P year‎ > ‎Neurology‎ > ‎

12.11.05 Clinic notes

MS
  • Course not thought to be related to relapse rate
  • EBV
    • Prevalence 90% in healthy population
    • Prevalence 100% in MS population
    • Mimicry?
  • 2 disease processes?
    • Inflammation
    • Neurodegeneration
  • Relapse rate 0.6-0.8 episodes per year
  • Subtype
    • RRMS
      • 85% of cases
      • No progression between relapses
    • PPMS
      • 10% of cases
      • No relapses
      • Continuous progression
    • PRMS
      • Clearly defined relapses
      • Disease progression between relapses
    • SPMS
      • Initial diagnosis is RRMS
      • But then continuous disease progression
  • DMT
    • IFNb
      • Blocks T-cell proliferation and tumor necrosis factor production
      • 3 different types
    • Glatiramer acetate
      • Immunomodulator
      • Inhibits T-cell activity
    • Mitoxantrone
      • Inhibits T-cell, B-cell, and macrophage proliferation
      • Decreases the secretion of pro-inflammatory cytokines and increases an anti-inflammatory response via promotion of the T-cell suppressor function
      • Inhibits macrophage-mediated myelin degradation
    • Natalizumab
      • Anti-alpha4-integrin
      • Inhibits the adhesion of leukocytes to their counter-receptor
      • Prevents cells crossing BBB
    • Fingolimod
      • First oral disease modifying drug
      • Sphingosine 1-phosphate receptor modulator
      • Sequesters lymphocytes in lymph nodes

Synamet
  • Combination of carbidopa and levodopa
  • Carbidopa is polar and so cannot cross the blood brain barrier
    • But prevents peripheral conversion of levodopa to dopamine
    • Thereby reduces the unwanted peripheral side effects of levodopa
      • Nausea + vomiting
    • Also increases the quantity of levodopa in the bloodstream that is available to enter the brain


Entacapone

  • Catechol-O-methyl transferase (COMT) inhibitor
  • Prevents COMT from metabolizing L-DOPA into 3-methoxy-4-hydroxy-L-phenylalanine (3-OMD) in the periphery, which does not easily cross the blood brain barrier (BBB)
  • May cause urine to turn reddish-brown


Stalevo

  • Anti-parkinsonian dopaminergic combination medication
  • Contains carbidopa, levodopa, and entacapone


Myotonic dystrophy

  • Army drink-drive guy
  • Chronic, slowly progressing, highly variable, inherited multisystemic disease
  • Trinucleotide expansion
  • Characterized by muscular dystrophy, cataracts, heart conduction defects, endocrine changes, and myotonia
  • Two types:
    • DM1, also called Steinert disease
      • Has a severe congenital form and a milder childhood-onset form
    • DM2, also called proximal myotonic myopathy (PROMM) or adult-onset form
      • Rarer than DM1 and generally manifests with milder signs and symptoms
  • Autosomal dominant inheritance
  • Presentation
    • Muscle wasting
    • Slow relaxation
    • Thenar tap test
    • Frontal hair loss
    • Hyporeflexia
    • Sunken face
  • A useful clinical clue for diagnosis is the failure of spontaneous letting go of the hands following strong handshakes due to myotonia


Notes

  • Leprosy is the most common global cause of neuritis
  • Sarcoidosis can have neurological manifestations
    • Neurologic findings are observed in about 5% of patients
    • Known as neurosarcoidosis
    • Cranial nerves are predominantly affected
      • Peripheral facial nerve palsy, often bilateral, is the most common neurological manifestation
  • Sildenafil-associated anterior ischemic optic neuropathy
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