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Huntington’s Disease

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Handbook of Pragmatic Language Disorders

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Abstract

Huntington’s disease is a neurodegenerative disorder with progressive motor, cognitive, and neuropsychiatric impairments. There is evidence that problems in both motor speech and language affect individuals’ ability to use language for active participation in everyday communication. Impaired non-verbal language use and comprehension deficits as well as lexical-semantic issues with word-finding difficulties impact the communicative interaction. As the disease progresses, discourse may be restricted by latencies and short, less elaborated utterances with an insufficient level of detail. Speech may become effortful, variable, and difficult to understand. This affects turn-taking along with topic management and also leads to an increased need for repair and adjustment from the communication partner. In this chapter, we adopt an emergentist perspective on pragmatic ability. Research on impairments in several different basic motor and cognitive systems and the effect of these impairments on pragmatic aspects of everyday communication will be presented along with snapshots from lived experiences of Huntington’s disease. Assessment methods used in the investigation of pragmatic aspects of communicative impairment in Huntington’s disease, as well as current communication interventions that may be beneficial, will also be described.

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References

  • Astell, A. J., Ellis, M. P., Berbardi, L., Alm, N., Dye, R., Gowans, G., et al. (2010). Using a touch screen computer to support relationships between people with dementia and caregivers. Interacting with Computers, 22(4), 267–275.

    Article  Google Scholar 

  • Aviezer, H., Bentin, S., Hassin, R., Meschino, W., Kennedy, J., Grewal, S., et al. (2009). Not on the face alone: Perception of contextualized face expressions in Huntington’s disease. Brain, 132(6), 1633–1644.

    Article  Google Scholar 

  • Bachoud-Lévi, A.-C., Ferreira, J., Massart, R., Youssov, K., Rosser, A., Busse, M., et al. (2019). International guidelines for the treatment of Huntington’s disease. Frontiers in Neurology, 10, 710. https://doi.org/10.3389/fneur.2019.00710

    Article  Google Scholar 

  • Baez, S., Herrera, E., Gershanik, O., Garcia, A., Bocanegra, Y., Kargieman, L., et al. (2015). Impairments in negative emotion recognition and empathy for pain in Huntington’s disease families. Neuropsychologia, 68(9), 158–167.

    Article  Google Scholar 

  • Beukelman, D. R., Fager, S., Ball, S., & Dietz, A. (2007). AAC for adults with acquired neurological conditions: A review. Augmentative and Alternative Communication, 23(3), 230–242.

    Article  Google Scholar 

  • Bilney, B., Morris, M. E., & Perry, A. (2003). Effectiveness of physiotherapy, occupational therapy and speech pathology for people with Huntington’s disease: A systematic review. Neurorehabilitation Neural Repair, 17(1), 12–24.

    Article  Google Scholar 

  • Bird, T. D. (1994). Poems: Huntington’s disease. The Western Journal of Medicine, 160(2), 179.

    Google Scholar 

  • Bora, E., Velakoulis, D., & Walterfang, M. (2016). Social cognition in Huntington’s disease: A meta-analysis. Behavioural Brain Research, 297, 131–140.

    Article  Google Scholar 

  • Braisch, U., Martinez-Horta, S., MacDonald, M., & Orth, M. (2016). Important but not enough—Information about HD related topics and peer and professional support for young adults from HD families. Journal of Huntington’s Disease, 5(4), 379–387.

    Article  Google Scholar 

  • Brown Moskowitz, C., & Rao, A. K. (2017). Making a measurable difference in advanced Huntington disease care. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 183–196). Elsevier.

    Google Scholar 

  • Buchholz, M., Ferm, U., & Holmgren, K. (2018). Support persons’ views on remote communication and social media for people with communicative and cognitive disabilities. Disability and Rehabilitation, 28, 1–9.

    Google Scholar 

  • Butters, N., Sax, D., Montgomery, K., & Tarlow, S. (1978). Comparison of the neuropsychological deficits associated with early and advanced Huntington’s disease. Archives of Neurology, 35(9), 585–589.

    Article  Google Scholar 

  • Calder, A., Keane, J., Young, A., Lawrence, A., Mason, S., & Barker, R. (2010). The relation between anger and different forms of disgust: Implications for emotion recognition impairments in Huntington’s disease. Neuropsychologia, 48(9), 2719–2729.

    Article  Google Scholar 

  • Carlozzi, N., Schilling, E., Lai, S., Perlmutter, G., Nance, J., Waljee, J., et al. (2016). HDQLIFE: The development of two new computer adaptive tests for use in Huntington disease, speech difficulties, and swallowing difficulties. Quality of Life Research, 25(10), 2417–2427.

    Article  Google Scholar 

  • Chenery, H., Copland, D., & Murdoch, B. (2002). Complex language functions and subcortical mechanisms: Evidence from Huntington’s disease and patients with non-thalamic sub-cortical lesions. International Journal of Language and Communication Disorders, 37(4), 459–474.

