Download clinical history - Neuropathology

“SOME BODIES IN THE BRAIN”
Noon Diagnostic Conference
11-20-2003
Ronald L. Hamilton, M.D.
Associate Professor of Neuropathology,
University of Pittsburgh
Some 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:
meningioma
Psammoma bodies
Derived from meningothelial
whorls
Psammomatous meningioma
spinal cord (females)
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
Verocay bodies
Schwannoma
Antoni A areas
Infrequent in acuostic and
cellular Schwannomas
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
Eosinophilic Granular Bodies (EGB)
Gangliogliomas, Pilocytic Astrocytomas, Pleomorphic
Xanthoastrocytoma
Degenerating tumor astrocytes
PAS-positive
Eosinophilic Granular Bodies (EGB)
Eosinophilic Granular Bodies (EGB)
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Negri bodies
Rabies encephalitis
Purkinje cells, CA-1 hippocampus
>10,000 human deaths per year
Negri bodies
Lewy bodies
Parkinson’s Disease
DLB, LBVAD, MSA, age
Lewy bodies
May be multiple
Lewy bodies
Cortical Lewy bodies
Entorhinal cortex,
cingulate gyrus,
insular cortex, other
neocortex
Lewy bodies
Cortical LB can be difficult to detect on H&E
Strongly ubiquitin-positive (vs. globose NFT)
Lewy bodies
Alpha-synuclein positive, specific and
sensitive
Lewy bodies
Hyaline bodies are abnormal
aggregates of AS
May be precursor to LB
Pick bodies
Pick’s disease
Fronto-temporal dementia
Severe neuronal loss and gliosis
(“knife-edge” atrophy)
Neocortex, dentate gyrus
Pick bodies
Tau
Strongly argyrophilic (silver
stains - Bielschowsky,
Bodian)
++tau, +ubiquitin
+/- Pick cells (balloon cells)
EM-straight filaments
Pick cell
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
MND-inclusion bodies
ubiquitin
ubiquitin
Negative silver stain
Ubiquitin positive
Negative for tau and alphasynucelin
Composition unknown
Pick bodies vs. mnd-inclusion bodies
Pick
Tau
I MND-inclusion
ubiquitin
Bielschowsky
Bunina bodies
Lower motor neurons
ALS
Unknown composition
ALS - other LMN inclusions
Hyaline bodies
Ubiquitin skeins
Corpora amylacea
Subpial and perivascular most common location
Corpora amylacea
Increased with age,
Neurodegeneration.
Olfactory bulb, base of brain,
spinal cord
Astrocytic inclusion
Corpora amylacea
PAS
PAS-positive and ubiquitin-positive
Ubiq
Lafora Bodies
Lofora Body Disease
Polyglucosan Body Disease
Myoclonic epilepsy
Autosomal recessive
Intraneuronal inclusions
Liver biopsy
Hirano bodies
Hippocampus CA-1, subiculum
Neuronal cytoplasmic inclusion
Actin and actin-related proteins
Non-specific
Increased with age esp. with AD
Hirano bodies
Hirano Body (x165,000)
Granulovacuolar bodies of Simchowicz
Non-specific, increased in aged and esp. AD
Hippocampus CA-1, subiculum, more rarely other
Granulovacuolar bodies of Simchowicz
Often multiple
Tau-positive
Unknown composition
Marinesco bodies
Non-specific
Substantia Nigra, LC
Aged, Parkinson’s
PAS-neg, pink on Masson’s
trichrome
Herring Bodies
Posterior pituitary, swollen axons, incidental
Herring bodies
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Buscaino Bodies
Artifact of formalin fixation
?<10%, ?not buffered
Do not mistake for edema or
storage disorder
Buscaino Bodies
“mucocytes”
PAS-positive
Zebra bodies
Lysosomal storage
disorder
Mucopolysaccharidos
es (MPS I, II and III)
Hunter-Hurler,
Sanfillipo
Cranial nerve nuceli,
liver, skin
PAS-positive, LFB-positive
Water soluble
Rare in metachromatic leukodystrophy
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)
Fingerprint bodies
EM
60,000-80,000
NCL
Curvilinear bodies
Skin biopsy
Lymphocytes (buffy
coat prep)
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
s
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