Chapter 14

Question 1

Amyotrophic Lateral Sclerosis (ALS)

Answer 1

ALS is a progressive, degenerative disease of unknown etiology

Involves the motor neurons of the brain and spinal cord

Question 2

Communication Symptoms ALS

Answer 2

Speech symptoms differ among individuals

80%-95% are unable to speak by the time of their death

Cognitive changes do occur in some people with ALS

Aphasia may occasionally be associated with ALS

Question 3

A Model for AAC Intervention - ALS

Answer 3

3-phase intervention model

Early phase – monitor, prepare, support; initial diagnosis through referral for an AAC assessment

Middle phase – assess, recommend, implement; time from referral for AAC assessment until AAC strategies are selected, purchased, and initial instruction is completed

Late phase – adapt, accommodate; time after initial AAC intervention until the individual’s death

Question 4

Early Phase ALS

Answer 4

1. Monitor speech performance –
   Use objective measures to monitor speaking rate and intelligibility
2. Make timely AAC referral -
   Refer when speaking rate reaches 125 words/minute
   For a small percentage severe dysarthria reduces speech intelligibility prior to significant rate reduction and it is recommended that referral for an AAC assessment be made when sentence intelligibility drops below 90% regardless of rate
3. Preserve natural speech effectiveness –
   amplify residual speech and allocate time for social or informational exchanges
4. Educate about AAC –
   Info about natural speech deterioration, timing of AAC assessment, low- and hig-tech AAC options, and technology purchases (funding)

Question 5

Middle Phase ALS

Answer 5

ID participation patterns and needs - Assess portability, telephone and internet access, aids to daily living

Current and anticipated capabilities –

Bulbar ALS – will typically be able to control AAC technology, carry and operate with direct selection, and then move towards head/eye tracking access

Spinal ALS – need for augmented writing system often precedes need for augmented conversational system; usually require mounted system with head/eye tracking access

Assess constraints – attitudes of family members and friends, availability of facilitators, funding

Evaluate intervention outcomes – to know which direction to go in, to meet funding requirements, to set precedent for a center or agency

Social and personal care supports

Selecting low- and high-tech AAC options

Question 6

Late Phase ALS

Answer 6

Adapt and Accommodate

Consider acceptance of mechanical ventilation

Within last few weeks of life people with ALS depend ore on low-tech options

Question 7

Multiple Sclerosis

Answer 7

Most common neurological condition of young and middle-age adults

Of unknown cause

Acquired, inflammatory, demyelinating disease of the central nervous system

Symptom patterns vary

Prognosis is worse:

• In males than females
• If the age at onset is greater than 35 years
• If a chronic, progressive pattern appears at onset

Question 8

Communication Symptoms MS

Answer 8

Dysarthria is most common but is not universal

Relatively small number of individuals with MS require AAC systems

Question 9

Early, Middle, and Late Phase Interventions MS

Answer 9

Early – may require supports like font enlargement, TTS, modified keyboard access (Ease of Access Center), memory and organizational supports

Middle – alphabet supplementation (ID first letter)

Late – AAC interventions are very personalized, depending on need and capabilities of person; primary communication needs of many people with MS are conversational and care related; some may require assistance with writing

Question 10

Assess Prior to AAC Intervention MS

Answer 10

Cognitive Skills – memory retrieval impairment, impaired conceptual reasoning, slowed information-processing time

Language Skills – aphasia occasionally reported (1%-3%), difficulty with word finding and changes in verbal or written organization

Sensory/Perceptual Skills – loss of vision presents problems in the context of AAC interventions and require auditory scanning options with speech feedback

Motor Skills – vary, common to have tremor, may need to stabilize body part to support access

Question 11

Guillain-Barre’ Syndrome

Answer 11

Results from progressive destruction and subsequent regeneration of the myelin sheath of peripheral nerve axons

Paralysis progresses from the lower extremities upward, maximal paralysis usually occurs within 1-3 weeks of onset

Nerve function and associated muscle strength gradually return as the myelin sheath slowly regenerates

Question 12

Communication Disorders GB

Answer 12

Weakness causes flaccid dysarthria and may cause anarthria

Question 13

Early Phase GB

Answer 13

Deterioration Phase/Loss of Speech

After a few weeks, those who require AAC intervention are usually unable to speak and receive respiratory support from a ventilator

Intervention starts with low-tech - establishing yes/no followed by eye-pointing/linking, creation of communication boards with social and health-related messages as well as letters and numbers

Question 14

Middle Phase GB

Answer 14

Prolonged Speechlessness, Spontaneous Recovery of Speech

Time frame varies (weeks/months)

Continue using low-tech but may opt for higher tech, scanning with eye control (funding? Borrow program?)

