Alzheimer
Brain diseases
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Tau
APP
Abeta
VCDN group
flag

Aging

ALS

ALS/PDC Guam
AGD
Bri
CBD
DLDH
DM1
Down S
GSS
FTD
FTDP-17
Huntington
Hallenvorden
IBM
Lewy BD
MSA
NPiD c
Parkinson D Guadeloupe
Parkinson
Dementia  in Parkinson 
Pick
Prion
PSP
PEP
Semantic  D
SSP
  ToD

Many thanks to Pr Dennis Dickson, Neuropathologist, Mayo Clinic, Jacksonville, USA for the information below

 

Feature

Pick’s disease

Corticobasal degeneration

Progressive supranuclear palsy

Argyrophilic grain disease

Insoluble tau

64 & 60kDa

3R tau

68 & 64 kDa; 4R tau

68 & 64 kDa; 4R tau

68 & 64 kDa; 4R tau

EM of filaments

14-16 nm straight filaments; 22-24 nm twisted ribbons

22-24 nm twisted ribbons

15-18 nm straight filaments

9-18 nm straight filaments


 

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

 

 

 

 

 

 

CBD

(4R)

 

Frontoparietal

 

 

 

Substantia nigra

 

 

PSP

(4R)

 

 

 

 

Subthalamic nucleus

Substantia

nigra

 

 

AGD

(4R)

CA2 & amygdala

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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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