Grand Rounds Presentation

Grand Rounds PresentationBy Scott Belliston DO PGY210/12/12DysphagiaElderly. right handed male with PMH of HTN, HLD, aneurysm in stomach 3cm, and GERDwas transferred from OSH for further evaluation of dysphagiaHx of dysphagia 3 years ago improved after esophageal dilatation No pain with swallowing

Where?Corticobulbar TractsMotor strip MedullaNuclei of Lower Cranial NervesEsophagus C-SpineNeuromuscular JunctionPresynapticPostsynapticWhat?Stroke ischemic or hemorrhagicIntracerebral mass (tumor, abscess, lesion, aneurysm)Amyotrophic lateral sclerosisMyasthenia Gravis Lambert-Eaton Myasthenic syndrome Multiple sclerosisParkinsons disease Toxins/Drugs (Botulism, snake venom, anticholinergics, antimuscarinics, tetrodotoxin)Foreign body internal or external compressionMore history3 years ago developed dysphagia was diagnosed with allergies to ragweed. GI procedure improved symptoms.One week before admission was eating and choked on a sandwich hasnt been able to eat or drink since. GI doctor told him no stricture that he had ALS. OSH neurology workup MRI brain no stroke, EMG of facial muscles and tongue negative. Acetylcholine receptor antibodies pending. Video swallow failed. Pt on TPN refusing PEG tube.LabsHbg 16.5WBCs 7.4Platelets 166INR 1.1UDS positive benzosSodium 132Potassium 3.8Chloride 106CO2 22BUN 12Creatinine 0.72Glucose 85HgbA1c 5.3TSH 1.980CPK 297ExamGeneral exam unremarkableNeuro examMental status alert and oriented x 4Speech moderate to severe flaccid dysarthria and dysphonia Dysphagia to solids and liquidsCN mild right eye ptosis, Obicularis oculi 3/5 Obicularis oris 2/5, unable to protrude tongue or place in cheek Motor Neck flexor 4/5 wrist flexors and hip flexors 4+/5

Edrophonium test Tensilon test (No longer available in US)Edrophonium a short acting IV acetylcholinesterace inhibitor

Risks/Side effects Edrophonium Test (cont)Prior to injectionAfter second dose of 5 mg

EMG/NCS

EMG/NCS

Other studiesAcetylcholine receptor antibodies Acetylcholine Binding AB 37.0, Acetylcholine Blocking AB 29, Acetylcholine Modulating AB 3485% of MG MuSK antibodies not checked40-70% of AChR-Ab negativeCT chest for thymoma negative1015% have a thymomaMyasthenia Gravis10 to 125 of every 1 million people worldwide Bimodal distribution 2nd and 3rd decades (mainly women) and 6th through 8th decades (mainly men).Antibodies to the muscle type of nicotinic cholinergic receptorsIncreased incidence of RA, SLE, and polymyositisCommon Presenting SymptomsOccular symptomsPtosis and or diplopia over 50%Bulbar symptomsDysarthria, dysphagia and fatigablechewing about 15%Proximal limb weakness alone less than 5%May get other isolated muscle weakness but are much less commonMyasthenia crisis is when the respiratory muscles are involvedNeuromuscular Junction

Physiology

Watch out for!Crisis!When to intubate?VC < 20ml/kg or NIF