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RACP Paeds Written Exam > General Neurology > Flashcards

General Neurology Flashcards

1
Q

3 most common causes of ataxia?

A

Acute cerebellar ataxia
Drug intoxication
Guillain-Barre Syndrome

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2
Q

2yo child presents with sudden inability to sit/walk with wide based gait. Coordination impaired. Viral illness 2/53 ago. Cause of ataxia?

A

Acute cerebellar ataxia

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3
Q

Treatment of acute cerebellar ataxia?

A

Supportive, usually improved within 2-3 weeks however can last up to 3 months
Can sometimes give steroids/IVIG

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4
Q

Most common degenerative ataxia?

A

Ataxia Telangiectasia

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5
Q

18mo child now walking with narrow based gait, nystagmus and apraxia of horizontal gaze. Recurrent sinopulmonary infections. Telangiectasias in conjunctivae and on bridge of nose. High AFP. What dx?

A

Ataxia Telangiectasia

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6
Q

Immune deficiency seen in Ataxia Telangiectasia?

A

Decreased secretory IgA

Diminished IgG2, Ig4 and IgE

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7
Q

What malignancy seen in ataxia telangiectasia and what RF to avoid?

A

Lymphoreticular tumours, brain tumours

Highly radiation sensitive (avoid unnecessary imaging)

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8
Q

Most common hereditary ataxia?

A

Friedrich’s Ataxia

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9
Q

Gene mutation and inheritance of Friederich’s Ataxia?

A

AR

Trinucleotide repeat expansion GAA causing mutation in mitochondrial frataxin protein

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10
Q

11yo child presents with ataxia, loss of lower limb reflexes and diminished sense of vibration in lower limbs. Dysarthric speech. Might have skeletal abnormalities or hypertrophic cardiomyopathy. What dx?

A

Friederich’s Ataxia

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11
Q

Management of Frederich’s Ataxia?

A

Antioxidant therapy - coenzyme Q10

Vitamin E

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12
Q

7yo boy presenting with fever, encephalopathy, ataxia, language disturbance. Recent ED presentation with enterovirus.
MRI shows hyper-intense white matter abnormalities - mostly subcortical
CSF - increased protein
What dx?

A

Acute Disseminated Encephalomyelitis (ADEM)

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13
Q

Management of ADEM?

A

?no management
Steroids - IV methylpred then a PO pred slow wean
IVIG if not responsive to steroids
Plasmapheresis if not responsive to either
Can consider rituximab, cyclophosphamide

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14
Q

Risk of MS after ADEM?

A

2-10%

60-90% of children make full recovery without sequelae

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15
Q

Child presents with bilateral leg weakness, hyperreflexia and clonus, urinary retention.
MRI shows demyelinating lesions of spinal cord with cord swelling and enhancement. Dx?

A

Transverse myelitis

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16
Q

Treatment of transverse myelitis?

A

IV steroids with a slow PO steroid wean
IDC if urinary retention
Plasma exchange if not responsive to steroids

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17
Q

Child presents with vision worsening over the last day or so, on testing has central scotoma and failed Ishihara tests with red numbers. Eye pain with eye movement. Had a viral illness 1/52 ago. Dx?

A

Optic neuritis

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18
Q

Optic neuritis treatment?

A

Steroids (MRI B should be completed prior to commencing)

19
Q

Two arterial circulations in the brain and the point at which they meet?

A

Anterior internal carotid
Vertebrobasilar
Meet at Circle of Willis

20
Q

Risk factors for arterial ischaemic stroke?

A

Arteriopathy (50%) - moyamoya
Cardiac (25%) - inc PFO, post cath/surg, arryhtmia/endocarditis
Haematological - sickle, prothrombotic diseases

21
Q

Child presents with headache, diplopia, lethargy, then has a seizure in ED. Reading through their history you see they have a promthrombotic condition/maybe they have leukaemia and chemo regimen includes L-aparaginase. What dx?

A

Cerebral Venous SInus Thrombosis (CVST)

22
Q

Risk factors for CVST?

