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know your neuro question bank > Random questions > Flashcards

Random questions Flashcards

1
Q

ALS patients are at risk of developing which dementia?

A

FTD - abnormal accumulation of TDP-43, including genetic forms associated with mutations in the genes C9ORF72, GRN, TARDBP, and VCP.

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2
Q

Middle medulla is primarily supplied by what?

A

Anterior spinal artery

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3
Q

What mutations can cause autosomal dominant partial epilepsy with auditory features (ADPEAF)?

A

LGI-1

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4
Q

What mutation is associated with benign familial neonatal epilepsy?

A

KCNQ2

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5
Q

What mutation is associated with familial frontal lobe epilepsy.

A

CNRNA4

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6
Q

A lesion to which hypothalamic nuclei can lead to hyperthermia?

A

anterior nuceli - temp regulation

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7
Q

lateral nucleus of hypothalamus regulates what?

A

thirst/hunger

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8
Q

Ventromedial nucleus of hypothalamus regulates what?

A

satiety

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9
Q

Dorsomedial nucleus of hypothalamus regulates what?

A

aggression

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10
Q

Charcot Marie Tooth is associated with what pathology?

A

Recurrent episodes of demyelination and remyelination of peripheral nerves lead to the concentric proliferation of Schwann cells around axons, also known as “onion bulb” formations.

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11
Q

What enzyme is mutated in Neimann-Pick>

A

sphinomyelinase

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12
Q

What enzyme is mutated in Tay-Sach’s disease?

A

Hexosamindase A deficiency

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13
Q

What enzyme is mutated in Krabbe disease?

A

Galactosylceramidase deficiency

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14
Q

What enzyme is mutated in Gaucher disease?

A

Glucosylceramidase deficiency

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15
Q

What enzyme is deficient in metachromatic leukodystrophy?

A

Arylsulfatase A deficiency

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16
Q

Zinc toxicity can cause deficiency of what?

A

Copper- present similarly to subacute combined degeneration

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17
Q

What med can be used for pseduobublar affect?

A

dextromethorphan-quinidine

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18
Q

One of the first choice agents for OCD?

A

Paroxetine

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19
Q

Are PSP and corticobaslar degeneration tau-opathies or alpha synucleinopathies?

A

Tau

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20
Q

Is Alzheimer’s alpha synuclein or tauopathy?

A

tau

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21
Q

What drugs can cause drug induced tremor?

A

Amiodarone, lithium, selective serotonin reuptake inhibitors, caffeine, valproic acid, immunosuppressants (tacrolimus, ciclosporin, and dopamine antagonists (haloperidol, thioridazine) are known to cause drug-induced tremors.

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22
Q

Hot cross bun sign on imaging is suggestive of what disorder?

A

MSA

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23
Q

Unlike other movement disorders, the hallmark of this disorder is autonomic dysfunction including urinary incontinence, erectile dysfunction, and orthostatic hypotension.

A

MSA

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24
Q

What transverses through the jugular foramen?

A

The glossopharyngeal nerve (CN IX), vagus nerve (CN X), and spinal accessory nerve (CN XI)

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25
Q

acroparesthesias (burning, tingling pain in the upper and lower extremities), hypohidrosis, and angiokeratomas. Which disease?

A

Fabry - X linked recessive lysosomal storage disorder
enzyme: A-galactosidase A

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26
Q

Lumbar stenosis symptoms are exacerbated with lumbar flexion or extension?

A

extension

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27
Q

What are the segments of the carotid artery?

A

cervical (C1), petrous (C2), lacerum (C3), cavernous (C4), clinoidal (C5), ophthalmic (C6), and communicating (C7).

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28
Q

Which triptan has the longest half life and is FDA approved for menstrual headaches?

A

Frovatriptan

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29
Q

Telencephalon develops into what?

A

cerebral hemispheres, the basal ganglia, the hippocampus, and the amygdala.

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30
Q

myelencephalon develops into what?

A

medulla

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31
Q

Diencephalon develops into what?

A

thalamus, hypothalamus, and retina.

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32
Q

Mesencephalon develops into what?

A

midbrain

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33
Q

Metencephalon develops into what?

A

pons and cerebellum

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34
Q

myelomeningoclee is associated with what condition?

A

Chiari II malformation

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35
Q

Oculomasticatory myorhythmia is pathognomic of which disease?

A

Whipple’s
**t.whipplei

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36
Q

non-epileptic paroxysm in newborn characterized by exaggerated startle response, tapping on nose/face?

A

hyperekplexa - 1/3 familial cases are associated with glycine receptor alpha 1 subunit gene mutation

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37
Q

anti-ma2 autoantibodies are most commonly associated with what tumor?

