Brain Structure and Function

, Volume 216, Issue 3, pp 275–288

Linking coordinative and executive dysfunctions to atrophy in spinocerebellar ataxia 2 patients

  • Federico D’Agata
  • Paola Caroppo
  • Andrea Boghi
  • Mario Coriasco
  • Marcella Caglio
  • Bruno Baudino
  • Katiuscia Sacco
  • Franco Cauda
  • Elisabetta Geda
  • Mauro Bergui
  • Giuliano Geminiani
  • Gianni Boris Bradac
  • Laura Orsi
  • Paolo Mortara
Original Article

DOI: 10.1007/s00429-011-0310-4

Cite this article as:
D’Agata, F., Caroppo, P., Boghi, A. et al. Brain Struct Funct (2011) 216: 275. doi:10.1007/s00429-011-0310-4
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Abstract

Spinocerebellar ataxias type 2 (SCA2) is a rare genetic disorder characterised by the degeneration of the Cerebellum, its connections and many Brainstem areas. A voxel-based morphometry (VBM) analysis was performed on 12 genetically determined SCA2 patients and 31 controls, normalising the brains with two different atlases: one was created in-house with DARTEL (a diffeomorphic registration method) and the other was SUIT (an exclusive Cerebellum atlas). We administered two versions of a popular executive/planning functions test: the Tower of London, in the traditional and in a computerised version that does not require the use of hands, to correlate the regional atrophy with the tests’ performances and to discover the different associations of Cerebellum’s areas to cognitive dysfunctions. SCA2 showed a diffuse infratentorial atrophy with the whole Cerebellum and Brainstem affected, the overall patterns were highly overlapping between atlases with some minor differences. The DARTEL VBM also allowed detecting two sovratentorial clusters of atrophy, one in the left Inferior Parietal Lobule and the other in the Corticospinal Tracts. Additional analyses revealed a partial involvement of many White Matter tracts and of the Thalamus in the pathology. The classical Tower of London version correlated maximally with the right Lobule IV–V, when the computerised version correlated with the right Crus 1. The correlations of different versions of the test suggested a dissociation between the dysfunctions in SCA2: the Posterior Cerebellum was linked to the executive dysfunction while the Anterior Cerebellum was linked to the coordinative dysfunction.

Keywords

Cerebellum Cerebellar atrophy Spinocerebellar ataxia SCA SCA2 Type 2 Voxel-based morphometry VBM Tower of London 

Abbreviations

ADCA

Autosomal dominant cerebellar ataxia

CSF

Cerebrospinal fluid

FWE

Family Wyse error

FWHM

Full width half maximum

FDR

False discovery rate

GM

Grey matter

ICARS

International Cooperative Ataxia Rating Scale

MMSE

Mini mental state examination

MNI

Montreal Neurological Institute

MRI

Magnetic resonance imaging

NS

Not significant

SCA

Spinocerebellar atrophy

SD

Standard deviation

SE

Standard error

TIV

Total intracranial volume

ToL

Tower of London

CPU

Tower of London computerised version

PEG

Tower of London Pegs version

VBM

Voxel-based morphometry

WM

White matter

Supplementary material

429_2011_310_MOESM1_ESM.pdf (3.7 mb)
Supplementary material 1 (PDF 3823 kb)

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Federico D’Agata
    • 1
    • 2
    • 3
    • 7
  • Paola Caroppo
    • 1
  • Andrea Boghi
    • 4
  • Mario Coriasco
    • 1
  • Marcella Caglio
    • 1
    • 2
  • Bruno Baudino
    • 5
  • Katiuscia Sacco
    • 2
    • 3
    • 6
  • Franco Cauda
    • 2
    • 3
  • Elisabetta Geda
    • 2
    • 3
  • Mauro Bergui
    • 1
  • Giuliano Geminiani
    • 2
    • 3
    • 6
  • Gianni Boris Bradac
    • 1
  • Laura Orsi
    • 1
  • Paolo Mortara
    • 1
    • 6
  1. 1.Department of NeuroscienceAOU S. Giovanni BattistaTurinItaly
  2. 2.Department of PsychologyUniversity of TurinTurinItaly
  3. 3.CCS-fMRI, Koelliker HospitalTurinItaly
  4. 4.Section of Neuroradiology, Department of RadiodiagnosticsASO S. CroceCuneoItaly
  5. 5.Nuclear Medicine, AOU S. Giovanni BattistaTurinItaly
  6. 6.NIT, Neuroscience Institute of TurinTurinItaly
  7. 7.Department of NeuroscienceUniversity of TurinTurinItaly

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