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Anesthesia for patient with neuromuscular disease

R1 .

Myasthenia gravis
skeletal muscle weakness fatigability : NM junction postsynaptic Ach.recepter ->receptor postsynaptic memb. fold NM junction nicotinic Ach. receptor Ab : Ocular muscle (m/c) . Rest : Infection,pregnancy,stress,surgery : Anticholinesterase (pyridostigmine) 2-4h + immunomodulating therapy corticosteroid->azathioprine,cyclosporine Cholinergic crisis :Sx(muccarinic effect).. Edrophonium.

MG. Anesthetic consideration

Respiratory m. oropharyngeal m. ->immunoglobulin , plasmapheresis Pre.Op consid. : , , Aspiration risk Premedi : opioid,benzodiazepine,.. NMBAs?? Ach. ?? -> .. Propopol : good!! ( ) Volatile agent : .. : cisatracurium,mivacurium ->monitorring -> 1.6 2.peak inspi. Pressure < 25cm H2O 3.VC < 4mg/kg 4.pyridostigmine >750mg

Lambert-eaton myasthenic syndrome

proximal m.weakness paraneoplastic syndrome : NM transmission presynaptic defect ->voltage gated Ca+channelAb Ach.release MG muscle weakness ,anticholine esterase drug Tx :guanidine hydrochloride&diaminopyridine ( plasmapheresis,immunosuppression)

Muscular dystrophies
1.Duchennes Muscular dystrophy XR: Male :1-3/10,000 3-5 Dystrophin:(muscle fiberd sarcolemmam protein) Muscle pseudohypertrophy(fatty infiltration) glucocorticoid->2-3 delay CK level10-100 Female carrier : CK level weakness Dx: muscle biopsy : pulmonary infection Kyphoscoliosis muscle wasting:restrictive ventilation defect

 Pul.HTN degeneration (cardiomyopathy: 10% ) MR:25% (papillaryM.dysfunction ) EKG: P-R interal QRS&ST seg. Atrial arrhythmia 15-25

Beckers muscular dystrophy

X-R & 1/30000 Duchennes muscular dystrophy (cardiomyopathy )

Myotonic dystrophy
Myotonia Myotonia: AD 1/8000 20-30 Cranial muscle -> Distal muscle prox.muscle CK leveld -

Myotonic dystrophy(2)
Multiple organ system involve (endocrine, cataract, sleep disorder) respiratory involvement:Vital capacity -> cor pulmonale GI hypomotility ->pul. Aspiration Uterine atony->labor Cardiac manifestation: atrial arrhythmia heart block

Myotonic dystrophy(3)
Antimyotic Tx & membrane-stabilizing Medication (Phenytoin,quinine sulfate,procainamode) Mexiletine&tocainide : MD Pace maker

Facioscapulohumeral dystrophy
Autosomal dominant variant 1-3/ 100,000 20-30 face,shoulder girdled weakness CK level: NL-slightly inc. Cardiac invoive : rare

Anesthetic consideration (A.Duchennes &Beckers muscular dystrophies)

Muscleweakness,cardiac&pulmonary Manifestation Sedative opioid Succi.: hyperkalemia NMBA: sensitive Volatile anesthesia: Vital capacity 30%

B.Myotonic dysthrophy
Perioperative Respiratory,cardiac Cx G/A : Opioid,sedative,inhalation,intravenous agent sensitive->apnea Succi.:intense myotonic contraction Myotonic contraction:ventilation Neostigmine,physostigmine->myotonia Resional Anes.(myotonic contracture )

 Troublesome myotonia:rare ->procaine i.m ->quinine hydrochroride(300-600mg) i.v NMBA: (Myotonic contraction ) NMBA reversal:myotonic contraction >short acting cisatracurium,mivacurium Post OP shivering:myotonic contraction ->meperidrine Thiopental.propopol,inhalation agent-> Pulmonary Cx:prolonged hypoventilation Atelectasis,pneumonia

Myotonias myotonia congenita

Generalized myotonia Skeletal m. mild,nonprogressive weakness Tx:phenytoin,mexiletine,procainamide.. (tocainide,dantrolene.taurine,)

Periodic paralysis
muscle weakness,paralysis spare :Resting potential partial depol. Muscle fiber K+ attack

1.voltage-gated calcium channelopathy (hypokalemic periodicparalysis)

Hyperthyroidism : ,stenuou exertion Kidney: Na+,K+,Cl- retain ->ICF volume , ECF volume Tx:2-10g oral potassium,acetazoleamide() Glucose solution : K+

2.Sodiumchannelopathy (hyperkalemic periodic paralysis)

potassium sodium channel abnormal inactivation paralysis Hypothermia,pregnancy,Glucocorticoid&K+ ->

Anesthetic consideration
1.EKG monitoring 2.K+ 3. (hypokalemic paralysis) 4.Neuromuscular function monitor 5.NMBA : Unpredictable 6.Succi: hyperkalemic paralysis 7.Hyperthermia