results in discoordination of body movements (ataxia), a gait disorder, and falls. All three aspects appear to be obviously interrelated; however, experimental evidence is sparse. This study systematically correlated the clinical rating of the severity of ataxia with dynamic stability measures and the fall frequency in patients with CA. Clinical severity of CA in patients with sporadic (n = 34) and hereditary (n = 24) forms was assessed with the Scale for the Assessment and Rating of Ataxia (SARA). Gait performance was examined during slow, preferred, and maximally fast walking speeds. Spatiotemporal variability parameters in the fore-aft and medio-lateral directions were analyzed. The fall frequency was assessed using a standardized interview about fall events within the last 6 months. Fore-aft gait variability showed significant speed-dependent characteristics with highest magnitudes during slow and fast walking. The SARA score correlated positively with fore-aft gait variability, most prominently during fast walking. The fall frequency was significantly associated to fore-aft gait variability during slow walking. Severity of ataxia, dynamic stability, and the occurrence of falls were interrelated in a speed-dependent manner: (a) Severity of ataxia symptoms was closely related to instability during fast walking. (b) Fall frequency was associated with instability during slow walking. These findings suggest the presence of a speed-dependent, twofold cerebellar locomotor control. Assessment of gait performance during non-preferred, slow and fast walking speeds provides novel insights into the pathophysiology of cerebellar locomotor control and may become a useful approach in the clinical evaluation of patients with CA.
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Literature
1.
go back to reference Bauby CE, Kuo AD (2000) Active control of lateral balance in human walking. J Biomech 33:1433–1440CrossRefPubMed Bauby CE, Kuo AD (2000) Active control of lateral balance in human walking. J Biomech 33:1433–1440CrossRefPubMed
2.
go back to reference Bloem BR, Grimbergen YA, Cramer M, Willemsen M, Zwinderman AH (2001) Prospective assessment of falls in Parkinson’s disease. J Neurol 248:950–958CrossRefPubMed Bloem BR, Grimbergen YA, Cramer M, Willemsen M, Zwinderman AH (2001) Prospective assessment of falls in Parkinson’s disease. J Neurol 248:950–958CrossRefPubMed
3.
go back to reference Brandt T, Strupp M, Benson J (1999) You are better off running than walking with acute vestibulopathy. Lancet 354:746CrossRefPubMed Brandt T, Strupp M, Benson J (1999) You are better off running than walking with acute vestibulopathy. Lancet 354:746CrossRefPubMed
4.
go back to reference Ebersbach G, Sojer M, Valldeoriola F, Wissel J, Muller J, Tolosa E, Poewe W (1999) Comparative analysis of gait in Parkinson’s disease, cerebellar ataxia and subcortical arteriosclerotic encephalopathy. Brain 122(Pt 7):1349–1355CrossRefPubMed Ebersbach G, Sojer M, Valldeoriola F, Wissel J, Muller J, Tolosa E, Poewe W (1999) Comparative analysis of gait in Parkinson’s disease, cerebellar ataxia and subcortical arteriosclerotic encephalopathy. Brain 122(Pt 7):1349–1355CrossRefPubMed
5.
go back to reference Fonteyn EM, Schmitz-Hubsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, Bunn L, Charles P, Durr A, Filla A, Giunti P, Globas C, Klockgether T, Melegh B, Pandolfo M, De Rosa A, Schols L, Timmann D, Munneke M, Kremer BP, van de Warrenburg BP (2010) Falls in spinocerebellar ataxias: results of the EuroSCA Fall Study. Cerebellum 9:232–239CrossRefPubMed Fonteyn EM, Schmitz-Hubsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, Bunn L, Charles P, Durr A, Filla A, Giunti P, Globas C, Klockgether T, Melegh B, Pandolfo M, De Rosa A, Schols L, Timmann D, Munneke M, Kremer BP, van de Warrenburg BP (2010) Falls in spinocerebellar ataxias: results of the EuroSCA Fall Study. Cerebellum 9:232–239CrossRefPubMed
6.
