Department of Neurology, Xiangya Hospital, Central South University, Changsha - 410 078, Hunan, P.R.China.
Neurol India. 2009 Sep-Oct;57(5):578-83. doi: 10.4103/0028-3886.57803.
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.
脑桥和小脑萎缩是磁共振成像(MRI)在脊髓小脑共济失调 3 型/马查多-约瑟夫病(SCA3/MJD)中最重要的特征。然而,脑桥和小脑萎缩与临床特征之间的相关性尚未得到很好的研究。
研究 SCA3/MJD 患者脑桥和小脑 MRI 特征与主要临床特征之间的相关性。
大学教学医院。
我们使用线性测量方法评估了 32 例 SCA3/MJD 患者,以研究脑桥和小脑形态数据与临床特征之间的相关性:疾病持续时间、发病年龄、国际合作共济失调评定量表(ICARS)总分;总共济失调评定量表(SARA)评分;ICARS 亚评分和 SARA 亚评分。
Pearson 相关检验。
中脑和脑桥前后径与 ICARS 总分、SARA 总分、ICARS 和 SARA 亚评分呈显著负相关(r=-0.381-0.57,P<0.05 或 0.01)和疾病持续时间(r=-0.42-0.51,P<0.05 或 0.01)。此外,小脑上、下径与 SARA 总分和 ICARS 及 SARA 亚评分呈负相关,除了两量表中姿势和步态共济失调(r=-0.37~-0.44,P<0.05)。第四脑室上、下径与发病年龄呈负相关(r=-0.45,P<0.05)。
SCA3/MJD 患者脑桥和小脑的影响与主要临床特征有关。