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Chapter 43: Demyelinating Diseases

Matthew McCoyd, MD; Arash Salardini, MD

INTRODUCTION

Multiple sclerosis (MS) is an autoimmune central nervous system (CNS) demyelinating disease characterized by inflammatory clinical relapses and gradual neurodegeneration. The etiology of MS is multifactorial and involves both genetic and environmental factors. MS diagnosis is made on clinical and imaging grounds. The basic paradigm was first introduced in the 1960s and is referred to as Schumacker’s1 criteria after its first author. Schumacker set out 6 criteria for the diagnosis of definite MS:

  • Age between 10 and 50 years

  • Presence of objective clinical signs

  • Lesions being predominantly in the white matter

  • Two or more lesions in the CNS separated in space

  • The attacks being separated in time

    • Episodes lasting more than 24 hours and spaced more than one month apart

    • Documented progression for more than 6 months.

  • The rule out of other pathologies

These have been revised (current criteria = McDonald Criteria—Table 43-1)2 with the advent of newer diagnostic modalities, but the paradigm remains the essence of MS diagnosis. The steps to diagnose MS are as follows:

  1. Take history, examine the patient, and document compatibility of the signs and symptoms with MS. Certain common presentations increase suspicion for the disease.

  2. Document the presence of white matter lesions, which are separated by time and space on imaging and clinical grounds.

  3. Rule out mimics of MS.

  4. Be aware of MS variants.3

Table 43-1.

2010 McDonald Criteria for the Diagnosis of Multiple Sclerosis8

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Table 43-1.

2010 McDonald Criteria for the Diagnosis of Multiple Sclerosis8
Clinical Presentation Additional Data Needed for Dx
≥2 attacks, objective clinical evidence of ≥2 or more lesions or objective clinical evidence of 1 lesion with reasonable historical evidence of a prior attack None
≥2 attacks, objective clinical evidence of 1 lesion Dissemination in space demonstrated by MRI or 2nd clinical attack
1 attack, objective clinical evidence of ≥2 or more lesions Dissemination in time demonstrated by MRI or second clinical attack
1 attack, objective clinical evidence of 1 lesion (clinically isolated syndrome) Dissemination in space and time demonstrated by MRI or second attack
Insidious neurologic progression suggestive of PPMS One-year disease progression (retrospective or prospective) AND 2 or 3: DIS in the brain, spinal cord, or OCBs

Adapted with permission from Polman CH, Reingold SC, Banwell B, et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 2011;69(2):292–302.

The goals of treatment are to reduce long-term disability and treat symptoms and exacerbations as follows:

  1. Institute disease-modifying medication.

  2. Treat exacerbations.

  3. Treat symptoms that arise from chronic changes caused by multiple exacerbations as well as progressive disease.

This chapter is organized around these themes:

  • Part 1 explores the diagnosis of MS.

  • Part 2 concentrates on mimic of MS.

  • Part 3 concentrates on treatment of exacerbations.

  • Part 4 outlines disease management.

  • Part 5 address MS and reproductive issues.

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