go back to reference Fonteyn EM, Schmitz-Hubsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, Bunn L, Giunti P, Globas C, Klockgether T, Melegh B, Pandolfo M, Schols L, Timmann D, van de Warrenburg BP (2013) Prospective analysis of falls in dominant ataxias. Eur Neurol 69:53–57CrossRefPubMed Fonteyn EM, Schmitz-Hubsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, Bunn L, Giunti P, Globas C, Klockgether T, Melegh B, Pandolfo M, Schols L, Timmann D, van de Warrenburg BP (2013) Prospective analysis of falls in dominant ataxias. Eur Neurol 69:53–57CrossRefPubMed
7.
go back to reference Greenberg SA (2012) Analysis of measurement tools of fear of falling for high-risk, community-dwelling older adults. Clin Nurs Res 21:113–130CrossRefPubMed Greenberg SA (2012) Analysis of measurement tools of fear of falling for high-risk, community-dwelling older adults. Clin Nurs Res 21:113–130CrossRefPubMed
8.
go back to reference Hauer K, Lamb SE, Jorstad EC, Todd C, Becker C (2006) Systematic review of definitions and methods of measuring falls in randomised controlled fall prevention trials. Age Ageing 35:5–10CrossRefPubMed Hauer K, Lamb SE, Jorstad EC, Todd C, Becker C (2006) Systematic review of definitions and methods of measuring falls in randomised controlled fall prevention trials. Age Ageing 35:5–10CrossRefPubMed
9.
go back to reference Ilg W, Giese MA, Gizewski ER, Schoch B, Timmann D (2008) The influence of focal cerebellar lesions on the control and adaptation of gait. Brain 131:2913–2927CrossRefPubMed Ilg W, Giese MA, Gizewski ER, Schoch B, Timmann D (2008) The influence of focal cerebellar lesions on the control and adaptation of gait. Brain 131:2913–2927CrossRefPubMed
10.
go back to reference Ilg W, Golla H, Thier P, Giese MA (2007) Specific influences of cerebellar dysfunctions on gait. Brain 130:786–798CrossRefPubMed Ilg W, Golla H, Thier P, Giese MA (2007) Specific influences of cerebellar dysfunctions on gait. Brain 130:786–798CrossRefPubMed
11.
go back to reference Ilg W, Timmann D (2013) Gait ataxia–specific cerebellar influences and their rehabilitation. Mov Disord 28:1566–1575CrossRefPubMed Ilg W, Timmann D (2013) Gait ataxia–specific cerebellar influences and their rehabilitation. Mov Disord 28:1566–1575CrossRefPubMed
12.
go back to reference Jahn K, Deutschlander A, Stephan T, Kalla R, Wiesmann M, Strupp M, Brandt T (2008) Imaging human supraspinal locomotor centers in brainstem and cerebellum. Neuroimage 39:786–792CrossRefPubMed Jahn K, Deutschlander A, Stephan T, Kalla R, Wiesmann M, Strupp M, Brandt T (2008) Imaging human supraspinal locomotor centers in brainstem and cerebellum. Neuroimage 39:786–792CrossRefPubMed
13.
go back to reference Jahn K, Strupp M, Schneider E, Dieterich M, Brandt T (2001) Visually induced gait deviations during different locomotion speeds. Exp Brain Res 141:370–374CrossRefPubMed Jahn K, Strupp M, Schneider E, Dieterich M, Brandt T (2001) Visually induced gait deviations during different locomotion speeds. Exp Brain Res 141:370–374CrossRefPubMed
14.
go back to reference Mori S, Matsuyama K, Mori F, Nakajima K (2001) Supraspinal sites that induce locomotion in the vertebrate central nervous system. Adv Neurol 87:25–40PubMed Mori S, Matsuyama K, Mori F, Nakajima K (2001) Supraspinal sites that induce locomotion in the vertebrate central nervous system. Adv Neurol 87:25–40PubMed
15.
go back to reference Morton SM, Bastian AJ (2003) Relative contributions of balance and voluntary leg-coordination deficits to cerebellar gait ataxia. J Neurophysiol 89:1844–1856CrossRefPubMed Morton SM, Bastian AJ (2003) Relative contributions of balance and voluntary leg-coordination deficits to cerebellar gait ataxia. J Neurophysiol 89:1844–1856CrossRefPubMed
16.
go back to reference Palliyath S, Hallett M, Thomas SL, Lebiedowska MK (1998) Gait in patients with cerebellar ataxia. Mov Disord 13:958–964CrossRefPubMed Palliyath S, Hallett M, Thomas SL, Lebiedowska MK (1998) Gait in patients with cerebellar ataxia. Mov Disord 13:958–964CrossRefPubMed
17.
