Random Questions 2 Flashcards
1
Q
MOA of ditans
A
agonist of 5HT-1F receptors in trigeminal ganglion
2
Q
Can you use triptans for abortive therapy for cluster headaches?
A
yes
3
Q
What is the first line treatment for blepharospasm?
A
botox
4
Q
Which thalamic nucleus is the relay center for sensory information of the BODY?
A
VPL
5
Q
Which thalamic nuclei is the relay center for sensory information of the face as well as taste?
A
VPM
6
Q
Do people with schizoid PD want to have friends?
A
no they like being alone
7
Q
Do people with avoidant pd want to have friends?
A
yes but they fear rejection
8
Q
Trinucleotide repeat for Friedrich ataxia?
A
GAA
*A.R.
9
Q
Pure sensory stroke is caused by damage to which thalamic nuclei?
A
VPL
10
Q
Common peroneal neuropathy presents how?
A
weakness in ankle dorsiflexion, eversion and toe extension
11
Q
Deep peroneal neuropathy presents how?
A
weakness in toe extension and ankle dorsiflexion
*can also have small patch of numbness between 1st and 2nd tos
12
Q
Does X- linked adrenoleukodystrophy have anterior or posterior white matter changes?
A
posterior
13
Q
How is schizotypal PD present?
A
eccentric, socially awkward, magical thinking
14
Q
Anterior should dislocation can cause injury to what nerve?
A
axillary
15
Q
In which patients should you not use dalfampridine in?
A
epilepsy, CKD
16
Q
Which 2 drugs ar eknown to exacerbate idiopathic generalized epilepsy?
A
oxcarb and carbamazepine
17
Q
What is apneustic breathing?
A
regular deep respirations, inspiratory pause and inadequate expiration
*pons
18
Q
What is ataxic breathing?
A
complete irregularity of breathing, increased periods of apnea and irregular pauses
*medulla
19
Q
Cheyne Stokes breathing is be due to lesions where?
A
midbrain, thalami, b/l cerebrum
20
Q
Eteplirsen is effective in treatment of Duchenne’s muscular dystrophy with which mutation?
A
out of frame mutation of exon 51
21
Q
How does botulism present?
A
DESCENDING weakness, areflexia and opthalmoplegia
22
Q
What is the enzyme deficiency in Pompe disease?
A
alpha glucosidase
23
Q
Phenotype for Pompe?
A
macroglossia, wide open eyes/mouth, hypotonia
24
Q
Phenotype for Zellweger syndrome?
A
hypotonia, midface hypoplasia, hypoplastic supraorbital ridges, prominent high forehead
25
amaurosis fugaux is due to occlusion of which artery?
ophthalmic
26
occlusion of branch of retinal artery causes what kind of vision loss?
segmental, not total loss
27
Treatment for toxo?
pyrimethamine and sulfadiazine
28
Treatment for neurocystercosis?
albendazole
29
treatment for malaria?
chloroquine
30
Bradyzoites and tachyzoites can be seen in what infection?
toxoplasmosis
31
Posterior cortical atrophy has what pathologic findings?
amyloid plaques and neurofibrillary tangles
*variant of Alzheimer's
*visual processing abnormalities
32
MOA of aducanumab?
monoclonal Ig-1 antibody binding to amyloid beta
33
Gene associated with early - onset Alzheimer's ?
Presenilin 1
34
What is the only adult anatomical structure that is derived from notocord?
nucleus pulposus of the intervertebral disc
35
36
What is the best treatment for lance adams?
depakote
37
How does congenital toxo present?
MACROocephaly, hypotonia, jaundice, rash, chorioretinitis, and diffuse scattered intracranial calcifications
38
How does congenital CMV present?
sensorineural deafness, hepatosplenomegaly, MICROcephaly and calcifications (mostly BG)
39
Refsum disease
peripheral neuropathy, palpable enlarged nerves, ataxia, retinitis pigmentosa, cardiac disease
40
What is the defective in Refsum disease?
defective alpha oxidation of phytanic acid
41
Kluvery Bucy syndrome is caused by what lesion?
bilateral temporal lobes
42
Expressive aprosodia (no emotion or meldoy in speech) is caused by a lesion where?
non-dominant hemisphere, specifically frontal lobe
43
What is the triad of Parinaud syndrome?
