National Prion Disease Pathology Surveillance Center
University Hospitals Case Medical Center
Pedro Ciarlini MDYezid Gutierrez MD PhDPierluigi Gambetti MD
Mark Cohen MD
2011 Diagnostic Slide Session Case 06
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.
Brain only autopsy,sent to NPDPSC
Prion immunoblotand IHC negative
Brain Weight = 1300g
Discussion
Harold Arnold Baylis(1889-1972)
Histopathologic Diagnosis
Necrotizing eosinophilic meningoencephalitis
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
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
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)
• 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%)
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
Parasitology Rule 2-4:Location, Location, Location
Baylisascaris transfuga
Baylisascaris procyonis
Baylisascaris
columnaris
Baylisascaris devosi
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%
Greetings from London!1 year old porcupine of
undetermined sex presented with four months of ataxia and circling gait.
