QB - DISEASES(3) Flashcards
1
Q
Postsynaptic NE receptor blockade
A
Beta-blockers
For migraine prophylaxis
2
Q
Dopamine & NE reuptakine inhibitor
A
Buproprion
For depression
3
Q
Presynaptic serotonin & NE reuptake inhibition
A
TCAs (amitripyline and venlafaxine)
Can be used as prophylaxis for migraines
4
Q
Presynaptic serotonin reuptake inhibition (3)
A
SSRIs for depression
Fluoxetine
Sertraline
Paroxetine
5
Q
Sites for corticospinal UMN lesions (4)
A
- corticospinal tracts
- midbrain, pons, medulla
- internal capsule
- precentral gyrus (primary motor cortex)
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
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
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
9
Q
Atrophy of caudate nucleus; what symptoms? What disease association?
A
Chorea, dementia, behavioral abnormalities
Huntington’s
10
Q
Phenelzine: fatal side effect and mechanism?
A
Phenelzine = MAO inhibitor –> severe hypertensive crisis when taken w/ tyramine-containing foods
11
Q
SSRI antidepressants (3); mechanism?
A
Fluoxetine
Sertraline
Paroxetine
Block reuptake of serotonin –> more serotonin counteracts depression
12
Q
TCAs: mechanism
A
Imipramine
Amitriptyline
Clomipramine
Block amine (NE or serotonin) reuptake pumps
13
Q
Poliomyelitus: what part of spinal cord affected; symptoms?
A
Anterior horn of spinal cord
LMN - flaccid paralysis, atrophy, areflexia, muscle fasciculation
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
15
Q
Difference btwn Duchenne & myotonic dystrophy
A
Duchenne: necrosis of muscle fibers and fibrofatty replacement
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
17
Q
Missle substance: function?
A
RER - protein synthesis
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
19
Q
Location of T-tubule
A
Junction of A and I bands in striated myocytes – faciliate depolarization to inside of cell
20
Q
Desmosomes: function
A
Protein aggregates - allow cell-cell adhesion btwn epithelial cells
21
Q
95% confidence interval: z-score equals?
A
1.96
22
Q
99% confidence interval: z-score
A
2.58
23
Q
Standard error of the mean (SEM)
A
SEM = SD/(n)^1/2
24
Q
Autonomic nervous system (ANS) regulations: Parasympathetic
A
Pregang –> Ach synapse on nicotinic –> Postgang –> Ach
25
Autonomic nervous system (ANS) regulations: Sympathetic
Pregang --> Ach synapse on nicotinic --> Postgang --> NE (cardiac & smooth muscle; glands, nerve terminals)
26
Exceptions to ANS regulation of symapthetics (2)
1. Adrenal glands: directly innervated by PREganglionic neurons
2. Sweat glands: both pregang & postgang release Ach
27
Somatic nervous system regulation
contain ONLY presynaptic neurons --> impulse to skeletal muscle via Ach on postsynaptic nicotinic receptors
28
Length constant (neuronal transmission)
How far along an axon the electrical impulse propagates --> myelin INCREASE length constant by reducing charge dissipation
29
Time constant (neuronal transmission)
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
30
Where can nerve summation NOT occur?
Axon
31
TSH & T3/T4 monitoring
Lithium
| Amiodarone
32
Renal failure toxicity
Aminoglycosides
| Vancomycin
33
Antipsychotic that causes prolonged QT
Ziprasidone
34
Schizophrenia drug that causes granulocytopenia
Clozapine - acts on D4 receptors
Do not have dopaminergic side effects like traditional antipsychotics (pseudoparkinsonism, tardive dyskinesia, hyperprolactinemia)
35
Hypertrophic cardiomyopathy: mode of inheritance & what is mutation responsible?
AD disorder
B-myosin heavy chain
36
Maple syrup urine disease (MSUD)
Defective breakdown of branched chain aa:
1) leucine
2) isoleucine
3) valine
Neurotoxicity
"Burned caramel" smell -- isoleucine
37
5 cofactors needed by branched chanin a-ketoacid dehydrogenase, pyruvate dehydrogenase, & a-ketoglutarate
"TLC for Nobody!"
1) Thiamine
2) Lipoate
3) Coenzyme A
4) FAD
5) NAD
38
Function of Pyridoxine (vitamin B6)
Transamination
Decarboxylation
Heme & neurotransmitter synthesis
39
What conditions require pyridoxine supplementation?
Sideroblastic anemia
| Hyperhomocysteinemia
40
Pathology of sideroblastic anemia
Inability to completely form heme in the mitochondria --> leads to accumulation of IRON around nucleus of immature RBC --> ringed sideroblast
41
Deficiency of tetrahydrobiopterin; disease?
Tetrahydrobiopterin necessary for phenylalanine hydroxylase (conversion of phenylalanine --> tyrosine)
No tetrahydrobiopterin --> inability to metabolize phenylalanine --> PKU
42
Difference btwn schizophrenia & schizophreniform
```
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
43
Adjustment disorder
Increased anxiety, depression, disturbed behavior within 3 months of IDENTIFIED stressor (and doesn’t last longer than 6 months)
44
Fragile X
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
Defect in DNA mismatch repair genes: prone to what disease?
