Longitudinal Progression of Dysphagia in Huntington’s Disease and Dentatorubral-Pallidoluysian Atrophy: ≥10 Years Retrospection in Twelve Patients
نویسندگان
چکیده
منابع مشابه
Corneal endothelial degeneration in dentatorubral-pallidoluysian atrophy.
BACKGROUND Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant spinocerebellar degeneration that exhibits a variety of neurologic manifestations. However, only a few reports have studied disturbances outside the central nervous system. We described 2 unrelated patients with DRPLA accompanied by corneal endothelial degeneration. PATIENTS AND METHODS A 52-year-old man presente...
متن کاملDentatorubral pallidoluysian atrophy in a Turkish family.
Dentatorubral pallidoluysian atrophy is a neurodegenerative disease that generally presents in adulthood. Although rare, it can be observed in childhood due to extreme expansion of the triplet repeat size during spermatogenesis. The diagnosis in childhood is very difficult in the absence of family history. Here we describe a 12-year-old girl with dentatorubral pallidoluysian atrophy who present...
متن کاملMR of childhood-onset dentatorubral-pallidoluysian atrophy.
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 of his father, who had adult-onset dentatorubral-pallidoluysian atrophy. Besides showing severe brain atrophy, especially of the brain stem tegmentum and cerebellum, MR showed diffuse periventricular hyperintensity on T2...
متن کاملDentatorubral-pallidoluysian atrophy in two Chinese families in Hong Kong.
We report on two Hong Kong Chinese families with dentatorubral-pallidoluysian atrophy. Two children in one family presented with progressive myoclonic epilepsy syndrome, and two children in the other family presented with ataxochoreo-athetoid symptoms. Early-onset childhood dentatorubral-pallidoluysian atrophy involved mental retardation, whereas myoclonic epilepsy was the predominant complaint...
متن کاملPearls & Oy-sters: Family history of Huntington disease disguised a case of dentatorubral-pallidoluysian atrophy.
Case report A 42-year-old woman of Caucasian ancestry presented with an episode of acute paranoid psychosis, an 8-month history of slowly progressive generalized choreoathetosis, gait and speech disturbance, as well as cognitive impairment. Her choreoathetosis remarkably improved due to olanzapine, and subsequently, cerebellar signs became obvious upon neurologic examination (video, http://link...
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ژورنال
عنوان ژورنال: Biomedical Journal of Scientific & Technical Research
سال: 2018
ISSN: 2574-1241
DOI: 10.26717/bjstr.2018.09.001833