impaired upward gaze, convergence retraction nystagmus, pupillary hyporeflexia
44
Where is the lesion in Parinaud syndrome?
doral midbrain
45
Which progressive language disorder presents with dysnomia, repetition errors, without deficits in comprehension?
logopenic progressive aphasia
46
What does SPECT scan show in logopenic progressive aphasia?
hypometabolism in dominant temporoparietal regeion
47
Non-fluent primary progressive aphasia exhibit what?
effortful, halting speech
48
What does SPECT show in non-fluent primary progressive aphasia?
hypometabolism in dominant posterior frontal and insular lesions
49
Is CMT type 1A a duplication or deletion of PMP22?
duplication
50
Is hereditary neuropathies with pressure palsies associated with deletion or duplication of PMP22?
deletion
51
Gerstmann syndrome symptoms?
finger agnosia, agraphia, acalculia, left/right confusion
52
What disease is associated with "extreme delta brush"
NMDA encephalitis
53
Where is hypometabolism in Alzheimer's disease?
temporoparietal
54
Progranulin protein (gene PGRN) is associated with what?
FTD
55
West nile presents how?
encephalitis, myalgia, acute flaccid paralysis, areflexia without sensory abnormalities
*asymmetric
56
Mutation sin progranulin protein (PGRN) is associated with what?
FTD
57
lissencephaly is due to impaired what?
cell migration
58
How does Giant axonal neuropathy present?
sensorimotor neuropathy, corticospinal tract involvement with UPMN signs, and optic atrophy, tightly curled hair
*walk on inner edges of feet
59
What are lewy bodies?
aggregates of folded alpha synuclein
60
Lewy bodies are primarily seen where in PD?
substansia nigra and locus coeruleus
61
Aggregation of hyperphosporylated tau protein is seen in which disease?
Alzheimers
62
Which antibodies is opsoclonus myoclonus syndrome associated with?
anti-HU and anti-Ri
63
When someone closes their eyes what happens on EEG?
postiive (downward) deflection
63
What happens when someone looks to the left on EEG?
negative (upward) deflection
64
Inability to make "ok sign" is due to damage to what nerve?
anterior interosseuous nerve
65
Is alzheimer's an alpha synucleinopathy or taoopathy?
tau
66
Pre-term infant with kernicterus can present how?
choreoathetosis
*bilateral cystic lesions in globus pallidi
67
What is the reversal agent for dabigatran?
Idarucizumab
68
Andexanet alfa is a reversal agent for what?
factor Xa inhibitors- rivaroxaban and apixaban
69
Which condition can exacerbate sx of diabetic polyneuropathy due to intermittent hypoxia and sleep fragmentation?
OSA
70
What must be done before doing electrocerebral inactivity testing?
tap testing, sensitivity must be set to <2 uV and double distance between electrodes
71
Benign hereditary chorea is caused by what gene mutation?
thyroid transcription factor (TITF1)
72
Autosomal dominant disease that presents with cold-induced myotonia?
Paramyotonia congenita
*SCN4A
73
Do sx of paratonia congenita worsen with activity or get better?
worsen with activity
74
Do symptoms of myotonia congenita worsen with activity or get better?
improve with activity
*CLCN1 gene
75
Intracranial AVM and mucosal telangiectasias is concerning for what syndrome?
osler-weber-rendu
76
What is a "never event"
a severe medical error that should never occur
*ie having surgery on wrong body part
77
Mutation in alternating hemiplegia of childhood?
ATP1A3
78
How does alternating hemiplegia of childhood present?
sudden transient episodes of paralysis affecting 1 side of the body which can alternate
79
Chordoma arises from the remnants of what?
notochord
80
Does Alexander disease have a anterior or posterior predominant demyelation?
Anterior- A
81
What is apomorphine used for?
*recently came out
sublingual med for "off periods" of PD
82
DMPK gene is associated with what?
myotonic dystophy
83
Does Gerstmann's localize to dominant or non-dominant hemisphere?
dominant
84
Difference between malingering and factitious disorder ?
malingering desire secondary gain
85
Movement disorder with progressive involuntary movements, cognitive decline, behavioral outbursts, elevated LFTs and CK?
chorea acanthocytosis
86
Which areas of the brain are most susceptible to injury from ischemia?
