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 Parkinson’s
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 can’t hear me
 People don’t understand me
 I can’t 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 Parkinson’s 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
 Don’t 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