Journals on Neurology Articles - BJSTR Journal

Abstract

#Huntington's disease (HD) and #Dentatorubral-pallidoluysian atrophy (DRPLA) are progressive and currently incurable neurodegenerative diseases which shares common features such as choreic involuntary movements, dementia, psychosis, and pathologic expansion of triplet nucleotide repeats in the causative genes [1,2]. In HD, the prevalence of dysphagia is approximately 30 % and several reports have been examined the features [3-5]. In DRPLA, #dysphagia can be clinically challenging considering a report that approximately 50 % of the DRPLA patients decease by aspiration pneumonia [6]. However, the data is scarce about the natural course of dysphagia in both HD and DRPLA in long term. Thereby, we examined the progression to clarify the features and to seek clues for potential treatments.The chart-review was concluded if the patients deceased, transferred to other institutions, or received #percutaneous endoscopic #gastrostomy (PEG). The onset of disease was defined as the initiation of movement abnormality, excluding the start of congnitive-#psychiatric symptoms or epilepsy. The level of nutritional modification was reviewed to express the severity of #dysphagia in the following five stages (the mildest: I, the severest: V); (I) unmodified, (II) partially modified (e.g. particular foods were minced), (III) pasted, (IV) high-caloric jelly, and (V) enteral feeding via PEG. The activity in daily lives (ADL), when they became wheelchair-bound and/or bedridden, was reviewed to express the systemic motor function. 

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Longitudinal Progression of Dysphagia in Huntington's Disease and Dentatorubral-Pallidoluysian Atrophy: ≥10 Years Retrospection in Twelve Patients by Hiroyuki Todo in BJSTR