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Overview of NM disorders: We want to decompensate the NM junction Locatlizatin of neuromusclluar disorders: Nm idsdseaess represent disorders at elvels extending form

the enatireoro horn cell to the uscle which is defined ath e motor unit which has the following componenents: 1. anterior horn 2. peiropheral nerve 3. peripheral nerve (myelin part) 4. NM junction 5. Muscle If has a problem with 1,2 or 3 -- > neuromuscular juniciton disorder 4 and 5 myopathic disorders evaluation approach: ALS: Genetic - 10% Non inherited possible mechanism: Defective glutamate metabolism Oxidative stress Mitochondrial abnormalities Abnormal protein aggregation Cytoskeletal abnormalities Axonal transport Neuroinflammation Peirpheral neruve 1. demyelnatin neuropathy ex. Gullai bafre syndroe 2. axonal nueropahty ex: diabetic, toxci nutritional a. due to hyperglycemia causes nerve damage b. DCCT, pancreatic transplant can result in irpovmeent in neuropathy 3. mononeuritis - ex vasculitis myelonionpahty - much quicker GBSWeakness, ascending Facial weakness in 50% Progresses over 2-4 weeks Minimal oective sensory findings Areflexia Preceding infection Psarking fo sphincter function

Autonomic dysfunction Monophasic illenss0 recovery wthin 2-4 weeks after progression stops Problems: autonomic problemesn , finetion, DVT< PE, hyponatremia, n=NM blockage ocmplciations, pain anxey, depression, nutrition issues Treatw iwht plamaphereis IVIG Neuromuscular junction Myasthenia grvais: flunctauign wekenaess Weakaness idstricution: focal, mutifocal Selectivity of affected im mslesn neck extensors, ;roxial mjscles, wrtist extensor Cranil anerves - innerved nuscle preliertion Muscles od breatehing Myopathy purely motor symptoms and signs Proximaldistrigubiton Absence of sneosry symptoms Ore.. Myopathy: nervconduction ormoal Electromyogram: myopathic CK: elecated Muscle gbipsy: morphololic evicence by histochemistry, EM immynocytohcimecal studies Classification acquired vs inherited

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