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Neuro > General neuro 0427Q > Flashcards

General neuro 0427Q Flashcards

1
Q

trisomy 21 prevalence

A

1 in 730 live births.
MOST FETUSES DIE IN UTERO.
most commonly caused by chromo nondisjunction during maternal meiosis 1.

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2
Q

Down syndrome screening

A

triple marker test
quadruple marker test
integrated test

verify with amniocentesis and chromo analysis of fetal cells

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3
Q

Down syndrome phenotype

A

flat facial features
excessive skin at nape of neck
slanted palpebral fissures
single transverse palmar crease

cardiac: AVSD, VSD
GI: duodenal atresia, Hirschsprung, TEF

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4
Q

distinguishing feature of Edwards syndrome

A

clenched hand with INDEX finger overriding middle finger, FIFTH FINGER overriding fourth finger

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5
Q

Patau syndrom

A 
cleft lip and palate
polydactyly
microcephaly
rocker bottom feet
umbilical hernia
cardiac and RENAL defect
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6
Q

stillborn fetus - Turner syndrome

A

edematous hands, feet
cystic hygroma of neck
coarctation of aorta

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7
Q

CMV in immunocompetent host

A

mostly subclinical infx.
MONONUCLEOSIS - fever, malaise, myalgia, atypical lymphocytosis, elevated LFT.

no heterophil Abs (monospot negative) (vs EBV)

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8
Q

CMV pneumonitis

A

after organ transplantation

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9
Q

vit A chronic overuse/toxicity

A

intracranial HTN: papilledema, pseudotumor cerebri

skin changes: alopecia, dry skin

hepatosplenomegaly, cirrhosis

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10
Q

vit A teratogenic effects

A

microcephaly
cardiac anomalies
fetal death (tri 1)

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11
Q

vit E overuse

A

higher mortality - hemorrhagic stroke in adults.

necrotizing enterocolitis in infants.

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12
Q

pernicious anemia - classic presentation

A

older, mentally slow northern European woman who is lemon colored (anemic, icteric) with a smooth shiny tongue (atrophic glossitis) and demonstrates shuffling broad-based gait

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13
Q

SSRI effect

A

takes 4 wks for noticeable therapeutic effect.
initial activating effects can lead to increased agitation and anxiety.

temporary course of BENZODIAZEPINE can help with anxiety-related sx (short-acting)

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14
Q

ALANINE

A

main AA responsible for transferring nitrogen to liver for disposal.

protein breakdown: amino groups transferred to ALPHA-KETOGLUTARATE to form glutamate [–> ammonia –> urea]. other product is pyruvate.

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15
Q

hydrocephalus in INFANTS

A

present with irritability, poor feeding, increased head circumference, enlarged ventricles on CT.

long term sequelae: lower extremity spasticity (stretching of periventricular pyramidal tracts - muscle hypertonicity, hyperreflexia). visual disturbances. learning disabilities.

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16
Q

cause of hydrocephalus in infants

A 
congenital (arnold chiari type II)
hereditary aqueductal stenosis
prenatal infx (toxoplasmosis)
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17
Q

neonatal intraventricular hemorrhage

A

common complication of prematurity.

occurs in fragile germinal matrix.

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18
Q

HIV polyprotein

A

encoded by structural genes (gag, pol, env).

env is glycosylated to gp160, proteolytically cleaved in ER and Golgi to form envelope gp120 and gp41 to aid virion absorption by target cells

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19
Q

gp41

A

transmembrane anchor for gp120, which mediates viral absorption by binding CD4 receptor on susceptible host cells

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20
Q

delusional disorder

A

one non-bizarre delusion but do not meet criteria for schizophrenia. can function w/o significant impairment to daily life.

*non-bizarre = situations that are unlikely but possible (being followed, cheated, poisoned)

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21
Q

staph epidermidis

A

common cause of FOREIGN BODY infx due to ability to produce BIOFILMS - foreign body coated in host proteins (fibrinogen, fibronectin), can then bind organism, which makes EC polysaccharide matrix to surround it.