A

testicular

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38
Q

CCM1 mutation is associated with what?

A

numerous cavernous malformations
*chromosome 7q

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39
Q

Leigh syndrome MRI?

A

symmetrical T2 hyperintense lesions in BG, periaqueductal grey and cerebral peduncles or brainstem

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40
Q

locus coeruleus is the primary source of what?

A

norepi

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41
Q

nucelus basalis of meynert produces what?

A

acetylcholine

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42
Q

Sturge-Weber has what MRI findings?

A

angiomas involving leptomeninges, choroid and face
*port wine stain
*refractory epilepsy

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43
Q

Tourette’s diagnosis

A

a patient must have two or more motor tics and at least one vocal tic for at least one year.

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44
Q

Depression with psychotic features

A

Patients who only experience psychotic symptoms during a major depression episode

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45
Q

Schizoaffective disorder

A

sx of schizophrenia with concurrent mood disorder. The psychotic sx must be present for at least 2 weeks without mood sx

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46
Q

what is the only thalamic artery to branch off the PCOM?

A

polar (tuberothalamic) artery

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47
Q

Donepezil MOA

A

AChE-inhibitor

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48
Q

Memantine MOA

A

Primarily via N-methyl-d-aspartate (NMDA) receptor blockade,

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49
Q

Kleine Levin syndrome

A

young males, requires at least 2 episodes of recurrent time limited hypersomia with behavioral (hyperphagia, irritability, sexual) or cognitive change with return to baseline between episodes

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50
Q

First dose bradycardia in MS drug?

A

Fingolomid

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51
Q

most common sider effect of dimethyl fumarate?

A

skin flushing
*can also cause nausea, diarrhea and leukopenia

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52
Q

Decorticate positioning lesion?

A

ABOVE the red nucelus (which is at the line of the midbrain)

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53
Q

Decerebrate positioning lesion?

A

At or BELOW red nucleus

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54
Q

Which meds should be avoided in Dravet syndrome?

A

Na channel blockers
*Dravet is a mutation in Na channel gene SCNA1

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55
Q

HSV encephalitis is known to trigger what?

A

NMDA encephalitis

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56
Q

Autosomal dominant nocturnal frontal lobe epilepsy

A

Sleep-related hyperkinetic AWARE seizures. Patients can have a prodrome of fear before the motor component of the seizure. *Mutations of nicotinic acetylcholine receptor genes CNRNA4 or CHRNB2 can cause this epilepsy syndrome.

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57
Q

NF-1 are at risk for developing what?

A

optic nerve/chiasm glioma, pilocytic astrocytoma, and neurofibromas.

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58
Q

Germinomas histology?

A

tumor cells are positive for cKIT, alkaline phosphatase, and SALL4.

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59
Q

Tangier disease

A

deficientin HDL, enlarged tonsils with an orange-yellow discoloration, and peripheral neuropathy. Patients often also have hepatosplenomegaly, lymphadenopathy, thrombocytopenia, anemia, coronary artery disease, and corneal clouding

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60
Q

Tolcapone and entacapone MOA?

A

COMT inhibitors- decrease the peripheral breakdown of levodopa

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61
Q

Epilepsy of Infancy with Migrating Focal Seizures (EIMFS).

A

Focal seizures that migrate between hemispheres and regression of milestones
Gene: KCNT1

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62
Q

Which Ab associated with dermatomyositis has the highest association with cancer?

A

Anti-TIF-1y

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63
Q

Gabapentin/Pregabalin MOA?

A

modulation of voltage gated Ca channels

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64
Q

Suprascapular nerve does what?

A

0-15 degree shoulder abduction/external rotation of arm

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65
Q

Pimavanersin MOA

A

Serotonin 5-HT2A receptor inverse agonist and antagonist

66
Q

Allele for asians and CBZ induced SJS?

A

HLA B1502

67
Q

Autonomic dysreflexia occurs with spinal injury at what level?

A

at or above T6
*intermediolateral cell column in thoracic cord which supplies sympathetic innervation

68
Q

Zellweger syndrome is caused by what

A

autosomal recessive mutation in PEX gene
*elevated very long chain fatty acids
*inappropriate assembly of peroxisomes

69
Q

Ropinirole MOA

A

direct dopamine agonist

70
Q

MOA of abciximab?

A

Glycoprotein IIB/IIIA inhibitor

71
Q

MOA of plavix?

A

ADP receptor inhibitor

72
Q

Dabigatran MOA?

A

Direct thrombin inhibitor

73
Q

Rivaroxaban and Apixaban MOA?

A

Factor Xa inhibitors

74
Q

Meissner corpuscles do what?

A

superficial light touch

75
Q

livedo reticularis is caused by what PD drug?

