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“SOME BODIES IN THE BRAIN” Noon Diagnostic Conference 11-20-2003
Ronald L. Hamilton, M.D. Associate Professor of Neuropathology, University of Pittsburgh
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Some 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: meningioma
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Psammoma bodies Derived from meningothelial whorls
Psammomatous meningioma spinal cord (females)
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Psammoma bodies melanotic Schwannomas - 50% are psammomatous
half of melanotic psammomatous Schwannomas have Carney complex Auto dominant mutation in Protein kinase A holoenzyme lentiginous facial pigmentation cardiac myxoma, calcifying Sertoli cell tumors endocrine overactivity Cushing syndrome multinodular adrenal hyperplasia acromegaly - pituitary adenoma
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Verocay bodies Schwannoma Antoni A areas
Infrequent in acuostic and cellular Schwannomas
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Verocay 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)
Gangliogliomas, Pilocytic Astrocytomas, Pleomorphic Xanthoastrocytoma Degenerating tumor astrocytes PAS-positive
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Eosinophilic Granular Bodies (EGB)
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Eosinophilic Granular Bodies (EGB)
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Negri bodies Rabies encephalitis Purkinje cells, CA-1 hippocampus
>10,000 human deaths per year
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Negri bodies
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Lewy bodies Parkinson’s Disease DLB, LBVAD, MSA, age
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Lewy bodies May be multiple
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Lewy bodies Cortical Lewy bodies
Entorhinal cortex, cingulate gyrus, insular cortex, other neocortex
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Lewy bodies Cortical LB can be difficult to detect on H&E
Strongly ubiquitin-positive (vs. globose NFT)
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Lewy bodies Alpha-synuclein positive, specific and sensitive
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Lewy bodies Hyaline bodies are abnormal aggregates of AS
May be precursor to LB
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Pick bodies Pick’s disease Fronto-temporal dementia
Severe neuronal loss and gliosis (“knife-edge” atrophy) Neocortex, dentate gyrus
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Pick bodies Tau Strongly argyrophilic (silver stains - Bielschowsky, Bodian) ++tau, +ubiquitin +/- Pick cells (balloon cells) EM-straight filaments Pick cell
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MND-inclusion bodies Motor Neuron Disease (MND) inclusion body
ALS, ALS with dementia or aphasia FTD (mnd-inclusion body dementia), Primary progressive aphasia Superficial neocortex, dentate gyrus NOT IN MOTOR NEURONS
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MND-inclusion bodies ubiquitin ubiquitin Negative silver stain
Ubiquitin positive Negative for tau and alpha-synucelin Composition unknown
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Pick bodies vs. mnd-inclusion bodies
Tau I MND-inclusion ubiquitin Bielschowsky
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Bunina bodies Lower motor neurons ALS Unknown composition
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ALS - other LMN inclusions
Hyaline bodies Ubiquitin skeins
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Corpora amylacea Subpial and perivascular most common location
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Corpora amylacea Increased with age, Neurodegeneration.
Olfactory bulb, base of brain, spinal cord Astrocytic inclusion
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Corpora amylacea Ubiq PAS PAS-positive and ubiquitin-positive
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Lafora Bodies Lofora Body Disease Polyglucosan Body Disease
Myoclonic epilepsy Autosomal recessive Intraneuronal inclusions Liver biopsy
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Hirano bodies Hippocampus CA-1, subiculum
Neuronal cytoplasmic inclusion Actin and actin-related proteins Non-specific Increased with age esp. with AD
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Hirano bodies
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Hirano Body (x165,000)
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Granulovacuolar bodies of Simchowicz
Non-specific, increased in aged and esp. AD Hippocampus CA-1, subiculum, more rarely other
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Granulovacuolar bodies of Simchowicz
Often multiple Tau-positive Unknown composition
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Marinesco bodies Non-specific Substantia Nigra, LC Aged, Parkinson’s
PAS-neg, pink on Masson’s trichrome
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Herring Bodies Posterior pituitary, swollen axons, incidental
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Herring bodies I
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Buscaino Bodies Artifact of formalin fixation ?<10%, ?not buffered
Do not mistake for edema or storage disorder
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Buscaino Bodies “mucocytes” PAS-positive
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Zebra bodies Lysosomal storage disorder
Mucopolysaccharidoses (MPS I, II and III) Hunter-Hurler, Sanfillipo Cranial nerve nuceli, liver, skin PAS-positive, LFB-positive Water soluble Rare in metachromatic leukodystrophy
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Fingerprint bodies Neuronal ceroid lipofuscinosis (NCL, types 1-7)
Batten’s Disease, et al. Autosomal recessive PAS-positive LFB, Sudan Black, Oil-red O Lipid stains remain in paraffin Autofluorescent EM - Skin biopsy, lymphocytes (buffy coat prep)
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Fingerprint bodies EM 60,000-80,000
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NCL Curvilinear bodies Skin biopsy Lymphocytes (buffy coat prep)
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Fingerprint bodies Infantile NCL -
granular osmiphilic deposits (GRODs) Late-infantile NCL - curvilinear bodies Juvenile NCL - FINGERPRINT BODIES Onset age 4-9 years old pigmentary retinopathy seizures, dementia, blindness spastic paraplegia death age 20-30
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