PT4 Flashcards

1
Q

Purpose of kinesin

A

Microtubule assoiated motor protein that functions in anterograde transport of intracellular vesicles and organelles - carries them away from cell body and towards the nerve terminal

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2
Q

Poliomyelitis

A

Enteroviral infection that damages motor neurons of anterior horn and is PURE LMN disease - flaccid paralysis, atrophy, fasciculations

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3
Q

Rabies infection

A

prodromal phase: fever, pharyngitis and pain around bite site
Acute encephalitis develops later
Ultimately: paralysis, confusion, coma

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4
Q

Huntingtons

A

AD disorder - atrophy of caudate nucleus and putamen -progressive dementia, behavioral changes, choreiform movements

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5
Q

Friedrich ataxia

A

AR disorder, children, ataxia (difficulty walking), associated with HYPERTROPHIC cardiomyopathy, kyphoscoliosis, foot abnormalities, diabetes mellitus

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6
Q

B12 deficiency

A

Causes subacute combined degeneration of the dorsal and lateral spinal columns

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7
Q

ALS

A

Loss of anterior horns (LMN) and demyelination of the Lateral corticospinal tract (UMN) - spaciticyt, weakness, atrophy, hyperreflexia

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8
Q

What’s derived from surface ectoderm

A

Rathke’s pouch (anterior pituitary), lens & cornea, inner ear sensory organs, olfactory, nasal and oral epi linings, epidermis, salivary, sweat, mammary glands

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9
Q

Derived from neural ectoderm

A

brain & spinal cord, posterior pituitary, pineal gland, retina

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10
Q

Derived from neural crest cell

A

Autonomic, sensory & celiac ganglia, Schwann cells, pia, arachnoid mater, AP septum, endocardial cushions, skull bones, melanocytes, adrenal medulla

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11
Q

Mesoderm derivatives

A

Muscles (all 3 types), connective tissue, bone, cartilage, CV system, blood, lymphatics, spleen, genitalia, kidney, ureters, adrenal CORTEX

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12
Q

Endoderm derivatives

A

GI tract, liver, pancreas, lungs, thymus, parathyroids, thyroid follicular cells, middle ear, bladder, urethra

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13
Q

Jaw pain that starts in the middle of eating is characteristic of…..
(esp. if accompanied by some vision loss problems)

A

TEMPORAL ARTERITIS - check via ESR (sed rate) - start on corticosteroids immediately if suspected to prevent vision loss

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14
Q

Syringomyelia pathophysiology

A

central cystic dilation in the cervical spinal cord (usually C8-T1)- slowly enlarges and causes damage to the ventral white commissure and anterior horns

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15
Q

Sx of syringomyelia

A

“cape-like” distribution loss of pain and temp with SPARING of fine touch and position (spares dorsal columns)

Expansion leads to muscle atrophy and weakness, impaired reflexes & horner syndrome (ptosis, miosis, anhidrosis)

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16
Q

Define focal dystonia and give example

A

focal dystonia: localized uncontrollable muscle contraction causing pain or discomfort as well as deformity in some cases; classic example = torticollis: cervical dystonia of SCM muscle

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17
Q

What can help treat focal dystonias

A

Injection of botulinism toxin - will relax muscle due to inhibition of ACh release from neuron

Used for focal dystonias, achalasias, spasms

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18
Q

Antiphagocytic capsule is a primary virulence factor for…

A

Strep pneumo, H. influ, and Neisseria bacteria

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19
Q

Hypervariable pili characteristic of…

A

N. meningitidis and N. gonorrhoeae

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20
Q

IgG binding outer membrane portion is a virulence factor for…

A

S. aureus - it’s called protein A virulence factor and binds the Fc portions of IgG, thereby preventing opsonization, phagocytosis, and complement fixation

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21
Q

Wernicke syndrome

A
Thiamine deficiency (usually from alcoholism) - focal hemorrhage and necrosis of the mamillary bodies
TRIAD: ophthalmoplegia, ataxia, confusion
Thiamine def. results in decreased glucose utilization
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22
Q

Thiamine (B1) cofactor for what enzymes?

A
  1. PDH (pyruvate to acetyl CoA)
  2. alpha-ketoglutarate dehydrogenase
  3. Transketolase (pentose to G3P)
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23
Q

How to treat Wernicke syndrome

A

Give glucose infusion BUT SUPPLEMENT WITH THIAMINE AS WELL

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24
Q

What product is increased in B12 deficiency?

