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 ORIGINAL ARTICLE
Year : 2009  |  Volume : 57  |  Issue : 5  |  Page : 578--583

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


1 Department of Neurology & Radiology, Xiangya Hospital, Central South University, Changsha - 410 078, China
2 Department of Neurology, Xiangya Hospital, Central South University, Changsha - 410 078; Neurodegenerative Disorders Research Center, Central South University, Changsha - 410 078, China
3 Department of Radiology, Xiangya Hospital, Central South University, Changsha - 410 078, China
4 Department of Neurology, Xiangya Hospital, Central South University, Changsha - 410 078, China
5 Department of Neurology, Xiangya Hospital, Central South University, Changsha - 410 078; Neurodegenerative Disorders Research Center, Central South University, Changsha - 410 078; National Lab of Medical Genetics of China, Changsha - 410 078, China

Correspondence Address:
Beisha Tang
Department of Neurology, Xiangya Hospital, Central South University, 87# Xiangya Road, Changsha - 410 008, Hunan, P. R
China
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Source of Support: This work was supported by series of grants including the National Science and Technology Pillar program in the Eleventh Five-year Plan Period (2006BAI05A07) (Dr. Tang); the National High-Tech Research and Development Program of China (863 Program) (2006AA02A408) (Dr. Tang); the Major State Basic Research Development Program of China (973 Program) (2006cb500700) (Dr. Tang); the National Key Technologies Research and Development Program of China (2004BA720A03) (Dr. Tang); the National Natural Science Foundation of China (30871354, 30971585, 30710303061, 30400262 and 30470619) (Dr. Jiang); Key Project in the Natural Science Foundation of Hunan Province (08JJ3048) (Dr. Jiang).,, Conflict of Interest: None


DOI: 10.4103/0028-3886.57803

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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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Online since 20th March '04
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