Top

Published in:

01-07-2014 | Original Communication

Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration

Authors: Naohisa Ueda, Yasuhito Hakii, Shigeru Koyano, Yuichi Higashiyama, Hideto Joki, Yasuhisa Baba, Yume Suzuki, Yoshiyuki Kuroiwa, Fumiaki Tanaka

Published in: Journal of Neurology | Issue 7/2014

Abstract

Spinocerebellar degeneration (SCD) is a progressive neurodegenerative disorder in which cerebellar ataxia causes motor disability. There are no widely applicable methods for objective evaluation of ataxia in SCD. An objective system to evaluate ataxia is necessary for use in clinical trials of newly developed medication and rehabilitation. The aim of this study was to develop a simple method to quantify the degree of upper-limb ataxia. Forty-nine patients with SCD participated in this study. Patients were instructed to trace an Archimedean spiral template, and the gap between the template spiral and the drawn spiral (gap area; GA) was measured using Image J software. Ataxia was rated using the Scale for the Assessment and Rating of Ataxia (SARA) and cerebellar volume was evaluated in 37 patients using an axial cross-section of magnetic resonance images that were obtained within 6 months of clinical evaluation. Regression analysis was performed to assess the relation between GA and patient age, disease duration, SARA score, and cerebellar volume. GA was significantly related to total SARA score (r = 0.660, p < 0.001), the posture and gait (r = 0.551, p < 0.001), speech (r = 0.527, p < 0.001), hand movements (r = 0.553, p < 0.001), and heel-shin slide (r = 0.367, p = 0.036) SARA subscores, and cerebellar volume (r = 0.577, p < 0.001) but was not related to patient age (r = 0.176, p = 0.227) or disease duration (r = 0.236, p = 0.103). GA is a simple, useful method to objectively quantify the degree of cerebellar ataxia, especially upper-limb ataxia, and can be widely adopted in various settings, including clinical trials.

Ilg W, Schatton C, Schicks J, Giese MA, Schöls L, Synofzik M (2012) Video game-based coordinative training improves ataxia in children with degenerative ataxia. Neurology 79:2056–2060CrossRefPubMed

Miyai I, Ito M, Hattori N, Mihara M, Hatakeyama M, Yagura H, Sobue G, Nishizawa M (2012) Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases. Neurorehabil Neural Repair 26:515–522CrossRefPubMed

Trouillas P, Takayanagi T, Hallett M, Currier RD, Subramony SH, Wessel K, Bryer A, Diener HC, Massaquoi S, Gomez CM, Coutinho P, Ben Hamida M, Campanella G, Filla A, Schut L, Timann D, Honnorat J, Nighoghossian N, Manyam B (1997) International COOPERATIVE Ataxia Scale for pharmacological assessment of the cerebellar syndrome. J Neurol Sci 145:205–211CrossRefPubMed

Schmitz-Hübsch T, Tezenas du Montcel S, 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, Schöls L, Szymanski S, van de Warrenburg BPC, Dürr A, Klockgether T (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717–1720CrossRefPubMed

Subramony SH, May W, Lynch D, Gomez C, Fischbeck K, Hallett M, Taylor P, Wilson R, Ashizawa T (2005) Measuring Friedreich ataxia. Interrater reliability of a neurologic rating scale. Neurology 64:1261–1262CrossRefPubMed

Schmitz-Hübsch T, Giunti P, Stephenson DA, Globas C, Baliko L, Saccà F, Mariotti C, Rakowicz M, Szymanski S, Infante J, van de Warrenburg BPC, Timmann D, Fancellu R, Rola R, Depondt C, Schöls L, Zdzienicka E, Kang JS, Döhlinger S, Kremer B, Melegh B, Filla A, Klockgether T (2008) SCA functional index. A useful compound performance measure for spinocerebellar ataxia. Neurology 71:486–492CrossRefPubMed

du Montcel ST, Charles P, Ribai P, Goizet C, Le Bayon A, Labauge P, Guyant-Maréchal L, Forlani S, Jauffret C, Vandenberghe N, N’guyen K, Le Ber I, Devos D, Vincitorio CM, Manto MU, Tison F, Hannequin D, Ruberg M, Brice A, Durr A (2008) Composite cerebellar functional severity score: validation of a quantitative cerebellar impairment. Brain 131:1352–1361CrossRefPubMed

Bastian AJ, Martin TA, Keating JG, Thach WT (1996) Cerebellar ataxia. Abnormal control of interaction torques across multiple joints. J Neurophysiol 76:492–509PubMed

Menegoni F, Milano E, Trotti C, Galli M, Bigoni M, Baudo S, Mauro A (2009) Quantitative evaluation of functional limitation of upper limb movements in subjects affected by ataxia. Eur J Neurol 16:232–239CrossRefPubMed

10.

Kamitani T, Kuroiwa Y, Wang L, Li M, Ikegami T, Matsubara S (2003) Event-related potentials during visual S1-S2 paradigm in multiple system atrophy: relation to morphologic changes on brain MRO measurement. Parkinsonism Relat Disord 10:93–100CrossRefPubMed

11.

Sullivan EV, Deshmukh A, Desmond JE, Lim KO, Pfefferbaum A (2000) Cerebellar volume decline in normal aging, alcoholism, and Korsakoff’s syndrome: relation to ataxia. Neuropsychology 14:341–352CrossRefPubMed

12.

Brenneis C, Bösch SM, Schocke M, Wenning GK, Poewe W (2003) Atrophy pattern in SCA2 determined by voxel-based morphometry. NeuroReport 14:1799–1802CrossRefPubMed

13.

