1. CNS

2. Classical Disease Patterns
Degenerative
Inflammatory
Neoplastic
Blood

3. CNS Normal Pathology (13 Questions) Neurons Glia Astrocytes
Oligodendrocytes
Ependymal Cells
Microglia
Pathology (13 Questions)
What is the fifth type of “glial” cell?

4. CNS DEGENERATIVE DISEASES
CORTEX (dementias)
BASAL GANGLIA and BRAIN STEM (parkinsonian)
SPINOCEREBELLAR (ataxias)
MOTOR NEURONS (muscle atrophy)
Question #10
FOUR classical areas for brain degeneration, a decent anatomic classification.

5. CNS DEGENERATIVE DISEASES
CORTEX (dementias)
ALZHEIMER DISEASE
Frontotemporal
Pick Disease (also primarily frontal)
Progressive Supranuclear Palsy (PSP)
CorticoBasal Degeneration (CBD)
Vascular Dementias (MID)
ALZHEIMER disease is many times more common than all the other dementias put together, except for MID.
Progressive supranuclear palsy (PSP) is a rare degenerative tauopathy involving the gradual deterioration and death of selected areas of the brain, chiefly basal ganglia and cortex. CBD is a similar tauopathy. Pick Disease is another tauopathy. Do you see a pattern here?
Are “tau”-opathies (i.e., tau protein buildups) generally synonymous with CNS degenerative diseases? YES.
CNS degenerative diseases were generally regarded as the same as “senility” until the PC police disallowed this word.

6. ALZHEIMER DISEASE CORTICAL (grey matter) ATROPHY
Commonest cause of dementias (majority)
Sporadic, 5-10% familial
CORTICAL (grey matter) ATROPHY
NEURITIC PLAQUES* (extraneuronal)
NEUROFIBRILLARY TANGLES (intraneuronal)
AMYLOID!!! (i.e., “BETA” amyloid)
* NB: NOT at all like MS plaques.

7. Normal sulci.

8. Prominent sulci in cortical atrophy
Prominent sulci in cortical atrophy. Why are the sulci, NOT the gyri, prominent in atrophy? Ans: cortical LOSS

9. Plaques and tangles and beta-amyloid of Alzheimer’s diaease

10. Neuritic plaques, stained with anti- beta amyloid immunostain
Plaques and tangles and beta-amyloid
Neuritic plaques
Neuritic plaques, stained with anti- beta amyloid immunostain

11. Amyloid with congo red stain (LEFT), and Amyloid with congo red stain under polarization (RIGHT). Remember the amyloid of Alzheimer’s disease is BETA amyloid, NOT the usual kind of ALPHA amyloid seen with immunoglobulinproliferative diseases such as myelomas.

12. Neurons with tangles displacing nucleus, H & E

13. Neurons with TANGLES, often displacing NUCLEUS.

14. OTHER CORTICAL DEMENTIAS (tau gene/protein, tau-opathies)
FRONTOTEMPORAL
PICK DISEASE (LOBAR ATROPHY)
PROGRESSIVE SUPRANUCLEAR PALSY (PSP)
CORTICOBASAL DEGENERATION (CBD)
VASCULAR DEMENTIA (MID)
Tau is a gene, Tau protein is a microtubule protein associated with hyperphosphorylation in tau-opathies. MANY cortical dementias are TAU protein, and therefore most cortical dementias are known as TAUOPATHIES.

15. CNS DEGENERATIVE DISEASES
BASAL GANGLIA and BRAIN STEM
Parkinsonism
Parkinson Disease
Multiple System Atrophy
Huntington Disease

16. Parkinsonism PARKINSON DISEASE Is a clinical “syndrome”, NOT a disease
Diminished facial expression
Stooped posture
Slowness of voluntary movement
“Festinating” gate (short, fast)
Rigidity (cogwheel)
“Pillrolling” tremor
The above clinical findings involve pathology of the SUBSTANTIA NIGRA, and include:
PARKINSON DISEASE
MULTIPLE SYSTEM ATROPHY
POSTENCEPHALIC PARKINSONISM
Progr. Supranuc. Palsy, Cort. Basal Degen. (cortical disorders)

17. PALLOR of the SUBSTANTIA NIGRA (and LOCUS COERULEUS)
PARKINSON DISEASE
PALLOR of the SUBSTANTIA NIGRA (and LOCUS COERULEUS)
LEWY BODIES (alpha-synuclein protein)

18. NORMALLY BLACK substantia nigra due to adequate dopamine.

19. PALE substantia nigra in Parkinson’s disease due to inadequate dopamine, normal on right.

20. Which patient has Parkinson’s disease. Ans: the RIGHT Why
Which patient has Parkinson’s disease? Ans: the RIGHT Why? Ans: decreased dopamine

21. Lewy bodies are commonly regarded as diagnostic of Parkinson’s disease also. The main substance of the eosinophilic inclusion is alpha-synuclein.

22. 主要内容 掌握神经系统的细胞及其基本病变； 掌握常见变性疾病的病理特点。 思考题： 1. Alzheimer病的病理特点？
2. Parkinson病的病理特点？
3. 脑组织中蛋白质异常聚集的机制有哪些？哪些疾病可能有蛋白质异常聚集？
竺可青
病理学与法医学研究所
神经科学研究所
浙江大学医学院中国人脑库