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

Research paper by Xiaochun X Liang, Hong H Jiang, Changqing C Chen, Gaofeng G Zhou, Junling J Wang, Shen S Zhang, Liwang L Lei, Xiaoyi X Wang, Beisha B Tang

Indexed on: 26 Nov '09Published on: 26 Nov '09Published in: Neurology India


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.To study the correlation between MRI features of the brainstem and cerebellum and predominant clinical features in SCA3/MJD.University teaching hospital.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.Pearson correlation test.There was a significant inverse correlation between anteroposterior diameter of the midbrain and pons and total ICARS scores, total SARA scores, ICARS and SARA subscores (r = -0.381 approximately -0.57, P < 0.05 or 0.01) and disease duration (r = -0.42 approximately -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 approximately -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).The effect on the cerebellum and brainstem is related to predominant clinical features in SCA3/MJD patients.