A

amantadine

76
Q

A positive wave is seen in what?

A

acute neuronal injury (radiculopathy) or muscle inflammation

77
Q

Ependymomas location?

A

most are around 4th ventricle
*perivascular pseudorosettes

78
Q

Medulloblastomas path?

A

Homer-wright rosettes
*also around 4th ventricles

79
Q

Do diffuse astrocytomas contrast enhance?

A

no
*unlike GBM

80
Q

Weber syndrome?

A

ipsilateral 3rd nerve palsy with contralateral hemiparesis
*medial midbrain

81
Q

Avastin targets what?

A

VEGF receptors

82
Q

Alemtuzumab targets what?

A

CD-52 receptor on activated T and B lymphocytes

83
Q

Natalizumab targets what?

A

Alpha 4 integrin on T lymphocytes

84
Q

Ocrelizumab targets what?

A

CD20 positive B cells

85
Q

High amplitude motor unit action potentials are indicative of acute or chronic problems?

A

chronic

86
Q

Signs of acute neuronal injury on EMG?

A

positive sharp waves, fibrillations and fasciculations

87
Q

Pompe disease deficiency?

A

alpha-glucosidase

88
Q

McArdle disease is due to deficiency in what?

A

myophosphorylase deficiency.

89
Q

phenobarbital MOA

A

increasing duration of GABA activated chloride channels

90
Q

What type of headache has a prominent circadian periodicity?

A

cluster

91
Q

Genes associated with association for Parkinsons?

A

GBA homozygotes and heterozygotes
LRKK2

92
Q

Gene associated with Wilsons?

A

ATP7B, PRNP

93
Q

Which 2 botulinum serotypes cleave SNAP 25?

A

A and B

94
Q

MOA of riluzole?

A

glutamate release inhibitor

95
Q

Where does manganese primarily accumulate in the brain during toxicity?

A

globus pallidus
*presents as dopamine resistant parkinsonism and dystonia

96
Q

Tetrabenazine is used for what?

A

chorea for huntington’s

97
Q

What meds are approved for TD?

A

valbenazine and deutetrabenazine
*selective VMAT2 inhibitors

98
Q

MOGAD pattern?

A

perivascular demyelination
KEY feature is preservation of oligodendrocytes

99
Q

well circumscribed contrast enhancing intraventricular mass near foramen of monroe with hx of TS?

A

subependymal giant cell astrocytoma

100
Q

What does a fasciculation sound like on EMG?

A

like popcorn pop

101
Q

patient with weakness in b/l lower limbs, particularly quads and finger flexers most likely has what

A

Inclusion body myositis

102
Q

what muscle is solely innervated by C5 nerve root

A

rhomboid

103
Q

what travels through the superior orbital fissure?

A

oculomotor, trochlear, abducens, V1 branch

104
Q

What artery supplies the LGN?

A

posterior choroidal

105
Q

What artery supplies the MGN?

A

thalamogeniculate

106
Q

What is cotard delusion?

A

a person denies the existence of their body. sometimes believe that they are dead.
*self starvation

107
Q

SMA type 1 presents how?

A

-early in life
-hypotonia
-narrow chest
-tongue fasciculations
-hypo or areflexia
*often progresses to respiratory failure and almost always fatal by age 2
*mutation in SMN1

108
Q

inititation of non-REM sleep is controlled by what area of the brain?

A

ventrolateral preoptic area

109
Q

NF2 people develop what brain tumors?

A

vestibular schwannomas and multiple meningiomas

110
Q

NF1 people develop what tumors

A

low grade gliomas

111
Q

Wilson’s disease has what on MRI

A

T2 FLAIR hyperintensities of caudate and putamen
*panda sign

112
Q

Drug of choice for paroxysmal kinesigenic dyskinesia?

A

carbamazepine or phenytoin

113
Q

What is Fahr’s disease

A

progressive movement disorder, often parkinsonism and/or neurpsych disorder
-CTH with b/l calcification of the BG

114
Q

oposoclonus myoclonus syndrome is associated with what?

A

usually paraneoplastic
kids: neuroblastoma
adults: neoplasms - medullary thyroid, breast adenocar, small cell lung cancer and paraneoplastic

115
Q

Closure of neural tube happens when

A

4th week of gestation

116
Q

Pinealoma can present how?

A

parinaud syndrome, insomnia, precocious puberty, gait disurbances, hydrocephalus

117
Q

How does juvenile HD differ from adult onset HD?

A

no chorea
but has dystonia and bradykinesia, cerebellar dysfunction and neuropsych symptoms

118
Q

most common cause of focal seizures in full term neonates?

A

hypoxic ischemic injury

119
Q

most common cause of focal seizures in pre-term infants?

