Nav: Home

Language disorders as indicators of the diagnosis and progression of Huntington's disease

February 20, 2020

Huntington's disease is a hereditary neurodegenerative disorder caused by a gene of chromosome 4 that affects a very important area of the brain, the striatum. People are born with the defective gene but symptoms do not appear until the age of 30 or 40.

This disease, in addition to motor impairments, cognitive and affective problems, also involves changes in language. A study shows that the first symptoms of the disease are revealed through linguistic changes in spontaneous speech.

So reveals a study published in Journal of Communication Disorders by Antonia Tovar as first author, led by Wolfram Hinzen, ICREA research professor, both researchers with the Department of Translation and Language Sciences at UPF, involving researchers from the universities of Paris-Saclay (Orsay, France), the UAB, the UB, together with the Hospital Mare de Déu de la Mercè in Barcelona and FIDMAG Sisters Hospitallers Research Foundation (Barcelona).

"Linguistic impairments in Huntington's disease arise before the onset of motor impairment and even when neuropsychological tests prove normal. This suggests that language may be a biomarker for the progression of Hungtinton's", Tovar and Hinzen explain.

Specific language patterns of Huntington's disease

In addition to showing that linguistic impairments in Huntington's disease arise before the onset of motor impairment, the authors have sought to characterize the nature of these specific changes in Huntington's disease. Thus "language needs to be assessed as a multi-dimensional construct organized at multiple levels (phonology, morphology, syntax, semantics, discourse), which are re-fitted together into an integrated functional whole", the authors point out.

The few existing linguistic studies on spontaneous speech in Huntington's disease have reported language shortcomings. For example patterns of reduced syntactic complexity, with fewer words and syntactic structures formed in short, simple sentence constructions, more paraphasic and grammatical errors, etc. These language deficits may form a distinctive signature profile of Huntington's as compared with Parkinson's disease or people with cerebral lesions.

To study the language profiles of Huntington's disease, the authors designed an experiment with 20 HD gene-carriers, all native Spanish speakers; 10 in the early stages of the disease and another 10 with pre-manifest Huntington's disease, and their respective neurotypical controls not carrying the gene, and compared them regarding the structure and function of language in spontaneous speech. The researchers also studied the relationship between language and non-linguistic cognitive impairment.

"This disease is generally accepted to begin with the onset of motor symptoms, but we have found differences in the language profile of subjects without motor symptoms", says Tovar, first author of the article.

In addition to proving that in Huntington's disease linguistic changes precede other cognitive and motor impairments, it has been shown that language impairments are in the areas of basic grammar organization and cannot be detected with standard neurocognitive tests. This study is important because it has managed to capture subtle changes in the patient's linguistic profile that could not be detected using the tests generally conducted in this population.

A more detailed and differentiated profile of the linguistic phenotype of this disease

The results showed that while the patients mentioned have "pauses" in their speech, at the beginning of Huntington's disease they tend to be filled through prolongations and repetitions. Therefore, the study provides further support for the disintegration of language in Huntington's disease and contributes to a more detailed and differentiated profile of the linguistic phenotype of the disease.

Since language skills mainly have an impact on communication skills, the authors conclude that special attention must be paid to the early detection of Huntington's disease by means of clinical linguistic tests and the sensitivity and specificity of language as a marker for the progression of the disease must continue to be systematically investigated.
Reference work:

Antonia Tovar, Aina Garí Soler, Jesús Ruíz-Idiago, Celia Mareca Viladrich, Edith Pomarol-Clotet, Joana Rosselló, Wolfram Hinzen (2020), " Language disintegration in spontaneous speech in Huntington's disease: a more fine-grained analysis", Journal of Communications Disorders, Volum 83, January-February,

Universitat Pompeu Fabra - Barcelona

Related Language Articles:

How effective are language learning apps?
Researchers from Michigan State University recently conducted a study focusing on Babbel, a popular subscription-based language learning app and e-learning platform, to see if it really worked at teaching a new language.
Chinese to rise as a global language
With the continuing rise of China as a global economic and trading power, there is no barrier to prevent Chinese from becoming a global language like English, according to Flinders University academic Dr Jeffrey Gil.
'She' goes missing from presidential language
MIT researchers have found that although a significant percentage of the American public believed the winner of the November 2016 presidential election would be a woman, people rarely used the pronoun 'she' when referring to the next president before the election.
How does language emerge?
How did the almost 6000 languages of the world come into being?
New research quantifies how much speakers' first language affects learning a new language
Linguistic research suggests that accents are strongly shaped by the speaker's first language they learned growing up.
Why the language-ready brain is so complex
In a review article published in Science, Peter Hagoort, professor of Cognitive Neuroscience at Radboud University and director of the Max Planck Institute for Psycholinguistics, argues for a new model of language, involving the interaction of multiple brain networks.
Do as i say: Translating language into movement
Researchers at Carnegie Mellon University have developed a computer model that can translate text describing physical movements directly into simple computer-generated animations, a first step toward someday generating movies directly from scripts.
Learning language
When it comes to learning a language, the left side of the brain has traditionally been considered the hub of language processing.
Learning a second alphabet for a first language
A part of the brain that maps letters to sounds can acquire a second, visually distinct alphabet for the same language, according to a study of English speakers published in eNeuro.
Sign language reveals the hidden logical structure, and limitations, of spoken language
Sign languages can help reveal hidden aspects of the logical structure of spoken language, but they also highlight its limitations because speech lacks the rich iconic resources that sign language uses on top of its sophisticated grammar.
More Language News and Language Current Events

Trending Science News

Current Coronavirus (COVID-19) News

Top Science Podcasts

We have hand picked the top science podcasts of 2020.
Now Playing: TED Radio Hour

Warped Reality
False information on the internet makes it harder and harder to know what's true, and the consequences have been devastating. This hour, TED speakers explore ideas around technology and deception. Guests include law professor Danielle Citron, journalist Andrew Marantz, and computer scientist Joy Buolamwini.
Now Playing: Science for the People

#576 Science Communication in Creative Places
When you think of science communication, you might think of TED talks or museum talks or video talks, or... people giving lectures. It's a lot of people talking. But there's more to sci comm than that. This week host Bethany Brookshire talks to three people who have looked at science communication in places you might not expect it. We'll speak with Mauna Dasari, a graduate student at Notre Dame, about making mammals into a March Madness match. We'll talk with Sarah Garner, director of the Pathologists Assistant Program at Tulane University School of Medicine, who takes pathology instruction out of...
Now Playing: Radiolab

What If?
There's plenty of speculation about what Donald Trump might do in the wake of the election. Would he dispute the results if he loses? Would he simply refuse to leave office, or even try to use the military to maintain control? Last summer, Rosa Brooks got together a team of experts and political operatives from both sides of the aisle to ask a slightly different question. Rather than arguing about whether he'd do those things, they dug into what exactly would happen if he did. Part war game part choose your own adventure, Rosa's Transition Integrity Project doesn't give us any predictions, and it isn't a referendum on Trump. Instead, it's a deeply illuminating stress test on our laws, our institutions, and on the commitment to democracy written into the constitution. This episode was reported by Bethel Habte, with help from Tracie Hunte, and produced by Bethel Habte. Jeremy Bloom provided original music. Support Radiolab by becoming a member today at     You can read The Transition Integrity Project's report here.