Pathophys test 2

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Pathophys test 2
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2011-03-07 19:22:04
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Pathophys test 2
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  1. _______: chronic degenerative disorder of the CNS that poduces movement problems. Most obvious changes are motor related, but cognitive symptoms can appear as well.
    Parkinson's Disease
  2. True or false: the cause of parkinson's is iatarogenic.
    False: idiopathic (also genetic and environmental component)
  3. _______ parkinsonism is not true PD, but the symptoms appear with other medical problems (meningitis, encephalitis, stroke, vascular disease, repeated head trauma etc.)
    secondary parkinsonism
  4. 4 risk factors for Parkinson's (they are very general)
    age, gender, genetic, exposure to environmental toxins
  5. Incidence of parkinson's over the age of 60 increases from a __-__% chance to __to__%.

    (men/women) are 1.5x more likely to develop PD.
    1-2%; 2-4%

    Men
  6. Pathophysiology of Parkinson's:
    Disruption of the ______ motor system. These tracts are associated with the _____ _____, which plays a big role in movement.
    • extrapyramidal motor system
    • basal ganglia (plays a big role because it contains the substantia nigra)
  7. The substantia nigra is filled with _____ secreting neurons. In PD, many of these neurons are destroyed. Dopamine is an (excitatory/inhibitory) neurotransmitter which helps control movement.
    • dopamine
    • inhibitory
  8. Decreased levels of dopamine causes the remaining neurons to be overy (inhibited/excited)?
    excited
  9. In addition to damage to dopamine producing neurons in PD, damage to ______ secreting neurons and the presence of ______ bodies can also occur.
    • norepinephrine (effects the ANS)
    • Lewy bodies (associated with Dementia and memory loss)
  10. ______ gait: common with PD, short, shuffling steps that increase in speed over time and often lead to falls.

    Patient's with PD often experience a ______ _____, where a person feels stuck and finds it difficult to start moving again.
    • Festinating (propulsive)
    • "Freezing Period"
  11. Describe typical standing posture of PD patient's: (essentially three things)
    stooped, leaning forward, head and neck flexed
  12. The most common diagnostic test for PD is and MRI/CAT scan. True or false
    false, no existing diagnostic test exists for PD
  13. Describe the 5 stages of PD:
    • Stage 1: unilateral symptoms
    • Stage 2: bilateral symptoms
    • Stage 3: balance trouble, but able to amb indep
    • Stage 4: assist needed for amb
    • Stage 5: wheel-chair or bed-bound
  14. What two drugs are used (most commonly) for PD?
    • carbidopa/levodopa
    • (carbidopa allows levadopa to cross the BBB)
  15. In alzheimer's disease, genetic mutations on chromosomes __,__, and __ are known to cause early onset or familial ALZ D which effects people between 30-60. People with ______ ______ have an increased chance of getting ALZ D.
    • 1,14, and 21
    • down's syndrome
  16. 1 in ___ persons over 65 have AD. (men or women) have a higher risk of having AD.
    • 1 in 8 persons
    • women
  17. Describe the 7 stages of AD:
    • 1. No impairment
    • 2. Very mild cognitive decline
    • 3. Mild cognitive decline
    • 4. Moderate cognitive decline
    • 5. moderately severe cognitive decline
    • 6. severe cognitive decline
    • 7. very severe cognitive decline
  18. ______ plaques: contain beta-amyloid proteins found in the extracellular deposits in the CNS. Interfere with functioning by stimulating production of free radicals.
    amyloid plaques
  19. ________ _______: paired helical filaments of tau proteins (which are normally important for growt hand devlopment of axons) When these proteins are hyperphosphorylated, they form _____ which leads to cell death.
    • neurofibrillary tangles
    • tangles
  20. With AD, neuron and synapse loss follows a pattern primarily affecting the pathways in:
    1.
    2.
    3.
    • Basalis Nucleus of Mevnert (cholinergic pathway/Ach which influences memory.
    • Median Raphe (serotonergic receptors)
    • Locus Ceruleus (adrenergic receptors which leads to loss of norepi)
  21. The only confirmatory test for AD is an?
    autopsy
  22. Early onset AD presents before the age of ___.
    60
  23. Name the 2 main types of drugs to treat AD?

