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The correlation between magnetic resonance imaging features of the brainstem and cerebellum and clinical features of spinocerebellar ataxia 3/Machado-Joseph disease

Author(s): Liang Xiaochun | Jiang Hong | Chen Changqing | Zhou Gaofeng | Wang Junling | Zhang Shen | Lei Liwang | Wang Xiaoyi | Tang Beisha

Journal: Neurology India
ISSN 0028-3886

Volume: 57;
Issue: 5;
Start page: 578;
Date: 2009;
Original page

Keywords: Clinical feature | correlation | magnetic resonance imaging | spinocerebellar ataxia type 3/Machado-Joseph disease

Background : Brainstem and cerebellar atrophy are the most important features in magnetic resonance imaging (MRI) in spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD). However, the correlation between brainstem and cerebellar atrophy and the clinical features has not been well studied. Aim : To study the correlation between MRI features of the brainstem and cerebellum and predominant clinical features in SCA3/MJD. Design and Setting : University teaching hospital. Patients and Methods : By using the linear measurement method, we assessed 32 patients with SCA3/MJD to study the correlations between the morphometric data of the brainstem and cerebellum and clinical features: Duration of the disease, age of onset, total international cooperative ataxia rating scale (ICARS) score; total scale for the assessment and rating of ataxia (SARA) score; ICARS subscores, and SARA subscores. Statistical Analysis : Pearson correlation test. Results : There was a significant invese correlation between anteroposterior diameter of the midbrain and pons and total ICARS scores, total SARA scores, ICARS and SARA subscores (r= -0.381~-0.57, P < 0.05 or 0.01) and disease duration (r=- 0.42~-0.51, P < 0.05 or 0.01). Additionally, superoinferior diameter of the cerebellum was inversely correlated with total SARA scores and ICARS and SARA subscores except for ataxia of posture and gait in both scales (r=- 0.37~-0.44, P < 0.05). The superoinferior diameter of the fourth ventricle was inversely correlated with age of onset (r=-0.45, P < 0.05). Conclusion : The effect on the cerebellum and brainstem is related to predominant clinical features in SCA3/MJD patients.
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