High intensity in the globus pallidus on proton and T2-weighted MRI in a case of dentato-ruburo-pallido-luysian atrophy of myoclonus epilepsy type.

Although marked atrophy of the tegmentum of the mid-brain is a known computed tomography (CT) and magnetic resonance imaging (MRI) feature in patients with dentato-ruburo-pallido-luysian atrophy (DRPLA), this is the first report of marked high signal intensity of the globus pallidus on proton and T2-weighted MR images in a patient in the early stage of DRPLA of the myoclonus epilepsy type. We suggest that the globus pallidus may be involved in the early stage of DRPLA of this type. Magnetic resonance imaging is considered to be useful for the early detection of involvement of the globus pallidus and in the clinical diagnosis of DRPLA.