A

Methylmalonic acid (product of FA oxidation)

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25
Q

Neuroblastoma

A

Most common tumor of adrenal medulla in CHILDREN - can occur anywhere along sympathetic chain; will see homovanillic acid in urine, associated with N-myc overexpression, associated with non-rhythmic conjugate eye movements and myoclonus

26
Q

Wilms tumor

A

nephroblastoma; palpable flank mass; resembles primitive metanephric tissue; associated with deletion of WT1 tumor suppressor gene

27
Q

Beckwith-Wiedemann syndrome/WAGR complex:

A

Wilms tumor
Aniridia (absence of iris)
GU malformation
Retardation (mental)

28
Q

Niemann-Pick disease

A

Sphingomyelinase deficiency (accumulate sphingomyelin); progressive neurodegeneration, hepatosplenomegaly, CHERRY RED SPOT ON MACULA (also seen in Tay-Sachs), foam cells (foamy histiocytes)

29
Q

Metachromatic leukodystrophy

A

Def. arylsulfatase A; accumulate cerebroside sulfate; central and peripheral demyelination with ataxia & dementia

30
Q

Tay-Sachs

A

Hexosaminidase A def; GM2 ganglioside accumulation; progressive neurodegeneration, cherry red spot on macula, onion-skin lysosomes, NO HEPATOSPLENOMEGALY (vs. Nieman Pick)

31
Q

Fabry’s disease

A

alpha-galactosidase A def; ceramide trihexoside accumulation; peripheral neuropathy of hands/feet, angiokeratomas, CV/renal disease

32
Q

Bacterial vs. viral/aseptic meningitis

A

Bacterial: WBC >1000, neutrophilic predom
Viral: WBC <500, lymphocytic predom

33
Q

Most common cause for viral meningitis

A

Enterovirus in kids (coxackievirus, echovirus, poliovirus)

34
Q

1 and #2 cause for bacterial meningitis

A
#1 Strep pneumo
#2 N. meningitidis
35
Q

2 nitrogen sources for urea cycle

A

NH3 and aspartate

36
Q

Rate limiting step in urea cycle & what activates the enzyme

A

Carbamoyl phosphate synthetase 1 (CPS 1) - generates carbamoyl phosphate

CPS 1 activated by NAG (N-acetylglutamate)

37
Q

Infectious process of N. meningitidis

A

Pharynx –> blood –> choroid plexus –> CNS /meninges

38
Q

Function of ventromedial nucleus of hypothal

A

satiety; stim. leptin; destruction = grow medial!

39
Q

Function of lateral nucleus of hypothal

A

mediates hunger; inhib by leptin; destruction leads to anorexia (shrink laterally!)

40
Q

Function of paraventricular nucleus

A

ADH, CRH, oxytocin & thyrotropin-releasing hormones

41
Q

Supraoptic nucleus

A

ADH and oxytocin secretion

42
Q

Suprachiasmatic nucleus function

A

Circadian rhythm & pineal gland function

43
Q

Anterior nucleus of hypothalamus

A

destruction = hyperthermia

44
Q

Posterior nucleus of hypothalamus

A

destruction = hypothermia

45
Q

Vomiting from chemo is triggered by…(and where is it located)

A

Chemoreceptor trigger zone (CTZ) - on dorsal surface of medulla at caudal end of 4th ventricle (aka area postrema)

46
Q

Subarachnoid hemorrhage

A

rupture of berry aneurysm or AV malformation; abrupt onset of severe headache (thunderclap headache), confusion, fever, nuchal rigidity

47
Q

Complication of resolved subarachnoid hemorrhage

A

50% develop secondary arterial vasospasm in the vessels surrounding ruptured aneurysm - can cause cerebral ischemia and present as new-onset Sx

48
Q

Treat/prevent vasospasm from a subarachnoid hemorrhage

A

Nimodipine - selective calcium channel blocker

49
Q

DNA pol 1

A

3-5 proofreading exonuclease activity

5-3 exonuclease activity (removes RAN primer and damaged DNA)

50
Q

DNA pol 3

A

Initiates DNA replication 3-5 direction

51
Q

Associations with ADPKD

A

Berry aneurysms can arise - lead to subarachnoid hemorrhage (thunderclap headache, sudden, nuchal rigidity)

52
Q

Intracerebral hemorrhage manifestation

A

headache, vomiting, sudden onset of focal neurological deficits (hemiplegia, hemianesthesia, cranial nerve deficits, etc.)

53
Q

Characteristics of uncal (transtentorial) herniation

A

Medial temporal lobe herniates; CN III (ipsi) compression - fixed & dilated pupil on side of compression; commonly caused from ipsi mass lesion

54
Q

12-48 hrs after ischemia

A

“red neurons” - eosinophilic cytoplasm, Nissl substances

55
Q

1-3 days after ischemia

A

Necrosis and neutrophilic infiltration

56
Q

3-5 days after ischemia

A

Macrophage infiltration + phagocytosis

57
Q

1-2 wks after ischemia

A

Liquefactive necrosis, reactive gliosis and vascular proliferation

58
Q

> 2 wks

A

glial scar, cystic area surrounded by gliosis

59
Q

Prophylactic agains invasive meningococcal diseases (N. meningitidis)

A

Rifampin

60
Q

Classic side effect for rifampin

A

orange discoloration of secretions (urine, breast milk, tears) - contact lenses will turn orange!

ALSO rifAMPin AMPlifies P450 - inc. metabolism of drugs (ex: wafarin) ,,j