SPMS may be defined as the progressive accumulation of disability after an initial relapsing course, with or without occasional relapses and minor remissions1

Many patients will progress along the spectrum to SPMS2
 

Diagnosis

Under the title 'Diagnosis' image of 3 circles representing patients transitioning to SPMS:a) 1 in 4 patients within 10 years post-onsetb) 1 in 2 patients within 20 years post-onsetc) 3 in 4 patients within 30 years post-onset

 

*From an analysis of 14,211 patients with MS. Amongst the 3169 patients who had at least 10 years of observation, 723 reached SPMS.3

Image of an arrow pointing upwards and a magnifying glass representing an increase in patients with RRMS transition to SPMS.
 
  • There are no consistently accepted diagnostic definitions for SPMS1
  • It is usually diagnosed retrospectively in the clinic by a history of gradual worsening after an initial course of RRMS1
  • No clinical imaging, immunological or pathological criteria can determine the transition point between RRMS and SPMS1

When transitioning to SPMS patients may notice a range of changes in disability, patients with lower relapse frequency but increasing disability could be transitioning to SPMS.4

Some common signs may indicate progression to SPMS:
 

mage with 6 icons indicating some of the common signs that may indicate progression to SPMS: 3 cognitive changes:a) Image icon of a head with arrows going around representing ‘Impaired concentration’b) Image of a speech box with question marks inside representing ‘Trouble forming words’c) Image of icon of a head with a clock next to it representing ‘Slower information processing’<br />
3 physical changes: a) Image icon of a person with Z's on top representing 'Increasing fatigue'b) Image icon of a toilet representing 'Accumulating bladder and bowel dysfunction'c) Image icon of person loosing balance representing 'Difficulty walking or balancing'5. Under the title 'Understanding progression' 2 image icons:a) Image icon of channels and cells passing through representing 'SPMS may be a result of inflammation that accumulates from the start'.b) Image icon of cells representing 'Central inflammation and neurodegeneration'.

Classification

  • The Lublin criteria classifies SPMS into active and non-active disease, which reflects whether inflammation and new lesion formation is ongoing1,8
  • Active disease may be defined clinically or on imaging1:
    • Clinical: relapses, acute or subacute episodes of new or increasing neurologic dysfunction followed by full or partial recovery, in the absence of fever or infection.
    • Imaging (MRI): occurrence of contrast-enhancing T1 hyperintense or new or unequivocally enlarging T2 hyperintense lesions.

Understanding progression

SPMS may be a result of inflammation that accumulates from the start9–12

Peripherally driven inflammation9

""

  • Occurs when circulating immune cells temporarily damage the brain
  • Leads to relapses
 

Central inflammation and neurodegeneration

""

  • The activation of CNS-resident immune cells is independent of peripheral inflammation and can worsen when immune cells become trapped after crossing the blood-brain barrier9,10
  • May lead to neurodegeneration, irreversibly damaging white and grey matter9
 

Line graph indicating the 'Two kinds of CNS inflammation affect patient with MS: Peripherally driven inflammation and Central inflammation and neurodegeneration.

SPMS with active disease is defined as the presence of relapses and/or the occurrence of T1 or new or enlarging T2 lesions.1

CNS, central nervous system; DMT, disease-modifying therapy; MRI, magnetic resonance imaging; MS, multiple sclerosis; RRMS, relapsing-remitting multiple sclerosis; SPMS, secondary progressive multiple sclerosis.

References

  1. Lublin FD, Reingold SC, Cohen JA, et al. Neurology. 2014;83(3):278–286;
  2. Larochelle C et al. Trends Neurosci. 2016;39(5):325–339.
  3. Khurana V, Medin J; Poster presented at: the 7th Joint ECTRIM-ACTRIMS Meeting, 25–28 October 2017, Paris, France;
  4. Lorscheider J, Buzzard K, Jokubaitis V, et al. Brain. 2016;139:2395-2405;
  5. Amato MP et al. J Neurol. 2013;260:1452–1468;
  6. Gross HJ, Watson C. Neuropsychiatr Dis Treat. 2017;13:1349–1357; 
  7. Achiron A et al. J Neurol Neurosurg Psychiatry. 2005;76:744–749; 
  8. Baecher-Allan C, Kaskow BJ, Weiner HL. Neuron. 2018;97(4):742–768;
  9. Dendrou CA et al. Nat Rev Immunol. 2015;15:545–558; 
  10. Kutzelnigg A et al. Brain. 2005;128(Pt 11):2705–2712;
  11. Ziemssen T et al. J Neurol. 2016;263:1053–1065;
  12. Lassman H et al. Nat Rev Neurol. 2012;8(11):647–656.
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