Guerrini L, Lolli F, Ginestroni A, Belli G, Nave RD, Tessa C, Foresti S, Cosottini M, Piacentini S, Salvi F, Plasmati R, Grandis DD, Siciliano G, Filla A, Mascalchi M (2004) Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study. Brain 127:1785–1790CrossRefPubMed

14.

Richter S, Dimitrova A, Maschke M, Gizewski E, Beck A, Aurich V, Timmann D (2005) Degree of cerebellar ataxia correlates with three-dimensional MRI-based cerebellar volume in pure cerebellar degeneration. Eur Neurol 54:23–27CrossRefPubMed

15.

Brenneis C, Boesch SM, Egger KE, Seppi K, Scherfler C, Schocke M, Wenning GK, Poewe W (2006) Cortical atrophy in the cerebellar variant of multiple atrophy: a voxel-based morphometry study. Mov Disord 21:159–165CrossRefPubMed

16.

Nave RD, Ginestroni A, Gianneli M, Tessa C, Salvatore E, Salvi F, Dotti MT, Michele GD, Piacentini S, Piacentini S (2007) Brain structural damage in Friedreich’s ataxia. J Neurol Neurosurg Psychiatry 79:82–85CrossRefPubMed

17.

Brandauer B, Hermsdörfer J, Beck A, Aurich V, Gizewski ER, Marquardt C, Timmann D (2008) Impairments of prehension kinematics and grasping forces in patients with cerebellar degeneration and the relationship to cerebellar atrophy. Clinical Neurophysiol 119:2528–2537CrossRef

18.

Ginestroni A, Nave RD, Tessa C, Gianneli M, Grandis DD, Plasmati R, Salvi F, Piacentini S, Piacentini S (2008) Brain structural damage in spinocerebellar ataxia type 1. A VBM study. J Neurol 25:1153–1158CrossRef

19.

Nave RD, Ginestroni A, Tessa C, Cosottini M, Gianneli M, Salvatore E, Sartucci F, Michele GD, Dotti MT, Piacentini S, Mascalchi M (2008) Brain structural damage in spinocerebellar ataxia type 2. A voxel-based morphometry study. Mov Disord 23:899–903CrossRefPubMed

20.

Nave RD, Ginestroni A, Tessa C, Salvatore E, Grandis DD, Plasmati R, Salvi F, Michele GD, Dotti MT, Piacentini S, Mascalchi M (2008) Brain white matter damage in SCA1 and SCA2. An in vivo study using voxel-based morphometry, histogram analysis of mean diffusivity and tract-based spatial statistics. Neuroimage 43:10–19CrossRefPubMed

21.

Reetz K, Lencer R, Hagenah JM, Gaser C, Tadic V, Walter U, Wolters A, Steinlechner S, Zühlke C, Brockmann K, Klein C, Rolfs A, Binkofski F (2010) Structural changes associated with progression of motor deficits in spinocerebellar ataxia 17. Cerebellum 9:210–217CrossRefPubMed

22.

Schulz JB, Borkert J, Wolf S, Schmitz-Hübsch T, Rakowicz M, Mariotti C, Schoels L, Timmann D, van de Warrenburg B, Dürr A, Pandolfo M, Kang JS, Mandly AG, Nägele T, Grisoli M, Boguslawska R, Bauer P, Klockgether T, Hauser TK (2010) Visualization, quantification and correlation of brain atrophy with clinical symptoms in spinocerebellar ataxia 1, 3 and 6. Neuroimage 49:158–168CrossRefPubMed

23.

Camargos ST, Marques-Jr W, dos Santos AC (2011) Brain stem and cerebellum volumetric analysis of Machado Joseph disease patients. Arq Neuropsiquiatr 69:292–296CrossRefPubMed

24.

Eichler L, Ballenberg B, Hahn HK, Köster O, Schöls L, Lukas C (2011) Quantitative assessment of brain stem and cerebellar atrophy in spinocerebellar ataxia types 3 and 6: impact on clinical status. AJNR 32:890–897CrossRefPubMed

25.

Walterfang M, Abel LA, Desmond P, Fahey MC, Bowman EA, Velakoulis D (2013) Cerebellar volume correlates with saccadic gain and ataxia in adult Niemann-Pick type C. Mol Genet Metab 108:85–89CrossRefPubMed

26.

Klockgether T, Schroth G, Diener HC, Dichgans J (1990) Idiopathic cerebellar ataxia of late onset: natural history and MRI morphology. J Neurol Neurosurg Psychiatry 53:297–305PubMedCentralCrossRefPubMed

27.

Schmahmann JD, Gardner R, MacMore J, Vangel MG (2009) Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS. Mov Disord 24:1820–1828PubMedCentralCrossRefPubMed

Title: Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration
Authors: Naohisa Ueda
Yasuhito Hakii
Shigeru Koyano
Yuichi Higashiyama
Hideto Joki
Yasuhisa Baba
Yume Suzuki
Yoshiyuki Kuroiwa
Fumiaki Tanaka
Publication date: 01-07-2014
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 7/2014
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-014-7353-4

At a glance: The STEP trials

Springer Medicine

Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 7/2014

Rate of intravenous thrombolysis for acute ischaemic stroke in the North-of-France region and evolution over time

Time intervals from subarachnoid hemorrhage to rebleed

Richard Bright (1789–1858)

Erratum to: Patterns of decline in upper limb function of boys and men with DMD: an international survey

Intracranial pressure in unresponsive chronic migraine

The incidence of clinically isolated syndrome in a multi-ethnic cohort