national prion disease pathology surveillance center university hospitals case medical center pedro...
TRANSCRIPT
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National Prion Disease Pathology Surveillance Center
University Hospitals Case Medical Center
Pedro Ciarlini MD
Yezid Gutierrez MD PhD
Pierluigi Gambetti MD
Mark Cohen MD
2011 Diagnostic Slide Session Case 06
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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.
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Brain only autopsy,sent to NPDPSC
Prion immunoblotand IHC negative
Brain Weight = 1300g
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Discussion
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Harold Arnold Baylis(1889-1972)
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Histopathologic Diagnosis
Necrotizing eosinophilic meningoencephalitis
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DDx of NEM
Infections Viral Rickettsial Helminthic
Immune-mediated Allergic fungal sinusitis Rheumatoid, Bechet,
Sarcoidosis Reactions to drugs &
devices
Neoplasms Myeloproliferative LM Carcinomatosis Glioblastoma
Hypereosinophilic syndrome
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Parasitology Rule #1: Size Matters
Paragonimus 4000-6000um
Gnathostoma 250-500um
Angiostrongylus 100-260um
Baylisascaris 30-80um
Strongyloides 30-60um
Trichinella 30-60um
Toxocara 15-20um
52um
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Helminth External Internal
BaylisascarisProminent bilateral cuticular
alae
•MN intestinal cells
•Large excretory columns (intestine to lateral cord)
•Y-shaped esophagus
Strongyloides1-2 m thick
w/fine transverse striations
•Intestine
•2 sections of reproductive tube
Trichinella
•Single reproductive tube
•Large glandular cells (stichocytes)
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• 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%)
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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
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Parasitology Rule 2-4:Location, Location, Location
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Baylisascaris transfuga
Baylisascaris procyonis
Baylisascaris
columnaris
Baylisascaris devosi
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Human Baylisascariasis (n = 16)
• 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%
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Greetings from London!
1 year old porcupine of undetermined sex presented with four months of ataxia and circling gait.
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