Amyotropic Lateral Sclerosis Flashcards
What can be defined as a progressive degeneration of the motor neurons of the central nervous system, leading to wasting of the muscles and paralysis?
Amyotrophic lateral sclerosis
What are the 2 classifications of ALS? Which is most common?
- sporadic (90%)
- familial (10%)
ALS incidence rate among Europe and North America ranges from __-__ for every 100,000 per year
1.5-2.7
What ethnic group has the highest rate of ALS?
Caucasians
Is ALS more common in males or females?
even
What age range has the highest chance of developing ALS?
40-75
There is a high prevalence in what 3 regions of the western Pacific?
- Guam
- West New Guinea
- Kii Peninsula Japan
What is the basic pathology behind ALS?
Motor neuron degeneration and death with gliosis replacing the neurons
Spinal cord atrophy associated with ALS causes what?
loss of peripheral innervation and muscle atrophy
What are the 6 signs and symptoms of ALS?
- Dysphagia
- Dysarthria
- Muscle weakness in hands, arms, and legs
- Fasciculation of muscles
- Difficulty projecting voice
- Shortness of breath
Symptoms begin in what muscles?
in the muscles of speech, swallowing, or in hands, arms, and legs
What is the average survival after diagnosis?
3-5 years
What is long-term survival associated with?
- Younger age at symptom onset
- Male gender
- Limb rather than bulbar symptom onset
What is stongly suggestive of ALS?
The presence of UMN and LMN signs
True or False
MRI is typically normal in patients with ALS
True
Levels of what are elevated in approximately 70% of patients diagnosed with ALS?
Creatine phosphokinase
A muscle biopsy is performed when ____ is suspected opposed to ALS
myopathy
Genetic testing is used to identify mutations in what 3 genes?
- SOD1
- TDP-43
- FUS
__% of persons with familial ALS will have a mutation in once of these genes
50
Pulmonary assessments must be done every _ months after diagnosis
3
If vital capacity is greater than __% respiratory management can be deferred
60
A vital capacity less than __% is often associated with respiratory failure and management is required
50
Vital capacity less than __-__% is often associated with respiratory failure and sudden death
25-30
What increases the risk for insufficient caloric and fluid intake/worsening of weakness and fatigue?
Dysphagia (difficulty swallowing)
What drugs can be prescribed in patients with intermittent dyspnea?
inhaled opiates (morphine) or IV midazolam
What drugs can be prescribed in patients with constant dyspnea?
IV morphine every 4 hours
What is debilitating fatigue treated with?
modafinil
What 3 drugs are used to treat muscle spasms?
- levetiracetam
- carbamazepine
- phenytoin
What 2 drugs are used to treat spasticity?
- Baclofen
- Tizanidine
What symptom is very common in ALS patients due facial muscle weakness & decreased swallowing ability?
Sialorrhea
What 4 drugs are used to treat sialorrhea?
- Atropine
- Hyoscyamine
- Amitriptyline
- Glycopyrrolate
Describe the pseudobulbar affect associated with ALS
Patients experience sudden uncontrollable outbursts of laughter or tearfulness
The pseudobulbar affects __% of patients
50
What 3 drugs are used to treat the pseudobulbar affect?
- combination of dextromethorphan-quinidine
- Amitriptyline
- Fluvoxamine
What is the only FDA regulated drug to treat ALS
Riluzole