HNPCC (hereditary non-polyposis colorectal cancer); extraintestinal malignancies
46
Young pt. dies from middle cerebral a. thrombosis; also suffered from vision problems & mental retardation; what supplement could have prevented this?
Vit B6 (Pyridoxine)
Pt. sufferings from homocystinuria: cystathionine synthetase deficiency --> thromboembolic episodes; also symptoms resembling Marfan's (ectopia lentis, elongated limbs, scoliosis, arachnodactyly)
47
Altruism (behavorial)
Guilty feelings alleviated through selfless service/giving to others (drunk driver volunteering at local hospital)
48
Reaction formation
Replacement of unpleasant or unacceptable thought/desire with its opposite (drunk driver lecturing against drunk driver)
49
Neonatal opioid withdrawal: symptoms & treatment?
Tremor, rhinorrhea, sneezing, nasal stuffiness, diarrhea; myoclonic jerks & seizures
Give opium solution
50
Sodium bicarbonate is given to fix what problems? (IV vs oral)
IV: acidosis & hyperkalemia
oral: alkalinizing agent/antacid
51
What ds DNA virus infects oligodendrocytes?
JC virus --> progressive multifocal leukoencephalopathy
52
Vascular lesion to thalamus: symptoms?
Thalamic syndrome
VPL and VPM nuclei --> sensory loss to CONTRA side of body
Severe proprioceptive defects --> unsteady gait & falls
53
Vascular lesion of internal capsule
Motor deficit
54
Vascular lesion to pons
```
Coma
Bilateral paralysis
Decerebrate rigidity
Pinpoint pupils
Death
```
55
Vascular lesion to caudate nucleus (learning & memory)
Transient hemiparesis (weakness of one side of body)
Frontal lobe deficits
56
Schizoaffective disorder
Presence of mood symptoms (major depressive, manic, mixed)
57
Hemangioblastomas in retina/cerebellum; congenital cysts in kidney, liver, pancreas -- disease?
Von Hippel - Lindau
58
Neurofibromas, optic nerve gliomas, Lisch nodules (pigmented nodules in iris), Café au lait spots
NF1
59
Bilateral cranial nerve VIII schwannomas, multiple meningiomas
NF2
60
Neurocutaneous disorder: facial angiomas, leptomeningeal angiomas
Ophthalmic and maxillary (V1 and V2) distributions of trigeminal n. affected
"tram track calcifications" on skull radiographs
Sturge-Weber
61
Cortical and subependymal HAMARTOMAS (benign neoplasm resembling tissue of origin); congenital cysts in kidney, liver, pancreas; what is main complication of condition?
Tuberous sclerosis (TS) -- different from von Hippel Lindau due to presence of hamartomas (instead of hemangioblastomas in VHL)
Seizures = main complication
62
Hereditary hemorrhagic telangiectasia --> epistaxis, GI bleeding, hematuria
Osler-Weber-Rendu
63
Role of salivary secretion via superior salivatory nucleus; what CN involved?
CN VII -- submandibular and sublingual gland secretion -- chorda tympani n., lingual n. involved
64
Role of salivary secretion via inferior salivatory nucleus; what CN involved?
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
Syringomyelia; what part of brain does it damage?
Disproportionate loss of P&T in arms and hands
Damages ventral white commissure & ventral horns
66
What tract decussates at ventral white commissure?
Lateral spinothalamic tract
67
Trace pathway of lateral spinothalamic tract: 1st order to 4th order neurons
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
Ventral horns: damage causes what symptoms?
Ventral horn - motor neurons from lateral CORTICOSPINAL tract (voluntary motor)
LMN lesion - flacid paralysis, fasciculations, atrophy of intrinsic muscles of hands
69
Ventral spinothalamic: damage causes what symptoms?
Light touch & pressure sensation
70
Dorsal horn: damage causes what symptoms?
2nd order neurons of lateral spinothalamic (P&T) and ventral spinothalamic (light touch and pressure) tracts located here
71
What aa becomes essential in pts. with PKU?
Tyrosine
Phenylalanine via dihydrobiopterin reductase (convert BH4 to BH2) & phenylalanine hydroxylase --> tyrosine --> DOPA or homogentisate (to fumarate --> TCA cycle)
72
What enzyme is missing in albinism?
Tyrosinase
73
Conversion of dopamine to NE; what enzyme necessary?
Dopamine hydroxylase
74
High amounts of homogentisic acid renally excreted; what disorder?
Alkaptonuria
75
Untreated/undiagnosed PKU -- what effects?
Mental retardation
Hypopigmentation (affects skin, eyes, BASAL GANGLIA)
76
How is infant w/ PKU normally described?
Fair-skinned, blond hair, blue eyes, musty odor
77
Subacute sclerosing panencephalitis (SSPE); associated w/ what microorganism?