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22
Q

avoidant personality disorder

A

maladaptive pattern of behavior - feelings of inadequacy, timidity, fear of rejection

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23
Q

TURNER baby

A

most prominent finding = lymphedema of hands and feet. also responsible for webbed neck and low posterior hair line.

abn nuchal lymphogenesis causes SQ nuchal edema and cystic hygroma in utero.

may have hydrops fetalis.

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24
Q

congenital toxoplasmosis

A

transplacental infx - acquired in utero.

TRIAD

  1. hydrocephalus (enlarged ventricles)
  2. intracranial calcifications
  3. chorioretinitis (cotton like white/yellow scars on retina)
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25
Q

alcohol withdrawal - 1st sx

A

tremulousness

others: GI, agitation, anxiety, autonomic disturbance (increase HR, RR, temp)

delirium tremens between 48-72 hrs after last drink

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26
Q

what receptors are downregulated with long term ethanol use?

A

GABA-A

  • NMDA-R upregulated
  • increase synthesis of excitatory mediators (NE, DA, 5HT)
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27
Q

loss of sensation - ulnar n. injury

A

medial 1.5 digits of hand

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28
Q

trochlear nerve palsy

A

characterized by vertical diplopia

CN IV

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29
Q

VZV infx of dorsal root ganglia

A

reactivation that results in painful vesicular skin eruption along SENSORY DERMATOMES (shingles - commonly in thoracic and trigeminal regions)

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30
Q

latent HSV-2

A

dsDNA virus infx of sacral sensory ganglia resulting in recurrent painful genital rash secondary to reactivation

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31
Q

reaction formation

A

person replaces UNPLEASANT or UNACCEPTABLE thought/desire with an EMPHASIS on the opposite

vs. altruism - mature, conscious mech to help others with the same problem w/o intention of covering up personal desire

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32
Q

most common cause of aseptic meningitis

A

enteroviruses - coxsackie, echo, entero, polio

*aseptic = lymphocytic CSF pleocytosis, modest prot elevation, other NORMAL parameters - usually VIRAL

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33
Q

Neimann-Pick

A

sphingomyelinase deficiency.
ceramide phospholipid SPHINGOMYELIN accumulates. foamy histiocytes.
neuro deterioration w/in yr 1.
massive hepatosplenomegaly.
deposits in retina = blindness (cherry red macula similar to Tay Sachs)

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34
Q

metachromatic leukodystrophy enzyme deficiency

A

arylsulfatase A.

sulfatides accumulate w/in tissue.

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35
Q

Tay Sachs enzyme deficiency

A

beta hexosaminidase A.

Gm2 ganglioside accumulates in neurons.

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36
Q

Fabry disease

enzyme deficiency

A

alpha galactosidase A.
ceramide trihexoside accumulates in tissue.
X-linked.

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37
Q

human sialidosis

enzyme deficiency

A

neuraminidase (sialidase).

is also the surface enzyme of influenza virus.

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38
Q

Farber disease

enzyme deficiency

A

ceramidase.

ceramide accumulates w/in neurons and granulomas in skin.

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39
Q

acute mania tx

A

mood stabilizing agent (lithium, valproate, carbamazepine)

PLUS

atypical antipsychotic (olanzapine)

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40
Q

meds that precipitate manic episodes

A

SSRI, bupropion

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41
Q

liquefactive necrosis following hypoxic CNS injury

A

complete lysosomal digestion and removal of necrotic tissue with formation of cystic cavity.

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42
Q

hemorrhagic infarcts

A

blood extravasation into necrotic areas.
often occurs in organs with DUAL bld supply (lung - pulmo and bronchial aa.)

in CNS - due to reperfusion injury

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43
Q

architecture of necrotic tissue is preserved in…?

A

coagulative necrosis

44
Q

methadone as DOC for heroin addiction/abuse

A

very potent mu receptor agonist.
LONG-ACTING opiate.
good oral bioavailability.

long half life allows for prolonged effects to suppress withdrawal sx.

