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Yoshio Murata, MD; Shinya Yamaguchi, MD; Hideshi Kawakami, MD; Yukari Imon, MD; Hirofumi Maruyama, MD; Tetsuo Sakai, MD; Toshinari Kazuta, MD; Toshiyuki Ohtake, MD; Masataka Nishimura, MD; Takahiko Saida, MD; Susumu Chiba, MD; Takekazu Oh-i, MD; Shigenobu Nakamura, MD

Arch Neurol. 1998;55:33-37.

Objective  To clarify the characteristic magnetic resonance imaging (MRI) findings in patients with Machado-Joseph disease (MJD) diagnosed by genetic analysis.

Patients and Methods  Using MRI, we examined 31 patients genetically diagnosed as having MJD, 20 patients with sporadic olivopontocerebellar atrophy, and 26 control subjects.

Results  The MRIs of patients with MJD disclosed remarkably reduced width of the superior cerebellar peduncles, atrophy in the frontal and temporal lobes, diminished transverse diameter of the globus pallidus, and decreased anteroposterior and transverse diameters of the pons, which correlated with the width of the middle cerebellar peduncle. The width of the superior cerebellar peduncles also correlated with the diameter of the dentate or red nucleus in patients with MJD, but not in controls or in patients with sporadic olivopontocerebellar atrophy. On T₂- and/or proton-weighted axial MR imaging, a high signal intensity in the transverse pontine fibers was observed in 14 (45.2%) of 31 patients with MJD and in all patients with sporadic olivopontocerebellar atrophy, but not in any controls.

Conclusion  Affected afferent and efferent cerebellar tracts and atrophy of the frontal and temporal lobes and globus pallidus are characteristics of MRI of patients with MJD.

From the Third Department of Internal Medicine, Hiroshima University School of Medicine, Hiroshima, Japan (Drs Murata, Yamaguchi, Kawakami, Imon, Maruyama, and Nakamura); the Department of Neurology, National Chikugo Hospital, Fukuoka, Japan (Dr Sakai); the Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan (Drs Kazuta and Ohtake); the Department of Neurology and Clinical Research Center, National Utano Hospital, Kyoto, Japan (Drs Nishimura and Saida); the Department of Neurology, Sapporo Medical University, Hokkaid[accent-mo], Japan (Dr Chiba); and the Third Department of Internal Medicine, Miyazaki Medical College, Miyazaki, Japan (Dr Oh-i). Dr Yamaguchi is now with the Department of Neurology, Suiseikai Kajikawa Hospital, Hiroshima, Japan.

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