    Article  Google Scholar 

  • Collis, J., & Bloch, S. (2012). Survey of UK speech and language therapists’ assessment and treatment practices for people with progressive dysarthria. International Journal of Language and Communication Disorders, 47(6), 725–737.

    Article  Google Scholar 

  • Coppens, E. M., Jacobs, M., Van der Zwaan, K. F., Middelkoop, H. A. M., & Roos, R. A. C. (2019). Visual object perception in premanifest and early manifest Huntington’s disease. Archives of Clinical Neuropsychology, 34(8), 1320–1328.

    Article  Google Scholar 

  • Crosson, B. (1999). Subcortical mechanisms in language: Lexical-semantic mechanisms and the thalamus. Brain and Cognition, 40(2), 414–438.

    Article  Google Scholar 

  • Crosson, B., & Nadeau, S. E. (1998). The role of subcortical structures in linguistic processes. In B. Stemmer & H. A. Whitaker (Eds.), Handbook of neurolinguistics (pp. 431–445). Academic.

    Chapter  Google Scholar 

  • Cruickshank, T. M., Thompson, J. A., Domínguez, J. F., Reyes, A. P., Bynevelt, M., Georgiou-Karistianis, N., et al. (2015). The effect of multidisciplinary rehabilitation on brain structure and cognition in Huntington’s disease: An exploratory study. Brain and Behavior, 5(2), e00312. https://doi.org/10.1002/brb3.312

    Article  Google Scholar 

  • Deb, A., Frank, S., & Testa, C. M. (2017). New symptomatic therapies for Huntington disease. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 199–207). Elsiever.

    Google Scholar 

  • de Gelder, B., Van Den Stock, J., deDiego-Balaguer, R., & Bachoud-Lévi, A.-C. (2008). Huntington’s disease impairs recognition of angry and instrumental body language. Neuropsychologia, 46(1), 369–373.

    Article  Google Scholar 

  • Diehl, S., & de Riesthal, M. (2019). Augmentative and alternative communication use by individuals with Huntington’s disease: Benefits and challenges of implementation. Perspectives of the ASHA Special Interest Groups, 4(3), 456–463.

    Article  Google Scholar 

  • Duffy, J. R. (2013). Motor speech disorders: Substrates, differential diagnosis and management (3rd ed.). Elsevier Mosby.

    Google Scholar 

  • Duncan, R., Gillam, L., Savulescu, J., Williamson, R., Rogers, J., & Delatycki, M. (2007). “Holding your breath”: Interviews with young people who have undergone predictive genetic testing for Huntington disease. American Journal of Medical Genetics Part A, 143A(17), 1984–1989.

    Article  Google Scholar 

  • Eddy, C., & Rickards, H. (2015). Theory of mind can be impaired prior to motor onset in Huntington’s disease. Neuropsychology, 29(5), 792–798.

    Article  Google Scholar 

  • Eggenberger, E., Heimerl, K., & Bennett, M. I. (2013). Communication skills training in dementia care: A systematic review of effectiveness, training content, and didactic methods in different care settings. International Psychogeriatrics, 25(3), 345–358.

    Article  Google Scholar 

  • Ekström, A., Ferm, U., & Samuelsson, C. (2017). Digital communication support and Alzheimer’s disease. Dementia, 16(6), 711–731.

    Article  Google Scholar 

  • Ewing, N., & Murphy, J. (2017). See what matters. Australian Journal of Dementia Care, 6(5), 25–27.

    Google Scholar 

  • Ferm, U., Eckerholm Wallfur, P., Gelfgren, E., & Hartelius, L. (2012). Communication between Huntington’s disease patients, their support persons and the dental hygienist using Talking Mats. In N. E. Tunali (Ed.), Huntington’s disease: Core concepts and current advances (pp. 532–554). InTechOpen.

    Google Scholar 

  • Ferm, U., Nilsson, S., Jenholt Nolbris, M., Linnsand, P., & Jonsson, A. (2018). Impact of a parent’s neurodegenerative disease and care on the daily life of children. In M. Mollaoglu (Ed.), Caregiving and home care (pp. 85–103). InTechOpen.

    Google Scholar 

  • Ferm, U., Sahlin, A., Sundin, L., & Hartelius, L. (2010). Using Talking Mats to support communication in persons with Huntington’s disease. International Journal of Language and Communication Disorders, 45(5), 523–536.

    Article  Google Scholar 

  • Ferm, U., & Saldert, C. (2013). Disagreements in dental hygienist consultations using Talking Mats: Interaction involving people with Huntington’s disease and their support persons. In N. Norén, C. Samuelsson, & C. Plejert (Eds.), Aided communication in everyday interaction (pp. 189–238). J&R Press.

