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Rev. Fac. Med. 2017 Vol. 65 No. 2: 343-8 343

REVIEW ARTICLE

DOI: http://dx.doi.org/10.15446/revfacmed.v65n2.57449

Speech, language and swallowing characteristics

in Huntington's disease
Speech, language and swallowing in Huntington 'Disease
Received : 05/16/2016. Accepted : 08/21/2016.

Maryluz Camargo-Mendoza 1 • Nicolás Castillo-Triana 1 • Juan Miguel Fandiño-Cardona 1 • Angélica Mateus-Moreno 1

Mariana Moreno-Martínez 1

1 Universidad Nacional de Colombia - Bogotá Campus - Faculty of Medicine - Department of Human Communication
- Bogotá DC - Colombia.

Correspondence: Maryluz Camargo-Mendoza. Department of Human Communication, Faculty of Medicine, National University
from Colombia. Carrera 30 No. 45-03, building 471, office 517. Telephone +57 1 3165000, extension 15079, 15194. Bogotá DC Colombia.
Email: mcamargom@unal.edu.co.

| Summary | to show shorter sentences, with much simpler syntactic structures,

Huntington's disease (HD) has been described as a condition
of genetic cause produced by a mutation in the repetition of the
nucleotide sequence CAG (cytosine-adenine-guanine). According
the stage of the disease, people can present
difficulties in speech, language and swallowing. The purpose of
This article is to expose in detail these difficulties, as well as their
speech therapy treatment. It is highlighted that in speech they are found
characteristics of hyperkinetic dysarthria due to
underlying choreic movements. In language, people
with HD they make shorter sentences and with syntactic structures
much simpler and have difficulties in tasks that require
complex cognitive processing. In swallowing, it is presented
a dysphagia that progresses as the disease progresses.
A timely, comprehensive and effective phonoaudiological intervention is
fundamental to improve people's quality of life and contribute
to your communicative well-being.
and difficulties in tasks that require complex cognitive processing.
Moreover, swallowing may present dysphagia that progresses as the
disease develops. A timely, comprehensive and effective speech-
language intervention is essential to improve the quality of life of
people and contribute to their communicative welfare.
Keywords: Huntington Disease; Speech-Language Pathology;
Speech; Language; Deglutition (MeSH).
Camargo-Mendoza M, Castillo-Triana N, Fandiño-Cardona JM,
Mateus-Moreno A, Moreno-Martínez M. [Speech, language and
swallowing in Huntington 'Disease]. Rev. Fac. Med. 2017; 65 (2): 343-8.
Spanish. doi: http://dx.doi.org/10.15446/revfacmed.v65n2.57449.
Introduction

Key words: Huntington's disease; Speech therapy;
Speaks; Language; Swallowing (DeCS).
Camargo-Mendoza M, Castillo-Triana N, Fandiño-Cardona JM, Mateus-
Moreno A, Moreno-Martínez M. Characteristics of speech, language and
swallowing in Huntington's disease. Rev. Fac. Med. 2017; 65 (2): 343-8.
Spanish. doi: http://dx.doi.org/10.15446/revfacmed.v65n2.57449.
| Abstract |
Huntington's disease (HD), also called chorea
Huntington (1) or San Vito's disease (1,2), has been described as a
disease of genetic cause produced by a mutation of the
number of repeats of the CAG nucleotide sequence (cytosine-
adenine-guanine) encoding the huntingtin protein in the short arm
of chromosome 4 (3-5). It is autosomal dominant with
full penetrance (4.6); that is, the person only needs to receive
the abnormal gene from one parent to inherit the disease (7,8),
which implies that it has a heritability percentage of 50%, with
a variable manifestation between 30 and 50 years of age (9).

