Term
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Definition
| Amyotrophic Lateral Sclerosis |
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Term
| Does ALS affect the central or peripheral nervous system? |
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Definition
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Term
| Which extremities are usually affected first? |
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Definition
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Term
| What is the most common motor neuron disease? |
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Definition
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Term
| What is the cause of ALS? |
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Definition
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Term
| What parts of the nervous system are affected by ALS? (2) |
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Definition
| anterior horn cells and the coricospinal tract. |
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Term
| What is the pathology of ALS? |
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Definition
| Destruction of motor neurons in the spinal cord and brainstem, and corticospinal/corticobulbar degeneration. |
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Term
| Will you see UMN or LMN dysfunction w/ ALS? How does it present clinically? |
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Definition
| both. So you will see both spasticity and flaccidity. |
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Term
| What are the differential diagnoses? (3) |
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Definition
| 1. progressive muscular atrophy. 2. primary lateral scerosis. 3. prgressive bulbar palsy. |
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Term
| What is progressive muscular atrophy (PMA)? |
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Definition
| A disease affecting the anterior horn cells that presents w/ purely LMN Sx. |
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Term
| What is primary lateral sclerosis? (PLS) |
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Definition
| A disease that produces purely UMN lesions. |
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Term
| What type of ALS is most common? |
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Definition
| Sporadic - no known etiology. |
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Term
| What is the most common age of sporadic ALS onset? |
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Definition
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Term
| What is the average survival rate of sporadic ALS? |
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Definition
| 3 years. Survival is longer if onset @ < 41 years old. |
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Term
| What is the mean onset of familial ALS? What is the impact of gender? |
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Definition
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Term
| What is the average survival of familial ALS pts? |
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Definition
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Term
| What are the four diagnostic tests used for Dx of ALS? |
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Definition
| 1. CPK levels (may be elevated) 2. EMG. 3. Muscle biopsy. 4. Genetic testing for familial ALS. |
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Term
| How do CPK levels relate to ALS? |
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Definition
| elevation of CPK doesn't Dx ALS, but is associated w/ it. Indicative of muscle wasting. |
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Term
| What EMG evidence will you find if the pt has ALS? |
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Definition
| evidence of fibrillations. Sometimes large motor unit potentials b/c of motor unit sprouting. |
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Term
| what is motor unit sprouting? |
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Definition
| healthy neurons take over for dying neurons next to them. |
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Term
| what will a muscle biopsy show? |
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Definition
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Term
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Definition
| It inhibits the release of glutamate. If there is excitotoxicity b/c of increased glutamate, this med reduces the release of the cytotoxin to decrease excitotoxicity. Prolongs the time before pt goes on ventilator by a few months. |
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Term
| What does pharmacological Tx of ALS consist of? |
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Definition
| Riluzole, and symptomatic meds. |
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Term
| What are the clinical signs and Sx of ALS? (6) |
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Definition
| 1. Upper motor neuron syndrom. 2. Lower motor neuron syndrome. 3. bulbar signs and sx. 4. Respiratory Sx. 5. Systemic. 6. Musculoskeletal. |
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Term
| What are the upper motor neuron syndrome Sx? (8) |
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Definition
| 1. loss of dexterity/coordination. 2. weakness. 3. stiffness. 4. slowness. 5. spasticity. 6 pathologic reflexes. 7. spastic bulbar palsy. 8. tonic flexor spasms |
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Term
| What pathologic reflex will you see w/ ALS? |
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Definition
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Term
| What are the lower motor neuron Sx? (6) |
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Definition
| 1. weakness (particularly trunk and cervical). 2. muscle atrophy. 3. hyporeflexia. 4. hyptonicity/flaccidity. 5. fasiculations. 6. muscle cramps. |
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Term
| Where and when are fasciculations commonly seen? |
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Definition
| in the tongue, calf and elbow flexors. They're present at rest and w/ movement, but most commonly at rest. |
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Term
| What are the bulbar signs and sx? (8) |
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Definition
| eye closure, orbicularis oris, tongue atrophy/deviations, jaw opening deviations, dysarthria, dysphagia, sialorrhea, aspiration/laryngospasm. |
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Term
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Definition
| poor production of speech. |
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Term
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Definition
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Term
| what is sialorrhea? what is the cause of it? |
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Definition
| drooling b/c of lack of jaw control & swallowing. |
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Term
| Why does aspiration/laryngospasm occur? |
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Definition
| incoordination of closing the glottis. |
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Term
| What is paroxysmal respiration? |
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Definition
| chest looks like its collapsing rather than rising b/c intercostals aren't expanding w/ the diaphragm. |
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Term
| what are the systemic signs and Sx you'll see? (3) |
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Definition
| weight loss, decreased muscle mass, fatigue. |
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Term
| why is weight loss commonly seen? |
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Definition
| dysphagia or loss of appetite. Also, loss of muscle mass will decrease weight. |
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Term
| What are the musculoskeletal signs? (3) |
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Definition
| hand and foot deformities, joint contracture, pain. |
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Term
| what are the common hand and foot deformities associated w/ als? (3) |
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Definition
| extensor stiffness in LEs, achilles tendon shortening, claw hand deformity. |
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