文档介绍：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
pressive 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
(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