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Motor neurons in the brainstem & spinal cord gradually degenerate idiopathically. Dead motor neurons cannot make or send singals to muscles, causing electric & chemical signals to not reach the muscles causing denervation and lack of stimulation
Treatment options are limited. There is not cure
Riluzole given P.O or through PEG tube - reduces damage to motor neurons by decreasing release of glutamate, slowing progression of disease
Edaravone given IV or P.O - exact MOA is yet to be discovered however it is a free radical scavenger
-dysphagia
-spontaneous clenching & biting
-dysarthria of lips, tongue & pharynx
-layrngospams
-tachypnea, dyspnea, orthopnea
-absent abdominal reflexe
-stiffness
-muscl cramps
-difficulty writing
-foot drop
-muscle atrophy
-fasciculation
There is no one test to diagnose ALS, it is mostly done througb process of elimination
usual diagnostics performed are
-MRI of head neck & lower spine
-EMG to test nerve conduction
-urinalysis
-spinal tap
The end result of ALS is inevitable
, before death almost every person with ALS may experience
-muscle wasting
-dysphagia
-aspirtion pneumonia
Some labs to look at would be
-an increased creatinine kinase level which could indicate sign of muscle damage
-facilitate commmunicaion between the patient and family
-reduce risk for aspiration
-early identification of respiratory insufficiency
-decrease pain from muscle weakness
-decrease risk for fall
-provide diversional activities like reading and companionship
-guide patient in use of moderate-intense endurance-type exercises for trunk & limbs to help reduce spacisity
-discuss advanced directives & artificial methods of ventiliation with patient & family
for ALS, H. C. (2021). Patient education: Learning about ALS. Massachusetts General Hospital. https://www.massgeneral.org/neurology/als/patient-education
Harding, M., Kwong, J., Roberts, D., Hagler, D., & Reinisch, C. (2020). Lewis’s medical-surgical nursing: Assessment and management of clinical problems (11th ed.). Elsevier, Inc.
What is Als?. The ALS Association. (n.d.). https://www.als.org/understanding-als/what-is-als