45
Q

neurofibromas

A

multiple SMALL cutaneous lesions (look like skin tags) distributed all over body.

NF1: AD with high penetrance.
cafe au lait spots (hyperpigmented).
Lisch nodules (asymptomatic hamartomas of iris).

also….
optic nerve gliomas (vision loss).
bony abn (sphenoid dysplasia, congenital pseudoarthrosis, scoliosis).
meningioma, glioma, pheochromocytoma.

46
Q

cricothyrotomy

A

when emergency airway is needed; oro or nasotracheal intubation can’t be done.

incision passes through:

  1. skin
  2. superficial cervical fascia (including SQ fat, platysma muscle)
  3. investing and pretracheal fascia of deep cervical fascia
  4. cricothyroid membrane (BELOW thyroid cartilage)
47
Q

PKU biochemical cause

A

deficient phenylalanine hydroxylase
OR cofactor tetrahydrobiopterin (BH4)

  • dihydropteridine reductase needed to regenerate BH4 from BH2
  • any of these 3 steps can result hyperphenylalaninemia
48
Q

brain component involved in PKU

A

hypopigmentation of basal ganglia.

ex: substantia nigra, locus ceruleus, vagal nucleus dorsalis

49
Q

albinism enzyme

A

depressed or absent TYROSINASE

tyrosine –[tyrosinase]—> melanin

50
Q

PKU manifestation

A

homozygous infant normal at birth but MR gradually develops in untreated baby - evident by 6 mos

51
Q

muscarinic effect in endo cells

A

cholinergic agonists bind muscarinic receptors on endo cells, promote release of NO (endothelial-derived relaxation factor, EDRF) - activate guanylyl cyclase, diminish endo calcium conc = VASODILATION (smooth muscle relaxation)

PARASYMPATHETIC

52
Q

cholinergic effect on smooth muscle

A

increase tone (stimulate contraction)

GI: increase motility and secretion.
salivation, sweating, lacrimation.
bladder (detrusor): urination

PARASYMPATHETIC

53
Q

cholinergic negative inotropy

A

decreased contractility due to decreased Ca current in cardiac myocytes.

more pronounced effect in atria (ventricles have more adrenergic innervation).

*similar effect on chronotropy - decrease Ca currents in SA and AV nodes to decrease conduction velocity (may reach heart block)

54
Q

sedative effect of allergy meds

A

1G H1-histamine receptor antagonists (diphenhydramine, chlorpheniramine, promethazine, hydroxyzine) - should not be used with BENZOS!!!

penetrate BBB and accumulate in CNS

55
Q

malignant otitis externa

A

most common cause: Pseudomonas

serious infx in elderly diabetic pts.
severe ear pain, drainage.
granulation tissue often seen in ear canal.
can progress to osteomyelitis of skull base w/cranial nerve damage.

56
Q

pseudomonas characteristics

A

nonlactose fermenting
motile
oxidase positive
gram neg rod

57
Q

chemoprophylaxis of meningococcal meningitis

A

RIFAMPIN
prescribed to ALL close contacts of pt w/active disease w/in 2 wks of dx.

penetrates resp tract - eliminates nasopharyngeal colonization.

*vaccine is given to at-risk groups (college freshmen) BEFORE exposure

58
Q

rifampin side effects

A

ORANGE discoloration of secretions - urine, tears, breast milk).

“amp”-lifies P450 - increase metab of warfarin and other drugs

59
Q

TREATMENT of meningococcal meningitis

A

penicillin

60
Q

orotic aciduria

A

impaired de novo pyrimidine synthesis.
(orotate to UMP)

tx: uridine supp (uridine converted to UMP, which can inhibit CPS II)

61
Q

orotic aciduria presentation

A

hypochromic megaloblastic anemia.
neuro abn.
growth retardation.
orotic acid in urine.

