Language impairment in Huntington's disease

Azambuja, Mariana Jardim; Radanovic, Márcia; Haddad, Mônica Santoro; Adda, Carla Cristina; Barbosa, Egberto Reis

doi:10.1590/s0004-282x2012000600006

Cited by 21 publications

(23 citation statements)

References 19 publications

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“…Syntactic processing was evaluated through two subtests from an extended version of the Boston Diagnostic Aphasia Examination (Goodglass, Kaplan, & Barresi, ): ‘Touching A with B’ and ‘Embedded Sentences’. These tasks have evinced subtle deficits in samples with frontostriatal alterations, including patients with HD (Azambuja et al ., ), patients with PD (Bocanegra et al ., ), and even asymptomatic PD gene carriers (García et al ., ). We calculated a global syntactic score by adding the participants’ scores in each subtest, and we also assessed their performance on each subtest separately.…”

Section: Methodsmentioning

confidence: 99%

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Action‐semantic and syntactic deficits in subjects at risk for Huntington's disease

García

Bocanegra

Herrera

et al. 2017

Journal of Neuropsychology

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Frontostriatal networks play critical roles in grounding action semantics and syntactic skills. Indeed, their atrophy distinctively disrupts both domains, as observed in patients with Huntington's disease (HD) and Parkinson's disease, even during early disease stages. However, frontostriatal degeneration in these conditions may begin up to 15 years before the onset of clinical symptoms, opening avenues for pre-clinical detection via sensitive tasks. Such a mission is particularly critical in HD, given that patients' children have 50% chances of inheriting the disease. Against this background, we assessed whether deficits in the above-mentioned domains emerge in subjects at risk to develop HD. We administered tasks tapping action semantics, object semantics, and two forms of syntactic processing to 18 patients with HD, 19 asymptomatic first-degree relatives, and sociodemographically matched controls for each group. The patients evinced significant deficits in all tasks, but only those in the two target domains were independent of overall cognitive state. More crucially, relative to controls, the asymptomatic relatives were selectively impaired in action semantics and in the more complex syntactic task, with both patterns emerging irrespective of the subjects' overall cognitive state. Our findings highlight the relevance of these dysfunctions as potential prodromal biomarkers of HD. Moreover, they offer theoretical insights into the differential contributions of frontostriatal hubs to both domains while paving the way for innovations in diagnostic procedures.

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Section: Methodsmentioning

confidence: 99%

“…Semantic and syntactic domains were evaluated through tasks that have revealed subtle deficits in neurodegenerative motor diseases, including HD (Azambuja et al, 2012; Kargieman et al, 2014), PD (Bocanegra et al, 2015;Cardona et al, 2014;Ib añez et al, 2013), and motor neuron disease (Bak & Hodges, 2004).…”

Section: Assessment Of Semantic and Syntactic Skillsmentioning

confidence: 99%

Action‐semantic and syntactic deficits in subjects at risk for Huntington's disease

García

Bocanegra

Herrera

et al. 2017

Journal of Neuropsychology

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“…Language comprehension deficits have also been reported (Teichmann et al, 2008; Azambuja et al, 2012). Moreover, late-stage HD has been associated with action–verb generation deficits (Peran et al, 2004), and action naming was found to be the task that best discriminated controls and HD patients (HDP) (Azambuja et al, 2012). Nevertheless, the relationship between action–language and motor action remains poorly explored.…”

Section: Introductionmentioning

confidence: 96%

“…In HD, typical motor alterations and cognitive deficits (Bachoud-Levi et al, 2001; Montoya et al, 2006) are accompanied by disorders in verbal production (Podoll et al, 1988; Murray and Lenz, 2001), including reduced verbal fluency (Chenery et al, 2002; Ho et al, 2002; Azambuja et al, 2012), affixation errors (Ullman et al, 1997), and syntactic processing difficulties (Teichmann et al, 2005, 2008). Language comprehension deficits have also been reported (Teichmann et al, 2008; Azambuja et al, 2012).…”

Section: Introductionmentioning

confidence: 99%

Motorâ€“Language Coupling in Huntingtonâ€™s Disease Families

Kargieman

Herrera

Báez

et al. 2014

Front. Aging Neurosci.

