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“SOME BODIES IN THE BRAIN” Noon Diagnostic Conference

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1 “SOME BODIES IN THE BRAIN” Noon Diagnostic Conference 11-20-2003
Ronald L. Hamilton, M.D. Associate Professor of Neuropathology, University of Pittsburgh

2 Some Bodies in the Brain
Identify these “bodies” that populate neuropathology

3 Psammoma bodies

4 Verocay body

5 Eosinophilic Granular Bodies (EGB)

6 Negri bodies

7 Cortical Lewy Body

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

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

10 Bunina bodies

11 Corpora amylacea

12 PAS stain Lafora body (polyglucosan body)

13 Hirano body

14 Granulovacuolar degeneration (Simchowicz bodies)

15 Marinesco body

16 Herring bodies

17 Buscaino bodies (mucocytes)

18

19 Zebra bodies

20

21 Fingerprint bodies

22 Psammoma bodies Psammoma bodies: meningioma

23 Psammoma bodies Derived from meningothelial whorls
Psammomatous meningioma spinal cord (females)

24 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

25 Verocay bodies Schwannoma Antoni A areas
Infrequent in acuostic and cellular Schwannomas

26 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

27 Eosinophilic Granular Bodies (EGB)
Gangliogliomas, Pilocytic Astrocytomas, Pleomorphic Xanthoastrocytoma Degenerating tumor astrocytes PAS-positive

28 Eosinophilic Granular Bodies (EGB)

29 Eosinophilic Granular Bodies (EGB)

30 Negri bodies Rabies encephalitis Purkinje cells, CA-1 hippocampus
>10,000 human deaths per year

31 Negri bodies

32 Lewy bodies Parkinson’s Disease DLB, LBVAD, MSA, age

33 Lewy bodies May be multiple

34 Lewy bodies Cortical Lewy bodies
Entorhinal cortex, cingulate gyrus, insular cortex, other neocortex

35 Lewy bodies Cortical LB can be difficult to detect on H&E
Strongly ubiquitin-positive (vs. globose NFT)

36 Lewy bodies Alpha-synuclein positive, specific and sensitive

37 Lewy bodies Hyaline bodies are abnormal aggregates of AS
May be precursor to LB

38 Pick bodies Pick’s disease Fronto-temporal dementia
Severe neuronal loss and gliosis (“knife-edge” atrophy) Neocortex, dentate gyrus

39 Pick bodies Tau Strongly argyrophilic (silver stains - Bielschowsky, Bodian) ++tau, +ubiquitin +/- Pick cells (balloon cells) EM-straight filaments Pick cell

40 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

41 MND-inclusion bodies ubiquitin ubiquitin Negative silver stain
Ubiquitin positive Negative for tau and alpha-synucelin Composition unknown

42 Pick bodies vs. mnd-inclusion bodies
Tau I MND-inclusion ubiquitin Bielschowsky

43 Bunina bodies Lower motor neurons ALS Unknown composition

44 ALS - other LMN inclusions
Hyaline bodies Ubiquitin skeins

45 Corpora amylacea Subpial and perivascular most common location

46 Corpora amylacea Increased with age, Neurodegeneration.
Olfactory bulb, base of brain, spinal cord Astrocytic inclusion

47 Corpora amylacea Ubiq PAS PAS-positive and ubiquitin-positive

48 Lafora Bodies Lofora Body Disease Polyglucosan Body Disease
Myoclonic epilepsy Autosomal recessive Intraneuronal inclusions Liver biopsy

49 Hirano bodies Hippocampus CA-1, subiculum
Neuronal cytoplasmic inclusion Actin and actin-related proteins Non-specific Increased with age esp. with AD

50 Hirano bodies

51 Hirano Body (x165,000)

52 Granulovacuolar bodies of Simchowicz
Non-specific, increased in aged and esp. AD Hippocampus CA-1, subiculum, more rarely other

53 Granulovacuolar bodies of Simchowicz
Often multiple Tau-positive Unknown composition

54 Marinesco bodies Non-specific Substantia Nigra, LC Aged, Parkinson’s
PAS-neg, pink on Masson’s trichrome

55 Herring Bodies Posterior pituitary, swollen axons, incidental

56 Herring bodies I

57 Buscaino Bodies Artifact of formalin fixation ?<10%, ?not buffered
Do not mistake for edema or storage disorder

58 Buscaino Bodies “mucocytes” PAS-positive

59 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

60 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)

61 Fingerprint bodies EM 60,000-80,000

62 NCL Curvilinear bodies Skin biopsy Lymphocytes (buffy coat prep)

63 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

64 s I


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