8.3.2 CNS Tumours Flashcards

1
Q

What are the two types of CNS tumours?

A

Primary
- Gliomas
- Parenchymal
- Meningeal
- Neuronal
- Poorly differentiated- medulloblastoma

Secondary
- Metastatic- paraneoplastic

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2
Q

What are some examples of gliomas?

A

Think of what glial cells actually are

-Astrocytic tumours
-Oligodendrogliomas
-Ependyomas
-Colloid cysts of 3rd ventricle

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3
Q

What are the types of parenchymals tumours?

A

CNS lymphomas
Germ cell

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4
Q

What are meningeal tumours called?

A

Meningiomas

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5
Q

What are the different types of neuronal tumours?

A

Ganglion cell tumours
Neuroblastomas

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6
Q

What are gliomas?

A

Malignant tumours
Graded 1-5

Astrocytic tumours
- Most common is astrocytic, low to high grade, 80%
- Glioblastoma multiforme- grade 4-5

Oligodendrogliomas
- 20% gliomas, grade 2-4

Ependymoma
- Ventricular system
- Often disseminated into CSF

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7
Q

What are lymphomas?

A

Diffuse, large B cell lymphomas

Associated with EBV

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8
Q

What are germ cell tumours?

A

Midline tumours, pineal and suprasellar

e.g. germinoma

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9
Q

Where do medulloblastomas occur?

A

Cerebellum

20% children
Radiosensitive

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10
Q

What are meningiomas?

A

Benign
Derived from arachnoid meningothelial cells

Can compress structures

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11
Q

How can infection enter into the CNS?

A

Direct- Air sinuses, skull fractures

Haematogenous- Arterial blood, venous sinuses

Iatrogenic- LP, surgery

Peripheral nerve- HZV, viruses

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12
Q

What is affected by infections entering the CNS?

A

Meninges
Aggregates of acute inflammation
Brain parenchyma

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13
Q

What is meningitis?

A

Inflammation of leptomeninges

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14
Q

What are the different types of meningitis?

A

Acute pyogenic- Bacterial
Aseptic- Viral, immunocompromised
Chronic- myco tuberculosis- fungi
Carcinomatosis- spread of cancer

May be due to septicaemia

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15
Q

How does meningitis present?

A

Headache
Photophobia
Irritability
Altered consciousness
Stiff neck- meningeal irritation
Focal neurological impairment

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16
Q

What investigations are done for meningitis?

A

CT scan
Lumbar puncture

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17
Q

What are the complications of meningitis?

A

Cerebral oedema
Cerebral infarction
Cerebral abscess / empyema
Epilepsy
Meningoencephalitis
Septicaemia

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18
Q

What infective organisms cause meningitis at each age?

A

Infants
- Escherichia Coli
- Group B streptococci

Young adults
- Neisseria meningitidis

Older adults
- Streptococcus pneumoniae
- Listeria monocytogenes

Chronic meningitis
- Mycobacterium tuberculosis

19
Q

What organisms causes a cerebral abscess?

A

Streptococci or staphylococci

20
Q

What organisms cause empyema?

A

Polymicrobial (staphylococci, anaerobic gram -ve)

21
Q

What is encephalitis?

A

Infection of the brain parencyma

22
Q

What are some features of encephalitis?

A

Usually viral
Neurone death by viruses
Prominent lymphocytes

23
Q

Give some examples of viruses causing encephalitis and what lobe they affect

A

Herpes Zoster- Temporal lobe

Polio
Spinal cord motor neurones

Rabies
Brainstem

24
Q

What viruses can cause encephalitic syndromes?

A

Herpes simplex virus, HSV-1 and HSV-2
CMV
HIV

25
Q

What are prion diseases?

A

Abnormal cellular protein accumulations which lead to cell injury in and out of the cell:
- Neurone cell death
- Synapse loss
- Microvacuolations
- Lack of inflammation

26
Q

Why are prion diseases infective?

A

Causes abnormal protein aggregates to accumulate in local cells

27
Q

What can cause prion diseases?

