MS+NMO(20190122).pdf

14 Multiple sclerosis.pdf

Background

• Characterized by inflammatory demyelination disseminated in different areas of the CNS (brain and spinal cord) and occurring at different time points.
• median age of onset is 23.5 y/o (年輕)
• more women than men
• 西方國家較多；北方國家較多

Pathophysiology

• Environmental: far from equator; northern european (vitaminD; Birth season); virus: EBV or HHV-6
• Genetics: 30% identical twins (5% for non-indentical twins); HLA-DR15, DQ6
• multifocal areas of demyelination with relative preservation of axons, loss of oligodendrocytes, and astroglial scarring.
  • may also involve axonal loss

Symptoms and signs

• four main clinical courses of MS
  • Clinically isolated syndrome (CIS)
  • Relapsing-remitting multiple scleosis (最常見85%)
  • Secondary progressive multiple sclerosis
  • Primary progressive multiple sclerosis

• focal neurological signs

  • the spinal cord, such as limb weakness, reduced or abnormal feelings of touch or skin sensations, the Lhermitte phenomenon (electrical sensation down spine upon neck flexion), a band-like sensation around the chest or abdomen, and problems with urination or bowel movements

  <aside> 💡 Uhthoff's phenomenon: a worsening of symptoms due to exposure to higher than usual temperatures

  </aside>

  • the optic nerve, such as impaired vision in 1 or both eyes and pain with eye movement

  <aside> 💡 50% having optic neuritis, 20-30% as first symptom, 30% opticneuritis will developed MS in 5 yrs

  </aside>

  • the brainstem, such as double vision and a jerking sensation in the visual field
  • the cerebellum, such as poor coordination, vertigo, tremors, facial muscle weakness, and reduced facial sensations

Diagnosis

• 2017 revised McDonald diagnostic criteria

  • A diagnosis of MS requires involvement of ≥ 2 areas of central nervous system (dissemination in space) at different time points (dissemination in time)
  • Diagnose relapsing-remitting MS in patients with either
    • ≥ 2 monophasic clinical attacks plus objective clinical evidence of ≥ 2 lesions or objective clinical evidence of 1 lesion and either:
      • clear-cut historical evidence of previous attack involving lesion in different anatomical location
      • demonstration of dissemination in space by magnetic resonance imaging (MRI) or an additional clinical attack implicating a different central nervous system (CNS) site
    • 1 monophasic clinical attack plus either of the following
      • objective clinical evidence of ≥ 2 lesions plus either demonstration of dissemination in time (by MRI or by an additional clinical attack) or presence of CSF-specific oligoclonal bands
      • objective clinical evidence of 1 lesion plus demonstration of dissemination in space and dissemination in time or presence of cerebrospinal fluid (CSF)-specific oligoclonal bands
  • Diagnose primary progressive MS in patients with both:
    • 1 year of steadily increasing, objectively documented neurologic disability independent of clinical relapse and ≥ 2 of the following:
      • at least 1 T2-hyperintense lesion characteristic of MS in periventricular, cortical, juxtacortical, or infratentorial brain regions
      • at least 2 T2-hyperintense lesions in spinal cord
      • presence of CSF-specific oligoclonal bands

• 簡單來說，就是要符合三個條件

  • History of the episode (不同時間點發生DIT + 侵犯不同地方 DIS)
  • Evidence of CNS abnormality on exam (MRI)
  • Lab diagnosis: CSF, evoked potentials

  <aside> 💡 DIT = disseminated in time DIS = disseminated in space

  </aside>

• DIT was defined as

  1. A new T2 and/or Gd+ (contrast) lesion(s) on follow-up MRI, with reference to a baseline scan, irrespective of the timing of the baseline MRI
  2. Simultaneous presence of asymptomatic Gd+ and Gd-lesions at any time