    Google Scholar 

  • Ford, R. A. (1989). The psychopathology of echo phenomena. Psychological Medicine, 19(3), 627–635.

    Article  Google Scholar 

  • Frank, H. L., Mcdade, H. L., & Scott, W. K. (1996). Naming in dementia secondary to Parkinson’s, Huntington’s, and Alzheimer’s diseases. Journal of Communication Disorders, 29(3), 183–197.

    Article  Google Scholar 

  • Frith, M., Togher, L., Ferguson, A., Levick, W., & Docking, K. (2014). Assessment practices of speech-language pathologists for cognitive communication disorders following traumatic brain injury in adults: An international survey. Brain Injury, 28(13–14), 1657–1666.

    Article  Google Scholar 

  • Gagnon, M., Barette, J., & Macoir, J. (2018). Language disorders in Huntington disease: A systematic literature review. Cognitive and Behavioral Neurology, 31(4), 179–192.

    Article  Google Scholar 

  • García, A., Bocanegra, Y., Herrera, E., Pino, M., Muñoz, E., Sedeño, L., et al. (2018). Action-semantic and syntactic deficits in subjects at risk for Huntington’s disease. Journal of Neuropsychology, 12(3), 389–408.

    Article  Google Scholar 

  • Giddens, C. L., Coleman, A. E., & Adams, C. M. (2010). A home program of speech therapy in Huntington’s disease. Journal of Medical Speech-Language Pathology, 18(2), 1–9.

    Google Scholar 

  • Gordon, W. P. (1985). Neuropsychological deficits in Huntington’s disease. International Neuropsychological Society.

    Google Scholar 

  • Gordon, W. P., & Illes, J. (1987). Neurolinguistic characteristics of language production in Huntington’s disease: A preliminary report. Brain and Language, 31(1), 1–10.

    Article  Google Scholar 

  • Hallberg, L., Mellgren, E., Hartelius, L., & Ferm, U. (2013). Talking Mats in a discussion group for people with Huntington’s disease. Disability and Rehabilitation: Assistive Technology, 8(1), 67–76.

    Google Scholar 

  • Hartelius, L., Carlstedt, A., Ytterberg, M., Lillvik, M., & Laakso, K. (2003). Speech disorders in mild and moderate Huntington’s disease: Results of dysarthria assessments of 19 individuals. Journal of Medical Speech-Language Pathology, 11(1), 1–14.

    Google Scholar 

  • Hartelius, L., Jonsson, M., Rickeberg, A., & Laakso, K. (2010). Communication and Huntington’s disease: Qualitative interviews and focus groups with persons with Huntington’s disease, family members, and carers. International Journal of Language and Communication Disorders, 45(3), 381–393.

    Article  Google Scholar 

  • Hamilton, A., Ferm, U., Heemskerk, A. W., Twiston-Davies, R., Matheson, K., Simpson, S. A., et al. (2012). Management of speech, language and communication difficulties in Huntington’s disease. Neurodegenerative Disease Management, 2(1), 67–77.

    Article  Google Scholar 

  • Hayes, C., Stevenson, R., & Coltheart, M. (2009). Production of spontaneous and posed facial expressions in patients with Huntington’s disease: Impaired communication of disgust. Cognition & Emotion, 23(1), 118–134.

    Article  Google Scholar 

  • Hinzen, W., Rosselló, J., Morey, C., Camara, E., Garcia-Gorro, C., Salvador, R., et al. (2018). A systematic linguistic profile of spontaneous narrative speech in pre-symptomatic and early stage Huntington’s disease. Cortex, 100, 71–83.

    Article  Google Scholar 

  • Ho, A. K., Sahakian, B. J., Robbins, T. W., Barker, R. A., Rosser, A. E., & Hodges, J. R. (2002). Verbal fluency in Huntington’s disease: A longitudinal analysis of phonemic and semantic clustering and switching. Neuropsychologia, 40(8), 1277–1284.

    Article  Google Scholar 

  • Holl, A., Wilkinson, L., Tabrizi, S., Painold, A., & Jahanshahi, M. (2013). Selective executive dysfunction but intact risky decision-making in early Huntington’s disease. Movement Disorders, 28(8), 1104–1109.

    Article  Google Scholar 

  • Illes, J. (1989). Neurolinguistic features of spontaneous language production dissociate three forms of neurodegenerative disease: Alzheimer’s, Huntington’s, and Parkinson’s. Brain and Language, 37(4), 628–642.

    Article  Google Scholar 

  • Ille, R., Holl, A. K., Kapfhammer, H.-P., Reisinger, K., Schäfer, A., & Schienle, A. (2011). Emotion recognition and experience in Huntington’s disease: Is there a differential impairment? Psychiatry Research, 188(3), 377–382.

    Article  Google Scholar 

  • Jensen, A. M., Chenery, H. J., & Copland, D. A. (2006). A comparison of picture description abilities in individuals with vascular subcortical lesions and Huntington’s disease. Journal of Communication Disorders, 39(1), 71–83.

