CNS: Degenerative and Demyelinating Diseases

Question 1

Normal weight of adult brain?

Answer 1

1250g in female

1400g in male

Question 2

Gross changes in brain due to ages

Answer 2

Narrowing of gyric and widening of sulci

Generalised enlargement of ventricular system

Loss of brain weight

Question 3

What is dementia

Answer 3

An acquired global brain impairment without affecting consciousness

Affects intellect, reason and personality

Question 4

how many people 75-84 have Alzheimers? Above 85?

Answer 4

75-84 20%

> 85, 50%

Affects F>M

Question 5

Microscopy findings with Alzheimer’s (6)

Answer 5

Beta A4 amyloid plaques
Neurofibrillary tangles (Tau protein)
Senile plaques
Amyloid angiopathy
Neuronal loss and reactive astrocytosis

Question 6

Gross finding for Alzheimers?

Answer 6

Narrowing of gyri, widening of sulci

Ventricular enlargment

Loss of brain weight

ATROPHIC HIPPOCAMPUS

Question 7

Two major histological features of Alzheimer’s

Answer 7

Beta A4 Amyloid plaques, widely distributed, can be stained using silver techniques or Conga Red Stain

Tau2 +ve Neurofibrillary tangles- highlighted using silver stains/

Question 8

Progression of vascular dementia

Answer 8

Stepwise progression corresponding to episodes of infarction

Question 9

Histology: Lacunar infarcts, no more than 15mm (cavitatory infarcts)

Answer 9

Vascular dementia

Question 10

Risks for vascular dementia

Answer 10

Hypertension

M?F

Question 11

Pick’s disease

Answer 11

EARLY ONSET behavioural and personality change due to frontal lobe atrophy

Speech changes due to temporal lobe atrophy

Question 12

Histology of Pick’s disease

Answer 12

Neuronal loss most severe in outer 3 layers of cortex

Neuronal swelling (Pick cells)

Question 13

What is Creutzfeldt Jakob disease?

Answer 13

A transmissible form of dementia caused by prions

Question 14

Clinical signs of CJD?

Answer 14

Myoclonus
Cerebella degeneration

(Due to prions)

Question 15

Histology: Vacuoles,spongiosis in grey matter. Numerous amyloid plaques and neuronal loss concentrated in the cerebellum.

Answer 15

CJD

Question 16

Prognosis of Creutzfeldt Jakob Disease?

Answer 16

Death after 3-12 months of symptoms

Question 17

What causes Parkinson’s disease?

Answer 17

Degeneration of dopaminergic neurons, especially within the substantia nigra

Question 18

Clinical features of Parkinson’s disease

Answer 18

Mask like face
Stooped posture
Resting pill rolling tremor
Rigidity
Bradykinesia
Festinating gait

Question 19

What type of gait does Parkinson’s cause?

Answer 19

Festinating

Question 20

Mean onset of Parkinson’s

Answer 20

61 years

Question 21

How many people with Parkinson’s get dementia?

Answer 21

10-15%

Question 22

How to manage early symptoms of Parkinson’s?

Answer 22

Levodopa

Question 23

Histology of Parkinson’s disease (2)

Answer 23

Pallor of substantia nigra and locus ceruleus

LEWY BODIES (intracytoplasmic inclusion)

Question 24

What causes Huntington’s disease?

Answer 24

Mutation on gene 4, CAG nucleotide repeat sequence from 37->100

No of repeats corresponds to age of onset and duration

CAG repeats increase through generations

Question 25

Clinical features of Huntington's

Answer 25

Chorea Rigidity Cognitive decline/progresses to dementia Increased infections eg bronchopneumonia

Question 26

Gross appearance: Cerebral atrophy and atrophy in caudate nucleus and putamen

Answer 26

Huntington's

Question 27

Histology of Huntington's

Answer 27

Cortical loss of neurons | Reactive astrocytosis

Question 28

Pathology of Multiple Sclerosis

Answer 28

CNS disease where myelin sheaths of neurons are destroyed resulting in PLAQUES Distinct episodes of neurological deficits separated in space and time NO PNS INVOLVEMENT

Question 29

Peak onset of MS? Which gender?

Answer 29

Females age 22-40

Question 30

Aetiology of MS?

Answer 30

Unknown but more prevalent in northern regions x15 risk with 1st degree relative IHC studies suggest a viral autoimmune reaction

Question 31

Clinical presentation of MS (7)

Answer 31

Can have brain, brainstem, or spinal cord involvement -OPTIC NEURITIS (unilateral sight loss due to CNII) Brain stem: - ataxia - nystagmus - diplopia Spinal cord: - parasthesia - sensory impairment - painful muscle spasms - incontinence

Question 32

Types of progression of MS? (4)

Answer 32

Relapsing remitting Secondary progressive: gradual deterioration +/- acute relapses in pt who previously had relapsing remitting Primary progression: gradual, continuous deterioration from the start Progressive relapsing: same as above but with subsequent superimposed relapses

Question 33

Signs of MS in CSF?

Answer 33

Increased protein Increased IgG OLIGOCLONAL BANDS ON ELECTROPHORESIS

Question 34

What bands are shown with MS on electrophoresis?

Answer 34

Oligoclonal bands

Question 35

Grossly: Grey-tan irregular plaques in white matter

Answer 35

MS

Question 36

Histology: Perivascular inflammation and macrophage reaction in assoc with myelin breakdown. Decrease in myelin forming cells with relative preservation of underlying neuronal axons.

Answer 36

MS

Question 37

Major immune cells involved in MS?

Answer 37

Perivascular lymphocytes and macrophages

Question 38

True/false: MS is a progressive disorder affecting both central and peripheral nervous systems

Answer 38

False, only affects CNS