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ALS (amyotrophic lateral sclerosis) or Lou Gehrig's Disease - Coggle…
ALS (amyotrophic lateral sclerosis) or Lou Gehrig's Disease
Risk Factors:
-Genetics (10% of cases)
-Oxidative stress
-Mitochondrial dysfunction
-Overactive immune system
-Glutamate toxicity and toxic exposures
-Lifestyle risk factors:
-Smoking
-Obesity
-Poor diet
-Environmental working conditions or toxins
Incidence/Prevalence
-Those between the ages of 40-70
-More commonly diagnosed in men than women
-More common in white populations rather than any other race.
-Develops with uniform frequency in major Western countries.
-Annual incidence is 2 per 100,000 population.
The prevalence in the US is 5 per every 100,000 people.
-Approx. 30,000 people have ALS in the US.
-More than 200,000 people in the world are living with ALS.
-80% of people diagnosed with ALS die within 2-5 years.
Pathogenesis:
-Pathogenesis remains largely unknown.
-What is known is there are multiple possibilities of potential mechanisms in regards to neurodegeneration.
-Characterized by progressive muscle paralysis determined by the degeneration of motoneurons in the motor cortex brainstem and spinal cord.
-The ALS pathogenetic mechanisms are still unclear, despite the wealth of studies demonstrating the involvement of several altered signaling pathways.
-ALS is characterized by upper motor neuron (corticospinal motor neurons) and lower motor neuron (bulbospinal motor neurons) degeneration and death as well as reactive gliosis replacing death neurons.
-The hallmark finding of lower motor neuron (LMN) pathology in ALS is the presence of intracellular inclusion bodies in neuronal soma and proximal dendrites as well as glia.
Sources:
What is ALS? Answers from the ALS therapy Development Institute. (n.d.). Retrieved February 17, 2021, from
https://www.als.net/what-is-als/?gclid=CjwKCAiAmrOBBhA0EiwArn3mfIzj9kBNNCycS4wuIdb1eY1GukRcfKaKLRHjsplfZXFSU6RMfpokkRoCQAsQAvD_BwE#!#who-gets-als
Ingre, C., Roos, P., Piehl, F., Kamel, F., & Fang, F. (2015, February 12). Risk factors for amyotrophic lateral sclerosis. Retrieved February 17, 2021, from
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4334292/
Morgan, S., & Orrell, R. (2016, September 09). Pathogenesis of amyotrophic lateral sclerosis. Retrieved February 17, 2021, from
https://academic.oup.com/bmb/article/119/1/87/1744530
Bonafede, R., & Mariotti, R. (2017, March 21). ALS pathogenesis and THERAPEUTIC approaches: The role of mesenchymal stem cells and Extracellular Vesicles. Retrieved February 17, 2021, from
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5359305/
Rossi, F., Franco, M., & Estevez, A. (2013, September 11). Pathophysiology of amyotrophic lateral sclerosis. Retrieved February 17, 2021, from
https://www.intechopen.com/books/current-advances-in-amyotrophic-lateral-sclerosis/pathophysiology-of-amyotrophic-lateral-sclerosis
“ALS Treatment.” Ucsfhealth.org, www.ucsfhealth.org/conditions/als/treatment.
“Progression.” ALS Society of Canada, 16 Feb. 2021, www.als.ca/what-is-als/about-als/progression/.
Diseases - als - medical management. (2019, April 30). Retrieved April 06, 2021, from
https://www.mda.org/disease/amyotrophic-lateral-sclerosis/medical-management
Diagnostics:
ALS can be difficult to diagnose due to its similarity to other diseases. However a neurologist can correctly diagnose a person with ALS based on these tests:
-Blood and urine studies
-Electrodiagnostic tests (EMG and NCV)
-MRI
-Muscle and nerve function tests
Genetic testing may also be helpful for individuals who have family members who have been diagnosed with ALS.
The majority (90%) of ALS cases are sporadic. This means that they occur at random with no clear risk factors or familial history of the disease.
The other 10% of ALS cases are familial. This means that a parent carried the gene responsible for the disease.
According to the Airlie House criteria a diagnosis of ALS requires the following:
1.Signs of degeneration of lower motor neurons, which are in the spinal cord and brainstem, by clinical examination or specialized testing
Signs of degeneration of upper motor neurons, which are in the brain, by clinical examination
Progressive spread of signs within a region to other regions
The absence of electrophysiological, pathological, and neuroimaging evidence of other disease processes that might explain the observed clinical signs.
Clinical Manifestations:
Early signs and symptoms:
-Tripping
-Dropping things
-Slurred or thick speech
-Difficulty swallowing
-Decreased muscle tone
-Feeling weak
-Fatigue
-Muscle stiffness or rigidity
-Muscle twitches in the arm, leg, shoulder, or tongue
*
MANY FIRST SIGNS APPEAR IN HAND OR ARM (could be simple tasks such as buttoning shirt or writing)
Later signs and symptoms:
-Difficulty walking
-Every early symptom but more severe
-Muscle Cramps
-Cognitive changes
ALS is progressive, this means that symptoms get worse over time.
Treatments:
There is no cure for ALS but these different treatments may slow the progression and help the symptoms of the disease.
-Riluzole: slows the progression of the disease; reduces damage to motor neurons by decreasing levels of glutamate
-Baclofen or diazepam: control spasticity
-Gabapentin: may help to control pain
Trihexyphenidyl or amitriptyline: help patients swallow.
-Physical therapy: help the muscles work in the best way possible as well as improve general health.
-Speech therapy: improve communications
-Edaravone has been shown to slow the decline in clinical assessment of daily functioning in persons with ALS.