Multiple sclerosis

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Background

  • CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction

Types

  • Relapsing/remitting (most common)
    • Relapse (days-months) followed by remission
  • Secondary progressive
    • Relapses and partial recoveries occur, but disability does not fade away between cycles
  • Primary progressive
    • Symptoms progress slowly and steadily without remission
  • Progressive relapsing
    • Similar to primary progressive but with superimposed flares

Clinical Features

  • Classic patient has multiple presentations for neuro symptoms of different areas of pathology
    • Patient often has resolution of the earlier symptoms
  • Symptoms worsen with increases in body temperature (Uhthoff's phenomenon[1])
  • Muscle/sensory signs:
    • Lower extremity weakness usually worse than upper extremity
    • Upper motor neuron signs:
      • Hyperreflexia
      • Positive Babinski
    • Decrease in proprioception / pain/temperature sensation
    • Lhermitte sign
      • Electric shock sensation radiating down back into arms/egs from neck flexion
  • Optic neuritis
    • Initial sign in 30% of patients
    • Vision loss (usually unilateral) often preceded by retrobulbar pain
    • Blurred vision
    • Afferent pupillary defect is pathognomonic for optic neuritis
    • Nystagus
    • Diplopia
  • Internuclear ophthalmoplegia
    • Abnormal eye adduction bilaterally and horizontal nystagmus
    • Convergence (both eyes center medially) is preserved
  • Dysautonomia

Differential Diagnosis

Weakness

Evaluation

Work-Up

  • LP
    • IgG
    • Albumin
    • Oligoclonal bands
    • myelin basic protein
    • Cell count
    • Glucose
    • Protein
    • Gm stain
  • CBC, Chemistry
  • Urinalysis
  • MRI with GAD of brain (+/- spine)

Evaluation

  • CSF
    • Elevated protein and gamma-globulin (increased oligoclonal bands)
  • MRI
    • Multiple lesions in supratentorial white matter, paraventricular area, spinal cord

Management

  • Fever must be reduced to minimize weakness associated with elevated temperature
  • Antibiotics for UTI/pyelo
  • High-dose steroid therapy for relapses
  • Suppression therapies
    • IFN B, Glatiramer, Estriol (usually not in ED)

Disposition

  • Hospitalization indicated for:
    • Any disease exacerbation associated with significant morbidity
    • IV antibiotics or steroid therapy required
    • Depression and significant risk of suicide

See Also

References

  1. Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.