    Article  Google Scholar 

  • Johnson, S. A., Stout, J. C., Solomon, A. C., Langbehn, D. R., Aylward, E. H., & Cruce, C. B. (2007). Beyond disgust: Impaired recognition of negative emotions prior to diagnosis in Huntington’s disease. Brain, 130(7), 1732–1744.

    Article  Google Scholar 

  • Kaplan, E., Goodglass, H., & Weintraub, S. (1983). The Boston naming test. Pro-Ed.

    Google Scholar 

  • Kargieman, L., Herrera, E., Baez, S., Garcia, A. M., Dottori, M., Gelormini, C., et al. (2014). Motor-language coupling in Huntington’s disease families. Frontiers in Aging Neuroscience, 6, 122. https://doi.org/10.3389/fnagi.2014.00122

    Article  Google Scholar 

  • Kay, C., Hayden, M. R., & Leavitt, B. R. (2017). Epidemiology of Huntington disease. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 31–46). Elsevier.

    Google Scholar 

  • Kent-Walsh, J., & McNaughton, D. (2005). Communication partner instruction in AAC: Present practices and future directions. Augmentative and Alternative Communication, 21(3), 195–204.

    Article  Google Scholar 

  • Kieburtz, K., & Huntington Study Group. (1996). Unified Huntington’s disease rating scale: Reliability and consistency. Movement Disorders, 11(2), 136–142.

    Article  Google Scholar 

  • Killoran, A., & Biglan, K. M. (2012). Therapeutics in Huntington’s disease. Current Treatment Options in Neurology, 14(2), 137–149.

    Article  Google Scholar 

  • Kirkwood, S. C., Siemers, E., Hodes, M. E., Conneally, P. M., Christian, J. C., & Foroud, T. (2000). Subtle changes among presymptomatic carriers of the Huntington’s disease gene. Journal of Neurology, Neurosurgery and Psychiatry, 69(6), 773–779.

    Article  Google Scholar 

  • Klager, J., Duckett, A., Sandler, S., & Moskowitz, C. (2008). Huntington’s disease: A caring approach to the end of life. Care Management Journals, 9(2), 75–81.

    Article  Google Scholar 

  • Klasner, E. R., & Yorkston, K. M. (2001). Linguistic and cognitive supplementation strategies as AAC techniques in Huntington’s disease: A case report. Augmentative and Alternative Communication, 17(3), 154–160.

    Article  Google Scholar 

  • Klasner, E. R., & Yorkston, K. M. (2005). Speech intelligibility in ALS and HD dysarthria: The everyday listener’s perspective. Journal of Medical Speech-Language Pathology, 13(2), 127–139.

    Google Scholar 

  • Kordsachia, C., Labuschagne, I., & Stout, J. (2017). Beyond emotion recognition deficits: A theory guided analysis of emotion processing in Huntington’s disease. Neuroscience and Biobehavioral Reviews, 73, 276–292.

    Article  Google Scholar 

  • Kordsachia, C., Labuschagne, I., & Stout, J. C. (2018). Visual scanning of the eye region of human faces predicts emotion recognition performance in Huntington’s disease. Neuropsychology, 32(3), 356–365.

    Article  Google Scholar 

  • Laakso, K., Brunnegård, K., Hartelius, L., & Ahlsén, E. (2000). Assessing high-level language in individuals with multiple sclerosis: A pilot study. Clinical Linguistics & Phonetics, 14(5), 329–349.

    Article  Google Scholar 

  • Lemiere, J., Decruyenaere, M., Evers-Kiebooms, G., Vandenbussche, E., & Dom, R. (2004). Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation. Journal of Neurology, 251(8), 935–942.

    Article  Google Scholar 

  • Lepron, E., Péran, P., Cardebat, D., & Demonet, J. F. (2009). A PET study of word generation in Huntington’s disease: Effects of lexical competition and verb/noun category. Brain & Language, 110(2), 49–60.

    Article  Google Scholar 

  • Lomas, J., Pickard, L., Bester, S., Elbard, H., Finlayson, A., & Zoghaib, C. (1989). The communicative effectiveness index: Development and psychometric evaluation of a functional communication measure for adult aphasia. Journal of Speech and Hearing Disorders, 54(1), 113–124.

    Article  Google Scholar 

  • Lundervold, A. J., Reinvang, I., & Lundervold, A. (1994). Characteristic patterns of verbal memory function in patients with Huntington’s disease. Scandinavian Journal of Psychology, 35(1), 38–47.

    Article  Google Scholar 

  • McDonald, S., Flanagan, S., Rollins, J., & Kinch, J. (2003). TASIT: A new clinical tool for assessing social perception after traumatic brain injury. Journal of Head Trauma Rehabilitation, 18(3), 219–238.