62
Q

atypical (malignant) PKU

A

deficiency of dihydrobiopterin reductase (BH2 to BH4 conversion).

affects:
Phe to Tyr (phenylalanine hydroxylase)
and
Tyr to DOPA (tyrosine hydroxylase)

*lack of dopamine disinhibits PRL

63
Q

atypical PKU with tyrosine supplementation

A

only catecholamine synth rxns DOWNSTREAM from tyrosine are compromised (includes low dopamine levels)

64
Q

fever + pharyngitis + lymphadenopathy in young adult

A

infectious mononucleosis - ATYPICAL LYMPHOCYTES (irreg border, abundant cytoplasm, multiple nucleoli) on peripheral smear

65
Q

EBV malignancies

A

increased incidence of Hodgkin and non-Hodgkin (Burkitt) lymphoma, nasopharyngeal carcinoma

66
Q

gingivostomatitis + fever + lymphadenopathy in child (age 1-3)

A

primary HSV1 infx - intranuclear inclusions are characteristic (enveloped dsDNA)

67
Q

cocaine abuse

A

NASAL FINDINGS- mucosal atrophy, partial septum destruction.

sx: increase BP, chest pain (coronary a. vasoconstriction), agitation (CNS activation), mydriasis but responsive to light

blocks NT reuptake in adrenergic sx (same moa as TCA)

68
Q

subacute combined degeneration

A

myelopathy assoc. w/B12 deficiency.
ascending dorsal column and descending corticospinal tracts.

abn myelin synthesis (vacuolization, axonal degen).

69
Q

axonal degeneration of PERIPHERAL nerves

A

numbness, paresthesias

70
Q

lead poisoning - enzymes

A
  1. delta-aminolevulinate dehydratase
  2. ferrochelatase

delta-ALA and protoporphyrin IX accumulate.
production of heme decreases.

71
Q

conditions assoc. with Down

A

acute leuk: AML, ALL

CHD: endocardial cushion defects, VSD, ASD

GI: duodenal atresia, Hirschsprung

72
Q

MPTP-induced damage of dopaminergic neurons

A

toxic metabolite of MPTP produced by MAO type B - prevent with SELEGILINE (used to delay progression of Parkinson)

73
Q

parkinson first line tx

A

combo of selegiline, anticholinergics, amantadine UNTIL they no longer control sx – THEN, use levodopa+carbidopa

74
Q

early changes in Alzheimer

A

slow memory loss.
language deficits (word-finding difficulties).
impaired visuospatial ability (misplacement of items).

trouble with everyday fxs comes later.

75
Q

brain atrophy in Alzheimer

A

most pronounced in temporoparietal lobes and HIPPOCAMPUS

76
Q

motion sickness

A

antimuscarinic agents (M1) - scopolamine.

antihistamines block H1 receptor activity by increasing the proportion of inactive H1 receptors (reverse blockade) - also have antimuscarinic, adrenergic, and serotonergic properties.
specifically: meclizine, dimenhydrate.

77
Q

antimuscarinic side effects

A 
blurry vision
dry mouth
palpitations
urinary retention
constipation
78
Q

PCP (phencyclidine)

A

hallucinogen - inhibits excitatory NMDA receptor.

moderate amts cause feelings of detachment/distance. slurred speech, loss of coordination, involuntary mvmt, exaggerated gait, nystagmus.

can induce paranoia and hallucinations.
users become hostile, aggressive - can’t distinguish from schizophrenia

79
Q

glutamate

A

excitatory NT that acts at NMDA receptor

80
Q

TCA-assoc. cardiac problems

A

hypertonic SODIUM BICARBONATE.

TCA can have QUINIDINE-like effect on conduction system (QRS and QT prolongation, cardiac dysrrhythmias - due to inhibition of fast Na channels)

81
Q

pain in temporal (giant cell) arteritis

A

jaw pain that starts in the middle of meal - art. lumen narrows and can’t respond to increased bld requirement.

may also have tongue pain (tongue claudication).

MOST COMMON SX is headache.
scalp tenderness.
polymyalgia rheumatica (muscle pain, morning stiffness) 50%

82
Q

blindness in temporal (giant cell) arteritis

A

painless
monocular
may be permanent

83
Q

dx of temporal (giant cell) arteritis

A

ESR - usu. > 100 mm/hr
biopsy of temporal a. is DEFINITIVE.
start corticosteroid tx ASAP to prevent vision loss.

