ALS

Question 1

What is the most common adult onset motor neuron disease?

Answer 1

ALS

Question 2

What does ALS stand for?

Answer 2

Amyotrophic lateral sclerosis

Question 3

ALS is known as a _____ illness

Answer 3

Terminal

Question 4

What does ALS cause?

Answer 4

Rapid progressive degeneration of both upper and lower nervous system motor neurons

Question 5

ALS: Life Expectancy

Answer 5

3-5 years after symptoms appear

Question 6

ALS: Etiology

Answer 6

Unknown

Question 7

First Document of ALS

Answer 7

Jean-Marie Charcot (1874)

Question 8

What is ALS also known as?

Answer 8

Lou Gehrig’s Disease

Question 9

ALS affects what age group?

Answer 9

55-65

Question 10

Possible Etiologies of ALS

Answer 10

• Genetic, environmental, and pathologic findings

- Smoking 4x increased risk

Question 11

What career groups of people are more likely to be diagnosed with ALS? Why?

Answer 11

US veterans and athletes; may be related to trauma, including traumatic brain injury, or exposure to a toxin, such as pesticide or heavy metal

Question 12

Risk Factors for ALS

Answer 12

Males>Females

Western Pacific

Question 13

Possible Risk Factors for ALS

Answer 13

Vigorous physical activity
Neurotoxins
Electrical works and industrial occupations
High fat intake

Question 14

ALS Pathophysiology

Answer 14

Messages from Upper motor neurons transmitted to motor neurons in the spinal cord and to lower motor neurons and from the spinal cord and motor nuclei of brain to a particular muscle or mucles.

Question 15

ALS: What happens when UMN and LMN die?

Answer 15

Muscles don’t function, gradually weaken, and atrophy

Question 16

ALS & Cognitive Impairment

Answer 16

Mild cognitive impairment, though they usually retain decisional capacity and complete awareness of their situation

Question 17

ALS Clinical Presentations

Answer 17

• Fasciculations (muscle twitches) in arm, leg, shoulder, or tongue
• Muscle cramps
• Spasticity
• Muscle weakness affecting an arm, a leg, neck or diaphragm
• Dysarthria
• Dysphagia
• Burn calories faster than person without ALS

Question 18

Despite extensive paresis, what are ALS patients able to maintain control of?

Answer 18

Eye movements and bladder/bowel function

Question 19

Define Dysarthia

Answer 19

Slurred and nasal speech

Question 20

Define Dysphagia

Answer 20

Difficulty chewing or swallowing

Question 21

Early stages of ALS

Answer 21

• Muscle weakness
• Muscle twitching
• Muscle cramping
• Fatigue
• Poor balance
• Slurred speech

Question 22

Middle Stages of ALS

Answer 22

• More severe muscle weakness
• Paralysis in some muscles
• Difficulty in swallowing, eating/chewing
• Breathing issues
• Bouts of uncontrollable laughter or crying

Question 23

Late Stages of ALS

Answer 23

• Paralysis in most muscles
• Extremely limited mobility
• Inability to speak
• Inability breath without assistance
• Inability to eat without assistance
• Inability to drink without assistance

Question 24

Diagnosis for ALS

Answer 24

• No one can test to provide a definitive diagnosis
• Detailed history of symptoms
• Tests to R/O other mimicking disease
• Neurologic exam
• Muscle & Imaging Tests: EMG & NCS

Question 25

Management for ALS

Answer 25

- No treatment - Physical therapy & Occupation therapy - Speech therapy - Nutritional Support - Breathing Support

Question 26

How can physical therapy help ALS patients?

Answer 26

Enhance an individual's independence and safety

Question 27

Cause of death of most ALS patients

Answer 27

Respiratory failure

Question 28

Two drugs used to treat ALS

Answer 28

Riluzole (Rilutek) & Edaravone (Radicava)

Question 29

ALS: Function of Riluzole (Rilutek)

Answer 29

- Reduces damage to motor neurons by decreasing levels of glutamate - Prolongs survival by a few months

Question 30

ALS: Function of Edaravone (radicava)

Answer 30

Slow decline in clinical assessment of daily functioning

Question 31

ALS Medications help manage what symptoms

Answer 31

- Muscle cramps, stiffness, excess saliva and phlegm & pseudobulbar affect - Pain, depression, sleep disturbances, and constipation

Question 32

ALS Oral Manifestations

Answer 32

- Wasting of the tongue muscles in bulbar-onset ALS - Tongue is disproportionately affect in comparison to other orophargyneal musculature - Note absence of palatal elevation present on vocalizaiton - Difficult with mouth opening - May have increased saliva (sialorrhea)

Question 33

ALS: Treatments for Sialorrhea

Answer 33

- Anticholinergic antidepressants - Anticholinergic drugs - Botulinum toxin injections - Radiation of salivary glands - Mouth care products - Suction

Question 34

ALS: Treatment for thickened saliva

Answer 34

- Natural remedies - Ensure adquate hydration - Saline nebulizers - Suctioning of the mouth - Mouth care

Question 35

ALS: Dental Management

Answer 35

- High risk of aspiration - Use of HVE, assistant, RD when possible - Avoid Cavitron if patient has dysphagia - Frequent hygiene vists for patients with g-tube - OHI and good home care - End stages will require pallative care

Question 36

ALS: What are ALS patient at a 6x greater risk for?

Answer 36

Suicide

Question 37

ALS: Speech Therapy Tools

Answer 37

Adapative strategies and computer based speech synthesizers that use eye-tracking

Question 38

ALS: Nutritional Support Tools

Answer 38

- Provide enough calories, fiber and fluid & avoid food that are difficult to swallow - Feeding tube reduces risk of choking and aspiration pneumonia

Question 39

ALS: Breathing Support Tools

Answer 39

- Noninvasive ventilation breathing support through a mask over the nose and/or mouth - Used at nighttime of full-time - Improves the quality of life and prolongs survival - Mechanical ventilation (respirators and tracheostomy)