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Speech & Language in HD
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Speech and Language
Of Huntington's Disease
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, distorted vowels
     
  • RESPIRATORY: sudden forced inspiration or exhalation
     
  • PROSODIC: very variable rate, short phrases, inappropriate silences, excess loudness variation, reduced stress
     
  • VOICE: hyper-nasality, 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:
     
  • initiating conversations
  • spontaneity
  • putting thoughts together
  • slow response time
  • understanding complex information
  • impaired reading and writing skills
  • poor attention
  • 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:
    • Feeding
    • Swallowing
    • Motor speech disorders
    • Loudness and articulation
    • Cognition
     
    Source University of Virginia
  • 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 sample.
    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)
  •  
    SPEECH TIPS
    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 devastating.

    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 patience and a little persistence. It is important for a patient with HD to maintain 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!