Neurology Flashcards
(103 cards)
1
Q
MRI sequences for stroke/ demyelination/myositis
A
DWI/FLAIR/STIR
2
Q
Abs for generalised myasthenia gravis
A
AChR (85%); MUSK (10%)
3
Q
Abs for occular Myasthenia
A
50% AChR
4
Q
Mgmt of thymoma/hyperplasia in MG
A
Resect
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
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
7
Q
Initial therapy for MG
A
Pyridostigmine
8
Q
MOA of pyridostigmine
A
Oral acetylcholinesterase inhibitor
9
Q
Rx for severe /rapidly worsening/ crisis MG
A
steroids, PLEX, IVIG, steroid sparing- MMF, Aza.
10
Q
Rx for refractory MG
A
Eculizumab, Rituximab
11
Q
MuSK MG Mgmt is less responsive to…
A
AChesterase inhibitors and thymectomy
12
Q
ADEM triggers
A
vaccination, infection
13
Q
ADEM Ab
A
Anti-MOG
14
Q
ADEM rx
A
Methylprednisolone
15
Q
Severe form of ADEM
A
Acute Hemorrhagic Leukoencephalitis
16
Q
Glatiramer MOA
A
4AA that mimics myelin basic protein, shifts TH1 -> Th2
17
Q
Natalizumab MOA
A
alpha 4 integrin inhibitor; molecule that traffics lymphocytes across BBB
18
Q
Ocrelizumab MOA
A
Anti-CD20
19
Q
Alemtuzumab MOA
A
Anti-CD52 (found on lymphocyte cell surface)
20
Q
Fingolimod MOA
A
SIP-1 receptor inhibitor; retains Th17 lymphocytes within LNs
21
Q
Fingolimod side effects
A
Bradycardia, PML, lymphopaenia
22
Q
Dimethyl Fumarate MOA
A
unknown
23
Q
Teriflunomide MOA
A
Pyrimidine synthesis inhibitor
24
Q
MOG Ab disease presentation in children vs. adults
A
kids- ADEM; adults- NMO
25
HMSN inheritance pattern
Any, depends on type
26
Characteristic findings of HMSN
pes cavus, stork leg
27
HMSN type 1 = CMT1 type/NCS finding/Biopsy
demyelinating/slowed conduction velocity/ onion bulbs
28
When 2 alleles are affected, get HMSN type 1; when 1 affected get...
Hereditary neuropathy with pressure palsy
29
HMSN type 2: type/ NCS
axonal/ reduced CMAP/SNAP
30
Main cancer that causes paraneoplastic syndrome
SCLC
31
Disease of Anti-Hu
Peripheral neuropathy w. limbic encephalitis- SCLC
32
Disease of Anti-Purkinje cell Ab
Cerebellar
33
Disease of anti-amphiphysin
Stiff man like syndrome
34
Disease of Anti-Ma2
Limbic Encephalitis (testicular)
35
Abs in LEMS are against...
Are against pre-synaptic voltage Calcium Channel channels
36
HLA of LEMs
HLAB8 DR3
37
Hallmarks of LEMs
Proximal weakness that improves with exercise, LL worse than UL, sensory neuropathy, arreflexia that improves with exercise, autonomic
38
NCS of LEMs
low CMAP that increases with exercise
39
LEMs mgmt
3-4 diaminopyridine: K channel blocker -> blocks efflux -> prolongs depol; IVIg, Prednisolone, rx of underlying Ca
40
Anti-NMDA features
oro-facial dyskinesias, seizures, drowsy, young female, psych
41
Anti-NMDA mgmt
high dose steroid, cyclophosphamide -> PLEX -> IVIg and rituximab. remove tumour
42
Huntington's new drug
Tominersin- anti-sense oligonucleotide, intrathecal
43
SMA 2 specific new rxs
Nusinersin- anti-sense oligonucleotide, intrathecal. Zolgensma- gene replacement by adenoviral vector
44
CJD hallmarks
rapidly progressive dementia, cerebellar, myoclonus, visual disturbance
45
CJD MRI changes
Are in putamen and head of caudate
46
CSF finding in CJD
14-3-3 protein
47
EEG in CJD
periodic sharp wave complexes
48
MND is associated with what dementia?
FTD
49
MND most common familial gene
C9-ORF, esp. assoc. w. FTD-ALS
50
MND specific drugs
Riluzole- inhibits glutamate excitatory damage; Edaravone- inhibits oxidative stress
51
AD genes increasing risk
PSEN 1 and 2, APP have 100% penetrance. APO E4/E4 increases risk 15x, Late onset AD.
