PT4 Flashcards by Shefali Pappu

Purpose of kinesin

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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Poliomyelitis

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

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Rabies infection

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

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Huntingtons

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

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Friedrich ataxia

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

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B12 deficiency

Causes subacute combined degeneration of the dorsal and lateral spinal columns

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ALS

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

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What’s derived from surface ectoderm

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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Derived from neural ectoderm

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

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Derived from neural crest cell

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

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Mesoderm derivatives

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

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Endoderm derivatives

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

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

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

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Syringomyelia pathophysiology

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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Sx of syringomyelia

“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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Define focal dystonia and give example

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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What can help treat focal dystonias

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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Antiphagocytic capsule is a primary virulence factor for…

Strep pneumo, H. influ, and Neisseria bacteria

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Hypervariable pili characteristic of…

N. meningitidis and N. gonorrhoeae

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IgG binding outer membrane portion is a virulence factor for…

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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Wernicke syndrome

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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Thiamine (B1) cofactor for what enzymes?

PDH (pyruvate to acetyl CoA)
alpha-ketoglutarate dehydrogenase
Transketolase (pentose to G3P)

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How to treat Wernicke syndrome

Give glucose infusion BUT SUPPLEMENT WITH THIAMINE AS WELL

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What product is increased in B12 deficiency?

Methylmalonic acid (product of FA oxidation)

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Neuroblastoma

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

Wilms tumor

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

Beckwith-Wiedemann syndrome/WAGR complex:

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

Niemann-Pick disease

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

Metachromatic leukodystrophy

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

Tay-Sachs

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

Fabry's disease

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

Bacterial vs. viral/aseptic meningitis

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

Most common cause for viral meningitis

Enterovirus in kids (coxackievirus, echovirus, poliovirus)

#1 and #2 cause for bacterial meningitis

``` #1 Strep pneumo #2 N. meningitidis ```

2 nitrogen sources for urea cycle

NH3 and aspartate

Rate limiting step in urea cycle & what activates the enzyme

Carbamoyl phosphate synthetase 1 (CPS 1) - generates carbamoyl phosphate CPS 1 activated by NAG (N-acetylglutamate)

Infectious process of N. meningitidis

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

Function of ventromedial nucleus of hypothal

satiety; stim. leptin; destruction = grow medial!

Function of lateral nucleus of hypothal

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

Function of paraventricular nucleus

ADH, CRH, oxytocin & thyrotropin-releasing hormones

Supraoptic nucleus

ADH and oxytocin secretion

Suprachiasmatic nucleus function

Circadian rhythm & pineal gland function

Anterior nucleus of hypothalamus

destruction = hyperthermia

Posterior nucleus of hypothalamus

destruction = hypothermia

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

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

Subarachnoid hemorrhage

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

Complication of resolved subarachnoid hemorrhage

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

Treat/prevent vasospasm from a subarachnoid hemorrhage

Nimodipine - selective calcium channel blocker

DNA pol 1

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

DNA pol 3

Initiates DNA replication 3-5 direction

Associations with ADPKD

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

Intracerebral hemorrhage manifestation

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

Characteristics of uncal (transtentorial) herniation

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

12-48 hrs after ischemia

"red neurons" - eosinophilic cytoplasm, Nissl substances

1-3 days after ischemia

Necrosis and neutrophilic infiltration

3-5 days after ischemia

Macrophage infiltration + phagocytosis

1-2 wks after ischemia

Liquefactive necrosis, reactive gliosis and vascular proliferation

> 2 wks

glial scar, cystic area surrounded by gliosis

Prophylactic agains invasive meningococcal diseases (N. meningitidis)

Rifampin

Classic side effect for rifampin

orange discoloration of secretions (urine, breast milk, tears) - contact lenses will turn orange! ALSO rifAMPin AMPlifies P450 - inc. metabolism of drugs (ex: wafarin) ,,j