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GCUH FRACP Writtens 2020 > Neurology > Flashcards

Neurology Flashcards

1
Q

What is the triad of symptoms associated with Lambert-Eaton Syndrome?

A

Proximal weakness (legs, arms, distal muscles then oculobulbar)

Areflexia (post-exercise facilitation)

Autonomic features (anticholingeric)

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2
Q

Which HLA allele is associated with 65% of autoimmune Lambert-Eaton cases?

A

HLA-B8-DR3

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3
Q

Which antibodies are typically seen in Lambert-Eaton?

A

Voltage Gated Calcium Channel (VGCC) Abs

Therefore, LEMS is pre-synaptic

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4
Q

Besides tumour removal and immunosuppression with IVIG and prednisolone, what treatment can you give for LEMS and how does it work?

A

3-4-diaminopyridine - blocks efflux of K+ ions, prolonging the duration of depolarisation

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5
Q

What is the MoA of botulinum toxicity?

A

Inhibition of presynaptic ACh release

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6
Q

What is the MoA of botulinum toxicity?

A

Inhibition of presynaptic ACh release

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7
Q

What skin changes are specifically associated with dermatomyositis? Name 5

A

Gottron papules (pathognomonic)

Heliotrope rash

V-sign

Shawl sign

Gottron’s sign

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8
Q

Which antibody is associated with rapidly progressive ILD in dermatomyositis?

A

Anti-MDA-5

Additionally, Anti-Jo-1 is seen in 50% or cases of inflammatory myositis with ILD

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9
Q

What factors confer an increased risk of malignancy in patients with dermatomyositis? Name 5

A

Male

Elderly

Shawl sign

Recurrent disease

Anti-TIF1 Abs

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10
Q

Which malignancies are associated with dermatomyositis?

A

Ovarian, lung, pancreatic, stomach, melanoma, NHL, colorectal

Note - low risk of malignancy in patients with ILD

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11
Q

Which antibody is associated with acute onset, severe rashes and good response to therapy in dermatomyositis?

A

Anti-Mi-2

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12
Q

What drug do you give to patients with dermatomyositis if they have ILD?

A

Cyclophosphamide

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13
Q

Which antibodies are associated with necrotising autoimmune myositis?

A

Anti-SRP (assoc with dilated cardiomyopathy)

Anti-HMG-CoAR (specific; can occur in patients not on statins)

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14
Q

Which HLA allele is associated with necrotising autoimmune myositis?

A

HLA-DRB1*1101

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15
Q

Which antibody is associated with inclusion body myositis?

A

Cytosolic 5-nucleotidase 1A (cN1A)

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16
Q

Which antibodies are seen in cancer-associated dermatomyositis?

A

Anti-T1Fy

Anti-NXP-2

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17
Q

Anti-synthetase syndrome is myositis incorporating 5 features. Please name them.

A

ILD

Fever

Polyarthritis

Mechanic hands

Raynaud’s

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18
Q

Which antibodies are seen in anti-synthetase syndrome?

A

Anti-Jo-1

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19
Q

Which form of epilepsy most often requires surgery?

A

Mesial temporal lobe epilepsy with hippocampal sclerosis

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20
Q

What is the treatment for juvenile myoclonic epilepsy?

A

Valproate

Lamotrigine

Zonisamide

21
Q

Which anti-epileptic is not recommended in the elderly, and what are considered viable alternatives instead?

A

Do not give carbamazepine

Consider lamotrigine or Keppra

22
Q

What is the recommended treatment for focal seizures?

A

Carbamazepine

23
Q

What are the recommended treatments for absence seizures?

A

Valproate

Ethosuximide

24
Q

What are the recommended treatments for idiopathic generalised epilepsy?

A

Valproate

Lamotrigine (first line for women of child-bearing age)

Zonisamide

25
Q

What condition should always be considered in patients with signs consistent with bilateral trigeminal neuralgia?

A

Multiple sclerosis

26
Q

What is Gerstmann Syndrome?

A

Dominant parietal lobe/angular gyrus lesion

Involves:

  • acalculia
  • agraphia
  • L-R disorientation
  • finger agnosia
27
Q

What might you expect to see with a basillar artery stroke? Name 5 aspects.

A

Staccato presentation

Dysarthria

Diplopia

Hemiparesis

Dropped GCS

28
Q

What might you expect to see with a basillar artery stroke? Name 5 aspects.