    Article  Google Scholar 

  • MacKenzie, C., Bennett, A., & Cairney, M. (2011). Active citizenship and acquired neurological communication difficulty. Disability and Rehabilitation, 33(3), 187–194.

    Article  Google Scholar 

  • Mehanna, R., & Jankovic, J. (2010). Respiratory problems in neurologic movement disorders. Parkinsonism and Related Disorders, 16(10), 628–638.

    Article  Google Scholar 

  • Murphy, J., & Boa, S. (2012). Using the WHO-ICF with Talking Mats to enable adults with long-term communication difficulties to participate in goal setting. Augmentative and Alternative Communication, 28(1), 52–60.

    Article  Google Scholar 

  • Murray, L. (2000). Spoken language production in Huntington’s and Parkinson’s diseases. Journal of Speech, Language, and Hearing Research, 43(6), 1350–1366.

    Article  Google Scholar 

  • Murray, L. L., & Lenz, L. P. (2001). Productive syntax abilities in Huntington’s and Parkinson’s diseases. Brain and Cognition, 46(1), 213–219.

    Article  Google Scholar 

  • Murray, L., & Stout, J. (1999). Discourse comprehension in Huntington’s and Parkinson’s diseases. American Journal of Speech-Language Pathology, 8(2), 137–148.

    Article  Google Scholar 

  • Nance, M. A. (2012). Therapy in Huntington’s disease: Where are we? Current Neurology and Neuroscience Reports, 12(4), 359–366.

    Article  Google Scholar 

  • Nasreddine, Z. S., Phillips, N. A., Bedirian, V., Charbonneau, S., Whitehead, V., Collin, I., et al. (2005). The Montreal Cognitive Assessment, MoCA: A brief screening tool for mild cognitive impairment. Journal of the American Geriatrics Society, 53(4), 695–699.

    Article  Google Scholar 

  • Nemeth, D., Dye, C., Sefcsik, T., Janacsek, K., Turi, Z., Londe, Z., et al. (2012). Language deficits in pre-symptomatic Huntington’s disease: Evidence from Hungarian. Brain and Language, 121(3), 248–253.

    Article  Google Scholar 

  • Pagan, F., Torres-Yaghi, Y., & Altshuler, M. (2017). The diagnosis and natural history of Huntington disease. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 63–97). Elsevier.

    Google Scholar 

  • Paterson, H. (2017). The use of social media by adults with acquired conditions who use AAC: Current gaps and considerations in research. Augmentative and Alternative Communication, 33(1), 23–31.

    Article  Google Scholar 

  • Paulsen, J. S., Miller, A. C., Hayes, T., & Shaw, E. (2017). Cognitive and behavioral changes in Huntington disease before diagnosis. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 69–91). Elsevier.

    Google Scholar 

  • Péran, P., Demonet, J. F., Pernet, C., & Cardebat, D. (2004). Verb and noun generation tasks in Huntington’s disease. Movement Disorders, 19(5), 565–571.

    Article  Google Scholar 

  • Perkins, M. R. (1998). Is pragmatics epiphenomenal? Evidence from communication disorders. Journal of Pragmatics, 29(3), 291–311.

    Article  Google Scholar 

  • Perkins, M. R. (2005a). Clinical pragmatics: An emergentist perspective. Clinical Linguistics and Phonetics, 19(5), 363–366.

    Article  Google Scholar 

  • Perkins, M. R. (2005b). Pragmatic ability and disability as emergent phenomena. Clinical Linguistics and Phonetics, 19(5), 367–377.

    Article  Google Scholar 

  • Perkins, M. R. (2007). Pragmatic impairment. Cambridge University Press.

    Book  Google Scholar 

  • Perkins, L., Whitworth, A., & Lesser, R. (1997). Conversation analysis profile for people with cognitive impairment (CAPPCI). Whurr Publishers Ltd..

    Google Scholar 

  • Phillips, W., Shannon, K., & Barker, R. (2008). The current clinical management of Huntington’s disease. Movement Disorders, 23(11), 1491–1504.

    Article  Google Scholar 

  • Philpott, A. L., Andrews, S. C., Staios, M., Churchyard, A., & Fisher, F. (2016). Emotion evaluation and social inference impairments in Huntington’s disease. Journal of Huntington’s Disease, 5(2), 175–183.

    Article  Google Scholar 

  • Piira, A., van Walsem, M. R., Mikalsen, G., Øie, L., Frich, J. C., & Knutsen, S. (2014). Effects of a two-year intensive multidisciplinary rehabilitation program for patients with Huntington’s disease: A prospective intervention study. PLoS Currents, 6, ecurrents.hd.2c56ceef7f9f8e239a59ecf2d94cddac. https://doi.org/10.1371/currents.hd.2c56ceef7f9f8e239a59ecf2d94cddac

    Article  Google Scholar 

  • Power, E., Anderson, A., & Togher, L. (2011). Applying the WHO ICF framework to communication assessment and goal setting in Huntington’s disease: A case discussion. Journal of Communication Disorders, 44(3), 261–275.