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3/5/2020 Speech, language and swallowing characteristics in Huntington's disease
Huntington's disease (HD) has been described as a genetic condition
caused by a mutation in the CAG (cytosine-adenine-guanine)
nucleotide sequence. Depending on the stage of the disease, people
may have difficulties in speech, language and swallowing. The
purpose of this paper is to describe these difficulties in detail, as well
as to provide an account on speech and language therapy approach
to this condition. Regarding speech, it is worth noticing that
characteristics typical of hyperkinetic dysarthria can be found due to
underlying choreic movements. The speech of people with HD tends
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344
Three stages of HD have been described: an initial stage that
characterized by presenting small changes in the coordination of
involuntary movements, difficulty solving problems, and
sometimes irritable (12) or depressed mood, apathy
and impulsivity (10); an intermediate stage in which the
choreic movements, speech and swallowing begin to show
affected and cognition is gradually decreased with
appearance of signs such as disorientation and memory loss of
short term (13); and a late stage, evidenced when the disease
has reached its highest level of expression, in which
severe choreic movements and periods of muscle stiffness,
swallowing problems are exacerbated, weight loss occurs and the
ability to walk and speak deteriorates considerably,
what causes the person to become dependent; However,
aspects such as language comprehension and recognition of
loved ones can remain intact (14).
The difficulties are described in more detail below.
in speech, language and swallowing, as well as intervention
speech therapy that is carried out in people with HD.
Speech characteristics
Finding speech disturbances in people with HD is something
so frequent (15,16), it is even mentioned that 90% of
patients present them (17). These alterations are associated with
the presence of the choreic movements and the hypotony that these
they generate, which can interfere with the contributing systems
to oral production (18), for example with the appearance of problems
in phonation (19-21), and can be given before the
diagnosis of the disease or of visible motor signs.
In general, speech difficulties in HD have been known and
framed in the group of dysarthria, specifically in dysarthria
hyperkinetic (15,18,20-22), which may include involvement of the
breathing, phonation, articulation, resonance and prosody
and lead the person to a reduction in communicative beginnings and to
mutism in the final stages of the disease (20).
Breathing
From the perceptual point of view, there is an alteration of the breathing
that is evidenced with a greater effort of the person during the
completion of the respiratory cycle (15). Sometimes it occurs
a transient breath (20), that is, a difficult breath
that appears unpredictably and with the presence of times
uncontrolled characterized by sudden inspiration or expiration
forced during rest, as well as during connected speech.
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HD is neurodegenerative and gives rise to multiple disorders
motor, cognitive and psychiatric (10,11); one of its main
characteristics is the presence of choreic movements that generate
loss of control over voluntary group movements
muscles such as those of the limbs, face, neck and
oral cavity (11), which impairs bodily functions such as
gait, manipulation of objects with hands and the processes of
feeding and swallowing. After the first symptoms appear
disease, the average survival of the person who
presents varies between 15 and 20 years (9).
Huntington: speech, language and swallowing: 343-8
in the periods of vibration caused by the presence of a flow of
turbulent air through the vocal cords.
Joint
People with HD may have distortion in the joint
in the production of both vowel and consonant sounds
(17-19). In diadocokinesis tasks repetition rates are presented
of diminished syllables (23) and difficulty in the tasks that require
alternating repetitive movements. During connected speech,
make shorter broadcasts, with fewer linguistic elements
and that they require less effort, so the duration of the
productions varies, reaching in some cases to be minimal.
Fluency and prosody
Fluency and prosody patterns can be severely found
affected with the presence of a greater number of silences, breaks
inappropriate within and between words, greater prolongation of sounds
and a slower speed or with many variations during speech
