MR findings in a 14-year-old boy with progressive myoclonic epilepsy, who was diagnosed
as having dentatorubral-pallidoluysian atrophy by DNA analysis, were compared with those …

Dentatorubral-pallidoluysian atrophy is a rare autosomal-dominant neurodegenerative
disorder caused by an expansion of a CAG repeat in the atrophin-1 gene on chromosome …

We describe a family showing dentatorubral-pallidoluysian atrophy. Three patients
appeared through three successive generations and displayed a wide variety of clinical …

We studied the frequency and characteristics of brain-stem and thalamic lesions in
dentatorubral-pallidoluysian atrophy using MRI. Of 15 subjects diagnosed by DNA analysis …

We describe the cases of 2 patients, a father and his son, with DRPLA who underwent MR
examinations prior to death and in whom postmortem examinations of the brain were …

We studied five families with a syndrome of myoclonus, epilepsy, dementia, ataxia, and
choreoathetosis. At autopsy, the major neuropathologic changes consisted of combined …

Hereditary dentatorubral-pallidoluysian atrophy (DRPLA) has recently been recognized as a
clinicopathological entity. It may be defined as a multisystem degenerative disease of …

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 …

We report a 48-year-old woman with dentatorubral-pallidoluysian atrophy (DRPLA). She is
the only patient in her 15 family members in two generations. She developed cerebellar …

A 22-year-old female with progressive myoclonus epilepsy (PME) considered to be due to
hereditary dentato-rubro-pallido-luysian atrophy (DRPLA) was reported. Some of her family …