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SBCP Pathology > CNS: Degenerative and Demyelinating Diseases > Flashcards

CNS: Degenerative and Demyelinating Diseases Flashcards

1
Q

Normal weight of adult brain?

A

1250g in female

1400g in male

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2
Q

Gross changes in brain due to ages

A

Narrowing of gyric and widening of sulci

Generalised enlargement of ventricular system

Loss of brain weight

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3
Q

What is dementia

A

An acquired global brain impairment without affecting consciousness

Affects intellect, reason and personality

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4
Q

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

A

75-84 20%

> 85, 50%

Affects F>M

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5
Q

Microscopy findings with Alzheimer’s (6)

A 
Beta A4 amyloid plaques
Neurofibrillary tangles (Tau protein)
Senile plaques
Amyloid angiopathy
Neuronal loss and reactive astrocytosis
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6
Q

Gross finding for Alzheimers?

A

Narrowing of gyri, widening of sulci

Ventricular enlargment

Loss of brain weight

ATROPHIC HIPPOCAMPUS

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7
Q

Two major histological features of Alzheimer’s

A

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

Tau2 +ve Neurofibrillary tangles- highlighted using silver stains/

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8
Q

Progression of vascular dementia

A

Stepwise progression corresponding to episodes of infarction

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9
Q

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

A

Vascular dementia

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10
Q

Risks for vascular dementia

A

Hypertension

M?F

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11
Q

Pick’s disease

A

EARLY ONSET behavioural and personality change due to frontal lobe atrophy

Speech changes due to temporal lobe atrophy

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12
Q

Histology of Pick’s disease

A

Neuronal loss most severe in outer 3 layers of cortex

Neuronal swelling (Pick cells)

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13
Q

What is Creutzfeldt Jakob disease?

A

A transmissible form of dementia caused by prions

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14
Q

Clinical signs of CJD?

A

Myoclonus
Cerebella degeneration

(Due to prions)

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15
Q

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

A

CJD

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16
Q

Prognosis of Creutzfeldt Jakob Disease?

A

Death after 3-12 months of symptoms

17
Q

What causes Parkinson’s disease?

A

Degeneration of dopaminergic neurons, especially within the substantia nigra

18
Q

Clinical features of Parkinson’s disease

A 
Mask like face
Stooped posture
Resting pill rolling tremor
Rigidity
Bradykinesia
Festinating gait
19
Q

What type of gait does Parkinson’s cause?

A

Festinating

20
Q

Mean onset of Parkinson’s

A

61 years

21
Q

How many people with Parkinson’s get dementia?

A

10-15%

22
Q

How to manage early symptoms of Parkinson’s?

A

Levodopa

23
Q

Histology of Parkinson’s disease (2)

A

Pallor of substantia nigra and locus ceruleus

LEWY BODIES (intracytoplasmic inclusion)

24
Q

What causes Huntington’s disease?

A

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

25
Q

Clinical features of Huntington’s

A

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

26
Q

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

A

Huntington’s

27
Q

Histology of Huntington’s

A

Cortical loss of neurons

Reactive astrocytosis

28
Q

Pathology of Multiple Sclerosis

A

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

29
Q

Peak onset of MS? Which gender?

A

Females age 22-40

30
Q

Aetiology of MS?

A

Unknown but more prevalent in northern regions

x15 risk with 1st degree relative

IHC studies suggest a viral autoimmune reaction

31
Q

Clinical presentation of MS (7)

A

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
32
Q

Types of progression of MS? (4)

A

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

33
Q

Signs of MS in CSF?

A

Increased protein
Increased IgG
OLIGOCLONAL BANDS ON ELECTROPHORESIS

34
Q

What bands are shown with MS on electrophoresis?

A

Oligoclonal bands

35
Q

Grossly: Grey-tan irregular plaques in white matter

A

MS

36
Q

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

A

MS

37
Q

Major immune cells involved in MS?

A

Perivascular lymphocytes and macrophages

38
Q

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

A

False, only affects CNS

SBCP Pathology (24 decks)

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