ALS: NCLEX Priorities

ALS: upper and lower motor neuron signs, progressive weakness, respiratory failure, BiPAP, riluzole.

ALS is progressive motor neuron disease. NCLEX tests symptoms, respiratory management, and end-of-life planning.

Recognition

Progressive muscle weakness, fasciculations, dysphagia, dysarthria. Both upper (spasticity, hyperreflexia) and lower (atrophy, fasciculations) motor neuron signs. Sensation and cognition preserved.

Respiratory

Cause of death. Monitor FVC. BiPAP nocturnally, then continuously. Eventually tracheostomy + ventilator (patient/family decision).

Meds

Riluzole, edaravone, modest slowing of progression. Symptomatic management of spasticity, sialorrhea, depression.

Nutrition

Aspiration risk. PEG tube often required. Speech therapy for safe swallowing.

End-of-life

Advance directives early. Hospice when appropriate. Caregiver support.

Frequently asked

Survival after diagnosis?

Median 2-5 years. Longer with respiratory support.

Cognitive impairment in ALS?

Up to 50% have some cognitive/behavioral changes (FTD spectrum). Most retain decision-making capacity.

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