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STEP1 - TLe > QB - DISEASES(3) > Flashcards

QB - DISEASES(3) Flashcards

1
Q

Postsynaptic NE receptor blockade

A

Beta-blockers

For migraine prophylaxis

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2
Q

Dopamine & NE reuptakine inhibitor

A

Buproprion

For depression

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3
Q

Presynaptic serotonin & NE reuptake inhibition

A

TCAs (amitripyline and venlafaxine)

Can be used as prophylaxis for migraines

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4
Q

Presynaptic serotonin reuptake inhibition (3)

A

SSRIs for depression

Fluoxetine
Sertraline
Paroxetine

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5
Q

Sites for corticospinal UMN lesions (4)

A
  1. corticospinal tracts
  2. midbrain, pons, medulla
  3. internal capsule
  4. precentral gyrus (primary motor cortex)
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6
Q

Drugs that are “pro-dopamine”

A

Bromocriptine & Pergolide: ergot (dopamine agonist)

Pramipexole & Ropinerole: non-ergot (dopamine agonist)

Selegiline: decrease central degradation of dopamine

COMT inhibitors & dopa decarboxylase inhibitor (DDI): decrease peripheral degradation of levodopa

Amantadine: enhance efects of endogenous dopamine (reduce tremors; improve motor symptoms); also antiviral for influenza

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7
Q

Hemiballism; what part of brain is affected? What condition caused this brain lesion?

A

CONTRA subthalamic nucleus lesion due to lacunar stroke (long standing HTN)

Lesion of SUBTHALAMIC NUCLEUS: involuntary flinging movements of one side of body (arm and/or leg) contra to side of brain lesion

Subthalamic nucleus part of basal ganglia

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8
Q

Lentiform nucleus: what two structures does it involve? What disease association?

A

Globus pallidus & putamen

Wilson disease

Wing-beating tremor, psychosis, & cirrhosis

Kayser-Fleischer rings in cornea

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9
Q

Atrophy of caudate nucleus; what symptoms? What disease association?

A

Chorea, dementia, behavioral abnormalities

Huntington’s

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10
Q

Phenelzine: fatal side effect and mechanism?

A

Phenelzine = MAO inhibitor –> severe hypertensive crisis when taken w/ tyramine-containing foods

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11
Q

SSRI antidepressants (3); mechanism?

A

Fluoxetine
Sertraline
Paroxetine

Block reuptake of serotonin –> more serotonin counteracts depression

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12
Q

TCAs: mechanism

A

Imipramine
Amitriptyline
Clomipramine

Block amine (NE or serotonin) reuptake pumps

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13
Q

Poliomyelitus: what part of spinal cord affected; symptoms?

A

Anterior horn of spinal cord

LMN - flaccid paralysis, atrophy, areflexia, muscle fasciculation

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14
Q

Myotonic dystrophy

A

AD disorder

Increased number of trinucleotide repeats (myotonia protein kinase gene) – CTG repeats

Sustained muscle contraction (mytonia); weakness, atrophy (type 1 fibers)

other features:
Frontal balding
Gonadal atrophy

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15
Q

Difference btwn Duchenne & myotonic dystrophy

A

Duchenne: necrosis of muscle fibers and fibrofatty replacement

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16
Q

Pufferfish toxin (Japanese delicacy): symptoms/mechanisms?

A

Prevents influx of Na+ into cell –> no depolarization

Paresthesias in face, weakness; can lead to respiratory depression

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17
Q

Missle substance: function?

A

RER - protein synthesis

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18
Q

Kinesin: function?

A

motor protein - anterograde transport of intracellular vesicles/organelles towards PLUS (rapidly growing) ends of MICROTUBULES

Kinesin uses energy from ATP hydrolysis

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19
Q

Location of T-tubule

A

Junction of A and I bands in striated myocytes – faciliate depolarization to inside of cell

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20
Q

Desmosomes: function

A

Protein aggregates - allow cell-cell adhesion btwn epithelial cells

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21
Q

95% confidence interval: z-score equals?

