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Flashcards in ALS Deck (26):

What does ALS stand for?

Amyotrophic Lateral Sclerosis


What are 2 other names for ALS?

1. Lou Gehrig's
2. Charcots


ALS is the most common what type of disorder?

Most common motor neuron problem


Is ALS progressive? How long is the duration?

Yes, it is very progressive and a short duration


Is ALS more common in men or women? By how much more?
What age group

2X more common in middle aged men


1. How quickly would a person die after acquiring ALS?
2. What would they typically die from?

1. 2-5 years
2. Respiratory complications


1. Bulbar ALS is a type of ALS, the damage in this ALS begins where?
2. What are the main 2 things affected in this ALS?

1. In the brainstem
2. Speech / swallowing


What is the Et of ALS?
(3) (what is the percentage of each?

1. Sporadic (90-95%)
2. Familial (5-10%)
3. Other Factors


What do the other factors include that can be an Et of ALS?

Autoimmune, viral


1. The sporadic form of ALS involves a mutation in what gene and on what Chromosome?
2. This gene codes for what enzyme?
3. What does this enzyme do?
4. Therefore... if the gene is mutated, therefore enzyme mutated, what occurs?

1. SOD1 (superoxide dismutase gene) gene mutation, on Chrm 21 (~5%)
2. Superoxide dismutase
3. Enzyme breaks up free radicals
4. Free radicals build up and can damage the neurons


1. The familal form ALS involves what gene on what Chrm?
2. How much of the familial form of ALS is affected by this gene?
3. There are other genes that can cause ALS, do we know them? What percentage is this?

1. SOD1 on Chrm 21
2. ~20% (therefore more so than the sporadic form)
3. Other genes (80%)


The Patho of ALS:
1. Involves what type of degeneration: motor or sensory neuron?
2. Proposed Free radical and or glutamate toxicity: is this m echanism clear?

1. Motor neuron degeneration
2. Unclear


The motor neuron degeneration is in what 3 places?

1. Anterior horn cells in spinal chord
2. motor nuclei in brain stem
3. Upper motor neurons in cerebral cortex


The anterior horn cells in the spinal chord house what type of neurons?

Motor neurons


1. The upper motor neurons in the cerebral cortex, run between ____ and _____?
2. What do the lower neurons run between?

1. brain and spinal chord
2. spinal chord and peripherals


The Patho of ALS can involve the motor neuron degeneration as well as what that involves glutamate toxicity?

Proposed free radical and or glutamate toxicity


What are the Mnfts of ALS? (3)

1. Muscle atrophy, weakness, fasciculations
2. Dysarthia
3. Acute complication: dysphagia and aspirations


What does fasciculations mean? What is this d/t?

Muscle twitching d/t inadequate stimulation


What does dysarthia mean?

Impaired speech


The dysphagia and aspirations (an acute complication of ALS) occurs why?

Inadquate stimulation of smooth muscle in esophagus


Are sensory fx and cognition affected in ALS?

No. Cognition and sensory function are still intact.


What is ALS Dx on? (2)

1. Clinical Presentation
2. Electromyography


What does electromyography measure? How does this work?

It measures nerve function.
Test is done through stimulating a muscle THROUGH a nerve, if muscle doesn't work then you know it is a nerve problem.


What is the Tx for ALS? (2)

1. The Tx is mostly supportive
2. Pharmacologic


What does the supportive treatment for ALS include? (2)

1. Helping with swallowing (PEG tube)
2. Ensuring vaccinations to limit respiratory infections


The pharmacologic Tx for ALS involves what drug? What does this drug do?

Riluzole. Protects the neurons, can extend an individuals life by a few months.