2011 Diagnostic Slide Session Case 06

Pedro Ciarlini MD Yezid Gutierrez MD PhD Pierluigi Gambetti MD Mark Cohen MD National Prion Disease Pathology Surveillance Center University Hospitals Case Medical Center

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:

aggregate is present

.”

“gray matter and leptomeninges with marked nonspecific gliosis. A single perivascular macrophage

• 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 (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

52um

Paragonimus Gnathostoma Angiostrongylus Baylisascaris Strongyloides Trichinella Toxocara

4000-6000um 250-500um 100-260um 30-80um 30-60um 30-60um 15-20um

Helminth External Internal

Baylisascaris Strongyloides Trichinella

Prominent bilateral cuticular alae 1-2  m thick w/fine transverse striations • MN intestinal cells • Large excretory columns (intestine to lateral cord) • Y-shaped esophagus •Intestine •2 sections of reproductive tube •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 devosi Baylisascaris procyonis

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.