A

1.96

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22
Q

99% confidence interval: z-score

A

2.58

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23
Q

Standard error of the mean (SEM)

A

SEM = SD/(n)^1/2

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24
Q

Autonomic nervous system (ANS) regulations: Parasympathetic

A

Pregang –> Ach synapse on nicotinic –> Postgang –> Ach

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25
Q

Autonomic nervous system (ANS) regulations: Sympathetic

A

Pregang –> Ach synapse on nicotinic –> Postgang –> NE (cardiac & smooth muscle; glands, nerve terminals)

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26
Q

Exceptions to ANS regulation of symapthetics (2)

A
  1. Adrenal glands: directly innervated by PREganglionic neurons
  2. Sweat glands: both pregang & postgang release Ach
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27
Q

Somatic nervous system regulation

A

contain ONLY presynaptic neurons –> impulse to skeletal muscle via Ach on postsynaptic nicotinic receptors

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28
Q

Length constant (neuronal transmission)

A

How far along an axon the electrical impulse propagates –> myelin INCREASE length constant by reducing charge dissipation

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29
Q

Time constant (neuronal transmission)

A

Time it takes for change in membrane potential to achieve 63% of new value –> myelin decreases time constant, therefore allow QUICKER change in membrane potention, INCREASING conduction speed

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30
Q

Where can nerve summation NOT occur?

A

Axon

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31
Q

TSH & T3/T4 monitoring

A

Lithium

Amiodarone

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32
Q

Renal failure toxicity

A

Aminoglycosides

Vancomycin

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33
Q

Antipsychotic that causes prolonged QT

A

Ziprasidone

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34
Q

Schizophrenia drug that causes granulocytopenia

A

Clozapine - acts on D4 receptors

Do not have dopaminergic side effects like traditional antipsychotics (pseudoparkinsonism, tardive dyskinesia, hyperprolactinemia)

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35
Q

Hypertrophic cardiomyopathy: mode of inheritance & what is mutation responsible?

A

AD disorder

B-myosin heavy chain

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36
Q

Maple syrup urine disease (MSUD)

A

Defective breakdown of branched chain aa:

1) leucine
2) isoleucine
3) valine

Neurotoxicity
“Burned caramel” smell – isoleucine

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37
Q

5 cofactors needed by branched chanin a-ketoacid dehydrogenase, pyruvate dehydrogenase, & a-ketoglutarate

A

“TLC for Nobody!”

1) Thiamine
2) Lipoate
3) Coenzyme A
4) FAD
5) NAD

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38
Q

Function of Pyridoxine (vitamin B6)

A

Transamination
Decarboxylation

Heme & neurotransmitter synthesis

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39
Q

What conditions require pyridoxine supplementation?

A

Sideroblastic anemia

Hyperhomocysteinemia

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40
Q

Pathology of sideroblastic anemia

A

Inability to completely form heme in the mitochondria –> leads to accumulation of IRON around nucleus of immature RBC –> ringed sideroblast

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41
Q

Deficiency of tetrahydrobiopterin; disease?

A

Tetrahydrobiopterin necessary for phenylalanine hydroxylase (conversion of phenylalanine –> tyrosine)

No tetrahydrobiopterin –> inability to metabolize phenylalanine –> PKU

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42
Q

Difference btwn schizophrenia & schizophreniform

A 
Both have same symptoms:
Hallucinations
Delusions
Disorganized thought
Blunted affect/social withdrawal/anhedonia

Symptoms for 1-6 months:
Schizophreniform

Symptoms >6months:
Schizophrenia

Brief psychotic disorder: same symptoms lasting less than 1 month

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43
Q

Adjustment disorder

A

Increased anxiety, depression, disturbed behavior within 3 months of IDENTIFIED stressor (and doesn’t last longer than 6 months)

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44
Q

Fragile X

A

FMR1 gene (Fragile X mental retardation 1)

CGG repeats –> hypermethylation of FMR1 –> gene inactivation

2nd most common genetic mental retardation in males (macroorchidism, long thin face, prominent jaw/forehead)

45
Q

Defect in DNA mismatch repair genes: prone to what disease?

