This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on ISI (23)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Radiologic Imaging  • Magnetic Resonance Imaging  • Alert me on articles by topic

Yoshio Murata, MD; Hideshi Kawakami, MD; Shinya Yamaguchi, MD; Masataka Nishimura, MD; Tatsuo Kohriyama, MD; Fumiko Ishizaki, MD; Zenjiro Matsuyama, MD; Yasuyo Mimori, MD; Shigenobu Nakamura, MD

Arch Neurol. 1998;55:1348-1352.

Objective  To clarify the characteristic magnetic resonance imaging (MRI) findings in patients with spinocerebellar ataxia 6 (SCA6) diagnosed by genetic analysis.

Patients and Methods  Using MRI, we examined 10 patients genetically diagnosed as having SCA6 and 40 control subjects.

Results  The mean (±SD) CAG repeat length in 10 patients with SCA6 was 22.9±1.3. There was a significant inverse correlation between the CAG repeat size and age at onset in the SCA6 group (r=-0.86, P =.003). In patients with SCA6, the areas of the cerebellar vermis and hemispheres in sagittal MRI were significantly smaller than those in the control subjects. In transaxial MRI, the anteroposterior diameter of the pons and the diameter of the middle cerebellar peduncle were mildly decreased and the red nucleus was slightly atrophied in patients with SCA6. There was no significant difference in the diameter of the midbrain, medulla oblongata, fourth ventricle, superior cerebellar peduncles, dentate nucleus, or globus pallidus between the SCA6 and control groups. A high-signal intensity in the transverse pontine fibers was not observed in any of the patients with SCA6 on T₂-weighted and/or proton-weighted axial MRI.

Conclusions  The cerebellum and its afferent and efferent systems were affected in patients with SCA6. These results seem to distinguish the MRI findings of SCA6 from those of other forms of spinocerebellar ataxia.

From the Third Department of Internal Medicine, Hiroshima University School of Medicine, Hiroshima, Japan (Drs Murata, Kawakami, Kohriyama, Matsuyama, Mimori, and Nakamura); Department of Neurology, Suiseikai Kajikawa Hospital, Hiroshima (Dr Yamaguchi); Department of Neurology, National Utano Hospital, Kyoto, Japan (Dr Nishimura); and Hiroshima Prefectural College of Health and Welfare (Dr Ishizaki).

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Morphological basis for the spectrum of clinical deficits in spinocerebellar ataxia 17 (SCA17)
Lasek et al.
Brain 2006;129:2341-2352.
ABSTRACT | FULL TEXT  

Infarcts in the posterior circulation territory in migraine. The population-based MRI CAMERA study
Kruit et al.
Brain 2005;128:2068-2077.
ABSTRACT | FULL TEXT  

Radiological characterization of spinocerebellar ataxia type 6
Butteriss et al.
Br. J. Radiol. 2005;78:694-696.
ABSTRACT | FULL TEXT  

Quantitative MRI measurement of superior cerebellar peduncle in progressive supranuclear palsy
Paviour et al.
Neurology 2005;64:675-679.
ABSTRACT | FULL TEXT  

Pontine atrophy precedes cerebellar degeneration in spinocerebellar ataxia 7: MRI-based volumetric analysis
Bang et al.
J. Neurol. Neurosurg. Psychiatry 2004;75:1452-1456.
ABSTRACT | FULL TEXT  

Quantitative Assessment of Cerebral Blood Flow in Genetically Confirmed Spinocerebellar Ataxia Type 6
Honjo et al.
Arch Neurol 2004;61:933-937.
ABSTRACT | FULL TEXT  

Clinical and Neuroradiological Features of Patients With Spinocerebellar Ataxias From Korean Kindreds
Bang et al.
Arch Neurol 2003;60:1566-1574.
ABSTRACT | FULL TEXT  

Metabolic Characterization of Spinocerebellar Ataxia Type 6
Soong et al.
Arch Neurol 2001;58:300-304.
ABSTRACT | FULL TEXT