    Which vitamin is also supplemented?
    • 1. Ach Inhibitors (keeps levels of Ach high)
    • 2. Memantine (NMDA antagonist which regulates glutamate (learning and memory))
    • Vitamin E
  24. ____ _____ ____ ____: may improve memory, mood, ciradian rhythm, and level of awareness of AD patients.
    transcutaneous electrical nerve stimulation
  25. _____ _____: progressive demyelination of neurons of the brain, spinal cord, and cranial nerves. Characterized by remissions and exacerbations.
    Multiple sclerosis
  26. What is the cause of MS?
    unknown, many believe it is autoimmune; appears to have a genetic component, immunologic, and environmental component as well.
  27. MS is most frequent in _____ in _____ zones (northern US and Canada)
    • caucasians (with european descent)
    • temperate (warm climates)
  28. True or false: MS is the most common cause of disability in the US.
    False: second to motor vehicle accidents
  29. MS is more common in (men or women) and onset of symptoms occur between ____ and _____ with a peak at ____.
    • women
    • 20 and 40
    • 30
  30. _____-_____ MS: most common form of the disease. Characterized by clearly defined exacerbations and remissions followed by partial or complete recovery. 85% of people with MS begin with this course.
    Relapsing-remitting MS
  31. _______ ________ MS: characterized by progression of disability of onset, without plateaus or remissions, but can have periods of stabilization. By definition, does not experience acute attacks. 10-15% of people.
    Primary progressive MS
  32. ______ _______ MS: begins as the relapsing remitting ms, followed by progression of disability that may include occasional relapses, minor remission, and plateaus. Less recovery following attacks, persistently worsening functioning during and between attackss, and fewer and fewer attacks.
    Secondary Progressive MS
  33. _______ _______ MS: least common form, shows progression of disability from onset but with clear acute relapses with or without full recovery.
  34. ______ ______: conduction of action potentials along myelinated axons from one node of Ranvier to the next.
    Saltatory conduction
  35. True or false: Earliest lesions in MS occur as inflammatory response when myelin is lost in the white matter of the brain and spinal cord. Later, larger areas are known as plaques because they are visible.
    True (plaques seen in lateral ventricles of brain, brain stem, and optic nerves)
  36. True or False: The neurological exam is conclusive for MS.
    False: no single test is conclusive
  37. ______ is a common first sign of MS follwoed by weakness in LE due to plaques in the corticospinal tract.
    blurred vision
  38. 3 common sensory abnormalities on clinical exam for MS:
    • impairment of vibration and joint position sense
    • decreased pain and light touch perception distally
    • patchy areas of reduced pain and light touch perception

    (pain also) but that makes four and i didn't want to go back and change my original question.
  39. 4 Goals of MS treatment:
    • improve speed of recovery from attacks
    • reduce number of attacks and lesions
    • slow progression
    • relieve other complications
  40. ______ _______ _____: progressive neurodegenerative disease that attacks nerve cells in the brain and spinal cord resulting in muscle weakness and atrophy.
    amyotrophic lateral sclerosis
  41. Etiology of ALS: 2 choices
    • unknown (90%)
    • genetic (10%)
  42. Average age of incidence of ALS ____-____ years old. (males or females) are 1.5 times more likely to have and (what ethnicity) is 1.6 more likely to have ALS.
    • 40-60
    • males
    • whites
  43. After being diagnosed with ALS, death usually occurs within __-__ years.
    3-5 years
  44. ALS degenerates both ____ and ____ neurons. As motor neurons degenerate, the _____ and _____ columns of the spinal cord are replaced by fibrous astrocytes.
    • upper and lower
    • anterior and lateral
  45. _______ degeneration: process by which motor axons die. Death occurs in the _____ horn of the cell body. Causes degeneration of associated motor axon. Surrounding Schwann cells breakdown the axon's myelin sheath, which results in the axons breaking into pieces.
    • Wallerian degeneration
    • anterior horn
  46. ALS causes degeneration of the _______ upper motor neurons that reside in the cerebral cortex specifically ____ _____. Loss of these upper motor neurons causes spastic paralysis, hyperreflexia, and stiffness.
    • corticospinal upper motor neuron
    • precentral gyrus
  47. ALS causes the degeneration fo the lower motor neurons that reside in the ______ ______ of the _____ ____ and _____. Loss of these motor neurons leads to flaccid paralysis, decreased muscle tone, decreased reflexes, muscle weakness, and muscle atrophy.
    • anterior horn
    • spinal cord and brainstem
  48. ALS does not affect: 4 things
    • Cranial Nerves III, IV, and VI
    • Sensory neurons
    • Cognitive function
    • Bowel and bladder function
  49. ______ is the only exogenous factor that may contribute to ALS.
    smoking
  50. _____ is the 1st and only drug that slows the disease ALS and prolongs life
    riluzole
  51. _____ _____ ______: syndrome where there is inflammation in the peripheral nervous system. Thought to be autoimmune. Occurs about 1-3 weeks after an infection, surgery, or vaccination.
    guillain-barre syndrome
  52. In GBS, peripheral nerves in the (arms or legs) are affected first. Critical period occurs when paralysis of the _____ and ______ begins.
    • legs
    • diaphragm and respiratory muscles
  53. GBS leads to impaired nerve conduction mainly in the _____ fibers. Axons can regenerate if the cell bodies stay intact.
    efferent (motor) fibers
  54. Recovery from GBS usually occurs __-__ years after the initial onset. Most people recover completely __%.
    • 2-3 years
    • 75%
  55. _____ _____: autoimmune disorder that leads to skeletal muscle weakness and rapid muscle fatigue. Acetylcholine receptors become impaired at the NMJ.
    myasthenia gravis
  56. MG in general is more common in (men or women).