A

HNPCC (hereditary non-polyposis colorectal cancer); extraintestinal malignancies

46
Q

Young pt. dies from middle cerebral a. thrombosis; also suffered from vision problems & mental retardation; what supplement could have prevented this?

A

Vit B6 (Pyridoxine)

Pt. sufferings from homocystinuria: cystathionine synthetase deficiency –> thromboembolic episodes; also symptoms resembling Marfan’s (ectopia lentis, elongated limbs, scoliosis, arachnodactyly)

47
Q

Altruism (behavorial)

A

Guilty feelings alleviated through selfless service/giving to others (drunk driver volunteering at local hospital)

48
Q

Reaction formation

A

Replacement of unpleasant or unacceptable thought/desire with its opposite (drunk driver lecturing against drunk driver)

49
Q

Neonatal opioid withdrawal: symptoms & treatment?

A

Tremor, rhinorrhea, sneezing, nasal stuffiness, diarrhea; myoclonic jerks & seizures

Give opium solution

50
Q

Sodium bicarbonate is given to fix what problems? (IV vs oral)

A

IV: acidosis & hyperkalemia
oral: alkalinizing agent/antacid

51
Q

What ds DNA virus infects oligodendrocytes?

A

JC virus –> progressive multifocal leukoencephalopathy

52
Q

Vascular lesion to thalamus: symptoms?

A

Thalamic syndrome

VPL and VPM nuclei –> sensory loss to CONTRA side of body

Severe proprioceptive defects –> unsteady gait & falls

53
Q

Vascular lesion of internal capsule

A

Motor deficit

54
Q

Vascular lesion to pons

A 
Coma
Bilateral paralysis
Decerebrate rigidity
Pinpoint pupils
Death
55
Q

Vascular lesion to caudate nucleus (learning & memory)

A

Transient hemiparesis (weakness of one side of body)

Frontal lobe deficits

56
Q

Schizoaffective disorder

A

Presence of mood symptoms (major depressive, manic, mixed)

57
Q

Hemangioblastomas in retina/cerebellum; congenital cysts in kidney, liver, pancreas – disease?

A

Von Hippel - Lindau

58
Q

Neurofibromas, optic nerve gliomas, Lisch nodules (pigmented nodules in iris), Café au lait spots

A

NF1

59
Q

Bilateral cranial nerve VIII schwannomas, multiple meningiomas

A

NF2

60
Q

Neurocutaneous disorder: facial angiomas, leptomeningeal angiomas

Ophthalmic and maxillary (V1 and V2) distributions of trigeminal n. affected

“tram track calcifications” on skull radiographs

A

Sturge-Weber

61
Q

Cortical and subependymal HAMARTOMAS (benign neoplasm resembling tissue of origin); congenital cysts in kidney, liver, pancreas; what is main complication of condition?

A

Tuberous sclerosis (TS) – different from von Hippel Lindau due to presence of hamartomas (instead of hemangioblastomas in VHL)

Seizures = main complication

62
Q

Hereditary hemorrhagic telangiectasia –> epistaxis, GI bleeding, hematuria

A

Osler-Weber-Rendu

63
Q

Role of salivary secretion via superior salivatory nucleus; what CN involved?

A

CN VII – submandibular and sublingual gland secretion – chorda tympani n., lingual n. involved

64
Q

Role of salivary secretion via inferior salivatory nucleus; what CN involved?

A

CN IX – glossopharyngeal n.

-- other fxns of nerve:
stylopharyngeus muscle (elevates and opens pharynx for food bolus to travel down); sensory of pharynx, posterios 1/3 of tongue; inner tympanic membrane
65
Q

Syringomyelia; what part of brain does it damage?

A

Disproportionate loss of P&T in arms and hands

Damages ventral white commissure & ventral horns

66
Q

What tract decussates at ventral white commissure?

