Week 6

Question 1

The clinician should advocate for a _____ for degenerative diseases

Answer 1

instrumental Ax

Question 2

What is ALS

Answer 2

progressive, fatal neurodegenerative disease that destroys corticospinal and corticobulbar tracts.
Eventual paralysis
AP present in ~15%
Huge QOL impairment, need early discussions about PEG and AAC

Question 3

What are the two onsets of ALS

Answer 3

Bulbar onset ( high impact of speech, voice, swallowing) - in 25-30% of cases

Spinal onset (gate/limbs affected first > speech/swallow impaired later) - more common

Question 4

What are typical swallowing signs of ALS?

Answer 4

Difficulty with solids early > progresses to diffuse pharyngeal residue, penetration and aspiration

Also: globus sensation
poor pharyngeal transit
UES dysfunction
Poor/oral tongue control (atrophy and fasciculation)

Question 5

What is HD?

Answer 5

Hereditary progessive ND disease of CNS
Chorea
50% chance of passing to children
no cure

Question 6

What is typically the cause of death in HD?

Answer 6

AP due to progressive dysphagia

Question 7

What is stage 1 (early stage) of HD progression?

Answer 7

Live at home
vocal quality changes
pharyngeal lodging or residue
Occasional coughing on food/drink
Slight mastication issues and lingual control reduction

Question 8

What is stage 2 (mid stage) of HD progression?

Answer 8

Darting lingual chorea
drooling
anterior spillage in oral stage
phonation during swallow
coughing with food/liquid
delayed/repetitive swallow

Question 9

What is stage 3 (late stage) of HD progression?

Answer 9

Speech is diminished/gone
Comprehension could be still intact
severe weight loss
Feeding/oral care dependency
non-oral feeding
Increased aspiration risk

Question 10

What is the preferred Ax for HD? why?

Answer 10

CSE

• chorea makes difficulty sitting still for instrumental
• FEES does not show oral phase which is important in HD

Question 11

What are some team-based approaches?

Answer 11

• Postural wedge to help with chorea
• Sour bolus for oral onset swallow
• Modified diet (caloric intake is huge with all the movement from chorea)
• Adaptive eating utensils
• Feeding assistance
• Enteral feeding

Question 12

What are the 4 clinical indicators for PD Dx

Answer 12

tremor
bradykinesia
muscle rigidity
postural instability

Question 13

What is the presenting symptom for PD?

Answer 13

Dysphagia and dysphonia

Question 14

What does dysphagia in PD look like?

Answer 14

slow oral phase
delayed swallow onset
prolonged pharyngeal and esophageal phases
residue

Question 15

What are swallowing characteristics of AD?

Answer 15

early disturbed olfaction
1/3 of pt aspirate
AP is most common cause of death
decline in memory, cognition and autonomy make treatment hard

Feeding apraxia
Agnosia for food
Delayed swallow initiation reflex
poor laryngeal excursion
poor swallow efficiency
low, slow, prolonged meals

Question 16

What structures are at risk of spine trauma and surgery

Answer 16

IX and XII above C3
Superior laryngeal nerve at C3-4
Cricopharyngeus at C5-6
RLN at C6 and below
Vagus nerve at risk for retraction injury