Progressive dementia, spasticity, seizures
Due to measles virus (myelin degeneration w/ viral inclusions)
78
CJD
Rapidly progressive dementia & myoclonic jerks
Multiple vacuoles in gray matter (spongioform encephalopathy)
79
Baby has problems feeding due to underdeveloped mandible; What other structure may be impaired? What is problem?
```
Mandible
Maxilla
Malleus, incus
Palate, vomer
TEMPORAL bone
```
All associated w/ 1st pharyngeal arch
Also associated w/ trigeminal n. (CNV)
80
2nd pharyngeal arch derivatives
Styloid process, stapes
CN VII involved
Muscles of facial expression, stylohyoid, stapedius, posterior belly of digastric
81
Pt. with damage to anterior horns & demyelination of corticospinal tracts; disease?
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
Most common cause of death in pts. w/ ALS
Respiratory complications
83
What macroscopic findings are present in ALS?
Thin anterior roots
Mild atrophy of precentral gyrus (motor deficits)
84
What mutation is present in pts. w/ ALS?
SOD1 (codes for copper-zinc superoxide dismutase)
85
Treatment of ALS
Riluzole
MOA: decrease glutamate release
86
Vitamin B12 or vitamin E deficiency; what happens to spinal cord?
Demyelination of dorsal columns, lateral corticospinal tracts, spinocerebellar (indicates position of limbs) tracts
87
Intracerebral hemorrhage; manifestations of deficits?
Sudden onset focal neurologic deficits (hemiplegia, hemianesthesia, cranial nerve deficits, aphasia)
88
Temporal arteritis: manifestations?
Scalp tenderness
Blurred vision, intermittent caludication of jaw
HIGH ESR
could be in setting of polymyalgia rheumatica
89
Tetanus diagnosis; what is most important?
History & physical
| not blood tests, no toxins secreted
90
Calcified cystic mass in children/young adult; what other symptoms involed? What cancer?
Craniopharyngioma
Calcified cystic masses filled w/ yellow vicuous fluid filled w/ cholesterol
Growth failure, bitemporal hemianopia
Remnant of Rathke's pouch
91
What structure has same origin as craniopharyngiomas?
Pituitary gland (adenohypophysis)
Posterior lobe of pituitary (neurohypophysis) develops from neuroectoderm
Craniopharyngioma is remnant of Rathke's pouch (precursor of anterior pituitary)
92
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?
Atropine poisoning (anticholinergic)
Treat w/ physostigmine
93
Massive hepatic necrosis after patient had uncomplicated surgery w/ general anesthesia; elevated AST, ALT, bilirubin; what was cause?
Massive centrilobular hepatic necrosis due to halogenated inhaled anesthetics (Halothane)
94
Microvesicular fatty change in liver of patient; what association?
Reye syndrome in children treated w/ salicylates (ASA)
95
Macrovesicular fatty change; what association?
Alcoholics (fatty liver)
96
Intrahepatic cholestasis; what association?
Biliary duct obstruction
Primary sclerosing cholangitis
Primary biliary cirrhosis
97
Drug-induced cholestasis
Contraceptives
Anabolic steroids
Chlorpromazine
98
Hepatic granulomatosis; what drug associations?
Methyldopa
Hydralazine
Quinidine
99
Release of what molecule is blocked by dopamine?
Ach release is INHIBITED by dopamine
In Parkinson's disease: low dopamine, high Ach levels
100
High potency antipsychotics: action regarding dopamine; what specific side effects?
High potency: Haloperidol, Fluphenazine, Pimozide
Action on D2 receptors of mesocortical-mesolimbic pathway: treats negative symptoms of schizophrenia (avolition, alogia, flat affect)
----
NEG side effects:
Block dopamine in NIGROSTRIATAL pathway: decreased dopamine --> increase Ach = motor disorders (Parkinsonism & acute dystonia)
101
Low potency antipsychotics: side effects
Chlorpromazine
Thioridazine
Anticholinergic and Antihistamine (not much extrapyramidal symptoms); Orthostatic hypotension via alpha adrenergic blockade (falls, syncope)
102
What is used to treat pseudoparkinsonism and acute dystonias (side effects of high potency antipsychotics)?
Benztropine
| Trihexyphenidyl
103
Side effect of histamine blockade?
Sedation & weight gain
104
Why is Zolpidem preferred for patient not wanting to get addicted to sleeping pills?
Zolpidem - similar to benzo (bind to GABA receptor) but is NOT a benzo
Zolpidem = short acting hypnotic for short term treatment of insomnia
105
Zolpidem: important properties
1. Less potential for tolerance/addiction (lower than benzos)
2. No anticonvulsant properties
3. No muscle relaxing effects (NOT used for anesthesia)
106
Why is Triazolam dangerous?
Short acting hypnotic - increase risk of physical dependence (bc of short half life)
107
Flurazepam: short or long acting?
Long acting benzo -- treats insomnia; more risk of tolerance/dependence than treatment w/ Zolpidem
108
Multiple sclerosis: findings
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