go back to reference Powell LE, Myers AM (1995) The activities-specific balance confidence (ABC) scale. J Gerontol Series A Biol Sci Med Sci 50:M28–M34CrossRef Powell LE, Myers AM (1995) The activities-specific balance confidence (ABC) scale. J Gerontol Series A Biol Sci Med Sci 50:M28–M34CrossRef
18.
go back to reference Saute JA, Donis KC, Serrano-Munuera C, Genis D, Ramirez LT, Mazzetti P, Perez LV, Latorre P, Sequeiros J, Matilla-Duenas A, Jardim LB (2012) Ataxia rating scales–psychometric profiles, natural history and their application in clinical trials. Cerebellum 11:488–504CrossRefPubMed Saute JA, Donis KC, Serrano-Munuera C, Genis D, Ramirez LT, Mazzetti P, Perez LV, Latorre P, Sequeiros J, Matilla-Duenas A, Jardim LB (2012) Ataxia rating scales–psychometric profiles, natural history and their application in clinical trials. Cerebellum 11:488–504CrossRefPubMed
19.
go back to reference Schmitz-Hubsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schols L, Szymanski S, van de Warrenburg BP, Durr A, Klockgether T, Fancellu R (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717–1720CrossRefPubMed Schmitz-Hubsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schols L, Szymanski S, van de Warrenburg BP, Durr A, Klockgether T, Fancellu R (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717–1720CrossRefPubMed
20.
go back to reference Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B (2006) Reliability and validity of the international cooperative ataxia rating scale: a study in 156 spinocerebellar ataxia patients. Mov Disord 21:699–704CrossRefPubMed Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B (2006) Reliability and validity of the international cooperative ataxia rating scale: a study in 156 spinocerebellar ataxia patients. Mov Disord 21:699–704CrossRefPubMed
21.
go back to reference Schniepp R, Strupp M, Wuehr M, Jahn K, Dieterich M, Brandt T, Feil K (2016) Acetyl-dl-leucine improves gait variability in patients with cerebellar ataxia—a case series. Cereb Ataxias 3:1CrossRef Schniepp R, Strupp M, Wuehr M, Jahn K, Dieterich M, Brandt T, Feil K (2016) Acetyl-dl-leucine improves gait variability in patients with cerebellar ataxia—a case series. Cereb Ataxias 3:1CrossRef
22.
go back to reference Schniepp R, Wuehr M, Ackl N, Danek A, Brandt T, Strupp M, Jahn K (2011) 4-Aminopyridine improves gait variability in cerebellar ataxia due to CACNA 1A mutation. J Neurol 258:1708–1711CrossRefPubMed Schniepp R, Wuehr M, Ackl N, Danek A, Brandt T, Strupp M, Jahn K (2011) 4-Aminopyridine improves gait variability in cerebellar ataxia due to CACNA 1A mutation. J Neurol 258:1708–1711CrossRefPubMed
23.
24.
go back to reference Schniepp R, Wuehr M, Neuhaeusser M, Benecke AK, Adrion C, Brandt T, Strupp M, Jahn K (2012) 4-aminopyridine and cerebellar gait: a retrospective case series. J Neurol 259:2491–2493CrossRefPubMed Schniepp R, Wuehr M, Neuhaeusser M, Benecke AK, Adrion C, Brandt T, Strupp M, Jahn K (2012) 4-aminopyridine and cerebellar gait: a retrospective case series. J Neurol 259:2491–2493CrossRefPubMed
25.
go back to reference Schniepp R, Wuehr M, Neuhaeusser M, Kamenova M, Dimitriadis K, Klopstock T, Strupp M, Brandt T, Jahn K (2012) Locomotion speed determines gait variability in cerebellar ataxia and vestibular failure. Mov Disord 27:125–131CrossRefPubMed Schniepp R, Wuehr M, Neuhaeusser M, Kamenova M, Dimitriadis K, Klopstock T, Strupp M, Brandt T, Jahn K (2012) Locomotion speed determines gait variability in cerebellar ataxia and vestibular failure. Mov Disord 27:125–131CrossRefPubMed
26.
