Diagnosis

  • PPMS can be diagnosed retrospectively or prospectively in patients with one year of disability progression and two of the following:1
    • One or more T2-hyperintense lesions characteristic of MS in one or more of the following brain regions: periventricular, cortical or juxtacortical, or infratentorial
    • Two or more T2-hyperintense lesions in the spinal cord
    • Presence of CSF-specific oligoclonal bands
  • Usually, but not always, PPMS is diagnosed in people in their 40s–50s2
  • Early symptoms of PPMS are often subtle problems with walking2,3

 

Image of a person walking with a magnifying glass above representing PPMS diagnosis.
 

Classification

  • The Lublin criteria classifies progressive MS into active and non-active disease, which reflects whether inflammation and new lesion formation is ongoing4
  • Additionally, progressive MS can remain relatively stable over periods of time and can also be classified clinically according to whether there has been evidence of progression over the past year4

 

Next to the title 'Classification', image of 4 arrows indicating different types of progressive MS classification a) Active and with progression b) Active but without progression c) Not active but with progression d) Not active and without progression (stable disease)
 

Management

Treatment of PPMS includes two broad categories5

Under the title 'Management', 2 images representing the 2 categories of treatment of SPMS. a) Image of dial representing slow disease progression with DMTs. b) Image of person with a magnifying glass representing symptomatic intervention.

Slowing disease progression with DMTs6,7

  • Limited DMTs are licensed for use in progressive disease, particularly in the later stages
  • In the future, strategies may evolve to prevent the development of late-stage progressive disease

Under the title 'Management', 2 images representing the 2 categories of treatment of SPMS. a) Image of dial representing slow disease progression with DMTs. b) Image of person with a magnifying glass representing symptomatic intervention.

Symptomatic intervention6,8–10 

  • Fatigue
  • Mood changes
  • Cognitive impairment
  • Visual difficulties
  • Motor and sensory deficits
  • Bowel and bladder dysfunction
 

CSF, cerebrospinal fluid; DMT, disease-modifying therapy; MS, multiple sclerosis; PPMS, primary progressive multiple sclerosis.

References

  1. Thompson AJ, Banwell BL, Barkhof F, et al. Lancet Neurol. 2018;17:162–173.
  2. MS Society. Primary Progressive MS. Available from: https://www.mssociety.org.uk/about-ms/types-of-ms/primary-progressive-ms [Last accessed: March 2021].
  3. National MS Society. Types of MS. Available from: https://www.nationalmssociety.org/What-is-MS/Types-of-MS [Last accessed: March 2021].
  4. Lublin FD, Reingold SC, Cohen JA, et al. Neurology. 2014;83(3):278–286.
  5. Willis MA, Fox RJ. Continuum (Minneap Minn). 2016;22(3):785–798.
  6. Hart FM, Bainbridge J. Am J Manag Care. 2016;22(6 Suppl):S159–S170.
  7. Lassman H. Mult Scler. 2017;23(12):1593–1599.
  8. NICE. Multiple sclerosis in adults: management. CG186. Available from: https://www.nice.org.uk/guidance/cg186 [Last accessed: March 2021].
  9. NICE. Faecal incontinence in adults. Management. CG49. Available from: https://www.nice.org.uk/guidance/cg49/resources/faecal-incontinence-in-a... [Last accessed: March 2021].
  10. NICE. Treatments to improve bladder storage in neurological disease. Available from: https://pathways.nice.org.uk/pathways/urinary-incontinence-in-neurologic... [Last accessed: March 2021].
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