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National Prion Disease Pathology Surveillance Center University Hospitals Case Medical Center

2011 Diagnostic Slide Session Case 06. National Prion Disease Pathology Surveillance Center University Hospitals Case Medical Center. Pedro Ciarlini MD Yezid Gutierrez MD PhD Pierluigi Gambetti MD Mark Cohen MD. Clinical History.

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National Prion Disease Pathology Surveillance Center University Hospitals Case Medical Center

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  1. 2011 Diagnostic Slide Session Case 06 National Prion Disease Pathology Surveillance CenterUniversity Hospitals Case Medical Center Pedro Ciarlini MD Yezid Gutierrez MD PhD Pierluigi Gambetti MD Mark Cohen MD

  2. Clinical History • 54 year old mentally retarded Missouri man with staggering gait and incontinence progressing to spastic quadriparesis in less than a week. • Normal CSF; significant cervical spinal stenosis • Decompressive laminectomy + high dose steroids • Failed to improve… • CSF: mild protein elevation (73 mg/dl), no pleocytosis • IVIg for possible stiff man syndrome • MRI: 1-2mm T2W/FLAIR bright foci in corona radiata, subcortical white matter, and thalami, bilaterally. • Right frontal lobe biopsy: “gray matter and leptomeninges with marked nonspecific gliosis. A single perivascular macrophage aggregate is present.” • Developed mild headache, low grade fever, rapidly declined and died 10 weeks after initial presentation.

  3. Brain only autopsy, sent to NPDPSC Prion immunoblot and IHC negative Brain Weight = 1300g

  4. Discussion

  5. Harold Arnold Baylis (1889-1972)

  6. Histopathologic Diagnosis Necrotizing eosinophilic meningoencephalitis

  7. Neoplasms Myeloproliferative LM Carcinomatosis Glioblastoma Hypereosinophilic syndrome DDx of NEM • Infections • Viral • Rickettsial • Helminthic • Immune-mediated • Allergic fungal sinusitis • Rheumatoid, Bechet, Sarcoidosis • Reactions to drugs & devices

  8. Parasitology Rule #1: Size Matters 52um

  9. Nematoda, superfamily Ascaridoidea • Middle Atlantic, Midwest, and Northeast regions of the US • Human disease rare, always entails sequelae or death • Highly prevalent in raccoons (est. 70-80%)

  10. Baylisascaris • B. procyonis first found in raccoons in the NY Zoological Park in 1931 [Ascaris columnaris] (G. McClure) • Genus Baylisascaris: J. F. A. Sprent (1968) • Currently, 7 relatively well studied species • (Partial) sequencing of 4

  11. Parasitology Rule 2-4:Location, Location, Location

  12. Baylisascaris columnaris Baylisascaris transfuga Baylisascaris procyonis Baylisascaris devosi

  13. 15 male, 1 female (nearly all within continental U.S.) 12 < 2.5 years of age All older patients had severe mental deficits 12 patients had pica, geophagia, or both (no information on 3) Visceral, cutaneous, or ocular larva migrans common Rapidly progressing lethargy, ataxia, paralysis Fever usually not prominent CSF eosinophilia 4-68%, PB 5-45% Human Baylisascariasis (n = 16)

  14. Greetings from London! 1 year old porcupine of undetermined sex presented with four months of ataxia and circling gait.

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