A

Staccato presentation

Dysarthria

Diplopia

Hemiparesis

Dropped GCS

29
Q

What tracts are affected in Brown-Sequard? 2 points

A

Loss of ipsilateral motor and dorsal columns at lesion level (LMN weakness and complete sensory loss)

Loss of contralateral pain and temperature sensation one or two segments below lesion

30
Q

How does Central Cord Syndrome present?

A

Loss of pain and temp in one or more adjacent dermatomes bilaterally at lesion level

Sensory loss in cape/vest distribution

Later - segmental LMN weakness at lesion level

Later - lateral corticospinal tracts affected meaning UMN weakness and temp/sensation loss below lesion

31
Q

Which tract is preserved in Anterior Cord Syndrome?

A

Dorsal columns

Anterior spinal artery supplies the anterior two-thirds of the spinal cord

Posterior cord syndrome is just a loss of the dorsal column

32
Q

For consideration of tPA, what BP constitutes exclusion criteria?

A

Pre-treatment blood pressure of:

SBP >185 mmHg

or

DBP >110 mmHg

33
Q

With regard to strokes, what is the difference between the penumbra and the core?

A

Penumbra - tissue is ischaemic but not dead

Core - tissue is dead (usually deep)

34
Q

What is acceptable blood pressure for stroke patients not receiving tPA?

A

SBP 220 and lower

DBP 120 and lower

35
Q

What should the blood pressure target be following ICH?

A

SBP 140 to 160 (closer to 140)

DBP 80 or so

36
Q

What is the most common pathological process for lobar haemorrhages?

A

Cerebral amyloid angiopathy

Note - genetic association with APOE e4 allele

37
Q

What are McDonald’s criteria for MS with respect to time? 3 points

A

2 separate attacks, or even a history of an attack

MRI with contrast enhancement of a lesion and no enhancement of another

Oligoclonal bands

38
Q

What are McDonald’s criteria for MS with respect to space? 2 points

A

2 different locations in the CNS through objective clinical evidence

2 different locations in the CNS through MRI

39
Q

Which areas on MRI are affected by MS? Name 4

A

Periventricular

Juxtacortical

Infratentorial

Spinal Cord

40
Q

Which injectable therapies are available for MS? Name 2

A

Interferon Beta

  • 1st line treatment
  • SE flu-like Sx, depression, leukopenia, liver and thyroid abnormalities

Glatiramer Acetate
- SE injection site reactions, post injection systemic reactions

41
Q

Which oral therapies are available for MS? Name 4

A

Laquinimod
- SE mild LFT derangement

Teriflunomide
- teratogenic, GI upset, hair thinning

Dimethyl fumarate/BG12
- SE flushing, diarrhoea, abdo pain, PML in psoriasis patients

Fingolimod

  • sphingosine-1-phosphate receptor modulator
  • SE 1st dose Brady, varicella reactivation, macular oedema, HTN, LFT, lymphopenia
42
Q

Which monoclonal therapies are available for MS? Name 3

A

Natalizumab

  • targets a4b1-integrin
  • SE PML, anxiety, pharyngitis, oedema

Alemtuzumab

  • targets CD52
  • SE herpes, Graves, anti-GBM, ITP

Ocrolizumab

  • CD20
  • PPMS with prior oligoclonal bands on CSF
43
Q

What treatments exist specifically for secondary progressive MS? Name 2.

A

Siponimod

  • selective sphingosine-1-phosphate receptor 1 and 5 moderator
  • SE 1st dose Brady, varicella reactivation, macular oedema, HTN, LFT, lymphopenia

Mitoxantrone
- chemo agent stopping B, T cell and macrophage proliferation
- SE cardiac toxicity
-

43
Q

Which genes are implicated in motor neuron disease? Name 6

A

SOD1

TARDBP

FUS

ANG

OPTN

C9orf72 (assoc FTD and PSP)

44
Q

Name 4 features of pre-symptomatic Parkinson’s

A

Anosmia

Constipation

REM sleep behaviour disorder

Mood changes/increased fatigue

45
Q

Which neuronal protein is thought to be implicated in Parkinson’s disease?

A

a-Synuclein

46
Q

What treatments can be given for rapid eye movement sleep behaviour disorder? Name 3

A

Withdrawing antidepressants

Melatonin alone

Melatonin and clonazepam in combination

Note - L-DOPA makes things worse

47
Q

Which 2 treatments confer a survival benefit in motor neuron disease?

A

Riluzole

  • glutamate inhibitor
  • 3-6 months

NIV at night
- 7 months

GCUH FRACP Writtens 2020 (14 decks)

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