Swallow Outcome in Three Female Siblings with Huntingtons Disease and Chorea

Abstract

The present study focuses on describing characteristics of swallow among Huntingtons Disease (HD) with Chorea before and after dysphagia therapy. 3 female siblings of 21, 22 and 33 years having juvenile type onset of HD with chorea were included. The patients were evaluated comprehensively for swallowing using Manipal Manual for Swallowing Assessment. Each patient was subjected to ingestion of solid, thin liquid and thick liquid of 5ml and 10 ml quantified using a standard measurable cup. Descriptive statistics was administered on the data using statistical package SPSS (Version 17). On observation, all 3 patients presented with sensory and motor issues in addition to posture instability with abrupt body movements, food spillage, piece meal deglutition, intra bolus retention, wet voice and cough. Following which cognitive approach and behavioural approach based intervention was initiated. The symptoms of intra bolus retention and cough decreased post therapy with no change in sensory aspects. The present study evidences three female siblings with severe cognitive deficits and dysphagia secondary to HD. Despite rehabilitation being provided, they could not completely waiver off the symptoms. These evidences highlight the importance of identifying and addressing swallow based treatment outcomes in HD with chorea.