ALS Flashcards

1
Q

What is the most common adult onset motor neuron disease?

A

ALS

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2
Q

What does ALS stand for?

A

Amyotrophic lateral sclerosis

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3
Q

ALS is known as a _____ illness

A

Terminal

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4
Q

What does ALS cause?

A

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

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5
Q

ALS: Life Expectancy

A

3-5 years after symptoms appear

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6
Q

ALS: Etiology

A

Unknown

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7
Q

First Document of ALS

A

Jean-Marie Charcot (1874)

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8
Q

What is ALS also known as?

A

Lou Gehrig’s Disease

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9
Q

ALS affects what age group?

A

55-65

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10
Q

Possible Etiologies of ALS

A
  • Genetic, environmental, and pathologic findings

- Smoking 4x increased risk

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11
Q

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

A

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

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12
Q

Risk Factors for ALS

A

Males>Females

Western Pacific

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13
Q

Possible Risk Factors for ALS

A

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

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14
Q

ALS Pathophysiology

A

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.

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15
Q

ALS: What happens when UMN and LMN die?

A

Muscles don’t function, gradually weaken, and atrophy

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16
Q

ALS & Cognitive Impairment

A

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

17
Q

ALS Clinical Presentations

A
  • 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
18
Q

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

A

Eye movements and bladder/bowel function

19
Q

Define Dysarthia

A

Slurred and nasal speech

20
Q

Define Dysphagia

A

Difficulty chewing or swallowing

21
Q

Early stages of ALS

A
  • Muscle weakness
  • Muscle twitching
  • Muscle cramping
  • Fatigue
  • Poor balance
  • Slurred speech
22
Q

Middle Stages of ALS

A
  • More severe muscle weakness
  • Paralysis in some muscles
  • Difficulty in swallowing, eating/chewing
  • Breathing issues
  • Bouts of uncontrollable laughter or crying
23
Q

Late Stages of ALS

A
  • Paralysis in most muscles
  • Extremely limited mobility
  • Inability to speak
  • Inability breath without assistance
  • Inability to eat without assistance
  • Inability to drink without assistance
24
Q

Diagnosis for ALS

A
  • 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
25
Q

Management for ALS

A
  • No treatment
  • Physical therapy & Occupation therapy
  • Speech therapy
  • Nutritional Support
  • Breathing Support
26
Q

How can physical therapy help ALS patients?

A

Enhance an individual’s independence and safety

27
Q

Cause of death of most ALS patients

A

Respiratory failure

28
Q

Two drugs used to treat ALS

A

Riluzole (Rilutek) & Edaravone (Radicava)

29
Q

ALS: Function of Riluzole (Rilutek)

A
  • Reduces damage to motor neurons by decreasing levels of glutamate
  • Prolongs survival by a few months
30
Q

ALS: Function of Edaravone (radicava)

A

Slow decline in clinical assessment of daily functioning

31
Q

ALS Medications help manage what symptoms

A
  • Muscle cramps, stiffness, excess saliva and phlegm & pseudobulbar affect
  • Pain, depression, sleep disturbances, and constipation
32
Q

ALS Oral Manifestations

A
  • 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)
33
Q

ALS: Treatments for Sialorrhea

A
  • Anticholinergic antidepressants
  • Anticholinergic drugs
  • Botulinum toxin injections
  • Radiation of salivary glands
  • Mouth care products
  • Suction
34
Q

ALS: Treatment for thickened saliva

A
  • Natural remedies
  • Ensure adquate hydration
  • Saline nebulizers
  • Suctioning of the mouth
  • Mouth care
35
Q

ALS: Dental Management

A
  • 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
36
Q

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

A

Suicide

37
Q

ALS: Speech Therapy Tools

A

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

38
Q

ALS: Nutritional Support Tools

A
  • Provide enough calories, fiber and fluid & avoid food that are difficult to swallow
  • Feeding tube reduces risk of choking and aspiration pneumonia
39
Q

ALS: Breathing Support Tools

A
  • 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)