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Neuronal Physiology, Physiology, USMLE Step 1 - Full Vignette with Extended Explanations

A 30-year-old man with metabolic and renal comorbidities develops acute-onset dysphagia, dysarthria, and dyspnea following a workplace potluck, progressing to severe bulbar, facial, neck, and proximal limb weakness with autonomic instability. Exam and nerve studies show a striking descending, symmetric paralysis with specific electrophysiologic findings. What clinical clues and diagnostic steps are critical to recognize this neurological emergency, and how should they guide immediate evaluation and management? VIDEO INFO Category: Neuronal Physiology, Physiology, USMLE Step 1 Difficulty: Hard - Advanced level - Challenges experienced practitioners Question Type: Diagnostic Failure Case Type: Critical Condition Explore more ways to learn on this and other topics by going to https://endlessmedical.academy/auth?h... QUESTION A 30-year-old man with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, autosomal dominant polycystic kidney disease, and biopsy-proven focal segmental glomerulosclerosis is brought to the ED for rapidly progressive dysphagia, dysarthria, and dyspnea that began after a workplace potluck dinner 18 hours ago. He had constipation and dry mouth overnight. He works intermittently in healthcare support and quit smoking 5 years ago after a 10-pack-year exposure (0.5 pack/day from age 15 to 25).... OPTIONS A. Collect serum and stool for reference-laboratory botulinum neurotoxin testing (Endopep-MS) and immediately contact the state health department/CDC to obtain heptavalent botulism antitoxin; administer antitoxin without waiting for laboratory confirmation. B. Order anti-P/Q-type voltage-gated calcium channel antibodies and schedule high-frequency RNS later in the week to confirm Lambert-Eaton myasthenic syndrome before starting any disease-specific treatment. C. Give an edrophonium IV challenge to document transient ocular improvement and begin pyridostigmine with atropine premedication while awaiting AChR/MuSK antibody results for seropositive myasthenia gravis. D. Treat presumptively as organophosphate poisoning with pralidoxime boluses and defer any antitoxin until red blood cell cholinesterase activity confirms cholinesterase inhibitor exposure. CORRECT ANSWER A. Collect serum and stool for reference-laboratory botulinum neurotoxin testing (Endopep-MS) and immediately contact the state health department/CDC to obtain heptavalent botulism antitoxin; administer antitoxin without waiting for laboratory confirmation. EXPLANATION Collect serum and stool for reference-laboratory botulinum neurotoxin testing (Endopep-MS) and immediately contact the state health department/CDC to obtain heptavalent botulism antitoxin; administer antitoxin without waiting for laboratory confirmation. This is correct because the patient has a symmetric, descending paralysis with prominent bulbar and autonomic involvement after a high-risk exposure window and electrophysiology showing low resting CMAPs with a marked post-exercise increment ( greater than 120%). That pattern is classic for botulism, a presynaptic SNARE-blocking intoxication that reduces ACh release and causes facilitation after exercise. In critical illness, public health notification is emergent to source heptavalent antitoxin.... --------------------------------------------------- Our cases and questions come from the https://EndlessMedical.Academy quiz engine - multi-model platform. Each question and explanation is forged by consensus between multiple top AI models (GPT, Claude, Grok, etc.), with automated web searches for the latest research and verified references. Calculations (e.g. eGFR, dosages) are checked via code execution to eliminate errors, and all references are reviewed by several AIs to minimize hallucinations. Important note: This material is entirely AI-generated and has not been verified by human experts; despite stringent consensus checks, perfect accuracy cannot be guaranteed. Exercise caution - always corroborate the content with trusted references or qualified professionals, and never apply information from this book to patient care or clinical decisions without independent verification. Clinicians already rely on AI and online tools - myself included - so treat this book as an additional focused aid, not a replacement for proper medical education. Visit https://endlessmedical.academy for more AI-supported resources and cases. This material can not be treated as medical advice. May contain errors. ---------------------------------------------------

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