    Article  Google Scholar 

  • Profant, O., Roth, J., Bureš, Z., Balogová, Z., Lišková, I., Betka, J., & Syka, J. (2017). Auditory dysfunction in patients with Huntington’s disease. Clinical Neurophysiology, 128(10), 1946–1953.

    Article  Google Scholar 

  • Prutting, C. A., & Kirchner, D. M. (1987). A clinical appraisal of the pragmatic aspects of language. Journal of Speech and Hearing Disorders, 52(2), 105–119.

    Article  Google Scholar 

  • Rees, E., Farmer, R., Cole, J., Henley, S., Sprengelmeyer, R., Frost, C., et al. (2014). Inconsistent emotion recognition deficits across stimulus modalities in Huntington’s disease. Neuropsychologia, 64, 99–104.

    Article  Google Scholar 

  • Robotham, L., Sauter, D. A., Bachoud-Lévi, A.-C., & Trinkler, I. (2011). The impairment of emotion recognition in Huntington’s disease extends to positive emotions. Cortex, 47(7), 880–884.

    Article  Google Scholar 

  • Roos, R. A. (2010). Huntington’s disease: A clinical review. Orphanet Journal of Rare Diseases, 5, 40. https://doi.org/10.1186/1750-1172-5-40

    Article  Google Scholar 

  • Ross, C. A., Aylward, E. H., Wild, E. J., Langbehn, D. R., Long, J. D., Warner, J. H., et al. (2014). Huntington disease: Natural history, biomarkers and prospects for therapeutics. Nature Reviews Neurology, 10(4), 204–216.

    Article  Google Scholar 

  • Rusz, J., Klempir, J., Tykalova, T., Baborova, E., Roman, C., Ruzicka, E., & Roth, J. (2014a). Characteristics and occurrence of speech impairment in Huntington’s disease: Possible influence of antipsychotic medication. Journal of Neural Transmission, 121(12), 1529–1539.

    Article  Google Scholar 

  • Rusz, J., Saft, C., Schlegel, U., Hoffman, R., & Skodda, S. (2014b). Phonatory dysfunction as a preclinical symptom of Huntington disease. PLoS One, 9(11), e113412. https://doi.org/10.1371/journal.pone.0113412

    Article  Google Scholar 

  • Saldert, C. (2017). Pragmatic assessment and intervention in adults. In L. Cummings (Ed.), Research in clinical pragmatics (pp. 527–558). Springer.

    Chapter  Google Scholar 

  • Saldert, C., Eriksson, E., Petersson, K., & Hartelius, L. (2010a). Interaction in conversation in Huntington’s disease: An activity-based analysis and the conversation partner’s view of change. Journal of Interactional Research in Communication Disorders, 1(2), 169–197.

    Google Scholar 

  • Saldert, C., Fors, A., Ströberg, S., & Hartelius, L. (2010b). Comprehension of complex discourse in different stages of Huntington’s disease. International Journal of Language and Communication Disorders, 45(6), 656–669.

    Article  Google Scholar 

  • Saldert, C., & Hartelius, L. (2011). Echolalia or functional repetition in conversation: A case study of an individual with Huntington’s disease. Disability and Rehabilitation, 33(3), 253–260.

    Article  Google Scholar 

  • Schegloff, E. A. (2007). Sequence organization in interaction: A primer in conversation analysis. Cambridge University Press.

    Book  Google Scholar 

  • Scholz, F., Yalcin, B., & Priestley, M. (2017). Internet access for disabled people: Understanding sociorelational factors in Europe. Cyberpsychology: Journal of Psychosocial Research on Cyberspace, 11(1), article 4. https://doi.org/10.5817/CP2017-1-4

    Article  Google Scholar 

  • Shoulson, G., & Fahn, S. (1979). Huntington’s disease. Clinical care and evaluation. Neurology, 29(1), 1–3.

    Article  Google Scholar 

  • Schuler, A. L. (1979). Echolalia: Issues and clinical applications. Journal of Speech and Hearing Disorders, 44(4), 411–434.

    Article  Google Scholar 

  • Simmons-Mackie, N., Raymer, A., & Cherney, L. R. (2016). Communication partner training in aphasia: An updated systematic review. Archives of Physical Medical Rehabilitation, 97(12), 2202–2221.

    Article  Google Scholar 

  • Simpson, S. A., & Rae, D. A. (2012). Standard of care for Huntington’s disease: Who, what and why. Neurodegenerative Disease Management, 2(1), 1–5.

