Most often patients diagnosed with Huntington's Disease have difficulty
speaking due to a motor speech disorder referred to as Hyperkinetic Dysarthria.
Hyperkinetic Dysarthria may sound as if normal speech is being attempted
but the extra movements involved in the disease (chorea) are interfering with the process and cause decreased intelligibility.
Speech characteristics of patient's with Huntington's Chorea are often:
ARTICULATORY: imprecise consonants, prolonged phonemes,
RESPIRATORY: sudden forced inspiration or exhalation
PROSODIC: very variable rate, short phrases, inappropriate
silences, excess loudness variation, reduced stress
VOICE: hypernasality, hoarse or strained voice quality,
rhythmic phonatory interruption, oscillations of vocal folds are seen with tremor or chorea.
Language skills are affected by the cognitive changes
consistent with Huntington's Disease.
The thought processes and cognitive skills affected by Huntington's Disease are needed
to formulate clear thoughts. Patients often have trouble:
putting thoughts together
slow response time
understanding complex information
impaired reading and writing skills
inability to reason, problem solve, and organize
All of the above examples may or may not happen in each case of HD and the unpredictably
can be frustrating to both speaker and listener.
A speech pathologists can provide assessment and
intervention in the following areas:
- Motor speech disorders
- Loudness and articulation
Speech and Articulation Evaluations:
- A patient will be evaluated via a language sample (perhaps Grandfather
passage) to determine any articulation errors.
- Patient will undergo an oral motor exam to determine any muscle weakness
and/or inadequate lingual movement.
- Patient can be asked to spontaneously respond to questions or describe
a picture to determine language function.
- Patient will be evaluated on loudness and rate via the above language
As well as the above, procedures Speech Language Pathologists are
encouraged to use standardized tests of intelligibility and receptive and expressive language such as:
DCT- Discourse Comprehension Test
Frenchay Test of Intelligibility
Arizona Battery for Communication Disorders of Dementia
Aphasia Diagnostic Procedures or other aphasia battery (although these
patients do not have a diagnosis of Aphasia this test can offer helpful information about language skills)
Communication Strategies for Patients and Caregiver dealing with
Hyperkinetic Dysarthria Associated with Huntington's Disease
For patients who are dealing with a degenerative disease there is often
a struggle for control of their life. The loss of independence is devestating.
Caregivers and family members must understand that the ability for their
loved ones with HD to communicate is integral to their mental health.
Communicating with someone with HD may take patiance and a little persistance.
It is important for a patient with HD to maintian some dignity and independence. Implementing communication strategies and
involving a Speech/Language Pathologist helps in creating a healthy and effective communicative environment.
Remember: patients with HD know what is going on around
them to a greater degree then some may expect so, BE RESPECTFUL!