“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
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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