    Article  Google Scholar 

  • Skodda, S., Grönheit, W., Lukas, C., Bellenberg, B., von Hein, S. M., Hoffman, R., & Saft, C. (2016). Two different phenomena in basic motor speech performance in premanifest Huntington disease. Neurology, 86(14), 1329–1335.

    Article  Google Scholar 

  • Smith, S., Butters, N., White, R., Lyon, L., & Granholm, E. (1988). Priming semantic relations in patients with Huntington’s disease. Brain and Language, 33(1), 27–40.

    Article  Google Scholar 

  • Snowden, J. S., Austin, N. A., Sembi, S., Thompson, J. C., Craufurd, D., & Neary, D. (2008). Emotion recognition in Huntington’s disease and frontotemporal dementia. Neuropsychologia, 46(11), 2638–2649.

    Article  Google Scholar 

  • Solomon, A., Stout, J., Johnson, S., Langbehn, D., Aylward, E., Brandt, J., et al. (2007). Verbal episodic memory declines prior to diagnosis in Huntington’s disease. Neuropsychologia, 45(8), 1767–1776.

    Article  Google Scholar 

  • Sparbel, K. J. H., Driessnack, M., Williams, J. K., Schutte, D. L., Tripp-Reimer, T., McGonigal-Kenney, M., et al. (2008). Experiences of teens living in the shadow of Huntington disease. Journal of Genetic Counseling, 17(4), 327–335.

    Article  Google Scholar 

  • Speedie, L. J., Brake, N., Folstein, S. E., Bowers, D., & Heilman, K. M. (1990). Comprehension of prosody in Huntington’s disease. Journal of Neurology, Neurosurgery and Psychiatry, 53(7), 607–610.

    Article  Google Scholar 

  • Stengel, E. A. (1947). Clinical and psychological study of echo-reactions. Journal of Mental Science, 93, 598–612.

    Article  Google Scholar 

  • Tannen, D. (2007). Talking voices. Cambridge University Press.

    Book  Google Scholar 

  • Teichmann, M., Dupoux, E., Kouider, S., & Bachoud-Lévi, A.-C. (2006). The role of the striatum in processing language rules: Evidence from word perception in Huntington’s disease. Journal of Cognitive Neuroscience, 18(9), 1555–1569.

    Article  Google Scholar 

  • Teichmann, M., Dupoux, E., Kouider, S., Brugiéres, P., Boissé, M.-F., Baudic, S., et al. (2005). The role of the striatum in rule application: The model of Huntington’s disease at early stage. Brain, 128(5), 1155–1167.

    Article  Google Scholar 

  • Thompson, J. C., Snowden, J. S., Craufurd, D., & Neary, D. (2002). Behavior in Huntington’s disease: Dissociating cognition-based and mood-based changes. Journal of Neuropsychiatry and Clinical Neurosciences, 14(1), 37–43.

    Article  Google Scholar 

  • Trinkler, I. (2016). Huntington’s disease. In W. Douglas & J. Panksepp (Eds.), Psychology and neurobiology of empathy (pp. 349–376). Nova Biomedical Books.

    Google Scholar 

  • Trinkler, I., Cleret de Langavant, L., & Bachoud-Lévi, A.-C. (2013). Joint recognition-expression impairment of facial emotions in Huntington’s disease despite intact understanding of feelings. Cortex, 49(2), 549–558.

    Article  Google Scholar 

  • Trinkler, I., Devignevielle, S., Achaibou, A., Ligneul, R., Brugières, P., Cleret de Langavant, L., et al. (2017). Embodied emotion impairment in Huntington’s disease. Cortex, 92, 44–56.

    Article  Google Scholar 

  • Ullman, M. T. (2001). A neurocognitive perspective on language: The declarative/procedural model. Nature Reviews Neuroscience, 2(10), 717–726.

    Article  Google Scholar 

  • Ullman, M. T., Corkin, S., Coppola, M., Hickok, G., Growdon, J. H., Koroshetz, W. J., et al. (1997). A neural dissociation within language: Evidence that the mental dictionary is part of declarative memory, and that grammatical rules are processed by the procedural system. Journal of Cognitive Neuroscience, 9(2), 266–276.

    Article  Google Scholar 

  • van Bruggen-Rufi, M., Vink, A., Achterberg, W., & Roos, R. (2017a). Improving quality of life in patients with Huntington’s disease through music therapy: A qualitative explorative study using focus group discussions. Nordic Journal of Music Therapy, 27(1), 44–66.

    Article  Google Scholar 

  • van Bruggen-Rufi, M., Vink, A., Wolterbeek, R., Achterberg, W. P., & Roos, R. (2017b). The effect of music therapy in patients with Huntington’s disease: A randomized controlled trial. Journal of Huntington’s Disease, 6(1), 63–72.

    Article  Google Scholar 

  • van Duijn, E. (2017). Medical treatment of behavioral manifestations of Huntington disease. In A. S. Feigin & K. E. Anderson (Eds.), Handbook of clinical neurology, volume 144: Huntington disease (pp. 129–139). Elsevier.

