“SOME BODIES IN THE BRAIN” Noon Diagnostic Conference 11-20-2003 Ronald L. Hamilton, M.D....

““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

Some Bodies in the BrainSome Bodies in the Brain

Identify these “bodies” that populate neuropathology

Psammoma bodies

Verocay body

Eosinophilic Granular Bodies (EGB)

Negri bodies

Cortical Lewy Body

Round basophilic inclusion #1(Pick body vs. mnd inclusion body)

Round basophilic inclusion #2(Pick body vs. mnd inclusion body)

Bunina bodies

Corpora amylacea

PAS stain

Lafora body (polyglucosan body)

Hirano body

Granulovacuolar degeneration (Simchowicz bodies)

Marinesco body

Herring bodies

Buscaino bodies (mucocytes)

Zebra bodies

Fingerprint bodies

Psammoma bodies Psammoma bodies

Psammoma bodies:meningioma

Psammoma bodiesPsammoma bodies

Derived from meningothelial whorls

Psammomatous meningiomaspinal cord (females)

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

Verocay bodiesVerocay bodies

SchwannomaAntoni A areas

Infrequent in acuostic and cellular Schwannomas

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

Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)

Gangliogliomas, Pilocytic Astrocytomas, Pleomorphic Xanthoastrocytoma

Degenerating tumor astrocytesPAS-positive

Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)

Eosinophilic Granular Bodies (EGB)Eosinophilic Granular Bodies (EGB)

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Negri bodiesNegri bodies

Rabies encephalitisPurkinje cells, CA-1 hippocampus>10,000 human deaths per year

Negri bodiesNegri bodies

Lewy bodiesLewy bodies

Parkinson’s DiseaseDLB, LBVAD, MSA, age

Lewy bodiesLewy bodies

May be multiple

Lewy bodiesLewy bodies

Cortical Lewy bodiesEntorhinal cortex,

cingulate gyrus, insular cortex, other neocortex

Lewy bodiesLewy bodies

Cortical LB can be difficult to detect on H&EStrongly ubiquitin-positive (vs. globose NFT)

Lewy bodiesLewy bodies

Alpha-synuclein positive, specific and sensitive

Lewy bodiesLewy bodies

Hyaline bodies are abnormal aggregates of AS

May be precursor to LB

Pick bodiesPick bodies

Pick’s diseaseFronto-temporal dementiaSevere neuronal loss and gliosis

(“knife-edge” atrophy)Neocortex, dentate gyrus

Pick bodiesPick bodies

Strongly argyrophilic (silver stains - Bielschowsky, Bodian)

++tau, +ubiquitin+/- Pick cells (balloon cells)EM-straight filaments Pick cell

Tau

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

MND-inclusion bodiesMND-inclusion bodies

Negative silver stainUbiquitin positiveNegative for tau and alpha-

synucelinComposition unknown

ubiquitin

ubiquitin

Pick bodies vs. mnd-inclusion bodiesPick bodies vs. mnd-inclusion bodies

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Pick

MND-inclusion

Tau

ubiquitinBielschowsky

Bunina bodiesBunina bodies

Lower motor neuronsALSUnknown composition

ALS - other LMN inclusionsALS - other LMN inclusions

Hyaline bodiesUbiquitin skeins

Corpora amylaceaCorpora amylacea

Subpial and perivascular most common location

Corpora amylaceaCorpora amylacea

Increased with age,Neurodegeneration.Olfactory bulb, base of

brain, spinal cordAstrocytic inclusion

Corpora amylaceaCorpora amylacea

PAS-positive and ubiquitin-positive

UbiqPAS

Lafora BodiesLafora Bodies

Lofora Body DiseasePolyglucosan Body DiseaseMyoclonic epilepsyAutosomal recessiveIntraneuronal inclusionsLiver biopsy

Hirano bodiesHirano bodies

Hippocampus CA-1, subiculumNeuronal cytoplasmic inclusionActin and actin-related proteinsNon-specificIncreased with age esp. with AD

Hirano bodiesHirano bodies

Hirano Body (x165,000)Hirano Body (x165,000)

Granulovacuolar bodies of SimchowiczGranulovacuolar bodies of Simchowicz

Non-specific, increased in aged and esp. ADHippocampus CA-1, subiculum, more rarely other

Granulovacuolar bodies of SimchowiczGranulovacuolar bodies of Simchowicz

Often multipleTau-positiveUnknown composition

Marinesco bodiesMarinesco bodies

Non-specificSubstantia Nigra, LCAged, Parkinson’sPAS-neg, pink on Masson’s

trichrome

Herring BodiesHerring Bodies

Posterior pituitary, swollen axons, incidental

Herring bodiesHerring bodies

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Buscaino BodiesBuscaino Bodies

Artifact of formalin fixation?<10%, ?not bufferedDo not mistake for edema or

storage disorder

Buscaino BodiesBuscaino Bodies

“mucocytes”PAS-positive

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

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)

Fingerprint bodiesEM60,000-80,000

NCLNCL

Curvilinear bodies

Skin biopsyLymphocytes

(buffy coat prep)

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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