Some Bodies in the Brain: A Diagnostic Conference

1. “SOME BODIES IN THE BRAIN”Noon Diagnostic Conference11-20-2003 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)

19. Zebra bodies

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) I

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 Strongly argyrophilic (silver stains - Bielschowsky, Bodian) ++tau, +ubiquitin +/- Pick cells (balloon cells) EM-straight filaments Tau 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 Negative silver stain Ubiquitin positive Negative for tau and alpha-synucelin Composition unknown ubiquitin

42. Pick bodies vs. mnd-inclusion bodies Pick I Tau 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 PAS-positive and ubiquitin-positive Ubiq PAS

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