Multiple sclerosis: Difference between revisions

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==Background==
==Background==
*CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction
*CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction<ref>Multiple sclerosis: a practical overview for clinicians. British Medical Bulletin, Volume 95, Issue 1, September 2010, Pages 79–104. https://doi.org/10.1093/bmb/ldq017</ref>


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


==Clinical Features==
==Clinical Features==
*Classic pt has multiple presentations for neuro symptoms of different areas of pathology  
*Classic patient has multiple presentations for neuro symptoms of different areas of pathology  
**Pt often has resolution of the earlier symptoms
**Patient often has resolution of the earlier symptoms
*Symptoms worsen w/ increases in body temperature (Uhthoff's phenomenon<ref>Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.</ref>)
*Symptoms worsen with increases in body temperature, classically after hot showers (Uhthoff's phenomenon<ref>Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.</ref>)
*Muscle/sensory signs:
*Muscle/sensory signs:
**Lower extremity weakness usually worse than upper extremity
**Lower extremity [[weakness]] usually worse than upper extremity
**Upper motor neuron signs:
**Upper motor neuron signs:
***Hyperreflexia
***Hyperreflexia
***Positive Babinski
***Positive Babinski
**Decrease in proprioception / pain/temp sensation
**[[numbness|Decrease]] in proprioception/pain/temperature sensation
**Lhermitte sign
**Lhermitte sign
***Electric shock sensation radiating down back into arms/egs from neck flexion
***Electric shock sensation radiating down back into arms/egs from neck flexion
*Optic neuritis
***If the discomfort is severe, carbamazepine or gabapentin may be beneficial for some patients.
*[[Optic neuritis]]
**Initial sign in 30% of patients
**Initial sign in 30% of patients
**Vision loss (usually unilateral) often preceded by retrobulbar pain
**[[Vision loss]] (usually unilateral) often preceded by retrobulbar pain
**Blurred vision
**[[Blurred vision]]
**Afferent pupillary defect is pathognomonic for optic neuritis
**Afferent pupillary defect is suggestive of optic neuritis
**Nystagus
**[[Nystagmus]]
**[[Diplopia]]
**[[Diplopia]]
*Internuclear ophthalmoplegia
*[[Internuclear ophthalmoplegia]]
**Abnormal eye adduction bilaterally and horizontal nystagmus
**Abnormal eye adduction bilaterally and horizontal nystagmus
**Convergence (both eyes center medially) is preserved
**Convergence (both eyes center medially) is preserved
*Dysautonomia
*Dysautonomia
**Urinary retention (increased risk of UTI/pyelo)
**[[Urinary retention]] (increased risk of [[UTI]]/[[pyelo]])
**Constipation or incontinence
**[[Constipation]] or incontinence
**Sexual dysfunction (males)
**Sexual dysfunction (males)


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{{Weakness DDX}}
{{Weakness DDX}}


==Diagnosis==
==Evaluation==
===Work-Up===
[[File:MS_MRI_1.jpg|thumb|MRI brain with contrast of a patient in her mid-20s with new onset MS. Large lesion in left parietal area.]]
*[[LP]]
[[File:MS_MRI_2.jpg|thumb|MRI brain with contrast of same patient with new onset MS with another lesion in the left cerebellum.]]
**IgG
*[[LP|CSF]]
**Albumin
**Oligoclonal bands
**myelin basic protein
**Cell count
**Glucose
**Protein
**Gm stain
*CBC, Chemistry
*UA
* MRI w/ GAD of brain (+/- spine)
 
===Evaluation===
*CSF
**Elevated protein and gamma-globulin (increased oligoclonal bands)
**Elevated protein and gamma-globulin (increased oligoclonal bands)
*MRI
*[[brain MRI|MRI]]
**Multiple lesions in supratentorial white matter, paraventricular area, spinal cord  
**Multiple lesions in supratentorial white matter, paraventricular area, spinal cord  
<gallery>
*[[brain MRI|MRI]] with GAD of brain (+/- spine) and orbits (if [[optic neuritis]] suspected)
File:MS_MRI_1.jpg|MRI brain with contrast of a patient in her mid-20s with new onset MS. Large lesion in L parietal area.
*CBC, Chemistry
File:MS_MRI_2.jpg|MRI brain with contrast of same patient with new onset MS with another lesion in the L cerebellum.
*[[Urinalysis]]
</gallery>


==Treatment==
==Management==
*Fever must be reduced to minimize weakness assoc w/ elevated temperature
*Fever must be reduced to minimize weakness associated with elevated temperature
*Abx for UTI/pyelo
===Infection Treatment===
*High-dose steroid therapy for relapses
*Antibiotics or surgical control of any infectious sources
===Steroids and Immunomodulators===
*High-dose [[Corticosteroids|steroid]] therapy for relapses in the form of oral or intravenous methylprednisolone (1000 mg)<ref>Le Page et al. Oral versus intravenous high-dose methylprednisolone for treatment of relapses in patients with multiple sclerosis (COPOUSEP): a randomised, controlled, double-blind, non-inferiority trial. Lancet. 2016 Jan 23;387(10016):340. https://core.ac.uk/download/pdf/52993687.pdf</ref>
*Suppression therapies
**IFN B, Glatiramer, Estriol (usually not in ED)


==Disposition==
==Disposition==
*Hospitalization indicated for:
*Hospitalization indicated for:
**Any disease exacerbation a/w significant morbidity
**Any disease exacerbation associated with significant morbidity
**IV abx or steroid therapy required
**IV antibiotics or steroid therapy required
**Depression and significant risk of suicide
**Depression and significant risk of suicide


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==References==
==References==
<references/>
<references/>


[[Category:Neurology]]
[[Category:Neurology]]

Latest revision as of 21:01, 16 November 2022

Background

  • CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction[1]

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[2])
  • 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
  • Internuclear ophthalmoplegia
    • Abnormal eye adduction bilaterally and horizontal nystagmus
    • Convergence (both eyes center medially) is preserved
  • Dysautonomia

Differential Diagnosis

Weakness

Evaluation

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.
  • CSF
    • Elevated protein and gamma-globulin (increased oligoclonal bands)
  • MRI
    • Multiple lesions in supratentorial white matter, paraventricular area, spinal cord
  • MRI with GAD of brain (+/- spine) and orbits (if optic neuritis suspected)
  • CBC, Chemistry
  • Urinalysis

Management

  • Fever must be reduced to minimize weakness associated with elevated temperature

Infection Treatment

  • Antibiotics or surgical control of any infectious sources

Steroids and Immunomodulators

  • High-dose steroid therapy for relapses in the form of oral or intravenous methylprednisolone (1000 mg)[3]
  • 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. ↑ Multiple sclerosis: a practical overview for clinicians. British Medical Bulletin, Volume 95, Issue 1, September 2010, Pages 79–104. https://doi.org/10.1093/bmb/ldq017
  2. ↑ Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.
  3. ↑ Le Page et al. Oral versus intravenous high-dose methylprednisolone for treatment of relapses in patients with multiple sclerosis (COPOUSEP): a randomised, controlled, double-blind, non-inferiority trial. Lancet. 2016 Jan 23;387(10016):340. https://core.ac.uk/download/pdf/52993687.pdf
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