A 
Pro-thrombotic conditions - V Leiden, protein C/S deficiency, anti phospholipid syndrome
Dehydration
Iron def anaemia
Drugs - L-asparaginse, COCP
Sepsis/DIC
Local infection - otitis media, mastoiditis
Trauma
Venous malformation
23
Q

Ix for CVST?

A

CT or MR venography

24
Q

Mx for CVST?

A

Heparin or enoxaparin, usually for 3 months with re-imaging for assessment.
30% have thrombus extension in first week
Regular ophthal exam for optic neuropathy secondary to raised ICP

25
Q

Epidural haematoma - what vessel injured?

A

Middle meningeal

26
Q

Subdural hameatoma - what vessel injured?

A

Bridging vein

27
Q

Subdural haematomas of variable age?

A

NAI

28
Q

Child presents with focal neurological deficit and psych sx, angiogram demonstrates features of vasculitis. Dx?

A

CNS vasculitis
- Childhood primary angitis of the CNS (cPACNS)
Secondary CNS vasculitis

29
Q

Bloods for Ix of CNS vasculitis?

A

Inflam markers - CRP ESR FBE
Endothelial markers - vWF proposed biomarker of vasculitis (correlates w disease activity)
Bacterial cultures/viral serology
Complement/autoantibodies
Pro-coagulant profile
LP - CSF and opening pressures, neuronal antibodies

30
Q

Treatment of CPACNS?

A

Corticosteroids (IV pulse therapy)

Antithrombotic therapy - esp if large/medium vessel involvement

31
Q

Child presents with headache, seizures. MRI demonstrates increased signal intensity of occipital lobe on T2 images. No hypertension. What dx?

A

Posterior reversible leukoencephalopathy syndrome (PRES)

32
Q

Child severely encephalopathic. Presented to ED 1 week ago and resp swab confirmed influenza. Bloods largely unremarkable however liver enzymes raised with normal ammonia level. MRIB shows symmetrical lesions in the thalami. What dx?

A

Acute necrotising encephalopathy
Often triggered by viral illness (HHV6, influenza) in genetically susceptible people
RANBP2 gene

33
Q

Treatment of acute necrotising encephalopathy?

A

Steroids & IVIG

34
Q

NMDA encephalitis mechanism?

A

IgG antibodies against NR1 subunit of NMDA receptor causing disruption of function to GABAergic neurons.

35
Q

NMDA encephalitis associated with what underlying disease?

A

Ovarian teratoma

36
Q

LP findings in autoimmune encephalitis?

A

Increased protein, lymphocytic pleocytosis

37
Q

Opsoclonus-myoclonus associated with what underlying disease?

A

Neuroblastoma

38
Q

CSF is produced where?

A

Choroid plexus

39
Q

CSF flow pattern?

A

Lateral ventricles

  • > through foramen of munro to 3rd ventricle
  • > via Silvian fissure to 4th ventricle
  • > via lateral foramina to basal cistern
40
Q

CSF resorbed by?

A

Mostly arachnid villi

Some lymphatic channels & choroid plexus

41
Q

Acute migraine management?

A 
Paracetamol, environmental change
Aspirin 1g (if >12yrs)
Sumitriptan IN (if >12yrs)
Chlorpromazine in 1L NS
42
Q

Migraine prophylaxis indications?

A

Frequent or long lasting migraines, causing sig disability for diminished QoL
Contraindication to acute therapies
Risk of medication overuse
Menstrual migraine

43
Q

Prophylaxis for migraine?

A

Flunarizine (Ca ch blocker)
Beta blockers (contraindicated in asthma/depression)
Cryohepatidine (antihistamine/serotonin antagonist, anticholinergic and blocks Ca channels)
Amitriptyline

44
Q

Child presents with off balance, reaching for furnitures. Might be sweaty and vomit during episode. Episode lasts about 1min then resolves, usually will happen intermittently for days/week then go a longer period with out any episodes. Fhx migraine. What dx?

A

BPPV

RACP Paeds Written Exam (15 decks)

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