Spinal Cord Damage in Machado-Joseph Disease

Spinal Cord Damage in Machado-Joseph Disease

Machado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in the disease and its clinical relevance. To evalu...

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Bibliographic Details
Published in:Cerebellum (London, England) Vol. 14; no. 2; pp. 128 - 132
Main Authors: Fahl, Camila N., Branco, Lucas Melo T., Bergo, Felipe P. G., D’Abreu, Anelyssa, Lopes-Cendes, Iscia, França, Marcondes C.
Format: Journal Article
Language:English
Published: Boston Springer US 01-04-2015
Springer Nature B.V
Subjects:
Atrophy
Biomedical and Life Sciences
Biomedicine
Cerebellum - pathology
Cervical Cord - pathology
Female
Humans
Imaging, Three-Dimensional
Machado-Joseph Disease - genetics
Machado-Joseph Disease - pathology
Magnetic Resonance Imaging
Male
Middle Aged
Neurobiology
Neurology
Neurosciences
Organ Size
Original Paper
Pattern Recognition, Automated
Severity of Illness Index
Trinucleotide Repeat Expansion
Ataxia
Spinal cord
Machado-Joseph disease
SCA3
MRI
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Summary:Machado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in the disease and its clinical relevance. To evaluate the spinal cord damage in SCA3 through quantitative analysis of MRI scans. A group of 48 patients with SCA3 and 48 age and gender-matched healthy controls underwent MRI on a 3T scanner. We used T1-weighted 3D images to estimate the cervical spinal cord area (CA) and eccentricity (CE) at three C2/C3 levels based on a semi-automatic image segmentation protocol. The scale for assessment and rating of ataxia (SARA) was employed to quantify disease severity. The two groups—SCA3 and controls—were significantly different regarding CA (49.5 ± 7.3 vs 67.2 ± 6.3 mm 2 , p  < 0.001) and CE values (0.79 ± 0.06 vs 0.75 ± 0.05, p  = 0.005). In addition, CA presented a significant correlation with SARA scores in the patient group ( p  = 0.010). CE was not associated with SARA scores ( p  = 0.857). In the multiple variable regression, we found that disease duration was the only variable associated with CA (coefficient = −0.629, p  = 0.025). SCA3 is characterized by cervical cord atrophy and antero-posterior flattening. In addition, the spinal cord areas did correlate with disease severity. This suggests that quantitative analyses of the spinal cord MRI might be a useful biomarker in SCA3.
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ISSN:1473-4222
1473-4230
DOI:10.1007/s12311-014-0619-7