go back to reference Schniepp R, Wuehr M, Schlick C, Huth S, Pradhan C, Dieterich M, Brandt T, Jahn K (2014) Increased gait variability is associated with the history of falls in patients with cerebellar ataxia. J Neurol 261:213–223CrossRefPubMed Schniepp R, Wuehr M, Schlick C, Huth S, Pradhan C, Dieterich M, Brandt T, Jahn K (2014) Increased gait variability is associated with the history of falls in patients with cerebellar ataxia. J Neurol 261:213–223CrossRefPubMed
27.
go back to reference Serrao M, Pierelli F, Ranavolo A, Draicchio F, Conte C, Don R, Di Fabio R, LeRose M, Padua L, Sandrini G, Casali C (2012) Gait pattern in inherited cerebellar ataxias. Cerebellum 11:194–211CrossRefPubMed Serrao M, Pierelli F, Ranavolo A, Draicchio F, Conte C, Don R, Di Fabio R, LeRose M, Padua L, Sandrini G, Casali C (2012) Gait pattern in inherited cerebellar ataxias. Cerebellum 11:194–211CrossRefPubMed
28.
go back to reference Stolze H, Klebe S, Petersen G, Raethjen J, Wenzelburger R, Witt K, Deuschl G (2002) Typical features of cerebellar ataxic gait. J Neurol Neurosurg Psychiatry 73:310–312CrossRefPubMedPubMedCentral Stolze H, Klebe S, Petersen G, Raethjen J, Wenzelburger R, Witt K, Deuschl G (2002) Typical features of cerebellar ataxic gait. J Neurol Neurosurg Psychiatry 73:310–312CrossRefPubMedPubMedCentral
29.
go back to reference van de Warrenburg BP, Steijns JA, Munneke M, Kremer BP, Bloem BR (2005) Falls in degenerative cerebellar ataxias. Mov Disord 20:497–500CrossRefPubMed van de Warrenburg BP, Steijns JA, Munneke M, Kremer BP, Bloem BR (2005) Falls in degenerative cerebellar ataxias. Mov Disord 20:497–500CrossRefPubMed
30.
go back to reference Wrisley DM, Marchetti GF, Kuharsky DK, Whitney SL (2004) Reliability, internal consistency, and validity of data obtained with the functional gait assessment. Phys Ther 84:906–918PubMed Wrisley DM, Marchetti GF, Kuharsky DK, Whitney SL (2004) Reliability, internal consistency, and validity of data obtained with the functional gait assessment. Phys Ther 84:906–918PubMed
31.
go back to reference Wuehr M, Schniepp R, Ilmberger J, Brandt T, Jahn K (2013) Speed-dependent temporospatial gait variability and long-range correlations in cerebellar ataxia. Gait Posture 37:214–218CrossRefPubMed Wuehr M, Schniepp R, Ilmberger J, Brandt T, Jahn K (2013) Speed-dependent temporospatial gait variability and long-range correlations in cerebellar ataxia. Gait Posture 37:214–218CrossRefPubMed
32.
go back to reference Wuehr M, Schniepp R, Pradhan C, Ilmberger J, Strupp M, Brandt T, Jahn K (2013) Differential effects of absent visual feedback control on gait variability during different locomotion speeds. Exp Brain Res 224:287–294CrossRefPubMed Wuehr M, Schniepp R, Pradhan C, Ilmberger J, Strupp M, Brandt T, Jahn K (2013) Differential effects of absent visual feedback control on gait variability during different locomotion speeds. Exp Brain Res 224:287–294CrossRefPubMed
33.
go back to reference Wuehr M, Schniepp R, Schlick C, Huth S, Pradhan C, Dieterich M, Brandt T, Jahn K (2014) Sensory loss and walking speed related factors for gait alterations in patients with peripheral neuropathy. Gait Posture 39:852–858CrossRefPubMed Wuehr M, Schniepp R, Schlick C, Huth S, Pradhan C, Dieterich M, Brandt T, Jahn K (2014) Sensory loss and walking speed related factors for gait alterations in patients with peripheral neuropathy. Gait Posture 39:852–858CrossRefPubMed
Metadata
Title
The interrelationship between disease severity, dynamic stability, and falls in cerebellar ataxia
Authors
Roman Schniepp
Cornelia Schlick
Cauchy Pradhan
Marianne Dieterich
Thomas Brandt
Klaus Jahn
Max Wuehr
Publication date
01-07-2016
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 7/2016
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-016-8142-z

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