Multiple sclerosis

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, classically after hot showers (Uhthoff's phenomenon)
• 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
    • If the discomfort is severe, carbamazepine or gabapentin may be beneficial for some patients.
• 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
  • Nystagmus
  • Diplopia
• Internuclear ophthalmoplegia
  • Abnormal eye adduction bilaterally and horizontal nystagmus
  • Convergence (both eyes center medially) is preserved
• Dysautonomia
  • Urinary retention (increased risk of UTI/pyelo)
  • Constipation or incontinence
  • Sexual dysfunction (males)

Differential Diagnosis

Weakness

• Neuromuscular weakness
  • Upper motor neuron:
    • CVA
    • Hemorrhagic stroke
    • Multiple sclerosis
    • Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
  • Lower motor neuron:
    • Spinal and bulbar muscular atrophy (Kennedy's syndrome)
  • Spinal cord disease:
    • Infection (Epidural abscess)
    • Infarction/ischemia
    • Trauma (Spinal Cord Syndromes)
    • Inflammation (Transverse Myelitis)
      • Acute flaccid myelitis
    • Degenerative (Spinal muscular atrophy)
    • Tumor
  • Peripheral nerve disease:
    • Guillain-Barre syndrome
    • Toxins (Ciguatera)
    • Tick paralysis
    • Diabetic peripheral neuropathy
  • Neuromuscular junction disease:
    • Myasthenia gravis crisis
    • Botulism
    • Organophosphate toxicity
    • Lambert-Eaton myasthenic syndrome
  • Muscle disease:
    • Rhabdomyolysis
    • Dermatomyositis
    • Polymyositis
    • Alcoholic myopathy
• Non-neuromuscular weakness
  • Can't miss diagnoses:
    • ACS
    • Arrhythmia/Syncope
    • Severe infection/Sepsis
    • Hypoglycemia
    • Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
      • Hypokalemic periodic paralysis
      • Thyrotoxic periodic paralysis
    • Respiratory failure
  • Emergent Diagnoses:
    • Symptomatic Anemia
    • Severe dehydration
    • Hypothyroidism
    • Polypharmacy
    • Malignancy
    • Aortic disease - occlusion, stenosis, dissection
  • Other causes of weakness and paralysis
    • Acute intermittent porphyria (ascending weakness)

Evaluation

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

• 

  MRI brain with contrast of a patient in her mid-20s with new onset MS. Large lesion in left parietal area.

• 

  MRI brain with contrast of same patient with new onset MS with another lesion in the left cerebellum.

• MRI with GAD of brain (+/- spine)
• CBC, Chemistry
• Urinalysis

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

• Optic Neuritis