Case 20134 Melike Pekmezci MD Arie Perry MD Conflict of interest None to disclose Clinical H istory 68yearold woman Missionary with recent travel history to Ghana Ocular toxoplasmosis on Bactrim ID: 1042342
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1. AANP Diagnostic Slide SessionCase 2013-4Melike Pekmezci, MDArie Perry, MD
2. Conflict of interestNone to disclose
3. Clinical History68-year-old woman Missionary with recent travel history to GhanaOcular toxoplasmosis- on BactrimPresented with neutropenia, odynophagia, and feverMental status subsequently declined and progressed to a comatose state. Neutropenia Bone marrow biopsy infarctsSickle cell trait
4. RadiologyAxial-T2 FRFSE(Fast Recovery Fast Spin Echo)Axial- T2 Gre(Gradient Echo)
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10. What is the differential diagnosis?What special studies would you perform?
11. Diagnostic ConsiderationsInfectiousCNS MalariaToxoplasmosisInflammatory/autoimmuneADEM/AHLLupus encephalitisToxicCarbon monoxide poisoning
12. Diagnostic ConsiderationsVasculitis/vasculopathyMicroscopic polyangiitisBinswanger (subcortical arteriosclerotic) encephalopathyCADASILCerebral amyloid angiopathy
13. Diagnostic ConsiderationsEmbolicDIC/TTPAir embolismFat embolismOtherSickle cell disease/crisis
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15. LFB-PASNeurofilament
16. GFAPCD68
17. Oil Red-O
18. Sudan IV
19. Predisposing Factors for Fat EmbolismTraumaticLong bones Viscera: LiposuctionNon-traumatic Metabolic (pancreatitis)Burn injuryParenteral nutritionSickle cell or thalassemias (with bone marrow infarcts)
20. PathogenesisMobilization of fat globules (or bone marrow) to microcirculationDelayed effects of fat embolism are secondary to toxic effects of free fatty acidsNitric oxide inducible NO synthase (iNOS)Phospholipase A2Free radical and inflammatory cytokines (TNFα, IL-1β, IL-6) Alveolar macrophages (iNOS), neutrophil elastase and myeloperoxidase
21. DiagnosisClinical suspicionAcute chest syndrome in Sickle cell disease…Cardiac defects increase the risk of CNS embolism, but are not required.
22. TreatmentGood arterial oxygenation (oxygen, PEEP, ventilator)Maintenance of intravascular volumeAlbuminBlood transfusionSteroids/Heparin/Alcohol/Dextranare not helpfulprovides intravascular volume + binds fatty acidsmay have a role in sickle cell or thalassemias
23. PrognosisMortality rate 5-15%, better when it is not associated with sickle cell disease.Rarely fulminant form may lead to death within hours of injuryHypoxia and neurologic deficits for days-weeksSubtle personality changes, memory problems
24. Thank you…
25. Sickle Cell Trait and crisisIncreased activityProfessional athletes and militaryHigh altitudeMedicationsBactrim 9 out of 26,627 side effects reported to FDA by June 2013All less than 1 month treatmentMostly children (10-19 y) one adult (50-59 y)Decongestants-pseudoephedrine