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ALS

QuestionAnswer
Lou Gehrig's Disease A progressive neurodegenerative disease causing degeneration of upper and lower motor neurons. Leads to muscle weakness, atrophy, and respiratory failure.
Demographics Most common in ages 40-60. Affects men slightly more than women. Not an autoimmune disease. No cure.
Classic Symptoms Progressive muscle weakness, fasciculations (twitching), spasticity, dysarthria (slurred, nasal speech), dysphagia.
Unique Finding Bowel and bladder function typically remain INTACT because autonomic nerves are not affected.
Respiratory Endpoint Respiratory failure from diaphragm weakness is the most common cause of death. May require mechanical ventilation.
Riluzole Glutamate antagonist. Modestly slows disease progression. Monitor liver function tests.
Drugs Baclofen/dantrolene for spasticity. Modafinil for fatigue. Diazepam for anxiety.
Nursing Priority Advanced care planning early: PEG tube, ventilator decisions, advanced directives, power of attorney.
Bulbar Onset Symptoms begin with muscles of speech and swallowing (dysarthria, dysphagia). Progresses faster than limb-onset. Higher aspiration risk.
UMN vs LMN Signs UMN: Spasticity, hyperreflexia. LMN: Fasciculations, atrophy, hyporeflexia. ALS features a mix of both.
Respiratory Monitor forced vital capacity (FVC), sniff test, and symptoms of hypoventilation (morning headache, fatigue, poor sleep).
Sialorrhea Excessive drooling from facial weakness. Treated with anticholinergics (glycopyrrolate), botox injections, or suctioning.
Dysphagia Modify diet to thicken liquids, use upright positioning, double swallow. PEG tube placement considered early for nutrition.
Communication Use of low-tech (alphabet boards) and high-tech (eye-gaze computer) devices as speech becomes unintelligible.
Multidisciplinary Team Care requires neurologist, pulmonologist, PT/OT, speech therapist, respiratory therapist, social worker, and palliative care.
Symptom of Pseudobulbar Affect Involuntary, uncontrollable episodes of laughing or crying. Treat with dextromethorphan/quinidine (Nuedexta).
Created by: Wasurenboh
 

 



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