Slide 2 Grand Rounds Presentation By Scott Belliston DO PGY2
10/12/12 Slide 3 Dysphagia Elderly. right handed male with PMH of
HTN, HLD, aneurysm in stomach 3cm, and GERDwas transferred from OSH
for further evaluation of dysphagia Hx of dysphagia 3 years ago
improved after esophageal dilatation No pain with swallowing Slide
4 Where? Corticobulbar Tracts Motor strip Medulla Nuclei of Lower
Cranial Nerves Esophagus C-Spine Neuromuscular Junction Presynaptic
Postsynaptic Slide 5 What? Stroke ischemic or hemorrhagic
Intracerebral mass (tumor, abscess, lesion, aneurysm) Amyotrophic
lateral sclerosis Myasthenia Gravis Lambert-Eaton Myasthenic
syndrome Multiple sclerosis Parkinsons disease Toxins/Drugs
(Botulism, snake venom, anticholinergics, antimuscarinics,
tetrodotoxin) Foreign body internal or external compression Slide 6
More history 3 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. Slide 7
Labs Hbg 16.5 WBCs 7.4 Platelets 166 INR 1.1 UDS positive benzos
Sodium 132 Potassium 3.8 Chloride 106 CO2 22 BUN 12 Creatinine 0.72
Glucose 85 HgbA1c 5.3 TSH 1.980 CPK 297 Slide 8 Exam General exam
unremarkable Neuro exam Mental status alert and oriented x 4 Speech
moderate to severe flaccid dysarthria and dysphonia Dysphagia to
solids and liquids CN 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 Slide 9
Edrophonium test Tensilon test (No longer available in US)
Edrophonium a short acting IV acetylcholinesterace inhibitor
Risks/Side effects Slide 10 Edrophonium Test (cont) Prior to
injection After second dose of 5 mg Slide 11 EMG/NCS Slide 12 Slide
13 Other studies Acetylcholine receptor antibodies Acetylcholine
Binding AB 37.0, Acetylcholine Blocking AB 29, Acetylcholine
Modulating AB 34 85% of MG MuSK antibodies not checked 40-70% of
AChR-Ab negative CT chest for thymoma negative 1015% have a thymoma
Slide 14 Myasthenia Gravis 10 to 125 of every 1 million people
worldwide Bimodal distribution 2 nd and 3 rd decades (mainly women)
and 6 th through 8 th decades (mainly men). Antibodies to the
muscle type of nicotinic cholinergic receptors Increased incidence
of RA, SLE, and polymyositis Slide 15 Common Presenting Symptoms
Occular symptoms Ptosis and or diplopia over 50% Bulbar symptoms
Dysarthria, dysphagia and fatigable chewing about 15% Proximal limb
weakness alone less than 5% May get other isolated muscle weakness
but are much less common Myasthenia crisis is when the respiratory
muscles are involved Slide 16 Neuromuscular Junction Slide 17
Physiology Slide 18 Watch out for! Crisis! When to intubate? VC
< 20ml/kg or NIF