(17-19). Of these, the decrease in the speed of speech is perhaps
the most obvious aspect and that people manifest the most
with HD (24).
Linguistic characteristics
Like speech, language can also be affected,
both in understanding and expression. When generating HD
a subcortical deficiency, as well as cognitive impairment, the
Linguistic processing is directly compromised.
In the understanding of language they have been found, in all
stages of the disease, difficulties in passive voice sentences,
with high information load, with ambiguous information (words
homonyms or metaphors) or that demand the elaboration of inferences,
the latter in the context of discourse (25); there are also problems in
Lexical-semantic manipulation (synonyms, word definitions,
absurd semantics and figurative language) and in the interpretation of
propositional meanings (26). Related problems so
close with difficulties in executive care functions
(selective, deferred and sustained), short-term memory and ability to
new learning (11,27) and which are also evident in the
inability to start or plan an activity, and difficulty in
change from one activity to another and in the appearance of behaviors
perseverative (28).
In expressive language there is a reduction in length
mean of the statements, the sentence structure tends to be simple and
give syntactic errors (29.30), so the discourses tend
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Phonation-resonance
The voice can be perceived tense (16,18,20), rough sometimes (19,22),
choppy, strangled, hoarse, with irregular fluctuations of the
tone, with uncontrolled variations in loudness (20) and silences
inappropriate, aspects caused by involuntary contractions of
vocal muscles. Also, the maximum phonation time can be
reduce due to insufficient air flow (20). In relation
with resonance, there may or may not be hypernasality in the different
stages of the disease, depending on the involvement that occurs
in the muscles that participate in the movement of the soft palate.
From an acoustic point of view, there is an increase in intensity
(18) and variability of the fundamental frequency (16,22); in addition,
Jitter and Shimmer measurements are increased, as is the
Measurement of voice signal noise (HNR) due to inaccuracies
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Rev. Fac. Med. 2017 Vol. 65 No. 2: 343-8
not very noticeable and irrelevant accompanied by sialorrhea. At the stage
intermediate, the difficulties become more noticeable and a
progressive dysphagia symptomatology, which evolves and is accentuated
towards the outcome of the disease in the final or late stage, moment
in which difficulties contribute significantly to the
detriment to the state of health of the person.
The main swallowing alterations reported in HD are
relate to neuromotor and behavioral aspects. With
with respect to the former, alterations in the oral phases are evident
and pharyngeal swallowing, such as lack of seal and
lip attachment, postural instability of the tongue, difficulty in
bolus management in the oral cavity and incomplete swallowing processes
which can manifest with residues in the oral cavity, vallecula or
pyriform sinuses (36). Similarly, and as a consequence of a
deterioration in the response of the submental muscle, can
present delayed laryngeal elevation, incomplete airway seal
inferior and low mobility of epiglottic cartilage (37). In the esophageal phase
swallowing, accompanied esophageal hypomotility may occur
spasticity, with the presence of an abnormal bolus transit
viscous, which is generally reported by people with HD
such as the sensation of retro-sternal permanence of the bolus and pain in
the chest; this pain is due in large part to inflammation
esophageal associated with gastroesophageal reflux. With respect to
the second, the behavioral, there may be modification of the
swallowing process due to the presence of eating patterns
fast and uncontrolled, known as tachyphagia (36,38,39).
Thus, in HD it is possible to find a wide range of signs
characteristic of different types of dysphagia, which can
present in isolation in intermediate and simultaneous stages
towards the final stages (Table 1) (40).
Table 1. Signs related to dysphagia in Huntington's disease.
Swallowing stages
Oral high school Oral Pharyngeal Esophageal
-Cough
-Postural instability Choking
-Back to the -Swallowing -Aspiration
pharynx due to voluntary Belching
postural changes deteriorated -Audible swallowing
abrupt -Incoordination -Aerophagia
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to be less informative
irrelevant. and sometimes