A

hemorrhage

120
Q

what is the essential finding between multifocal motor neuron disease and ALS?

A

conduction blck

121
Q

MOA of natalizumab?

A

inhibits T cell transmigration across the BBB by targeting alpha 4 integrin

122
Q

lissencephaly is what

A

complete absence of sulci–VERY SMOOTH

123
Q

Which MS drug is known to develop neutralizing antibodies?

A

interferon beta

124
Q

What tumor is most commonly associated with cerebellar mutism?

A

medulloblastoma

125
Q

What is a hypnic headache?

A

only occurs during sleep and causes awakening
“alarm clock headache”

126
Q

Which type of headache responds to caffeine and lithium?

A

hypnic headache

127
Q

which antibioitcs cause mania?

A

macrolidses

128
Q

What age can sleep spindles first be seen?

A

44 weeks GA

129
Q

Typical treatment for cerebral toxo?

A

pyrimethamine and sulfadiazine

130
Q

1p/19q co deletion is seen in which brain tumor?

A

oligiodendroglioma

131
Q

Raphe nucleus produces what?

A

serotonin

132
Q

which bacteria is classically associated with rhomboencephalitis?

A

listeria monocytogenes

133
Q

lead poisoning can produce what type of neuropathy?

A

motor- predominately radial nerve

134
Q

logopenic pirmary progressive aphasia is characterized by what?

A

difficulty with word finding retrieval and repetition

135
Q

semantic primary progressive aphasia is characterized by what?

A

impaired comprehension or single words and confrontation

136
Q

what are the 3 most common causes of meningitis in neonate?

A

group b strep, e.coli, listeria monocytogenes

137
Q

most common causes of bacterial menginitis in children ?

A

strep pneumo, neisseria, h.flu

138
Q

most common cause of meningitis in adolescents/adults?

A

neisseria, strep pneumo

139
Q

most common cause of bacterial meningitis in adults >50?

A

strep pneumo, neisseria, listeria

140
Q

AQP4 channels are found on which cell type?

A

astrocytes

141
Q

Psammoma bodies (little onion looking things) are seen in which brain tumor on path?

A

meningioma

142
Q

what is a thalamic aphasia?

A

dominant hemisphere
fluency predominant deficit and less impressive difficulties with comprehension

143
Q

Autofluorescent curvilinear bodies are characteristic of what disease?

A

neuronal ceroid lipofuscinoses
*seizures, retinopathy with vision loss, dementia

144
Q

What med can be used in patients who are s/p TBI and lack motivation?

A

methylphenidate

145
Q

Fatal fetal insomnia is characterized by what?

A

-inherited prion disease
-loss of K complexes and sleep spindles (abnl stage 2 sleep)
-delirium, hallucinations, clumsiness

146
Q

What is seen on a muscle biopsy in steroid myopathy?

A

type 2 fiber atrophy

147
Q

Triad of spasmus nutans?

A

torticollis, head bobbing and nystagmus

148
Q

MOA of clonidine?

A

alpha 2 receptor agonist

149
Q

What is the vertical gaze center and where is it located?

A

rostral MLF in the rostral midbrain

150
Q

Recurrent meningitis in someone with a dimple in their lumboscacral region?

A

dermal sinus tract

151
Q

How does leber hereditary optic neuropathy present?

A

20s-30s, sudden onset painless central vision loss with subsequent vision loss in the other eye weeks to months later
*usually caused by mitochondrial disorder

152
Q

Neurosarcoidoisis manifests how?

A

papilledema, cranial mono/polyneuropathies, peripheral neuropathy, neuroendocrine dysfunction

153
Q

Lipoatrophy is a side effect of which MS drug?

A

glatiramer acetate

154
Q

Anti-amphiphysin paraneuplastic syndrome can result in what two conditions?

A

encephalomyelitis and stiff person syndrome

155
Q

anti-Hu paraneoplastic syndrome can cause what?

A

pure sensory neuropathy

156
Q

What class of meds when given with statins increase risk of statin induced myopathy?

A

calcium channel blockers, cyclosporine, niacin, fibrates

157
Q

What does using steroids with bacterial meningitis help with?

A

decreases risk of hearing loss

158
Q

Gene associated with HYPOkalemic periodic paralysis?

A

CACNA1S

159
Q

what triggers HYPOKalemic periodic paralysis?

A

large carb meals, salt, insulin, steroids, rest after exercise

160
Q

Gene most commonly associated with HYPERkalemic period paralysis?

A

SCN4A

161
Q

What it the only oral CGRP (gepant) approved for abortive therapy for cluster headaches?

A

Galcanezumab

know your neuro question bank (3 decks)

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