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Myasthenia Gravis
| Question | Answer |
|---|---|
| Patho | Chronic autoimmune disorder. Antibodies destroy or block acetylcholine (ACh) receptors at the neuromuscular junction. |
| Key Symptoms | Ptosis (drooping eyelid), diplopia, facial weakness, dysphagia, dysphonia (voice impairment). Weakness worsens with activity. |
| Tensilon Test | Diagnostic for MG. IV edrophonium improves muscle strength (e.g., ptosis) for 5 minutes. Requires atropine at bedside. |
| Pyridostigmine | Cholinesterase inhibitor (Mestinon). Increases ACh at NMJ. Give 30-45 minutes BEFORE meals for swallowing strength. |
| Myasthenic Crisis | Too little ACh. Severe weakness, respiratory distress (dry). Often triggered by infection. Treat with IVIG or plasmapheresis. |
| Cholinergic Crisis | Too much ACh. From overdose of pyridostigmine. Symptoms: salivation, lacrimation, bradycardia, bronchospasm (wet). Antidote: Atropine. |
| Meal Planning | Eat at peak medication effect. Consume largest meal in morning. Sit upright, use soft foods, have suction available. |
| Respiratory | Weakness of intercostals/diaphragm can lead to respiratory failure. KEY SIGN: Inability to handle secretions/drooling. |
| Nuance | Antibodies block acetylcholine (ACh) receptors at neuromuscular junction, preventing muscle contraction. |
| Repetitive Nerve Stimulation | Shows a decremental response: muscle action potentials decrease with repeated nerve stimulation. |
| ACth Antibodies | Positive in 85-90% of generalized MG patients. Negative in some ocular MG or with MuSK antibodies. |
| Thymus Gland | 75% of MG patients have thymic abnormalities (hyperplasia, thymoma). Thymectomy can improve symptoms, especially if thymoma present. |
| Medication Timing Critical | Pyridostigmine (Mestinon) given 30-45 minutes BEFORE meals to maximize strength for chewing and swallowing. |
| Myasthenic Crisis Precipitants | Infection (most common), surgery, stress, pregnancy, medications (aminoglycosides, beta-blockers, magnesium). |
| Cholinergic Crisis Symptoms | From too much ACh: SLUDGE (Salivation, Lacrimation, Urination, Diarrhea, GI upset, Emesis) + muscle fasciculations, bradycardia. |
| Test Procedure | Atropine must be at bedside. IV edrophonium given; improvement in ptosis within 30-60 seconds confirms MG. |
| Plasmapheresis | Removes circulating antibodies. Provides short-term improvement (days-weeks). Used for crisis or pre-op stabilization. |
| IVIG | Modulates immune response. Alternative to plasmapheresis. Effects last weeks. Monitor for fluid overload, headache. |