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Transcript of “SOME BODIES IN THE BRAIN” Noon Diagnostic Conference 11-20-2003 Ronald L. Hamilton, M.D....
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““SOME BODIES IN THE BRAIN”SOME BODIES IN THE BRAIN”
Noon Diagnostic ConferenceNoon Diagnostic Conference11-20-2003 11-20-2003
Ronald L. Hamilton, M.D.Associate Professor of Neuropathology,
University of Pittsburgh
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Some Bodies in the BrainSome Bodies in the Brain
Identify these “bodies” that populate neuropathology
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Psammoma bodies
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Verocay body
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Eosinophilic Granular Bodies (EGB)
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Negri bodies
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Cortical Lewy Body
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Round basophilic inclusion #1(Pick body vs. mnd inclusion body)
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Round basophilic inclusion #2(Pick body vs. mnd inclusion body)
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Bunina bodies
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Corpora amylacea
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PAS stain
Lafora body (polyglucosan body)
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Hirano body
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Granulovacuolar degeneration (Simchowicz bodies)
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Marinesco body
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Herring bodies
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Buscaino bodies (mucocytes)
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Zebra bodies
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Fingerprint bodies
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Psammoma bodies Psammoma bodies
Psammoma bodies:meningioma
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Psammoma bodiesPsammoma bodies
Derived from meningothelial whorls
Psammomatous meningiomaspinal cord (females)
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Psammoma bodiesPsammoma bodies
melanotic Schwannomas - 50% are psammomatoushalf of melanotic psammomatous Schwannomas have Carney complex Auto dominant mutation in Protein kinase A holoenzyme
lentiginous facial pigmentationcardiac myxoma, calcifying Sertoli cell tumorsendocrine overactivity
Cushing syndromemultinodular adrenal hyperplasia
acromegaly - pituitary adenoma
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Verocay bodiesVerocay bodies
SchwannomaAntoni A areas
Infrequent in acuostic and cellular Schwannomas
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Verocay bodiesVerocay bodies
Bilateral acoustic schwannomas = NF II autosomal dominant 22q12 - merlin (schwannomin)
similar to cytoskeletal proteins moesin, ezrin, radixin (MER)+meningiomas, spinal ependymomas, posterior lens opacities, meningioangiomatosis
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Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)
Gangliogliomas, Pilocytic Astrocytomas, Pleomorphic Xanthoastrocytoma
Degenerating tumor astrocytesPAS-positive
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Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)
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Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)
I
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Negri bodiesNegri bodies
Rabies encephalitisPurkinje cells, CA-1 hippocampus>10,000 human deaths per year
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Negri bodiesNegri bodies
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Lewy bodiesLewy bodies
Parkinson’s DiseaseDLB, LBVAD, MSA, age
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Lewy bodiesLewy bodies
May be multiple
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Lewy bodiesLewy bodies
Cortical Lewy bodiesEntorhinal cortex,
cingulate gyrus, insular cortex, other neocortex
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Lewy bodiesLewy bodies
Cortical LB can be difficult to detect on H&EStrongly ubiquitin-positive (vs. globose NFT)
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Lewy bodiesLewy bodies
Alpha-synuclein positive, specific and sensitive
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Lewy bodiesLewy bodies
Hyaline bodies are abnormal aggregates of AS
May be precursor to LB
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Pick bodiesPick bodies
Pick’s diseaseFronto-temporal dementiaSevere neuronal loss and gliosis
(“knife-edge” atrophy)Neocortex, dentate gyrus
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Pick bodiesPick bodies
Strongly argyrophilic (silver stains - Bielschowsky, Bodian)
++tau, +ubiquitin+/- Pick cells (balloon cells)EM-straight filaments Pick cell
Tau
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MND-inclusion bodiesMND-inclusion bodies
Motor Neuron Disease (MND) inclusion body
ALS, ALS with dementia or aphasia FTD (mnd-inclusion body dementia),Primary progressive aphasiaSuperficial neocortex, dentate gyrusNOT IN MOTOR NEURONS
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MND-inclusion bodiesMND-inclusion bodies
Negative silver stainUbiquitin positiveNegative for tau and alpha-
synucelinComposition unknown
ubiquitin
ubiquitin
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Pick bodies vs. mnd-inclusion bodiesPick bodies vs. mnd-inclusion bodies
I
Pick
MND-inclusion
Tau
ubiquitinBielschowsky
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Bunina bodiesBunina bodies
Lower motor neuronsALSUnknown composition
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ALS - other LMN inclusionsALS - other LMN inclusions
Hyaline bodiesUbiquitin skeins
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Corpora amylaceaCorpora amylacea
Subpial and perivascular most common location
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Corpora amylaceaCorpora amylacea
Increased with age,Neurodegeneration.Olfactory bulb, base of
brain, spinal cordAstrocytic inclusion
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Corpora amylaceaCorpora amylacea
PAS-positive and ubiquitin-positive
UbiqPAS
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Lafora BodiesLafora Bodies
Lofora Body DiseasePolyglucosan Body DiseaseMyoclonic epilepsyAutosomal recessiveIntraneuronal inclusionsLiver biopsy
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Hirano bodiesHirano bodies
Hippocampus CA-1, subiculumNeuronal cytoplasmic inclusionActin and actin-related proteinsNon-specificIncreased with age esp. with AD
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Hirano bodiesHirano bodies
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Hirano Body (x165,000)Hirano Body (x165,000)
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Granulovacuolar bodies of SimchowiczGranulovacuolar bodies of Simchowicz
Non-specific, increased in aged and esp. ADHippocampus CA-1, subiculum, more rarely other
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Granulovacuolar bodies of SimchowiczGranulovacuolar bodies of Simchowicz
Often multipleTau-positiveUnknown composition
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Marinesco bodiesMarinesco bodies
Non-specificSubstantia Nigra, LCAged, Parkinson’sPAS-neg, pink on Masson’s
trichrome
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Herring BodiesHerring Bodies
Posterior pituitary, swollen axons, incidental
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Herring bodiesHerring bodies
I
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Buscaino BodiesBuscaino Bodies
Artifact of formalin fixation?<10%, ?not bufferedDo not mistake for edema or
storage disorder
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Buscaino BodiesBuscaino Bodies
“mucocytes”PAS-positive
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Zebra bodiesZebra bodiesLysosomal storage
disorderMucopolysaccharid
oses (MPS I, II and III)
Hunter-Hurler, Sanfillipo
Cranial nerve nuceli, liver, skin
PAS-positive, LFB-positiveWater solubleRare in metachromatic leukodystrophy
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Fingerprint bodiesFingerprint bodiesNeuronal ceroid lipofuscinosis(NCL, types 1-7)Batten’s Disease, et al.Autosomal recessivePAS-positiveLFB, Sudan Black, Oil-red OLipid stains remain in paraffinAutofluorescentEM - Skin biopsy, lymphocytes
(buffy coat prep)
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Fingerprint bodiesEM60,000-80,000
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NCLNCL
Curvilinear bodies
Skin biopsyLymphocytes
(buffy coat prep)
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Fingerprint bodiesFingerprint bodiesInfantile NCL - granular osmiphilic deposits
(GRODs)Late-infantile NCL -
curvilinear bodiesJuvenile NCL - FINGERPRINT
BODIESOnset age 4-9 years old
pigmentary retinopathyseizures, dementia, blindnessspastic paraplegiadeath age 20-30
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ss
I