Neurology Flashcards

1
Q

MRI sequences for stroke/ demyelination/myositis

A

DWI/FLAIR/STIR

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2
Q

Abs for generalised myasthenia gravis

A

AChR (85%); MUSK (10%)

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3
Q

Abs for occular Myasthenia

A

50% AChR

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4
Q

Mgmt of thymoma/hyperplasia in MG

A

Resect

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5
Q

Characteristics of ACh-ve and MUSK +ve MG

A

Female, any age, oculobulbar form (not purely ocular); no thymic pathology and uncertain role of thymectomy

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6
Q

Thymectomy is recommended in whom in MG

A

in absence of thymoma when generalised MG and AChR Ab +ve aged <60. And those with thymoma

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7
Q

Initial therapy for MG

A

Pyridostigmine

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8
Q

MOA of pyridostigmine

A

Oral acetylcholinesterase inhibitor

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9
Q

Rx for severe /rapidly worsening/ crisis MG

A

steroids, PLEX, IVIG, steroid sparing- MMF, Aza.

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10
Q

Rx for refractory MG

A

Eculizumab, Rituximab

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11
Q

MuSK MG Mgmt is less responsive to…

A

AChesterase inhibitors and thymectomy

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12
Q

ADEM triggers

A

vaccination, infection

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13
Q

ADEM Ab

A

Anti-MOG

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14
Q

ADEM rx

A

Methylprednisolone

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15
Q

Severe form of ADEM

A

Acute Hemorrhagic Leukoencephalitis

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16
Q

Glatiramer MOA

A

4AA that mimics myelin basic protein, shifts TH1 -> Th2

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17
Q

Natalizumab MOA

A

alpha 4 integrin inhibitor; molecule that traffics lymphocytes across BBB

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18
Q

Ocrelizumab MOA

A

Anti-CD20

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19
Q

Alemtuzumab MOA

A

Anti-CD52 (found on lymphocyte cell surface)

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20
Q

Fingolimod MOA

A

SIP-1 receptor inhibitor; retains Th17 lymphocytes within LNs

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21
Q

Fingolimod side effects

A

Bradycardia, PML, lymphopaenia

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22
Q

Dimethyl Fumarate MOA

A

unknown

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23
Q

Teriflunomide MOA

A

Pyrimidine synthesis inhibitor

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24
Q

MOG Ab disease presentation in children vs. adults

A

kids- ADEM; adults- NMO

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25
Q

HMSN inheritance pattern

A

Any, depends on type

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26
Q

Characteristic findings of HMSN

A

pes cavus, stork leg

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27
Q

HMSN type 1 = CMT1 type/NCS finding/Biopsy

A

demyelinating/slowed conduction velocity/ onion bulbs

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28
Q

When 2 alleles are affected, get HMSN type 1; when 1 affected get…

A

Hereditary neuropathy with pressure palsy

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29
Q

HMSN type 2: type/ NCS

A

axonal/ reduced CMAP/SNAP

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30
Q

Main cancer that causes paraneoplastic syndrome

A

SCLC

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31
Q

Disease of Anti-Hu

A

Peripheral neuropathy w. limbic encephalitis- SCLC

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32
Q

Disease of Anti-Purkinje cell Ab

A

Cerebellar

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33
Q

Disease of anti-amphiphysin

A

Stiff man like syndrome

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34
Q

Disease of Anti-Ma2

A

Limbic Encephalitis (testicular)

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35
Q

Abs in LEMS are against…

A

Are against pre-synaptic voltage Calcium Channel channels

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36
Q

HLA of LEMs

A

HLAB8 DR3

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37
Q

Hallmarks of LEMs

A

Proximal weakness that improves with exercise, LL worse than UL, sensory neuropathy, arreflexia that improves with exercise, autonomic

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38
Q

NCS of LEMs

A

low CMAP that increases with exercise

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39
Q

LEMs mgmt

A

3-4 diaminopyridine: K channel blocker -> blocks efflux -> prolongs depol; IVIg, Prednisolone, rx of underlying Ca

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40
Q

Anti-NMDA features

A

oro-facial dyskinesias, seizures, drowsy, young female, psych

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41
Q

Anti-NMDA mgmt

A

high dose steroid, cyclophosphamide -> PLEX -> IVIg and rituximab. remove tumour

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42
Q

Huntington’s new drug

A

Tominersin- anti-sense oligonucleotide, intrathecal

43
Q

SMA 2 specific new rxs

A

Nusinersin- anti-sense oligonucleotide, intrathecal. Zolgensma- gene replacement by adenoviral vector

44
Q

CJD hallmarks

A

rapidly progressive dementia, cerebellar, myoclonus, visual disturbance

45
Q

CJD MRI changes

A

Are in putamen and head of caudate

46
Q

CSF finding in CJD

A

14-3-3 protein

47
Q

EEG in CJD

A

periodic sharp wave complexes

48
Q

MND is associated with what dementia?

