The correlation between magnetic resonance imaging features of the brainstem and cerebellum and clinical features of spinocerebellar ataxia 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.


OBJECTIVE
To study the correlation between MRI features of the brainstem and cerebellum and predominant clinical features in SCA3/MJD.


METHODS
University teaching hospital.


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.


METHODS
Pearson correlation test.


RESULTS
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).


CONCLUSIONS
The effect on the cerebellum and brainstem is related to predominant clinical features in SCA3/MJD patients.
Liang
Xiaochun
X
Department of Neurology, Xiangya Hospital, Central South University, Changsha - 410 078, Hunan, P.R.China.
Jiang
Hong
H
Chen
Changqing
C
Zhou
Gaofeng
G
Wang
Junling
J
Zhang
Shen
S
Lei
Liwang
L
Wang
Xiaoyi
X
Tang
Beisha
B
D000328:Adult; D000067699:Ataxin-3; D001933:Brain Stem / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology*; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D012097:Repressor Proteins / Q000235:genetics; D012720:Severity of Illness Index; D013223:Statistics as Topic; D019680:Trinucleotide Repeat Expansion / Q000235:genetics; D055815:Young Adult
D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D012097:Repressor Proteins; C092341:ATXN3 protein, human; D000067699:Ataxin-3