Quantifying the Onset of Unintended Weight Loss in Huntington’s Disease: A Retrospective Analysis of Enroll-HD

Background: Unintended weight loss and decreased body mass indexes (BMIs) are common symptoms of individuals with manifest HD. It is unknown at what point during disease progression weight loss starts to accelerate relative to a healthy individual’s weight and when recommended interventions should be initiated to have the strongest impact on patient care. Objective: The objective of this study was to identify a point in time relative to age at motor onset when the decline in weight in HD starts to accelerate relative to a non-HD population. The relationship between initiation of weight loss interventions and changes in weight loss was also explored. Methods: Participants from the fifth version of the Enroll-HD study were identified for this research. Linear mixed-effects piecewise regression models were used to estimate the point in time relative to the reported age of motor onset in which BMI started to decline in participants with HD compared to healthy non-HD controls. A post-hoc descriptive analysis was performed to look at when nutritional supplements and swallow therapy were initiated in participants with HD relative to motor onset. Results: BMI decline in the HD group began to accelerate compared to controls approximately 5.7 years after the reported age of motor onset (95% CI: 4.7–6.9). The average initiation times of swallow therapy and nutritional supplements were 7.7 years (SD = 5.5 years) and 6.7 years (SD = 6.5 years) after motor onset, respectively. Conclusion: Our findings suggest a potential point for intervention of nutrition programs or therapies used to prevent future weight loss.

Development of Swallowing Function in Infants with Oral Feeding Difficulties

Background. Discharge of preterm infants is often delayed because of their oral feeding difficulties. Independent oral feeding is the last obstacle to pass after managing acute and chronic morbidities. We conducted this study to determine the prevalence, characteristics, and risk factors of swallowing dysfunction and suggest proper interventions to reduce aspiration and chronic lung injury. Methods. Infants admitted to the neonatal intensive care unit (January 2016 to December 2018) who were performed modified barium swallow study due to oral feeding difficulties were enrolled. Modified barium swallow study was done≥postmenstrual age 37 weeks to limit radiation exposure. Clinical data were collected retrospectively. Swallowing dysfunction was defined as inadequate epiglottic closure, laryngeal penetration, or tracheal aspiration according to result of the modified barium swallow study. Results. Among a total of 54 infants enrolled, nine (16.7%) were term infants, 13 (24.1%) were late preterm infants (gestational age, 34-36 weeks), and 32 (59.3%) were early preterm infants (gestational age<34 weeks). Gestational age and birth weight were smaller in infants with swallowing dysfunction. Total duration of mechanical ventilation and duration of invasive ventilation were longer in infants with swallowing dysfunction. The risk of swallowing dysfunction increased by 11.2 times for infants with gestational age<29 weeks compared to infants with gestational age≥29 weeks. Swallowing dysfunction was improved in most infants after they became matured. They showed different time and rate of maturation with the help of rehabilitation through swallow therapy and dietary modification with thickened formula. Conclusion. Preterm infants with gestational age<29 weeks or with longer ventilation duration are at a higher risk of aspiration. Rehabilitation of swallow therapy and dietary modification with thickened formula can be helpful interventions to prevent aspiration and chronic lung injury and reassure parents until independent oral feeding is possible.

Changes in Timing of Swallow Events in Parkinson’s Disease

Objectives: The prevalence of Parkinson’s disease (PD) increases as the population ages. Dysphagia and subsequent aspiration pneumonia are common causes of morbidity and mortality in those with PD. To maximize the benefit of swallowing therapy, protocol design should be based on an understanding of the physiologic swallowing deficits present in the PD population. The aim of this study was to compare the timing of swallow events in a cohort of patients with PD with that in normal age-matched control subjects to characterize variations in the coordination of structural displacement and bolus movement that may contribute to dysphagia. Methods: This retrospective study included 68 adults with diagnoses of PD. Liquid bolus swallows during modified barium swallow studies were analyzed and compared with those from an age- and sex-matched cohort of 48 adults without PD. Results: Patients with PD were significantly slower in initiating and completing airway closure. Hyoid elevation was prolonged in this patient population. Conclusions: Patients with PD demonstrate slower initiation of airway closure and a delay in relaxation of hyoid elevation during swallow. Delays increased with larger boluses. These findings may be related to impaired pharyngeal sensation and increased muscular rigidity. The results of this study will be helpful in guiding swallow therapy for patients with PD.