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

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 immunoblot and IHC negative Brain Weight = 1300g

Discussion

Harold Arnold Baylis ( )

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 um Gnathostoma um Angiostrongylus um Baylisascaris30-80um Strongyloides30-60um Trichinella30-60um Toxocara15-20um 52um

HelminthExternalInternal Baylisascaris Prominent bilateral cuticular alae MN intestinal cells Large excretory columns (intestine to lateral cord) Y-shaped esophagus Strongyloides 1-2  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 %)

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.