Background Huntington’s disease (HD) is a genetic neurodegenerative disorder characterised with motor, cognitive and psychiatric disturbances. As it progress patients experience reduced language and communication skills.
Objective To characterise the language and communication abilities of HD patients as reflected in tasks involving naming, verbal fluency, working memory and pragmatics.
Method The study included 13 HD participants (7 males), mean age 48 ± 12 from the movement disorders unit at Tel Aviv Medical centre. All participants underwent United Huntington’s Disease Rating Scale (UHDRS), Montreal Cognitive Assessment (MoCA), Naming Test (NT), the Phonemic Fluency (PF) and Semantic Fluency (SF) subtests and the Auditory Word Matching Span (AWMS) test. Each session was video recorded and each participant’s pragmatic behaviour was estimated by 3 judges, according to the Pragmatic Protocol (PP). The scores were compared to the tests’ norms.
Results Mean disease duration 8.15 ± 3.67, disease severity 36.67 ± 14.47, number of repeats 45.08 ± 4.8 and MoCA 19.17 ± 5.17. There was a significant difference between the HD patients tests scores and the tests norms: NT 40.45 ± 6.61 (p = 0.011); PF 10 ± 5.66; SF 22.33 ± 11.34; AWMS 2.20 ± 1.48; PP 43.33 ± 22.77 (p < 0.001 respectively). A significant difference was found between PF and SF scores (p < 0.001). The verbal/paralinguistic/non-verbal pragmatic aspects that had the highest scores were ‘verbal or non-verbal feedback to speaker’/‘Intonation’/‘Physical proximity’ and ‘Physical contacts’ 69.23 ± 0.48/61.54 ± 0.51/100 ± 0 and 100 ± 0 respectively. The lowest scores were ‘Topic change’/‘Vocal quality’/‘Foot/leg and hand/arm movements’ 7.69 ± 0.28/23.08 ± 0.44/0 ± 0 respectively. A significant correlation was found between UHDRS and SF scores (p < 0.05) and between MoCA and AWMS scores (p < 0.01).
Conclusion Reduced language and communication skills can be an indication of disease severity and cognitive decline. These findings may help develop specific language intervention program as well as serve as a possible marker of disease progression.
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