Q1

What is the most frequent cause of clinically significant subarachnoid haemorrhage?

  • Rupture of a saccular (berry) aneurysm

Pass criteria:

  • Placental ischaemia PLUS 1 other point

Where are saccular aneurysms commonly located?

  • 40% ant comm art
  • 34% middle cerebral art
  • 20% int carotid/PICA
  • 4% Basilar/Posterior Cerebral (most likely to cause problems with vasospasm)

Pass Criteria:

  • At least anterior circulation to pass

What is the aetiology of saccular aneurysms?

  • Generally unknown
  • Not ‘congenital’,
  • Some genetic risk (Polycystic kidney, Ehlers Danlos type 4, Neurofibromatosis type 1, Marfans),
  • Predisposing factors (Smoking, Hypertension)

Pass criteria:

  • 2 to pass

What are the consequences of subarachnoid haemorrhage?

  • Early (Vasospasm and additional ischemic injury)
  • Later (Meningeal fibrosis & scarring, CSF obstruction)

Pass criteria:

  • Placental ischaemia PLUS 1 other point

 


Q2

What is multiple sclerosis?

  • Autoimmune demyelinating disorder characterised by distinct episodes of neurological deficits separated in time, attributable to white matter lesions that are separated in space

Pass criteria:

  • Bold to pass

Describe the pathogenesis of multiple sclerosis.

  • Cellular immune response inappropriately directed against components of myelin sheath. (Not clear how cellular reaction initiated – infection +/- genetics; CD4 and TH1 T-cells; Secrete cytokines…activate macrophages; Activated macrophages and products cause demyelination)
  • Genetic and environmental influences (greater incidence in first degree relatives)

Pass Criteria:

  • Bold to pass

What are the typical cerebrospinal fluid changes found in multiple sclerosis?

  • Elevated protein
  • Mild pleocytosis (33%)
  • Increased gamma globulin
  • Oligoclonal bands (B cell proliferation)

Pass criteria:

  • At least 2

 


Q3

Describe the clinical features of Parkinsonism.
Prompt: How do Parkinsonian patients look?

  • Diminished facial expression, stooped posture, slowness of voluntary movement, festinating gait (progressively shortened, accelerated steps), rigidity and a “pill-rolling” tremor

Pass criteria:

  • 3 out of 6

What are the causes of Parkinsonism?

  • Conditions that cause damage to the nigrostriatal dopaminergic system
    • Parkinson disease
    • Post-encephalitic
    • Familial forms (rare – auto dominant & recessive)
    • trauma/ injuries
    • Drugs – dopamine antagonists/toxins/pesticides
    • Multiple system atrophy, progressive supranuclear palsy

Pass criteria:

  • Bold PLUS 2 others

Outline the possible pathogenesis of Parkinson’s Disease.

  • Possible pathogenesis – no unifying pathogenic mechanism identified
    • Misfolded protein/stress response triggered by alpha-synuclein aggregation
    • Defective proteosomal function due to the loss of the E3 ubiquitin ligase parkin
    • Altered mitochondrial function caused by the loss of DJ-1 and PINK1
    • Genetic variants with gene defects
    • Possible damage to dopaminergic cells from toxins drugs/AI conditions

 


Q4

What are clinical features of Multiple Sclerosis?

  • Distinct episodes of neurological deficits separated by time.
  • Myriad of presentations as lesions separated by space.
  • Unilateral visual impairment (optic neuritis) is common, brainstem, cord lesions

Pass criteria:

  • Bold to pass

 

What is the pathogenesis of Multiple Sclerosis?

  • Exact etiology not established
  • Autoimmune, demyelinating disorder, to white matter lesions separated in space.
  • Genetic linkage, microbial / viral triggers.  CD4+ Th1 T cells react against myelin antigens, release cytokines, activate macrophages.  Inflammatory cells create plaques

Pass Criteria:

  • Pass – 2 out of 3 groups must include biogenic amines and example of each

What might be found in CSF of a patient with MS?

  • Mildly elevated protein; moderate pleocytosis; increased proportion of gamma globulin, oligoclonal bands – reflects B cells

Pass criteria:

  • Bold to pass

 


Q5

What is the morphology of a berry aneurysm?

Medial muscular layer thins as approaches neck and gets thickened hyalinised intima, covered with normal adventitia

What are the common sites of berry (saccular) aneurysms?
Prompt: Anterior or posterior circulation?

Anterior circulation around Circle of Willis at arterial junctions — internal carotid; middle cerebral; anterior cerebral and anterior communicating.

May be multiple (20-30%).

Extra: Anterior circulation around Circle of Willis

What is the natural history of a ruptured berry aneurysm?

1.            Acute onset of severe headache, often with loss of consciousness.

2.            25-50% die at the time.

