Background Huntington’s disease (HD) is a genetic neurodegenerative disease clinically manifested by motor, cognitive and psychiatric disturbances. With disease progress patients experience increasing swallowing difficulty.
Objective To characterise the swallowing disturbances of HD patients and evaluate the relation to cognition, disease duration and severity.
Method HD patients from the movement disorders unit at Tel Aviv Medical Centre were recruited to the study. All patients underwent United Huntington’s Disease Rating Scale (UHDRS), Montreal Cognitive Assessment (MoCA), filled the swallowing disturbances questionnaire (SDQ) and underwent fibre optic endoscopic evaluation of swallowing (FEES).
Results Fourteen patients were recruited (4 males, mean age 47.5 ± 11.28 years, disease duration 10.38 ± 6.25). The UHDRS score and number of CAG Trinocleotide repeats were 36.75.61 ± 17.52 (14–58) and 45.61 ± 4.31 (41–56) respectively. MoCA mean score was 20.14 ± 4.12 and SDQ was 13.21 ± 7.5. Oral motor evaluation revealed reduced rate of the diodikinetic task ± 7.22 3.9 seconds and reduced lips and tongue movements in 63% of patients. FEES showed a delayed swallowing reflex trigger, reflex was initiated when the food bolus reached the vallecula in 50% of patients. Food residues were seen in 38% of patients in one location in the pharynx and 15.5% of patients presented all over the pharynx. Pre spillage was noted in 83.3% of patients and post spillage in 87.5%. Significant correlations were found between volitional cough strength, ability to initiate volitional swallow and cognitive status (p < 0.01, p < 0.05, respectively), volitional cough and disease duration, diodekinetik task rate and numbers of CAG Trinocleotide repeats (p = 0.058 and p < 0.01 respectively).
Conclusion HD patients exhibit prominent unique oropharyngeal dysphagia apparently due to dyssynergia between the swallowing phases, worsened by the cognitive decline. Dysphagia may serve as a marker and relatively objective indicator of disease progression. A specific swallowing intervention program should be developed addressing the unique swallowing disturbances of HD patients.
- Swallowing disorders