A

Lateral spinothalamic tract

67
Q

Trace pathway of lateral spinothalamic tract: 1st order to 4th order neurons

A

1st order - dorsal root ganglia

2nd order - dorsal horn
axons DECUSSATE at ventral white commissure –> ascend CONTRA lateral funiculus

3rd order - ventral posterolateral (VPL) nucleus of thalamus

4th order - primary somatosensory cortex (parietal lobe)

68
Q

Ventral horns: damage causes what symptoms?

A

Ventral horn - motor neurons from lateral CORTICOSPINAL tract (voluntary motor)

LMN lesion - flacid paralysis, fasciculations, atrophy of intrinsic muscles of hands

69
Q

Ventral spinothalamic: damage causes what symptoms?

A

Light touch & pressure sensation

70
Q

Dorsal horn: damage causes what symptoms?

A

2nd order neurons of lateral spinothalamic (P&T) and ventral spinothalamic (light touch and pressure) tracts located here

71
Q

What aa becomes essential in pts. with PKU?

A

Tyrosine

Phenylalanine via dihydrobiopterin reductase (convert BH4 to BH2) & phenylalanine hydroxylase –> tyrosine –> DOPA or homogentisate (to fumarate –> TCA cycle)

72
Q

What enzyme is missing in albinism?

A

Tyrosinase

73
Q

Conversion of dopamine to NE; what enzyme necessary?

A

Dopamine hydroxylase

74
Q

High amounts of homogentisic acid renally excreted; what disorder?

A

Alkaptonuria

75
Q

Untreated/undiagnosed PKU – what effects?

A

Mental retardation

Hypopigmentation (affects skin, eyes, BASAL GANGLIA)

76
Q

How is infant w/ PKU normally described?

A

Fair-skinned, blond hair, blue eyes, musty odor

77
Q

Subacute sclerosing panencephalitis (SSPE); associated w/ what microorganism?

A

Progressive dementia, spasticity, seizures

Due to measles virus (myelin degeneration w/ viral inclusions)

78
Q

CJD

A

Rapidly progressive dementia & myoclonic jerks

Multiple vacuoles in gray matter (spongioform encephalopathy)

79
Q

Baby has problems feeding due to underdeveloped mandible; What other structure may be impaired? What is problem?

A 
Mandible
Maxilla
Malleus, incus
Palate, vomer
TEMPORAL bone

All associated w/ 1st pharyngeal arch

Also associated w/ trigeminal n. (CNV)

80
Q

2nd pharyngeal arch derivatives

A

Styloid process, stapes

CN VII involved

Muscles of facial expression, stylohyoid, stapedius, posterior belly of digastric

81
Q

Pt. with damage to anterior horns & demyelination of corticospinal tracts; disease?

A

ALS – both UMN and LMN lesions

Anterior horn destruction (LMN)

Demyelination of corticospinal tract (UMN)

Loss of neurons in motor nuclei of CN V, IX, X, XII

82
Q

Most common cause of death in pts. w/ ALS

A

Respiratory complications

83
Q

What macroscopic findings are present in ALS?

A

Thin anterior roots

Mild atrophy of precentral gyrus (motor deficits)

84
Q

What mutation is present in pts. w/ ALS?

A

SOD1 (codes for copper-zinc superoxide dismutase)

85
Q

Treatment of ALS

A

Riluzole

MOA: decrease glutamate release

86
Q

Vitamin B12 or vitamin E deficiency; what happens to spinal cord?

A

Demyelination of dorsal columns, lateral corticospinal tracts, spinocerebellar (indicates position of limbs) tracts

87
Q

Intracerebral hemorrhage; manifestations of deficits?

A

Sudden onset focal neurologic deficits (hemiplegia, hemianesthesia, cranial nerve deficits, aphasia)

88
Q

Temporal arteritis: manifestations?