Also, grammatical imprecise,
errors can be foundincomplete and
such as the omission of verbs and articles (31) or the inappropriate handling of
verbal suffixes or conjugations (32). When people face
to word production tasks in certain time frames (60
seconds), there is a decrease in production that worsens as
disease progression (33) and there may be a marked difficulty in
those words that refer to lifeless objects (34), an aspect that
It is associated with lexicon recovery problems (30).
Swallowing characteristics
Swallowing problems can be seen from early stages
of HD, even when motor impairment has not become apparent
(35). Thus, in the initial stage there are difficulties in swallowing
3. 4. 5
Swallowing problems are perhaps those that affect life the most
of people with HD, since it is considered that about 86% of
they die from aspiration pneumonia (41) and those who
they do not present respiratory problems, they usually reach states
malnutrition.
Both speech and language difficulties and swallowing
they are usually evaluated and diagnosed in the phonoaudiological consultation
when the person has been confirmed to have HD. Bliss
evaluation, and subsequent treatment, which in principle seeks to contribute
with communicative well-being and improving the quality of life, consists of
different actions described below.
Speech evaluation
Speech assessment includes taking subjective tests
and objective. Among the first is the valuation
perceptual voice through sustained vocalizations,
spontaneous speech, repetition and reading tasks (15, 19);
likewise, carrying out tests of oromotor skills,
intelligibility of speech, of communication functionality,
of participation and interaction (42). In relation to
second, an acoustic analysis of the voice is carried out, which includes
the measurement of the fundamental frequency and the measurements of
disturbance of Shimmer, Jitter and HNR (15-17,19,20), as well as
calculating the number of tonal breaks and analyzing the
fluctuations of the vocal cycle (16). Similarly, within
These tests include acoustic measurement of the speed of the
speech and diadocokinesis index.
Swallowing evaluation
The swallowing evaluation also includes the performance of
subjective and objective tests. Among the first are,
In addition to the physical examination of the phonoarticulating structures, the
cranial nerve examination, behavioral observation
of swallowing, cervical auscultation before, during and after
of swallowing and some tests with different foods
consistencies, textures and volumes. The latter include
cinedeglución, videofluoroscopy, surface electromyography e
gauge impedance.
Therefore, it is of great importance to determine in which phase
or phases are the swallowing difficulties, since, although
mostly presents an oropharyngeal dysphagia, has also been
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-Hyperextension in the in swallowing -Laryngeal lift
found that the anticipatory phases may be compromised,
head and trunk -Short time prolonged
preparatory and esophageal, which compromise safe transit and
-Difficulty oral transit -Difficulty in
control speed -Deglutitions pharyngeal descent -Reflux food efficient.
and the amount of repetitive -Fonation during -Threw up On the other hand, the determination of the degree of severity by which
food in one take -Latens in the swallowing -Early satiety
Dysphagia is a fundamental aspect in the evaluation, for which
-Fast consumption swallowing Pharyngeal stasis -Motility
which are self-report scales that allow you to do it in a way
and impulsive of the -Waste -Function esophageal
food then cryopharyngeal abnormal agile and fast (Table 2) (43).
Signs / symptoms
-Chewing to swallow deteriorated -Korea
inadequate foods -Disability diaphragmatic Language assessment
-Poor lingual control solids to stop the
-Long tongue protrusion -Retention breathing
-Transfer intraoral -Vocal quality
For the evaluation of the alterations in the production of language
premature bolus wet own HD tests are often used that include the description
liquids -Transfer -Korea laryngeal sheet to determine spontaneous production skills
-Delay in lingual -Absence of (29); among them, the Aphasia Diagnostic Profiles and Boston tests
lingual transfer segmented the inclination
Diagnostic Examination for Aphasia. Within the analyzes that
-Korea lingual posterior inferior of the
epiglottis
are made, the average length of the statements is calculated, the
total number of words used, sentence structure and the
Source: Elaboration based on Heemskerk & Roos (40). percentage of correct units of information (Table 3).

