ALS Flashcards
1
Q
What is the pathophisiology of ALS?
A
- Rapidly progressing, fatal CNS disease
- Affects voluntary muscle control (not senses/ability to think)
- Both upper and lower neurons degenerate and die
2
Q
ALS affects
A
Diaphragm and chest wall, leads to respiratory compromise, pneumonia and death
3
Q
Causes of ALS
A
- Genetics
- smoking
- Athleticism
- Metals
- Solvents
- Radiation
- Electromagnetic fields!
- Pesticides!
- Viruses!
4
Q
Early symptoms of ALS
A
- Tongue atrophy
- Dysphagia
- Dysarthria
5
Q
Late symptoms of ALS
A
- Nasal quality of speech
- Fasciculation (Twitching) of the face
- Spasticity
- Weakness of the hands and arms
- Muscle atrophy
- Eventual respiratory muscle involvement
6
Q
What are the diagnostic tests used to diagnose ALS?
A
No single test can be used to diagnose
- S/S
- EMG and nerve conduction studies
- CT / MRI of neck and head
- Lumbar puncture
- Genetic testing
- Swallow studies
7
Q
ALS medical management
A
No cure, but 2 drugs may slow progression
- Riluzole (Rilutek)
- Edaravone (Radivaca)
8
Q
Riluzole (Rilutek) mode of action
A
- Reduces damage to motor neurons by decreasing the release of glutamate
- Well tolerated
- Delays need for trach by 3-6 months
9
Q
Riluzole (Rilutek) adverse effects
A
- Asthenia
- GI reactions
- Dizziness/vertigo
- Decreased lung fx
- Liver injury
- Neutropenia
10
Q
Edaravone (Radivaca) MOA
A
- Relieves effects of oxidative stress
- Slows loss of physical function in early stages
11
Q
Edaravone (Radivaca) adverse effects
A
- Hives
- Swelling of lips, tongue, face
- Fainting
- Breathing problems (wheezing, asthma attack)
- Trouble swallowing
- Dizziness
12
Q
Complications of ALS
A
- Aspiration
- Resp. Failure
- Pneumonia
- Pressure ulcers
- DVT
- PE
- Constipation
- Depression
- Weight loss
13
Q
ALS interventions
A
- Palliative treatment
- Respiratory! Prevent aspiration, provide treatments and support
- Psychosocial support
- Compassionate end of life care