3.            Rebleeding is common.

4.            Vasospasm in vessels other than the bleeding site can cause secondary ischaemic injury.

5.            In the healing phase, meningeal fibrosis and scarring can cause secondary hydrocephalus.

Pass: 3 of 4

 


Q6

What is Haemophilia A?
Prompt: Do patients with haemophilia A always have a family history? How is it inherited?

A reduction in amount or activity of factor VIII.

Factor VIII is a cofactor for factor IX in the activation of factor X.

X-linked recessive trait
Therefore males and homozygous females
30% have no family history so probably due to a new mutation

Pass: Bold

Why do patients with Haemophilia A bleed?

Lack of factor VIII affects the intrinsic pathway, inappropriate fibrinolysis, inadequate coagulation.
Normally the extrinsic pathway produces initial burst of thrombin activation, that activates the intrinsic pathway. Unable to do so in Haemophilia.

Thrombin activated the intrinsic pathway via factors XI & XII.

Thrombin activate TAFI (Thrombin activatable fibrinolysis inhibitor) which inhibits fibrinolysis.

 


Q7

What are the causes of cerebral infarction?
Prompt: What causes obstruction of blood supply?

Hypoxia and ischaemia—

Thrombotic / Embolic

(Prompt — What are the causes of thrombosis and/or embolus)

Atherosclerosis

Arteritis —

Syphilis, TB, PAN,

Opportunistic infections

(Toxoplasmosis, aspergillosis, CMV)

Dissection.

Primary angiitis of CNS

Hypercoagulation state

Drug abuse (cocaine, amphetamine, heroin)

Embolic cardiac

(Mural: Myocardial infarction, valve, AF)

Arterial, carotid Paradoxical and other (tumour, fat, gas, cost surgical)

 


Q8

Describe the process of peripheral nerve repair following traumatic injury
Prompt: What would you see microscopically?

  1. Death of distal part (+/- some of proximal)
  2. Axonal Cone of Growth 1-2 mm per day
  3. Growth through Schwann cell structure
  4. regenerating Clusters

 


Q9

What are the types of cerebral ischaemic injury?

PROMPT – Describe the patterns of cerebral ischaemic injury.

  • Global cerebral ischaemia (ischaemic/hypoxic encephalopathy) when there is generalised reduction of cerebral perfusion
  • Focal cerebral ischaemia follows reduction of blood flow to a localised area of the brain

Pass Criteria:

  • Both types and description

Toggle Title

PROMPT – Give examples.

  • Embolic
    • From cardiac mural thrombi
    • Thromboemboli from arteries, especially carotid
    • Paradoxical association with cardiac anomalies
    • Tumour, fat, air
  • Thrombotic arterial occlusion/ in situ thrombosis (large vessel disease)
  • Vasculitis (small vessel disease)
    • Infectious – immunosuppression and aspergillus, CMV encephalitis, syphilis, TB
    • Non-infectious e.g. Polyarteritis nodosa, primary angiitis
  • Others
    • e.g. Amphetamines, cocaine, heroin
    • Dissecting aneurysm extracranial arteries
    • Hypercoaguable states

Pass Criteria:

  • 3 causes plus 1 example of each 

Toggle Title

  • Lacunar infarcts (in lenticular nucleus, thalamus, internal capsule, deep white matter, caudate nucleus, pons)
  • Slit haemorrhages
  • Hypertensive encephalopathy
  • Massive intracerebral haemorrhage

Pass Criteria:

  • 4 out of 4

Q10

What changes occur in the spinal cord after a traumatic injury?

  • Acute phase:
    • Haemorrhage
    • Necrosis
    • Axonal swelling in the surrounding white matter at level of injury
  • Late phase:
    • Area of neuronal destruction becomes cystic and gliotic
    • Secondary Wallerian degeneration involving long white matter tracts
    • Liquefactvie necrosis often see in CNS

Pass Criteria:

  • 1 acute, 1 late

What are the features of irreversible injury at the cellular level?

  • Mitochondrial damage:
    • Failure of oxidative phosphorylation –> ATP depletion –> failure of energy dependent cellular functions
  • Membrane damage:
    • Plasma membrane –> enzyme leakage –> cell necrosis

Pass Criteria:

  • 3 out of 4 bold

What are the acute clinical consequences of a cervical spinal cord injury?
Prompt: What happens in a high cervical level injury?

  • Complete or incomplete
  • Spinal shock – Quadriplegia/flaccid paralysis, total anaesthesia, areflexia
  • If above C4 –> respiratory compromise (diaphragmatic paralysis)
  • Neurogenic shock
    • Hypotension
    • Bradycardia
    • Warm dry skin, etc
  • Incomplete syndromes
    • Ie: anterior cord, central cord etc

Pass Criteria:

  • Bold

Neurology 11 to 20