    Google Scholar 

  • van Walsem, M. R., Howe, E. I., Frich, J. C., & Andelic, N. (2016). Assistive technology for cognition and health-related quality of life in Huntington’s disease. Journal of Huntington’s Disease, 5(3), 261–270.

    Article  Google Scholar 

  • Veenhuizen, R. B., Kootstra, B., Vink, W., Posthumus, J., van Bekkum, P., Zijlstra, M., et al. (2011). Coordinated multidisciplinary care for ambulatory Huntington’s disease patients. Evaluation of 18 months of implementation. Orphanet Journal of Rare Diseases, 6, 77. https://doi.org/10.1186/1750-1172-6-77

    Article  Google Scholar 

  • Veenhuizen, R. B., Nijsten, H., van Roosmalen, P., Lammertsen, K., Stor, T., de Jager, L., et al. (2018). Huntington’s disease outpatient clinic for functional diagnosis and treatment: Coming to consensus: How long term care facility procedures complement specialist diagnosis and treatment. Journal of Huntington’s Disease, 7(2), 189–191.

    Article  Google Scholar 

  • Vogel, A., Shirbin, C., Churchyard, A. J., & Stout, J. C. (2012). Speech acoustic markers of early stage and prodromal Huntington’s disease: A marker of disease onset? Neuropsychologia, 50(14), 3273–3278.

    Article  Google Scholar 

  • Wetherby, A. M., Prizant, B. M., & Schuler, A. L. (2000). Understanding the nature of communication and language impairments. In A. M. Wetherby & B. M. Prizant (Eds.), Autism spectrum disorders: A transactional developmental perspective (pp. 109–141). Paul H. Brookes Publishing.

    Google Scholar 

  • Wiig, E., & Secord, W. (1989). Test of language competence (Expanded edition). Psychological Corporation.

    Google Scholar 

  • Williams, J., Driessnack, M., Barnette, J., Sparbel, K., Leserman, A., Thompson, S., et al. (2013). Strategies used by teens growing up in families with Huntington disease. Journal of Pediatric Nursing, 28(5), 464–469.

    Article  Google Scholar 

  • Williams, J., Hamilton, R., Nehl, C., McGonigal-Kenney, M., Schutte, D., Sparbel, K., et al. (2007). “No one else sees the difference”: Family members’ perceptions of changes in persons with preclinical Huntington disease. American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics, 144B(5), 636–641.

    Article  Google Scholar 

  • World Health Organization. (2001). International classification of functioning, disability and health. World Health Organization.

    Google Scholar 

  • Yorkston, K. M., & Beukelman, D. R. (2007). AAC intervention for progressive conditions. In D. R. Beukelman, K. L. Garrett, & K. M. Yorkston (Eds.), Augmentative communication strategies for adults with acute or chronic medical conditions (pp. 317–345). Paul H. Brookes Publishing.

    Google Scholar 

  • Zinzi, P., Salmaso, D., De Grandis, R., Graziani, G., Maceroni, S., Bentivoglio, A., et al. (2007). Effects of an intensive rehabilitation programme on patients with Huntington’s disease: A pilot study. Clinical Rehabilitation, 21(7), 603–613.

    Article  Google Scholar 

  • Zinzi, P., Salmaso, D., Frontali, M., & Jacopini, G. (2009). Patients’ and caregivers’ perspectives: Assessing an intensive rehabilitation programme and outcomes in Huntington’s disease. Journal of Public Health, 17(5), 331–338.

    Article  Google Scholar 

  • Zraick, R., Davenport, D., Tabbal, S., Hutton, T., Hicks, G., & Patterson, J. (2004). Reliability of speech intelligibility ratings using the unified Huntington disease rating scale. Journal of Medical Speech-Language Pathology, 12(1), 31–40.

    Google Scholar 

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Authors and Affiliations

  1. University of Gothenburg, Gothenburg, Sweden

    Charlotta Saldert & Lena Hartelius

  2. Sahlgrenska University Hospital, Gothenburg, Sweden

    Ulrika Ferm

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  1. Charlotta Saldert
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  2. Ulrika Ferm
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Correspondence to Charlotta Saldert .

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  1. Department of English and Communication, The Hong Kong Polytechnic University, Kowloon, Hong Kong

    Louise Cummings

Appendix: Transcription Key

Appendix: Transcription Key

⌈ ⌉ Large brackets link an ongoing utterance with an overlapping utterance

⌊ ⌋

=  Latched utterance with no gap between the adjacent utterances

but-  A dash indicates a cut-off

(0.6) Pauses or gaps in tenths of a second within parentheses

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Saldert, C., Ferm, U., Hartelius, L. (2021). Huntington’s Disease. In: Cummings, L. (eds) Handbook of Pragmatic Language Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-74985-9_18

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