    women are usually most susceptible between the ages __-__.
    men are usually over the age of __?
    • women
    • 20-30
    • 50
  57. In MG, ____ autoantibodies bind to ach receptors and block or destroy the sites. If ach cannot bind to the receptor site, muscle stimulation can't occur.
    Muscles of the ____ and ____ are the first to be affected.
    • IgG
    • face and eyes
  58. group of inherited disorders characterized by degeneration of skeletal muscle
    Muscular Dystrophy
  59. MD is a metabolic defect caused by a deficit of ______ resulting in cell degeneration and necrosis
    dystrophin
  60. T/F: Few muscular dystrophies are inherited and involve a mutation in genes
    False: all involve a mutation
  61. Can MD be caused by spontaneous mutations?
    yeppers
  62. What are the 3 types of inheritance for MD?
    • Autosomal Dominant
    • Autosomal Recessive
    • X-linked Recessive
  63. In MD, muscle appears ______ because muscle fibers are replaced with ____ & _______ ______ tissue
    • hypertrophic
    • fat & fibrous connective tissue
  64. holds the cytoskeleton to the extracellular matrix
    dystrophin
  65. Know inheritance, age of onset, distribution & speed of progression for:
    Duchenne's MD
    Fascioscapulo humeral MD
    Myotonic MD
    Limb Girdle MD
    • Duchenne's - x-linked recessive, 2-3 years, hips legs & shoulder girdle, rapid
    • FH MD - autosomal dominant, before 20, shoulder neck & face, slow to moderate
    • Myotonic - autosomal dominant, birth to 50 yrs, face & hands, slow
    • Limb Girdle - autosomal recessive, all ages, shoulders & pelvis, varied
  66. 3 ways to test for MD
    • Physical Exam
    • Muscle Biopsy
    • Blood test for creatine kinase (elevated with death of muscle)
  67. an autosomal recessive neurodegenerative disease characterized by degeneration of spinal cord motor neurons, atrophy of skeletal muscle & muscle weakness
    Spinal Muscular Atrophy (SMA)
  68. cause of SMA
    mutation of SM1 gene (survival motor neuron 1)
  69. SMN protein from the SM1 gene is crucial for the survival and health of __-motor neurons
    alpha
  70. type ___ SMA: Werdnig-Hoffman disease
    most severe, affects infants (<8 months), death before 2 years, never able to sit
    type I
  71. type ___ SMA: less severe, children progressively weaker, 7-18 months, death after 2 years, never stands
    type II
  72. type ___ SMA: least severe, can begin as a child or an adult, >18 months, death as an adult, is able to stand and walk
    type III
  73. 4 general tests for SMA
    • genetic (pre conception and blood for post)
    • prenatal (chronic villi sampling & amniocentesis)
    • electromyography
    • biopsy

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