Exam 4: MG, GBS, ALS Flashcards
Myasthenia Gravis
Autoimmune disorder, affects motor not sensation
Affects vision and face
Pyridostigmide bromide to treat
Clinical manifestations of MG
Ocular: Diplopia and ptsosis (80% of patients), cranial nerves 3, 4, 6
Generalized: Weakness of facial and throat muscles, swallowing (dysphagia), and voice impairment (dysphonia), generalized weakness, cranial nerve 9
- Aspiration and choking risk, soft diet
Diagnosis of MG
Acetylcholinesterase inhibitor test (if eye drooping resolves within 30 seconds = MG, cannot do with hx or CV condition or asthma)
Ice test
Blood test for acetylcholine antibodies
Repetitive nerve stimulation (non-invasive)
Single fiber electromyography (EMG) - detects delay or failure of neuromuscular transmission
MRI (enlarged thymus)
Management of MG
Goal: Improve function and reduce and remove circulating antibodies
Pyridostigmine: Long-term therapy
IVIG: Acute/severe exacerbations (crisis), must start very slowly and only lasts 28 days
TPE: Plasma exchange for acute/severe exacerbation
Thymectomy: Complete removal of thymus glands, 30% attains complete remission
No cure for MG
Pyridostigmine bromine
First line of therapy for MG blocks ACh
Adverse effects: Severe: Bradycardia, cardiac arrest, hypotension, bronchospasm; Cholinergic effects (diarrhea and weakness)
Antidote: Atropine (anti-cholinergic)
Teaching: Take on time in evenly spaced doses Set Alarm, Wear/carry medical alert bracelet, monthly labs
Report severe diarrhea and worsening weakness to provider, stop medication and assess airway, IVIG or plasmic exchange
Myasthenic Crisis
Disease exacerbation or precipitating event, respiratory infection, stress, too much physical activity, lack of sleep
Impairment of function of CN 9, 10, 11, 12
Can’t cough up secretions to clear airway, may need to intubate despite high GCS (PT is neurologically intact)
Management of myasthenic crisis
PT education on s/s (dysphagia, dysarthria, ptosis, diplopia, prominent muscle weakness, dyspnea)
Ensure airways and respiratory support
Avoid sedatives and tranquilizers
Guillian Barre Syndrome (GBS)
Occurs 2-4 weeks after a viral illness, could be a reaction to a vaccination
Immune system attacks peripheral nervous system, progressive muscle weakness that may lead to paralysis (autoimmune)
Starts in feet and travels up (feet tingling and leg numbness), completely cognitively aware
Does not affect cognitive function or LOC
GBS assessment
Minor febrile illness 2-4 weeks before current symptoms
Symmetrical paresthesia in the legs
Progression of symptoms to the arms, trunk, and finally face
Stiffness and pain in the legs and back
Labile HR and BP
LFT may be elevated and creatinine and ESR may be elevated
GBS treatment
IVIG #1 treatment, crisis
Low sedation if intubated because you need a good neuro assessment
Pain is not the priority, it is not deadly. Airway and low BP are the priority because they are DEADLY
Major goal is to improve respiratory function
Need to avoid immunizations during acute phase and for approx. 1 year after onset of disease
ALS
Chronic, progressive, degenerative neurological disease. Incurable and fatal. Autosomal dominant
Weakness beings in the limbs, affects cognition at the end. Results in death in most patients within 5 years of diagnosis
ALS Pathophysiology
Impacts upper (brain) AND lower (spinal cord) motor neurons will eventually effect cognition
Progressive degeneration of axons causes loss of myelin, spinal cord atrophies
Muscles affected leading to denervation and atrophy
Always causes depression due to neurotransmitters in brain
Happens much quicker than MS
Creatinine kinase MAY be elevated (breakdown of muscle)
Riluzole
Slows disease progression of ALS, brings down glutamate levels (not everyone with ALS has high glutamate levels)
Hepatotoxic = Monitor AST/ALTs