BG, thalami, occipital and perirolandic cortex
87
The laterality of the INO is based on the eye that cannot what?
ADDuct
88
How does Isaac's syndrome present?
muscle cramps, pain, percussion myotonia
*AB against voltage gated K channels leading to hyperexcitability and continuous firing of muscle fibers
89
Fragile X cardiac abnormalities?
aortic arch dilation and MVP
90
What is the MOA of Eculizumab
binds to the human terminal complement protein C5, inhibiting its enzymatic cleavage and consequently preventing the formation of the C5b-induced membrane attack complex (MAC).
91
Which AED is associated with low birth weight?
topiramate
92
"Superior" ocular muscles are responsible for what?
intorsion
93
"inferior" ocular muscles are responsible for what?
extorsion
94
What do you see on EMG with colchicine exposure?
myopathy and neuropathy
95
Patient with behavioral changes and with new habits, new activity?
FTD
96
Where do you put the DBS for epilepsy?
anterior nucleus
97
Where do you put DBS for PD patients?
GPi and STN
98
HINTS exam for central veritgo?
No corrective saccade, skew deviation present, nystagmus is vertical and horizontal
99
HINTS exam for peripheral veritgo?
corrective saccade present, no skew deviation and unidirectional; horizontal nystagmus
100
CSF in AD
elevated total tau, phosphorylated tau
decreased beta amyloid 42
101
What is Fregoli syndrome?
Erroneously perceive different people as being familiar individuals in disguise, often leading to confusion or confrontation.
102
Gene mutation associated with alternating hemiplegia of childhood?
ATP1A3
103
What structures don't have intact BBB?
area postrema, pineal gland, choiroid plexus, posterior pituitary and other circumventricular organs
104
Classic MRI findings for transient global amnesia?
restricted diffusion DWI/ADC in CA1 area of hippocampus
105
Which genetic test should you get before starting Siponimod?
CYP2C9 test
*CYP2C9*3/*3 genotype, in particular, is associated with significantly reduced enzymatic activity.
106
What nuclei is the relay center for sensory info of the BODY?
VPL
107
What nucleus is the relay center for the face/taste?
VPM
108
Bilateral exotropia can be caused by what lesion?
Bilateral MLF lesion
109
The trochlear nucleus sends nerve fibers to which muscle?
Trochlear nerve decusssates prior to exiting the brainstem so innervation is to the contralateral side
110
bulbar complaints, LMN findings, and gynecomastia. Disease?
Kennedy's disease
111
Gene mutation in Kenndy's disease?
CAG repeats on androgen receptor on X chromosome
*x-linked
112
Krabbe disease demyelination
spares U fibers
113
How does Krabbe's present?
infantile onset, developmental regression, spasticity, irritability, exaggerated startle response, pale optic discs
114
Submaximal stimulation is applied when you want to elicit what on EMG?
H-reflex -- so that only sensory nerves are stimulated
115
Tetrabenazine carries a block box warning for what?
depression and suicidality
116
Blackbox warning for carbamazepine?
agranulocytosis and aplastic anemia
117
Most common gene associated with primary generalized dystonia?
TOR1A
118
How does primary generalized dystonia present?
action induced dystonia in early childhood
119
Mutation in Sagawa syndrome aka dopa responsive dystonia?
GCH1 (GTP cyclohydrolase I)
120
Gene mutation in choreoacanthoyctosis?
VPS13A
121
What is a "never event"
Something that should've never happened
ie. doing surgery on wrong body part
122
What artery supplies the posterior limb of the internal capsule?
anterior choroidal (branch directly off ICA)
123
Lissencephaly is a disorder of what
cell migration
124
lateral temporal lobe seizure manifests how?
auditory
ALSO vertigo is another ictal sign
125
depression with psychotic featuers
only experience psychotic features DURING a major depression episode
126
What does non-arteritic optic neuropathy mean?
that the cause of the optic neuropathy is not arteritic in nature
127
What do you see on fundoscopic exam with central retinal artery occlusion?
CHERRY RED SPOT
128
what does the optic disc look like if there is a short posterior cilliary artery occlusion?
pale, swollen
*no cherry red spot on fundoscopy
129
kernicterus (elevated bilirubin) in newborn with abnormal movements?
athetoid cerebral palsy