A

FTD

49
Q

MND most common familial gene

A

C9-ORF, esp. assoc. w. FTD-ALS

50
Q

MND specific drugs

A

Riluzole- inhibits glutamate excitatory damage; Edaravone- inhibits oxidative stress

51
Q

AD genes increasing risk

A

PSEN 1 and 2, APP have 100% penetrance. APO E4/E4 increases risk 15x, Late onset AD.

52
Q

AD protective gene

A

APOE2

53
Q

AD specific drugs x2 and MOA

A

Donepezil- Cholinesterase inhibitors, NOT for MCI; memantine- NMDA antagonist

54
Q

Stiff person syndrome sx

A

trunk mainly- pain, increased tone and spasms

55
Q

Stiff person aetiology

A

idiopathic, autoimmune

56
Q

Abs in stiff person

A

Anti-GAD (glutamic acid decarboxylase, Anti-GAD/other Abs inhibit GABA receptors -> stiffness)

57
Q

EMG of stiff person

A

continuous activity in trunk and limbs

58
Q

Stiff person mgmt

A

Benzos, anti-spasmodics, immunosuppress if life threatening

59
Q

Myotonic Dystrophy Type 1 pattern of weakness

A

facial, distal limb, and myotonia

60
Q

Myotonic dystrophy Type 1 extra-muscle features

A

frontal balding, cataracts, DM, low IQ

61
Q

McArdles Disease MOA

A

Glycogen storage disease V; myophosphorylase deficiency

62
Q

McArdle’s classical presentation

A

exercise induced myalgia, stiffness, cramping, rhabdo and myoglobinuria

63
Q

Duchenne’s muscular dystrophy inheritance

A

X linked

64
Q

Duchenne’s muscular dystrophy weakness pattern

A

proximal and limb girdle

65
Q

Duchenne’s gene affected

A

Dystrophin on X chromosome

66
Q

Becker’s mutation

A

Dystrophin gene Xp21

67
Q

Keppra MOA

A

inhibits SV2A

68
Q

AED Na channel blockers

A

Phenytoin, CBZ, valproate, lamotrigine

69
Q

AED for generalised

A

Valproate, Keppra

70
Q

Best AED for focal seizures

A

CBZ, lamotrigine

71
Q

Best AED for pregnancy

A

lamotrigine in pregnancy

72
Q

AED causing weight gain

A

valproate

73
Q

AED longest half life

A

Phenytoin

74
Q

AED that induces own metabolism

A

CBZ

75
Q

AED that causes hyperammonaemic encephalopathy

A

Valproate

76
Q

Most significant two AED interaction

A

Lamotrigine and Valproate (are a good combo for refractory epilepsy, VA doubles serum Lam)

77
Q

Sx of Lamotrigine toxicity

A

dizziness/ ataxia

78
Q

Lamotrigine metabolism

A

via glucoronidation -> produces inactive metabolite that is renally excreted

79
Q

OCP decreases concentration of which AED

A

Lamotrigine

80
Q

Which AED should be avoided with Carbapenems?

A

Na Val (get 0-100% decrease in Na Val level)

81
Q

AED for absence seizures only

A

Ethosuxamide; can use Lamotrigine for absence seizures

82
Q

JME AED

A

Na Val/ Lamotrigine if child bearing demographic

83
Q

What is a Jacksonian March?

A

focal seizure that progresses up limb

84
Q

EEG findings of HSV encephalitis

A

temporal spike and wave discharged with diffuse b/g slowing

85
Q

Treatment of seizures in neurocystericercosis

A

Rx seizures before rx the parasite

86
Q

AED that worsens JME

A

CBZ

87
Q

Alpha synucleinopathies

A

PD, MSA, LBD

88
Q

Tau-opathies

A

PSP and CBD

89
Q

PSP MRI sign

A

Hummingbird

90
Q

In Encephalitis what is the biggest predictor of mortality?

A

conscious state

91
Q

Top viral causes of encephalitis

A

HSV, VZV, enteroviruses

92
Q

What is the most common form of HSV to cause encephalitis?

A

HSV1

93
Q

Nerve injured in THR

A

sciatic

94
Q

Gerstman’s symptoms

A

acalculia, agraphia, finger agnosia, L-R disorientation

95
Q

Gerstmann’s affects what area

A

dominant parietal lobe

96
Q

1st order neuron in cervical sympathetic chain is from

A

hypothalamus to C-spine (C8-T2)

97
Q

Second order neuron in cervical sympathetic chain is from

A

T1 -> synapse at superior cervical ganglion at bifurcation of common carotid

98
Q

Third order neuron in cervical sympathetic chain is from

A

superior cervical ganglion to eye

99
Q

PSP defining sx

A

backwards falls, applause sign, supranuclear palsy/ophthalmoplegia w. slowed vertical saccades first

100
Q

PSP differences from PD

A

Resting tremor RARE, symmetric

101
Q

MSA defining features

A

pre-motor issues ++ behaviour/urinary dysfunction/REM sleep, Autonomic instability profound, then stridor/ parkinsonism/cerebellar features

102
Q

MSA subtypes

A

Parkinsonism; cerebellar

103
Q

CBD defining features

A

MARKEDLY asymmetric parkinsonism; can barely use affected limb- myoclonus, dystonia; prominent cognitive/behavioural problems