As speech returns may use a ventilator and a tracheostomy tube for breathing; some use an oral-type electrolarynx with a head switch

May use AAC to set topic then use residual speech

May use AAC as a repair strategy

Question 15

Late Phase GB

Answer 15

Long-Term Residual Motor Speech Disorder

For a small population, speech interventions to maximize the effectiveness of their natural speech are appropriate

Question 16

Parkinson’s Disease

Answer 16

Syndrome composed of a cluster of motor symptoms that include tremor at rest, rigidity, paucity, and impaired postural reflexes

PD results from a loss of dopaminergic neurons in the basal ganglia and the brainstem

Onset is insidious (gradual but with harmful effects)

Medication is greatly changing the lifestyles of people with PD

During the time when AAC intervention is needed, symptoms may be difficult to manage with medication

Question 17

Communication Symptoms PD

Answer 17

Dysarthria is common

Reduced pitch variability, reduced overall loudness (AT for that), decreased use of all vocal parameters for achieving stress and emphasis

Imprecise articulation and produced at variable rates

Harsh and often breathy voice quality

Speech disorder are not uniform

For numerous reasons speech becomes increasingly more difficult to understand

AAC techniques are typically used with natural speech

Question 18

Early, Middle, and Late Phase Intervention PD

Answer 18

Early
AAC supports are usually not necessary
May require assistance for computer control, internet usage, and recreation
Near the end of this phase, people with PD often complete instruction in the Lee Silverman Voice Treatment (distance tx)

Middle
AT and AAC supports may be necessary to supplement natural speech
Delayed auditory feedback (iPad) helps control speaking rate
Portable vocal amplifiers (Chattervox, Mini Voice Amp)

Late
Use AAC to resolve communication breakdowns, communicate in adverse situations, or for most of their messages
Choose technology with speech synthesis output accessed through letter-by-letter spelling

Question 19

Participation Patterns and Communication Needs PD

Answer 19

People with PD typically need communication for the social environments of their retirement

People with PD may require extensive physical assistance, and this influences access to AAC

Question 20

Assess Prior to AAC Intervention PD

Answer 20

Cognitive/Linguistic Skills – are typically literate, unsure about presence of dementia, may require help compensating for learning or memory difficulties

Sensory/Perceptual Skills – usually N/A

Motor Skills – motor control problems may influence AAC interventions
Reduced range and speech of movement – reduce size of selection display (on alphabet board)
May require keyguard

Question 21

Brainstem Stroke

Answer 21

Strokes that disrupt the circulation serving the lower brainstem often cause severe dysarthria or anarthria

Severe damage to the brainstem may impair motor control of the limbs as well as of the face and mouth

Question 22

Communication Symptoms Brainstem Stroke

Answer 22

Vary based on level and extent of damage

Some people are dysarthric but can communicate partial or complete messages through speech

Usually flaccid type of dysarthria but may have spastic component

Nearly all require AAC initially

Question 23

Participation Patterns and Communication Needs Brainstem Stroke

Answer 23

Medical and lifestyle issues as well as the extent of their communication disorders influence communication needs

Usually unable to work and are cared for at home, others may be in independent living centers  nursing homes

Question 24

Early Phase Brainstem Stroke

Answer 24

Begins as soon as the referral is received

Goal is to develop a functional yes/no response and the ability to access a call system
Share information among all communication partners

Later in the early phase, may introduce low-tech techniques

Consider eye-linking, eye-gaze, or partner-assisted scanning strategies

Dependent on accuracy of individual, willingness and capability of communication partners

Question 25

Eye-linking Brainstem Stroke

Answer 25

low-tech communication option in which multiple images are placed or mounted on a transparent sheet. The communication partner visually focuses at the eyes of the person with AAC needs, who looks at the image of interest. The communication partner then moves the sheet until their eyes meet because they are both looking at a shared item. At this point, the communication partner confirms the meaning or message to be conveyed.

Access Interface Strategies - http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3663592/
Susan Fager, PhD, CCC-SLP, David R. Beukelman, PhD, CCC-SLP, Melanie Fried-Oken, PhD, CCC-SLP, Tom Jakobs, PE, and John Baker

Question 26

Middle Phase Brainstem Stroke

Answer 26

Selection of AAC Technology

Begins with acceptance of idea that AAC will be needed long-term

Formal AAC assessment (assess communication needs, abilities, and match with features of AAC systems)

Conduct trials with devices

Is motor control affected? May need to consider eye or head pointing

Question 27

Late Phase Brainstem Stroke

Answer 27

Implementation of AAC Technology into Daily Life

Receive device and commence intense instruction and practice

Question 28

Intervention Stages to Restore Natural Speech

Brainstem Stroke

Answer 28

Stage 1: No Useful Speech – Many folks must rely on AAC, may primarily focus on individual speech subsystems

Stage 2: Reestablish Subsystem Control for Speech – work to develop voluntary control of their respiratory, phonatory (vocal), velopharyngeal, and articulatory subsystems; strengthen muscles; AAC system will be used early in this stage to support the majority of communication interactions

Stage 3: Independent Use of Natural Speech – speech intervention focuses on speech intelligibility; goal is to meet all communication needs through natural speech; may use alphabet supplementation early in this stage; may be for repairing communication breakdowns

Question 29

Locked-In Syndrome

Answer 29

Also known as ventral pontine syndrome

Results in a conscious quadriplegic state in which the individual’s only voluntary movement consists of vertical eye movement and perhaps eye blinks

Usual cause is basilar artery stroke, tumor, or trauma/damage to the upper pons or occasionally midbrain

Low- and high-tech strategies have been used

When high-tech –
switch access sites included the head, mouth, fingers, and hands

AAC technology was used for communication, internet access, email, writing, telephone, games, and vocational duties