52
AD protective gene
APOE2
53
AD specific drugs x2 and MOA
Donepezil- Cholinesterase inhibitors, NOT for MCI; memantine- NMDA antagonist
54
Stiff person syndrome sx
trunk mainly- pain, increased tone and spasms
55
Stiff person aetiology
idiopathic, autoimmune
56
Abs in stiff person
Anti-GAD (glutamic acid decarboxylase, Anti-GAD/other Abs inhibit GABA receptors -> stiffness)
57
EMG of stiff person
continuous activity in trunk and limbs
58
Stiff person mgmt
Benzos, anti-spasmodics, immunosuppress if life threatening
59
Myotonic Dystrophy Type 1 pattern of weakness
facial, distal limb, and myotonia
60
Myotonic dystrophy Type 1 extra-muscle features
frontal balding, cataracts, DM, low IQ
61
McArdles Disease MOA
Glycogen storage disease V; myophosphorylase deficiency
62
McArdle's classical presentation
exercise induced myalgia, stiffness, cramping, rhabdo and myoglobinuria
63
Duchenne's muscular dystrophy inheritance
X linked
64
Duchenne's muscular dystrophy weakness pattern
proximal and limb girdle
65
Duchenne's gene affected
Dystrophin on X chromosome
66
Becker's mutation
Dystrophin gene Xp21
67
Keppra MOA
inhibits SV2A
68
AED Na channel blockers
Phenytoin, CBZ, valproate, lamotrigine
69
AED for generalised
Valproate, Keppra
70
Best AED for focal seizures
CBZ, lamotrigine
71
Best AED for pregnancy
lamotrigine in pregnancy
72
AED causing weight gain
valproate
73
AED longest half life
Phenytoin
74
AED that induces own metabolism
CBZ
75
AED that causes hyperammonaemic encephalopathy
Valproate
76
Most significant two AED interaction
Lamotrigine and Valproate (are a good combo for refractory epilepsy, VA doubles serum Lam)
77
Sx of Lamotrigine toxicity
dizziness/ ataxia
78
Lamotrigine metabolism
via glucoronidation -> produces inactive metabolite that is renally excreted
79
OCP decreases concentration of which AED
Lamotrigine
80
Which AED should be avoided with Carbapenems?
Na Val (get 0-100% decrease in Na Val level)
81
AED for absence seizures only
Ethosuxamide; can use Lamotrigine for absence seizures
82
JME AED
Na Val/ Lamotrigine if child bearing demographic
83
What is a Jacksonian March?
focal seizure that progresses up limb
84
EEG findings of HSV encephalitis
temporal spike and wave discharged with diffuse b/g slowing
85
Treatment of seizures in neurocystericercosis
Rx seizures before rx the parasite
86
AED that worsens JME
CBZ
87
Alpha synucleinopathies
PD, MSA, LBD
88
Tau-opathies
PSP and CBD
89
PSP MRI sign
Hummingbird
90
In Encephalitis what is the biggest predictor of mortality?
conscious state
91
Top viral causes of encephalitis
HSV, VZV, enteroviruses
92
What is the most common form of HSV to cause encephalitis?
HSV1
93
Nerve injured in THR
sciatic
94
Gerstman's symptoms
acalculia, agraphia, finger agnosia, L-R disorientation
95
Gerstmann's affects what area
dominant parietal lobe
96
1st order neuron in cervical sympathetic chain is from
hypothalamus to C-spine (C8-T2)
97
Second order neuron in cervical sympathetic chain is from
T1 -> synapse at superior cervical ganglion at bifurcation of common carotid
98
Third order neuron in cervical sympathetic chain is from
superior cervical ganglion to eye
99
PSP defining sx
backwards falls, applause sign, supranuclear palsy/ophthalmoplegia w. slowed vertical saccades first
100
PSP differences from PD
Resting tremor RARE, symmetric
101
MSA defining features
pre-motor issues ++ behaviour/urinary dysfunction/REM sleep, Autonomic instability profound, then stridor/ parkinsonism/cerebellar features
102
MSA subtypes
Parkinsonism; cerebellar
103
CBD defining features
MARKEDLY asymmetric parkinsonism; can barely use affected limb- myoclonus, dystonia; prominent cognitive/behavioural problems