A

Sporadic
Familial
Iatrogenic
Ingested (think of House episode with the water)

28
Q

What are the different types of prion disease?

A
  • Creutzfeldt- Jakob Disease and variant CJD
  • Scrapies (sheep)
  • Bovine spongiform encephalopathy (Mad Cow disease)
29
Q

What happens to proteins in prion disease?

A

Normal brain proteins are rich in alpha helices

Initiation
Prion disease ingested. inoculated or sporadic, this causes conformational change to beta pleated sheet

Propagation
Abnormal proteins cause surrounding proteins to undergo conformational change from alpha helices to beta pleated sheets

Aggregation
Abnormal proteins aggregate forming amyloid plaques and spongiform brain

30
Q

What are the features of Creutzfeld Jakob Disease?

A
  • Normally sporadic (familial form)
  • 1 per million very rare
  • Rapidly progressive dementing illness
  • Subtle changes in memory
  • Cerebellar ataxia
  • Global dementia
  • Definitive diagnosis- post mortem
  • Often brain looks normal, may have atrophy or ventricular enlargement
31
Q

What are the features of variant Creutzfeld Jakob Disease?

A
  • Affects young adults
  • Slower progression
  • Starts with behavioural issues
  • Exposure due to cattle- e.g. meat and blood transfusions
  • Prolonged incubation, 15 years
32
Q

What causes neurodegenerative diseases?

A

Loss of neurones
Accumulation of protein aggregates

33
Q

What happens in neurodegenerative diseases affecting the hippocampus and cerebral cortices?

A

Changes in:
- Cognitive
- Memory
- Behaviour
- Language

34
Q

What happens in neurodegenerative diseases affecting the Basal ganglia?

A

Movement disorders, hypo/hyperkinetic

35
Q

What happens in neurodegenerative diseases affecting the cerebellum?

A

Ataxias, motor neurones can also be hit

36
Q

Who is affected by Alzheimer’s disease?

A

3% of 65-74 year olds, increases with age

Sporadic 90% of the time

Can be familial which causes earlier onset, 5-10%

37
Q

What happens in Alzheimer’s?

A

Alpha beta plaques and neurofibrillary tangles (tau) form

This accumulation causes neuronal damage, this leads to loss of neurones

Loss of neurones causes cortical atrophy and shrunken brain

Affects frontal, temporal and parietal lobes

38
Q

How do plaques and tangles form?

A

Amyloidogenic pathway
- Transmembrane protein on chromosome 21 cleaved to alpha beta monomer by beta and and gamma secretase
- Alpha beta monomers clump together forming oligomers, these then aggregate forming fibrils which then form tangles
- Tau aggregates are formed by alpha beta oligomers becoming hyperphosphorylated, this causes neuronal damage

Non-amyloidogenic pathway
- Alpha and gamma secretase cleave

39
Q

Why are people with Down syndrome at increased risk of Alzheimer’s disease?

A

Protein which causes Alzheimer’s is found on chromosome 21

In Down syndrome, there are 3 chromosome 21s, therefore more likely to have plaque and tangle formation

40
Q

What are the signs and symptoms of Alzheimer’s?

A

Impaired memory
Impaired intellectual function
Altered mood and behaviour
Disorientated

41
Q

What causes Parkinson’s disease?

A

Loss of dopaminergic neurones in the substantia nigra pars compacta

Lewy body involvement (alpha synuclein)

42
Q

What are the symptoms of Parkinson’s?

A

Sometimes TRAPPED D*ck Means Femoral Hernia

Shuffling gait

Tremor
Rigidity
Akinesia
Pill-rolling tremor
Postural instability
Expressive aphasia
Dementia

Depression
Micrographia
Facial weakness
Hypokinesia

43
Q

What causes Huntington’s?

A

Autosomal dominant condition

Increased CAG repeats

Mutant protein broken down to aggregates which causes damage

Loss of inhibitory neuronal projection to corpus striatum

44
Q

What are the signs of Huntington’s?

A

CDCD

Chorea
Distonia
Co-ordination loss
Decline in cognition