ALS Flashcards
What is the most common adult onset motor neuron disease?
ALS
What does ALS stand for?
Amyotrophic lateral sclerosis
ALS is known as a _____ illness
Terminal
What does ALS cause?
Rapid progressive degeneration of both upper and lower nervous system motor neurons
ALS: Life Expectancy
3-5 years after symptoms appear
ALS: Etiology
Unknown
First Document of ALS
Jean-Marie Charcot (1874)
What is ALS also known as?
Lou Gehrig’s Disease
ALS affects what age group?
55-65
Possible Etiologies of ALS
- Genetic, environmental, and pathologic findings
- Smoking 4x increased risk
What career groups of people are more likely to be diagnosed with ALS? Why?
US veterans and athletes; may be related to trauma, including traumatic brain injury, or exposure to a toxin, such as pesticide or heavy metal
Risk Factors for ALS
Males>Females
Western Pacific
Possible Risk Factors for ALS
Vigorous physical activity
Neurotoxins
Electrical works and industrial occupations
High fat intake
ALS Pathophysiology
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.
ALS: What happens when UMN and LMN die?
Muscles don’t function, gradually weaken, and atrophy
ALS & Cognitive Impairment
Mild cognitive impairment, though they usually retain decisional capacity and complete awareness of their situation
ALS Clinical Presentations
- 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
Despite extensive paresis, what are ALS patients able to maintain control of?
Eye movements and bladder/bowel function
Define Dysarthia
Slurred and nasal speech
Define Dysphagia
Difficulty chewing or swallowing
Early stages of ALS
- Muscle weakness
- Muscle twitching
- Muscle cramping
- Fatigue
- Poor balance
- Slurred speech
Middle Stages of ALS
- More severe muscle weakness
- Paralysis in some muscles
- Difficulty in swallowing, eating/chewing
- Breathing issues
- Bouts of uncontrollable laughter or crying
Late Stages of ALS
- Paralysis in most muscles
- Extremely limited mobility
- Inability to speak
- Inability breath without assistance
- Inability to eat without assistance
- Inability to drink without assistance
Diagnosis for ALS
- 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
Management for ALS
- No treatment
- Physical therapy & Occupation therapy
- Speech therapy
- Nutritional Support
- Breathing Support
How can physical therapy help ALS patients?
Enhance an individual’s independence and safety
Cause of death of most ALS patients
Respiratory failure
Two drugs used to treat ALS
Riluzole (Rilutek) & Edaravone (Radicava)
ALS: Function of Riluzole (Rilutek)
- Reduces damage to motor neurons by decreasing levels of glutamate
- Prolongs survival by a few months
ALS: Function of Edaravone (radicava)
Slow decline in clinical assessment of daily functioning
ALS Medications help manage what symptoms
- Muscle cramps, stiffness, excess saliva and phlegm & pseudobulbar affect
- Pain, depression, sleep disturbances, and constipation
ALS Oral Manifestations
- 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)
ALS: Treatments for Sialorrhea
- Anticholinergic antidepressants
- Anticholinergic drugs
- Botulinum toxin injections
- Radiation of salivary glands
- Mouth care products
- Suction
ALS: Treatment for thickened saliva
- Natural remedies
- Ensure adquate hydration
- Saline nebulizers
- Suctioning of the mouth
- Mouth care
ALS: Dental Management
- 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
ALS: What are ALS patient at a 6x greater risk for?
Suicide
ALS: Speech Therapy Tools
Adapative strategies and computer based speech synthesizers that use eye-tracking
ALS: Nutritional Support Tools
- Provide enough calories, fiber and fluid & avoid food that are difficult to swallow
- Feeding tube reduces risk of choking and aspiration pneumonia
ALS: Breathing Support Tools
- 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)
