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 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.