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NE10 Other neurological disorders

Deleted user 16 questions Updated 11 Oct 2019

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Questions in this quiz (16)

  1. 1By the end of this lecture you will be able to:
    1. Describe the pathophysiology of motor neurone disease and multiple sclerosis
    2. Describe available treatments for these disorders
    • Mechanisms of action
    • Main unwanted effects

    [blank_start]OK[blank_end]
    1. AOK
  2. 2Motor neurone disease is [blank_start]progressive degeneration[blank_end] of motor neurons. The most affected group is [blank_start]middle aged males[blank_end].
    Motor neurons - Upper ones originate in brain, lower ones originate in spinal cord. MND affects [blank_start]both[blank_end] upper and lower. Also know as:
    • [blank_start]Lou Gehrig's[blank_end] Disease
    • Charcot's Disease (in Europe).
    • Amyotrophic Lateral [blank_start]Sclerosis[blank_end] (in US)
    1. Aprogressive degeneration
    2. Bmiddle aged males
    3. Cboth
    4. DSclerosis
    5. ELou Gehrig's
  3. 3Which of these is NOT a presentation of MND?
    1. AStumbling
    2. BDropping, difficulty holding things
    3. CHoarse voice/slurred speech
    4. DMuscle cramps
    5. EDysphagia
    6. FFatigue
    7. GBehavioural/emotional changes
    8. HSevere cognitive difficulties
  4. 4What causes MND?
    Most cases [blank_start]sporadic[blank_end]...
    • [blank_start]Viral[blank_end] infection
    • [blank_start]Immune[blank_end] mediated damage
    • Premature motor neuron [blank_start]aging[blank_end]
    • Loss of [blank_start]growth factors[blank_end]
    [blank_start]5-10[blank_end]% hereditary ([blank_start]SOD[blank_end] mutation).

    Pathophysiology?
    • TDP-43 [blank_start]RNA splicing[blank_end] dysfunction
    • Excessive [blank_start]glutamate R[blank_end] activation
    • Intracellular [blank_start]Ca2+ overload[blank_end]
    • Mitochondrial damage
    • [blank_start]Excitotoxic[blank_end] cell death
    • Oxidative stress ([blank_start]free radicals[blank_end])
    1. Asporadic
    2. BViral
    3. CImmune
    4. Daging
    5. Egrowth factors
    6. F5-10
    7. GSOD
    8. Hglutamate R
    9. ICa2+ overload
    10. JExcitotoxic
    11. Kfree radicals
    12. LRNA splicing
  5. 5Tx for motor neurone disease:
    1. Riluzole
    Slows progression, prolongs median survival time by [blank_start]2-3 months[blank_end]
    Multiple effects on [blank_start]glutamate[blank_end] neurotransmission.
    Presynapic: inhibits [blank_start]release[blank_end] - inhibits [blank_start]voltage gated Na+[blank_end] channels
    Postsynaptic: prevents [blank_start]action[blank_end] - non competitive [blank_start]blockade of NMDA[blank_end] Rreceptors
    Contraindicated: hepatic impairment
    Caution: [blank_start]neutropenia[blank_end] (fever, sore-throat, malaise)

    2. Endaravone (not available in NZ). First FDA approved MND therapy in [blank_start]22[blank_end] years. Free [blank_start]radical[blank_end] scavenger.
    1. A2-3 months
    2. Bglutamate
    3. Cvoltage gated Na+
    4. Drelease
    5. Eaction
    6. Fblockade of NMDA
    7. Gneutropenia
    8. H22
    9. Iradical
  6. 6Which of these is not a common unwanted effect of riluzole?
    1. ANausea and vomiting
    2. BLethargy
    3. CDizziness
    4. DDiarrhoea
  7. 7Multiple Sclerosis is an [blank_start]autoimmune[blank_end] disorder that affects nerve [blank_start]conduction[blank_end]. There are multiple areas of [blank_start]scarring[blank_end] in the brain, and spinal cord. Inflammation causes relapse. [blank_start]Demyelination and axon damage[blank_end] cause:
    Optic neuritis, weakness, spasticity, ataxia, [blank_start]bladder and bowel[blank_end] dysfunction.

    Epidemiology:
    - Affects 1 in 1000
    - [blank_start]Young[blank_end] adults (average age 37 years old)
    - [blank_start]Women[blank_end] three times more than men
    • [blank_start]Caucasians[blank_end]
    • People in [blank_start]cooler[blank_end] climates
    1. Aautoimmune
    2. Bconduction
    3. Cscarring
    4. DDemyelination and axon damage
    5. Ebladder and bowel
    6. FYoung
    7. GWomen
    8. HCaucasians
    9. Icooler
  8. 8Pathology of MS:
    Trigger = [blank_start]peripheral[blank_end] immune response. Primed [blank_start]T and B[blank_end] lymphocytes cross [blank_start]BBB[blank_end]. Upregulation of [blank_start]integrins[blank_end] causes [blank_start]adhesion[blank_end],

    Inflammation -
    [blank_start]Th1 lymphocytic[blank_end] autoimmune response involving INF-g, IL-17, TNF-b.

    [blank_start]B[blank_end] cell mediated [blank_start]antibodies[blank_end] - [blank_start]Initiate[blank_end] myelin destruction

    [blank_start]Macrophages[blank_end] -
    Destroy myelin [blank_start]sheath[blank_end], damage [blank_start]oligo[blank_end]dendrocytes. Demyelination leads to [blank_start]axon[blank_end] damage
    1. Aperipheral
    2. BT and B
    3. CBBB
    4. Dintegrins
    5. Eadhesion
    6. FTh1 lymphocytic
    7. GB
    8. Hantibodies
    9. IInitiate
    10. JMacrophages
    11. Ksheath
    12. Loligo
    13. Maxon
  9. 9Drug treatment for MS:
    Reduction of symptoms.
    15% of patients have [blank_start]primary[blank_end] progressive MS, 85% [blank_start]relapsing- remitting[blank_end] MS ([blank_start]secondary[blank_end] progressive MS).
    • Modulating immune response early reduces [blank_start]disability[blank_end]
    1. Aprimary
    2. Brelapsing- remitting
    3. Csecondary
    4. Ddisability
  10. 10Fingolimob
    • Oral [blank_start]prodrug[blank_end] (reversible [blank_start]phosphorylation[blank_end])
    • Agonist of [blank_start]sphingosine-1-phosphate[blank_end] R
    • Then internalisation prevents [blank_start]lymphocyte[blank_end] egress, reduced [blank_start]migration[blank_end] to lesions
    Immunomodulator - [blank_start]infections[blank_end] risk
    UEs: Headache, migraine, [blank_start]dizziness, paraesthesia, depression[blank_end]
    Caution: patients with [blank_start]CV[blank_end] risk – transient bradycardia, heart block.
    1. Aprodrug
    2. Bphosphorylation
    3. Csphingosine-1-phosphate
    4. Dlymphocyte
    5. Emigration
    6. Finfections
    7. Gdizziness, paraesthesia, depression
    8. HCV

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User has deleted their subject information Quiz on NE10 Other neurological disorders, created by Deleted user on 11/10/2019.
Author
Deleted user
Language
English
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Created on 11 Oct 2019 · updated on 11 Oct 2019
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