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346 Huntington: speech, language and swallowing: 343-8

Table 2. 11-item scale to determine dysphagia in Phonoaudiological treatment

Huntington.
Treatment is a complex process in all diseases
Number Item
neurodegenerative, including HD, since its purpose is
one Do you drool during the day? improve the person's quality of life and seek independence
The most time possible. Also, the complexity is that
Do you feel that you have too much food in your mouth and therefore cannot
2 you must link an interdisciplinary work with different professionals
swallow more?
of health that work in coordination on a plan of
3 Does the food come out of your mouth?
adequate treatment and based on common goals, which
4 Does food or drink come out of your nose?
includes the incorporation of activities not only with the person,
but also with your family or caregivers in different contexts that
5 Is the passage of food or drinks successful for you? allow transfer to everyday life (44).

6 Have you choked on any food or drink?

Speech and communication
7 Do you cough while passing any food or drink?

Within the intervention program, a retraining must be carried out

8 Do you sneeze while passing any food or drink?
respiratory to increase the efficiency of breathing during
Do you sometimes unexpectedly breathe while eating food or speech (45) and, with this, improve the aspects of the voice affected. Of the
9
drinks? that is, do you breathe suddenly when you swallow?
Similarly, the orofacial musculature must be worked to preserve
Does the food or drink sometimes reappear in your mouth, even their mobility both in speech and in swallowing and thus preserve
10
when I had already passed them?
also facial expression. When problems arise in the
eleven Do you feel like there is sometimes a lump in your throat? intelligibility due to an increase in the speed of speech, in addition
to do specific work during treatment in this regard,
Response options:
Questions 1-4 and 6-11: 1. No, almost never; 2. Yes, rarely; 3. Yes, sometimes; 4. Yes, with it is suggested to work with the family or caregivers to monitor
frequency; 5. Yes, almost always. on the person speed and remind him to speak slower; in cases
Question 5: 1. Yes, almost always; 2. Yes, frequently; 3. Yes, sometimes; 4. Yes, rarely; 5. No, where speech becomes very difficult, training is done in
rarely.
the use of, for example, shorter sentences, gestures or
Source: Elaboration based on Heemskerk et al. (43). Augmentative and Alternative Communication (CAA) (23).
In general, the treatment in speech and communication
Table 3. Measures to assess the complexity of oral productions in in HD it can be summarized in the following stages, without forgetting that
Huntington's disease. each person is unique and that HD manifests itself differently
in each subject according to context, interlocutors and difficulties
Words that make sense in context are accurate
Percentage of
in relation to an image or a topic and relevant and cognitive that occur (18):
correct units
informative on the content. The percentage is calculated
of information
over the total number of words used. Initial stage: muscle relaxation, breathing exercises,
It is calculated by dividing the number of informative statements sound and prosodic work.
on the total number of statements. A statement is considered Intermediate stage: counseling to family or caregivers
Proportion of informative as long as it provides different information, emphasis on communication settings in school activities
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statements accurate and represented in the image or theme.
informative Incomplete, irrelevant productions with information
imprecise, repeated or with personal beliefs are rated
as non-informative.
Number of A sentence is considered correctly stated
statements produced and with descending intonation.
It is the number of words used in the description of the
Number of words
image. Interjections are not included, eg. ex. Ehhh.
Structure of
a) Simple: they have canonical structure subject + verb + object
and b) complex: non-canonical structures follow, for example
prayers
in passive voice or subordinate sentences.
Average length of
The number of morphemes is calculated. Excluded from count
perseverative productions, false starts,
statement
interjections and incomplete words or false words.
Source: Elaboration based on Murray (29).
Regarding the assessment of understanding, it is necessary to perform
tests that determine the person's performance in tasks with
elaborate language such as metaphors, logical sentences-
grammatical and with inferential meanings. This is because in
routine language comprehension tests, such as those
assess aphasia, people with HD tend to perform
suitable (25).
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Rev. Fac. Med. 2017 Vol. 65 No. 2: 343-8
neck neutral - or with the use of the Chin Tuck maneuver - to
reduce the range of movement of the phonoarticulating organs
and make food intake safer (46).
Sensory stimulation: the use of cold and acidic substances
stimulates swallowing in its oral (improves its onset) and pharyngeal phases
(reduces its delay), while reducing movements
involuntary oropharyngeal (47) and the aspiration frequency of the
food bolus (48).
Modifications in food consistency: this aspect is
fundamental since there is a close relationship between viscosity
of food and the probability of aspiration, the more viscous the
food, less probability of penetration into the lower airway,
even when laryngeal residue is present (49).
In addition to the above strategies, it is recommended that
people with HD do the following (28):
1. Avoid or reduce the consumption of anticholinergics due to
dryness produced in the oral cavity (should be consulted
this aspect with the treating doctor).
2. Limit the duration of the meal to 30 minutes,
this with the purpose of reducing the fatigue and frustration that
It can produce a long feeding process.
3. Avoid distractions during the meal, since the process of
feeding becomes more complex and its success depends largely
part of the concentration of the person with HD and the caregiver.
4. After the meal is over, they should keep
a vertical position to prevent regurgitation.
Finally, swallowing treatment not only improves
active participation of the person (independence) during
feeding, but reduces the risk of food aspiration and
gastroesophageal reflux (50).
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daily life.
Late stage: implementation of CAA strategies and systems
to increase not only communicative effectiveness through visual support
they represent (23), but also to enable the person
participate in social and recreational activities. This is because, as
As already mentioned, oral communication may not be possible due to
neurodegeneration of the structures required for speech (16).
Swallowing
In people with HD, the application of
certain strategies to facilitate and guarantee an effective swallowing and
safe. Among these are:
Compensatory maneuvers: aimed at the re-education of the
swallowing and modifying eating behaviors
such as bringing solid food directly to the
the molars to ensure that they are crushed and do not remain
in a quantity / size that may exacerbate difficulties
swallowing (40).
Postural compensatory techniques: aimed at minimizing
involuntary movements (38) by positioning
proper limb and posture assurance
347
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conclusion
HD is a neurodegenerative disease that affects
direct speech and language functions in people who
present, which may include difficulties in breathing, voice,
articulation, resonance, fluency, prosody, understanding
and expression of language, higher executive functions and gait
food insurance.
The detection of these difficulties is carried out from tests
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Conflict of interests
None declared by the authors.
Financing
This review was carried out within the framework of the “National Program
of Research, Creation and Innovation Seedbeds of the
Universidad Nacional De Colombia 2013-2015 ”of the Faculty of
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Thanks
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