Neuromuscular diseases leading to respiratory failure.pptVIP

55 F Acute worsening of dyspnea on 8/10, 1996 Present illness General weakness, SOB, dysphagia for 2 months BW loss 20 kg/2 months Cathay General Hospital Severe restrictive lung disease Gastric erosion (PES) Past history: DM for 5 years NE ABG Neurological exam Clear consciousness No ptosis EOM: OK MP: 3/3 DTR: ++/++ ABG 8/10 8/11 pH 7.366 7.185 PO2 166.3 113.5 PCO2 58.0 88.0 HCO3 33.4 33.4 SaO2 99.4 96.7 Blood gas Chest PA Laboratory tests Glu(pc) 435 T3 54.8 CPK 402 Ketone + T4 6.8 AchRAb 46.01 Cr 0.7 TSH 0.14 ESR 15/hr Na 145 Hb 16.5 EF 55% K 3.8 Platelet 263K LA 52mm Osmol 309 WBC 9200 EKG NSR Mediatinal CT Contrast Foodborne Botulism Food Contaminated with spores in anaerobic conditions Home canned vegetable/potato preserved sea food Toxin Resistant to proteolysis in stomach Absorption Alkaline pH of intestine dissociates toxin from proteins ? Absorption into circulation Usually adults Foodborne Botulism in Taiwan 1986; 9 cases (2 fatalities) in Chang-Hwa city Type A foodborne botulism Canned peanuts from a unlicensed cannery Malaise, ptosis, diplopia, dysphagia, dysarthria and proximal weakness Implications Poor governmental supervision of canned food Inadequate quantities of orphan drug stored Inefficient system for recalling the problem products Delayed broadcasting of warnings to the public Wound Botulism Toxin is produced locally Drug abuse: most common cause IM or SC heroin for subcutaneous abscesses (50%) Incubation: 4-14 D, longer when wound is debrided Onset: blurred vision bulbar weakness Progression Generalized weakness Dysarthria; dysphagia Pupillary reactivity ? Botulinum types A B Treatment - Botulism Supportive care: respiratory; wound debridement Early Emetics: avoid magnesium containing Lavage Enemas: not when paralytic ileus Antitoxin Most useful in 1st 24 hours Use on clinical diagnosis Lowers fatality rate shortens illness (Type A) Complications (immune): 9% ? 3,4-diaminopyridine Prognosis - Botulism Ventilator depe