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Many thanks to Pr Dennis Dickson,
Neuropathologist, Mayo Clinic, Jacksonville, USA for the information below
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Historical
term |
Clinical |
Distribution |
Histopathology |
Current Term
|
|
Type A |
Personality
changes, amnestic syndrome, dementia |
Frontal and temporal
poles and limbic structures |
Pick bodies & ballooned neurons |
PiD |
|
Type B |
Focal cortical
signs, extrapyramidal signs, dementia |
Superior
frontal and parietal cortices; basal ganglia; substantia nigra |
Ballooned neurons |
CBD |
|
Type C |
Dementia,
personality changes |
|
Neither Pick
bodies nor ballooned neurons |
FTLD (DLDH) |
|
Feature |
Pick’s disease |
Corticobasal
degeneration |
Progressive
supranuclear palsy |
Argyrophilic grain
disease |
|
Gross appearance |
Sharply circumscribed
lobar (frontotemporal) atrophy |
Circumscribed
atrophy, parasagittal frontal and parietal |
Premotor and frontal
atrophy |
Variable or none,
temporal atrophy |
|
Cortex |
Status spongiosis |
Superficial
spongiosis |
Minimal or none |
None |
|
Ballooned neurons |
Abundant |
Abundant |
None or sparse,
limbic lobe (see AGD) |
Frequent, limbic lobe |
|
Tau- or
Gallyas positive neuronal lesions |
Pick body |
Pre-tangles,
skein-like, NFT-like, Pick body-like |
Globose NFT,
pretangles |
Pretangles, grains |
|
Threads and thread-like lesions |
Sparse to variable |
Numerous, gray & white matter cortical, striatal & brainstem |
Variable, basal
ganglia & diencephalon |
Minimal, medial
temporal lobe |
|
Glial pathology |
Glial fibrillary
tangles, Pick body -like inclusions in oligodendrocytes |
Astrocytic plaques,
coiled bodies |
Tufted astrocytes,
coiled bodies |
Coiled bodies, tau-positive
astrocytes |
|
Subthalamic nucleus |
Minimal |
Minimal |
Marked neuronal loss |
Minimal |
|
Substantia nigra |
Variable |
Marked |
Marked |
None |
|
Pons & medulla |
Mild; pontine Pick
bodies |
Mild; pontine threads
and pretangles |
Marked pathology,
tegmental; pontine nuclei NFT |
None |
|
Cerebellum |
None |
Mild, Purkinje cell
torpedoes |
Moderate, grumose
degeneration in dentate |
None |
|
Disease |
Limbic lobe |
Association
cortices |
Motor cortex |
Basal ganglia |
Thalamus |
Midbrain |
Pons & medulla |
Cerebellum
(dentate) |
|
PiD
(3R) |
CA1,
dentate & amygdala
|
Limbic & frontotemporal |
|
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CBD
(4R) |
|
Frontoparietal |
|
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|
Substantia nigra |
|
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PSP
(4R) |
|
|
|
|
Subthalamic nucleus |
Substantia
nigra |
|
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AGD
(4R) |
CA2 & amygdala
|
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The intensity of the
shading reflects the severity of the neuronal and glial tau pathology in a given
region. Note that in both PiD and CBD, the brunt of the pathology is in
cortical areas, but that the distribution is different; limbic/frontotemporal in
PiD versus frontoparietal in CBD. AGD is a tauopathy limited to the temporal
lobe. PSP is a tauopathy with the brunt of the pathology in brainstem and
diencephalon
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