Huntington’s Disease:
Speech and Voice Intervention
Cross Section Of A Normal Brain
Cross Section Of A Brain Affected By Huntington's Disease
SLPs are involved in the treatment of individuals with Huntington’s Disease (HD) by
managing swallowing and communication abilities, including treatment of
respiration, articulation, phonation, prosody, and resonance as they pertain to
production of voice for speech. HD is rare in that every component of speech
production may be affected, whereas in most other speech disorders, relative
strengths can be noted. Hyperkinetic dysarthria presents in almost every individual with HD.
The following communication problems are found in many individuals with HD:
§ Muscle weakness, slowness, or incoordination of the lips, tongue, throat, and jaw (dysarthria)
§ Disruption in programming and sequencing muscle movements for speech (apraxia)
§ Diminished rate of control (talking too fast or too slowly)
§ Poor voice quality (hoarse/harsh, breathy, volume too low or too high)
§ Problems coordinating breathing and voice
§ Word-finding difficulties
§ Short length of utterance (person only responds with one or two words)
§ Incorrect pronunciation of sounds
§ Problems initiating conversation
§ Getting "stuck" on certain words or phrases, repeating them often and at inappropriate
times (perseveration)
§ Repeating statements (echolalia)
§ Difficulty monitoring pragmatic skills (turn-taking in conversation; reduced ability to
maintain a topic or to switch topics appropriately)
§ Inability to speak
§ Difficulty beginning a word or sentence, with repetition of sounds (stuttering)
§ Difficulty understanding information
§ Difficulty reading and writing
The following list details the most common speech/voice production errors in 30
individuals with hyperkinetic dysarthria of chorea:
· Imprecise consonants
· Prolonged intervals
· Variable pitch
· Monopitch
· Harsh voice quality
· Inappropriate silences
· Distorted vowels
· Excess loudness variation
· Prolonged phonemes
· Monoloudness
· Short phrases
· Irregular articulatory breakdown
· Excess and equal stress
· Hypernasality
· Reduced stress
· Strained-strangled quality
Early intervention is ideal in patients with HD. In the early stages of the disease,
SLPs may teach patients compensatory strategies for the challenges they
experience. These are best when learned in the early stages of HD and then applied throughout the course of the disease. In the later stages of the disease, SLPs are likely to be involved in maintaining and preserving the communication and swallowing abilities of the individual with HD as much as
possible. SLPs may be involved in designing an AAC program if appropriate for a particular patient.
They coach family members and teach communication facilitation techniques
that address speech, language, voice, and cognitive problems.
In the early stages of HD, a patient can be taught the following strategies to
enhance speech and voice to allow functional communication:
· Working on easy, forward prosody
· Maintaining a rate close to 150 syllables per minute seems to help patients achieve a normal
voice to the extent possible. DAF and metronomic pacer of the Facilitator are tools used to help patients learn and maintain a slower speaking rate. A slower, more controlled speaking rate seems to help patients voice become smoother, with less jerkiness which is so troubling for both the speaker and listener.
· The yawn-sigh has been used successfully to open the vocal tract and help a patient
develop ease of voice production.
· Greater intensity also helps many patients enhance their speech and
voice.
In the later stages of the disease, cognition declines, and it is usually futile to
work on speech and voice. Speech intelligibility becomes impossible as severe airflow interruptions and flailing movements worsen dramatically. Improving speech, voice, and functional communication in HD patients is usually not possible after the first few years after onset.
Speech and voice therapy for chorea is largely compensatory in nature.
Respiratory retraining exercise, vocal fold adduction exercise, and oral
motor exercise supplementing pharmacological treatment have been met with
positive clinical outcomes. Patients reported improved breathing,
vocal control, and improved swallow. To maximize speech intelligibility, interaction should be face-to-face
with good eye contact and elimination of background noise when possible.
Speech and voice therapy for Huntington’s chorea is best accomplished when aimed at the
root of the problem, usually phonation and respiration.
Instead of treating the symptom, namely articulation, SLPs will be more
likely to meet with success when targeting compensatory strategies for improved
respiration and phonation.
Everyone involved in the care of the person with HD should be aware of what strategies
are being used to help him or her communicate. Consistency will help the patient
maximize functional communication.
According to Freed, there is little for an SLP to do regarding a patient’s speech in the
early stages of HD, besides maintaining normal prosody and optimal rate. In the middle stages, SLPs may work on speaking on exhalation and not inhalation. Unfortunately, progressive dementia
affects carryover of skills learned in this stage. SLPs may also teach rhythmic breathing and relaxation exercises to maximize intelligibility. They work with family members and other communication partners to facilitate communication using new strategies. Traditional speech therapy may be implemented in this stage, but it should be simplified. SLPs should prioritize goals and select one or two which will most help the individual become a more intelligible and functional communicator.
Talking Mats have been used successfully to support communication in individuals with
Huntington’s Disease by enhancing social interaction and enabling expression of
opinions and decisions.
To help a patient communicate, he or she should be encouraged by SLPs, family
members, and others to do the following (taken from www.asha.org:
§ Speak more slowly
§ Say one word at a time
§ Repeat the word or sentence when necessary
§ Rephrase the sentence
§ Exaggerate the sounds
§ Speak louder (taking a deep breath before speaking)
§ Describe what he or she is trying to say if he or she can' t think of the word
§ Indicate the first letter of the word
§ Use gestures
§ Keep sentences short
§ Use alternative techniques such as word boards, alphabet boards, picture boards, or
electronic devices
As a listener, family members, SLPs, and others should keep in mind the following
strategies to help the person with HD communicate better (taken from
www.asha.org):
§ Eliminate distractions (TV's, radio, large groups of people)
§ Keep questions/statements simple
§ Ask one question at a time
§ Use yes/no question format as much as possible
§ Pay attention to gestures and facial expressions/changes
§ If you do not understand what is being said, don't pretend that you do. Ask for
clarification, or repeat what you think was said in the form of a question, such
as, "Did you say...?"
§ Try to keep to familiar topics
§ Encourage the speaker to use his/her specific compensatory
strategies
§ Allow enough time for the person to convey his/her message
§ Most important, be patient with the speaker
The following video is entitled “Management of Speech Language and Swallowing Disorders in Huntington's
Disease”:
members, and others to do the following (taken from www.asha.org:
§ Speak more slowly
§ Say one word at a time
§ Repeat the word or sentence when necessary
§ Rephrase the sentence
§ Exaggerate the sounds
§ Speak louder (taking a deep breath before speaking)
§ Describe what he or she is trying to say if he or she can' t think of the word
§ Indicate the first letter of the word
§ Use gestures
§ Keep sentences short
§ Use alternative techniques such as word boards, alphabet boards, picture boards, or
electronic devices
As a listener, family members, SLPs, and others should keep in mind the following
strategies to help the person with HD communicate better (taken from
www.asha.org):
§ Eliminate distractions (TV's, radio, large groups of people)
§ Keep questions/statements simple
§ Ask one question at a time
§ Use yes/no question format as much as possible
§ Pay attention to gestures and facial expressions/changes
§ If you do not understand what is being said, don't pretend that you do. Ask for
clarification, or repeat what you think was said in the form of a question, such
as, "Did you say...?"
§ Try to keep to familiar topics
§ Encourage the speaker to use his/her specific compensatory
strategies
§ Allow enough time for the person to convey his/her message
§ Most important, be patient with the speaker
The following video is entitled “Management of Speech Language and Swallowing Disorders in Huntington's
Disease”:
Research completed by Miriam Balsam November 2012.