Speech and Swallowing Issues

in Wilson Disease
Kristin Larsen, MA CCC-SLP
Communication Sciences and
Disorders
Northwestern University

Speech Problems in Wilson

Disease
Common sign of neurological involvement
Dysarthria: refers to speech disorders of a
neurological origin resulting from disturbances in
muscular control of the speech mechanism
May be hypokinetic, spastic, ataxic--usually a
combination

Hypokinetic Dysarthria
Caused by damage to the basal ganglia
control circuit
Most frequently found in Parkinsons
Disease or other related CNS degenerative
disorders
Hypokinetic refers to decreased mobility or
range of motion--decreased amplitude of
speech

Hypokinetic Dysarthria
Characteristics

Reduced loudness
Monopitch
Monoloudness
Imprecise consonant
articulation
Fast speech rate

Short rushes of speech

Lower pitch
Palilalia
Breathy voice quality

Hypokinetic Dysarthria-patient
perceptions
People cant hear me
People dont understand me
I cant communicate well in large groups or
in public places
My spouse/parent needs a hearing aid!

Hypokinetic Dysarthria
Treatment
Generally focuses on increasing
loudness/effort, reducing speech rate and
improving articulation
Key focus: Think loud, Be loud
LSVT: Speech/voice treatment program
developed for Parkinsons disease, but has
proven useful in related diseases

Spastic Dysarthria
Caused by damage to direct and indirect
activation pathways of the CNS-bilaterally
Found in vascular disorders, inflammatory
diseases and degenerative disorders
Spastic refers to excessive muscle tone

Spastic Dysarthria Characteristics

Strained-strangled
voice quality
Harshness
Slow rate
Imprecise consonant
articulation
Distorted vowels

Hypernasality
Short Phrases
Pitch breaks
Excess and equal
stress
Monopitch
Monoloudness

Spastic Dysarthria-patient
perceptions

It takes more effort to speak

I speak so slowly
I get tired quickly from talking
My speech sounds nasal
Difficulty controlling emotional expression
Often complains of difficulty chewing or
swallowing as well

Spastic Dysarthria-Treatment

Reduce muscle tone

Relaxation techniques
Easy onset of phonation
Gentle stretching/range of motion exercisesnot to the point of fatigue

Ataxic Dysarthria
Caused by damage to the cerebellar control
circuit
Found in degenerative diseases, vascular
disorders, neoplastic disorders, toxicmetabolic conditions and trauma
Characterized by reduced coordination of
speech

Ataxic Dysarthria Characteristics

Irregular articulatory breakdown

Vowel distortions
Prolonged sounds
Slow rate
Monopitch/monoloudness
Excess and equal stress

Ataxic Dysarthria-patient
perceptions

Slurred speech
Drunken sounding speech
Stumbling over words
Reduced coordination with chewing

Ataxic Dysarthria Treatment

Focuses on modifying rate and prosody
Slow down!
Pitch control

Speech Therapy
Diagnosis of speech problem: will
determine treatment plan
Treatment: will focus on compensation,
augmentation or exercise program as
appropriate
Compensations must be practiced
frequently to be habituated

General Communication
Strategies for Dysarthria

Slow down
Take a breath before you start talking
Pause for a new breath as needed
Exaggerate your speech
Control your environment--avoid competing
noise when possible

General Communication
Strategies for Dysarthria

Set the context: what is the main idea?

Modify the length of the utterance
Monitor listener comprehension
Use letter/word/picture board or gestures to
supplement verbal communication

Strategies for the Listener

Modify the environment-reduce excess
noise/distractions, maintain adequate
lighting
Maintain eye contact
Repeat or clarify the message--let the
speaker know what parts you understood
Ask focused questions to clarify message

More Strategies for the Listener

Establish how and when to provide
feedback
Encourage use of appropriate strategies
Model appropriate strategies
Encourage use of augmentative
communication as needed

Augmentation-when useful
speech is limited:low tech

Writing
Letter/picture board
Personalized communication book
Develop consistent yes/no response
Use gestures

Augmentation-when useful
speech is limited: high tech
Alternative and augmentative
communication (AAC) devices
Computer systems: variable expense, level
of difficulty
Speech software for existing computers
Smart phone applications

AAC Device Considerations

Input or access features: how to select
letters/words/pictures--direct or scanning.
Output features: voice or readable
Portability
Cost/funding and insurance coverage
Training or learning curve: how easy is it to
operate?

Dysphagia
Difficulty with any phase of swallowing
May result in aspiration:food or liquid
entering the airway-can lead to pneumonia
May result in inefficiency-can lead to
longer mealtimes, weight loss, malnutrition

Dysphagia in Wilson Disease

Swallowing difficulty is a common
complaint with neurologic manifestation of
Wilson Disease
Can vary from mild to severe
May or may not be accompanied by
difficulty with secretion
management/drooling

Dysphagia in Wilson Disease

Can involve any stage of swallowing: oral
prep/chewing, oral transit, or pharyngeal
Involvement of the basal ganglia can impair the
coordination of chewing and swallowing
Dystonia affecting head or neck muscles can
affect ability to swallow safely
Pseudobulbar palsy-weakness in lips, tongue or
throat muscles can reduced efficiency and lead to
aspiration

Role of SLP in Dysphagia

Management
Assessment: clinical, endoscopic or
videofluoroscopic
Develop appropriate treatment plan:
compensations (postures, maneuvers), diet
modifications
Monitor progression of swallowing
problems
Monitor need for possible non-oral nutrition

Early Signs of Dysphagia

Longer mealtimes
Coughing with liquids
Difficulty with chewier foods
Difficulty with mixed consistencies (cereal
in milk, chunky soups)
Feeling food or pills sticking in throat
Coughing during or after meals

Signs of Advanced Dysphagia

Aspiration
Decrease in caloric intake (weight loss,
malnutrition)
Decrease in fluid intake (dehydration)
Fatigue or excessive inefficiency with
mealtimes--unable to meet nutritional needs

Swallowing Guidelines: Posture

Sit as upright as possible
Keep head in a neutral or slightly chin down
position if indicated/possible
Stay sitting upright for 30 minutes after
meals to allow time for all the food to go
down (if any food remaining in mouth or
throat

General Swallowing Guidelines

Eat and drink slowly-allow plenty of time
for meals
Chew thoroughly
Focus on the task of eating-eliminate
distractions like TV
Dont talk with food or liquid in your mouth
Swallow everything in your mouth before
taking a new bite/sip

Diet Modification Guidelines

Caution with mixed consistencies

May need to choose softer foods
May need to thicken liquids
Smaller, more frequent meals if fatigued
Nutritional supplements--drinks or puddings
(try to avoid ones with added copper)

Non-oral Nutrition
If aspiration, malnutrition, dehydration or
inefficiency become a problem
Surgical placement of a gastrostomy or
jejeunostomy tube for nutrition
Highly personal decision, quality of life
considerations
May still be able to take some foods/liquids by
mouth

Drooling/Saliva Management

Medications
Botox
Radiation to salivary glands
Maintain adequate hydration
Use suctioning as needed
Secretions management techniques

Secretion Management
Techniques
SWALLOW! Remind yourself to slurp and
swallow throughout the day--especially
before you speak
If able, try to sip water frequently
If able, chewing gum or sucking on a hard
candy can increase swallow frequency