A

Scalp tenderness
Blurred vision, intermittent caludication of jaw

HIGH ESR
could be in setting of polymyalgia rheumatica

89
Q

Tetanus diagnosis; what is most important?

A

History & physical

not blood tests, no toxins secreted

90
Q

Calcified cystic mass in children/young adult; what other symptoms involed? What cancer?

A

Craniopharyngioma

Calcified cystic masses filled w/ yellow vicuous fluid filled w/ cholesterol

Growth failure, bitemporal hemianopia

Remnant of Rathke’s pouch

91
Q

What structure has same origin as craniopharyngiomas?

A

Pituitary gland (adenohypophysis)

Posterior lobe of pituitary (neurohypophysis) develops from neuroectoderm

Craniopharyngioma is remnant of Rathke’s pouch (precursor of anterior pituitary)

92
Q

Gardener working outside has sudden blurred vision, tachycardia, flushed skin, dry oral mucosa, dilated pupils that are non-reactive to light; what is condition and how to treat it?

A

Atropine poisoning (anticholinergic)

Treat w/ physostigmine

93
Q

Massive hepatic necrosis after patient had uncomplicated surgery w/ general anesthesia; elevated AST, ALT, bilirubin; what was cause?

A

Massive centrilobular hepatic necrosis due to halogenated inhaled anesthetics (Halothane)

94
Q

Microvesicular fatty change in liver of patient; what association?

A

Reye syndrome in children treated w/ salicylates (ASA)

95
Q

Macrovesicular fatty change; what association?

A

Alcoholics (fatty liver)

96
Q

Intrahepatic cholestasis; what association?

A

Biliary duct obstruction

Primary sclerosing cholangitis
Primary biliary cirrhosis

97
Q

Drug-induced cholestasis

A

Contraceptives
Anabolic steroids
Chlorpromazine

98
Q

Hepatic granulomatosis; what drug associations?

A

Methyldopa
Hydralazine
Quinidine

99
Q

Release of what molecule is blocked by dopamine?

A

Ach release is INHIBITED by dopamine

In Parkinson’s disease: low dopamine, high Ach levels

100
Q

High potency antipsychotics: action regarding dopamine; what specific side effects?

A

High potency: Haloperidol, Fluphenazine, Pimozide

NEG side effects:
Block dopamine in NIGROSTRIATAL pathway: decreased dopamine –> increase Ach = motor disorders (Parkinsonism & acute dystonia)

101
Q

Low potency antipsychotics: side effects

A

Chlorpromazine
Thioridazine

Anticholinergic and Antihistamine (not much extrapyramidal symptoms); Orthostatic hypotension via alpha adrenergic blockade (falls, syncope)

102
Q

What is used to treat pseudoparkinsonism and acute dystonias (side effects of high potency antipsychotics)?

A

Benztropine

Trihexyphenidyl

103
Q

Side effect of histamine blockade?

A

Sedation & weight gain

104
Q

Why is Zolpidem preferred for patient not wanting to get addicted to sleeping pills?

A

Zolpidem - similar to benzo (bind to GABA receptor) but is NOT a benzo

Zolpidem = short acting hypnotic for short term treatment of insomnia

105
Q

Zolpidem: important properties

A
  1. Less potential for tolerance/addiction (lower than benzos)
  2. No anticonvulsant properties
  3. No muscle relaxing effects (NOT used for anesthesia)
106
Q

Why is Triazolam dangerous?

A

Short acting hypnotic - increase risk of physical dependence (bc of short half life)

107
Q

Flurazepam: short or long acting?

A

Long acting benzo – treats insomnia; more risk of tolerance/dependence than treatment w/ Zolpidem

108
Q

Multiple sclerosis: findings

A

IgG – oligoclonal band on protein electrophoresis

autoimmune demyelination of axons (white matter); plaques show LOSS of myelin sheaths, depletion of oligodendrocytes

Astrocyte proliferation (repair cells) in response to injury occuring in MS

deficit found in female pts.; mutliple neurological lesions on both sides of body and on upper and lower trunk

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