Pathophysiology: Amyotrophic Lateral Sclerosis
Terms in this set (46)
ALS is also known as
Lou Gehrig's Disease
Familial Amyotrophic Lateral Sclerosis (FALS)
Over 100 different SODI mutations have been seen in FALS but not all pts. with a SODI mutation develop FALS.
Is the disease greater in males or females?
What race is ALS more prevalent in?
What increases the incidence of ALS?
Most Common Course
Typical Life Expectancy After Dx
-ALS is a progressive neurodegenerative disease.
-ALS degenerates both upper and lower motor neurons
-As motor neurons degenerate the anterior and lateral columns of the spinal cord are replaced by fibrous astrocytes causing harden of these areas
-Wallerian degeneration is the process by which motor axons die
-Death occurs in the anterior horn cell body
-Causes degeneration of associated motor axon
-Surrounding Schwann cells breakdown the axon's myelin sheath
-Causing the axon to break into pieces
-The axon debris is cleaned up by macrophages
Upper Motor Neurons
ALS causes degneration of the corticospinal upper motor neurons that reside in the cerebral cortex specifically the precentral gyrus
Loss of UMN leads to:
1. spastic paralysis
Lower Motor Neuron
ALS causes the degeneration of the lower motor neurons that reisdes in the anterior horn of the spinal cord and in the brainstem
Loss of LMN leads to:
1. flaccid paralysis
2. decreased muscle tone
3. decreased reflexes
4. muscle weakness
5. muscle atrophy
Other neurodegenerative disease can evolve into ALS, such as:
1. progressive bulbar palsy
2. progressive muscular atrophy
3. primary lateral sclerosis
4.adult-onset spinal muscular atrophy
Progressive Bulbar Palsy
bulbar involvement (facial muscles)
Progressive Muscular Atrophy
pure lower motor neuron degeneration
Primary Lateral Sclerosis
pure upper motor neuron degeneration
Adult-Onset Spinal Muscular Atrophy
includes a broad range of primary motor neuron disease by pattern of inheritance, distribution of weakness, or age of onset
ALS does not affect:
-Cranial Nerves III, IV, and VI (Eye muscles)
-Bowel and Bladder Function
-no direct tests
-tests are used to rule out other diseases
Early Stage Clinical Features
-Painless weakness and difficulty breathing due to motor neuron degeneration.
-Soft or spastic muscles
-Twitches (fasciculation) and cramps due to imbalance of antagonistic muscles.
-Atrophy of muscles especially in hands and forearms
-Symptoms localized or mild and sporadic
-Imbalance from muscle weakness causing gait abnormalities
-Weak grip and fine motor deterioration
Middle Stage Clinical Features
-Symptoms spread and worsen
-Some muscle paralysis
-Joint pain and deformity
-May have gag reflex with dysphagia (difficulty swallowing) which can become severe in advanced stages and therefore at risk for aspiration.
-Lung weakness can cause respiratory insufficiency
-May have inappropriate or flat affect.
Final Stage Clinical Features
-Paralysis of most voluntary muscles
-Often severe breathing insufficiency
-Susceptible to lung infection like pneumonia
-Possible complete speech failure
-Inability to eat or drink on own due to dysphagia
-Death after 3-5 years usually due to respiratory failure or cachexia (wasting away of body).
-most common (90-95%)
-may affect anyone
-believed to be connected to a defecive SODI gene on chromosome 21.
-age of onset 20 years younger than Sporadic
an extremely high incidence of ALS was observed in Guam & the Trust Territories of the Pacific in the 1950's
Relevant Patient History
Past family history
Other motor neuron diseases
-considered only exogenous factor
1. service in the US military
2. deployment to the Persian Gulf in the First Persian Gulf War
3. being an Italian soccer player
no known prevention factors
Majority of treatment is to control pain, symptoms, & to improve quality of life.
-Tube feeding placement-for problems with choking
-Nutritionist- helps pts maintain weight
-Assistive devices and orthotics- muscle function and control
-Breathing devices-esp during the night
-Speech Language Pathologist
1st Drug of Choice
-slows the disease process
-only drug approved by FDA
Medications can be prescribed to help relieve other symptoms such as:
Baclofen and diazepam-Spasticity
Trihexyphenidyl or amitriptyline-helps swallow saliva
Medications are also prescribed to help the patient improve their quality of life such as medication to treat:
Improves pts quality of life
Provide special equipment
Help pts conserve energy
Examples of equipment that ALS pts use :
ROM and Strengthening exercises
-Prevent contracture of muscles
Low impact aerobics: swimming, walking, stationary bike
-Strengthen unaffected muscles
Emerging Medical Treatment
1. Stem Cell
2. Gene Therapy
-The hope is that stem cells will be able to be used to create new motor neurons.
-The fear is that these new cells won't be able to stand up to the disease just like the old cells.
-Gene therapy is when genetic instructions are used to try to make a protein to help treat ALS
-Could be beneficial if it could develop a protein that could save dying nerve cells.
-Even if they do find a protein to help some ALS patient's it may not be a cure for all patients.
Examples of Non-Traditional Medical Treatment
Astragalus- Chinese herb
Other non-tradional medical treatment includes anything they can do to improve their quality of life.
ex. If they like Yoga they are likely to use that to ease their pain.
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