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Traditionally, Huntington’s disease (HD) has been known as a movement disorder, characterized by motor, psychiatric, and cognitive impairments. Recent studies have shown that motor and action–language processes are neurally associated. The cognitive mechanisms underlying this interaction have been investigated through the action compatibility effect (ACE) paradigm, which induces a contextual coupling of ongoing motor actions and verbal processing. The present study is the first to use the ACE paradigm to evaluate action–word processing in HD patients (HDP) and their families. Specifically, we tested three groups: HDP, healthy first-degree relatives (HDR), and non-relative healthy controls. The results showed that ACE was abolished in HDP as well as HDR, but not in controls. Furthermore, we found that the processing deficits were primarily linguistic, given that they did not correlate executive function measurements. Our overall results underscore the role of cortico-basal ganglia circuits in action–word processing and indicate that the ACE task is a sensitive and robust early biomarker of HD and familial vulnerability.

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“…23 HH'li hastada (15 tanesinin genetik tanısı doğrulanmış) dil fonksiyonlarını incelemek için farklı dil testleri uygulamışlar [10]. HH'li grupta sözel akıcılık, sözel anlama, tekrarlama, oral esneklik, okuma anlama ve öyküleyici yazım performansları daha kötü bulunmuş.…”

Section: Discussionunclassified

Evaluation of language functions in Huntington's disease

Gültekın¹

2018

Pau Med J

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ÖzetAmaç: Bu çalışmanın amacı; genetik tanıları doğrulanmış olan HH'li hastalarda lisan fonksiyonlarının kontrol grubu ile karşılaştırarak değerlendirmektir. Gereç ve Yöntem: Çalışmaya HH tanısı genetik test ile doğrulanan 15 HH ve 15 sağlıklı kontrol grubu alındı. Katılımcıların lisan işlevlerini değerlendirmeye yönelik Addenbrook Kognitif Muayenesi (ACE-R, sözel akıcılık ve dil bölümleri) ve Mini Mental Durum Değerlendirmesi (MMDM) testlerini kapsayan bir batarya oluşturulup uygulandı. Bulgular: Her iki grupta 9 erkek ve 6 kadın bireyden oluşuyordu. Hasta ve kontrol grubunun yaş ortalaması 48 bulundu. Hasta grubunda ortalama hastalık süresi 5 yıldı. Hastaların ortalama CAG tekrar sayıları 43.3 ± 3.5 bulundu. ACE-R dil testi performansına göre gruplar karşılaştırıldığında HH'li hastaların skorları istatistiksel olarak daha düşük bulundu. ACE-R sözel akıcılık testinde ise benzer olarak yine hasta grubunun performans skorları daha düşük bulundu. Ancak bu fark istatistiksel olarak anlamlı değildi. Hastaların CAG tekrar sayıları artıkça, tüm test süreleri de daha fazla zaman alıyordu. Ancak dil testi ve sözel akıcılık arasında pozitif korelasyon bulunmadı. Sonuç: HH'da lisan fonksiyonları kliniği hafif olan ve CAG tekrar sayısı az olan olgularda bile bozulmaktadır. Bu durum sadece iletişimi değil değil aynı zamanda hastanın günlük yaşam aktivitelerini ve hastalık yükünü de olumsuz etkilemektedir. Pam Tıp Derg 2018;11(2):145-149Anahtar sözcükler:Huntington hastalığı, lisan, kognisyon Abstract Purpose: The purpose of this study is to evaluate language functions in Huntington's disease (HD) patients whose genetic diagnoses confirmed and compared to control group. Materials and methods: Fifteen patients confirmed with genetic testing and 15 healthy group were included in the study. A battery containing the Addenbrook Cognitive Examination (ACE-R, verbal fluency and language part) and the Mini Mental State Examination (MMSE) tests were used to assess participants' language functions. Results: There were 9 males and 6 females in each group. The average age of the patient and control group was 48. The mean duration of disease in the patient group was 5 years. Mean CAG repetetion of the patients was found 43.3 ± 3.5. When the groups were compared according to ACE-R language test performance, scores of HD patients were found to be statistically lower. Also, the performance scores of the patient group in ACE-R verbal fluency test was similarly lower. However, this difference was not statistically significant. If the patients' CAG repeat numbers were high, all the test times were taking longer time. As a result there was no positive correlation between language test and verbal fluency. Conclusion: Language functions in HD are disturbed even in patients with mild clinic and low CAG repetition count. This affects not only the communication but also the daily living activities and disease burden.

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Language impairment in Huntington's disease

Cited by 21 publications

References 19 publications

Action‐semantic and syntactic deficits in subjects at risk for Huntington's disease

Action‐semantic and syntactic deficits in subjects at risk for Huntington's disease

Motorâ€“Language Coupling in Huntingtonâ€™s Disease Families

Evaluation of language functions in Huntington's disease

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