84
Q

rhabdovirus

A

RABIES - ssRNA virus enveloped in bullet-shaped capsule studded with glycoprotein spikes that bind NICOTINIC ACH RECEPTORS. virus bind ACh receptors on peripheral nerve axons and travel retrograde to CNS.

85
Q

rabies encephalitis

A 
agitation
disorientation
photophobia 
hypersalivation (mouth foaming)
pharyngospasm (painful swallowing, breathing) - may cause dysphagia and avoidance of food, water
leading to coma, death.
86
Q

atropine side effect

A

increased intraocular pressure - precipitate acute closed-angle glaucoma in susceptible pts

87
Q

atropine cardiac use

A

block vagal influence on SA and AV node (increase HR in bradycardic pts)

88
Q

acute closed-angle glaucoma presents as….?

A

unilateral severe eye pain and visual disturbances (halo)

at risk: inuits and asians

89
Q

inferior MI

A

often due to block of right coronary artery - responsible for SA and AV node perfusion so BRADYCARDIA is often involved

90
Q

gingival hyperplasia with phenytoin

A

drug causes increased PDGF expression.
gingival macrophages exposed to increased PDGF will stimulate proliferation of gingival cells and alveolar bone

other tox: mainly cerebellar and vestibular system (ataxia and nystagmus)

91
Q

phenytoin and folic acid

A

drug interferes with metabolism of folic acid - cause megaloblastic anemia

92
Q

cystic hygroma

A 
(Turner syndrome, esp at birth)
tumor on neck/posterior triangle.
cystic spaces separated by CT rich in lymphoid aggregates.
due to abn lymphatic outflow.
swelling decreases w/age.
93
Q

primary amenorrhea in Turner

A

due to ovarian underdevelopment (streak ovaries). fail to develop secondary sex characteristics.

94
Q

Bell’s palsy = unilateral facial paralysis plus…?

A

decreased tearing
hyperacusis
and/or loss of taste sensation over ant 2/3 tongue

95
Q

specific findings of Bells palsy

A 
sudden onset
impaired eye closure
eyebrow sagging
inability to smile/frown on affected side
disappearance of nasolabial fold
mouth drawn to nonaffected side
96
Q

freidreich ataxia neuro involvement

A

ascending and descending spinocerebellar tract degeneration (gait ataxia)

dorsal column and dorsal root ganglia degeneration (lose position and vibration sense)

97
Q

cardiac embolism to brain

A

causes multiple infarcts within diff vascular territories

98
Q

hypertensive encephalopathy

A

HA, vomiting, confusion
may lead to coma, death
if death, brain edema and petechial hemorrhages seen in autopsy

99
Q

tabes dorsalis

A

part of neurosyphilis
affects dorsal columns and dorsal roots
loss of position, vibration, pain sensation

100
Q

JC virus

A

polyomavirus - PML in severely immunocompromised pts

101
Q

typical meningioma location

A

adjacent to falx cerebri, compressing medial surface of hemisphere (parasagittal region)

102
Q

candida oral thrush

A

white patches on oral mucosa
pseudomembranous
can be scraped off
KOH prep for yeast and pseudohyphae

assoc. with dentures, DM, immunosuppression

103
Q

ubiquitin-proteasome system in neurodegenerative d/o

A

ubiquitin undergoes ATP-dependent attachment to other prots to mark them for degradation.

impairment of system can contribute to parkinson, alzheimer - protein misfolding, aggregation, obstruction of molecular trafficking

104
Q

carbidopa

A

reduce most peripheral side effects of levodopa BUT behav changes can worsen bc more dopamine becomes available to brain (anxiety, agitation)

105
Q

adrenergic regulation of insulin

A

alpha receptors inhibit insulin secretion.
beta receptors stimulate insulin secretion.

pretreatment with alpha blocker results in predominance of beta effects (ex: with epinephrine)

Neuro (15 decks)

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