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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
What condition should always be considered in patients with signs consistent with bilateral trigeminal neuralgia?
Multiple sclerosis
26
What is Gerstmann Syndrome?
Dominant parietal lobe/angular gyrus lesion Involves: - acalculia - agraphia - L-R disorientation - finger agnosia
27
What might you expect to see with a basillar artery stroke? Name 5 aspects.
Staccato presentation Dysarthria Diplopia Hemiparesis Dropped GCS
28
What might you expect to see with a basillar artery stroke? Name 5 aspects.
Staccato presentation Dysarthria Diplopia Hemiparesis Dropped GCS
29
What tracts are affected in Brown-Sequard? 2 points
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
How does Central Cord Syndrome present?
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
Which tract is preserved in Anterior Cord Syndrome?
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
For consideration of tPA, what BP constitutes exclusion criteria?
Pre-treatment blood pressure of: SBP >185 mmHg or DBP >110 mmHg
33
With regard to strokes, what is the difference between the penumbra and the core?
Penumbra - tissue is ischaemic but not dead Core - tissue is dead (usually deep)
34
What is acceptable blood pressure for stroke patients not receiving tPA?
SBP 220 and lower DBP 120 and lower
35
What should the blood pressure target be following ICH?
SBP 140 to 160 (closer to 140) DBP 80 or so
36
What is the most common pathological process for lobar haemorrhages?
Cerebral amyloid angiopathy Note - genetic association with APOE e4 allele
37
What are McDonald's criteria for MS with respect to time? 3 points
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
What are McDonald's criteria for MS with respect to space? 2 points
2 different locations in the CNS through objective clinical evidence 2 different locations in the CNS through MRI
39
Which areas on MRI are affected by MS? Name 4
Periventricular Juxtacortical Infratentorial Spinal Cord
40
Which injectable therapies are available for MS? Name 2
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
Which oral therapies are available for MS? Name 4
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
Which monoclonal therapies are available for MS? Name 3
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
What treatments exist specifically for secondary progressive MS? Name 2.
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
Which genes are implicated in motor neuron disease? Name 6
SOD1 TARDBP FUS ANG OPTN C9orf72 (assoc FTD and PSP)
44
Name 4 features of pre-symptomatic Parkinson's
Anosmia Constipation REM sleep behaviour disorder Mood changes/increased fatigue
45
Which neuronal protein is thought to be implicated in Parkinson's disease?
a-Synuclein
46
What treatments can be given for rapid eye movement sleep behaviour disorder? Name 3
Withdrawing antidepressants Melatonin alone Melatonin and clonazepam in combination Note - L-DOPA makes things worse
47
Which 2 treatments confer a survival benefit in motor neuron disease?
Riluzole - glutamate inhibitor - 3-6 months NIV at night - 7 months

GCUH FRACP Writtens 2020 (14 decks)

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