. 2022 Oct 25;30(1):9–21. doi: 10.1111/ene.15593

TABLE 1.

Criteria used to define progressive multiple sclerosis phenotypes

Source	Definition
US NMSS [2]	Progressive disease (primary or secondary) Clinical: steadily increasing objectively documented neurological dysfunction/disability without unequivocal recovery (fluctuations and phases of stability may occur) Imaging (MRI): imaging measures of progression are not established or standardised and not (yet) useful as phenotype descriptors for individual patients. Under consideration are increasing number and volume of T1‐hypointense lesions, brain volume loss and changes in magnetic transfer imaging and diffusion tensor imaging
Lorscheider et al. 2016 [14]	SPMS Three‐strata progression magnitude (an increase in EDSS by 1.5 points if the last EDSS before conversion to SPMS was 0, an increase by 1 point if the EDSS was 1–5.5, or an increase by 0.5 points if the EDSS was >5.5), in the absence of a relapse, and A minimum EDSS of ≥4, and A pyramidal FS score of ≥2 confirmed over at least 3 months including within the leading functional system
2017 McDonald criteria [11]	PPMS 1 year of disability progression (retrospectively or prospectively) determined, independent of clinical relapse, plus Two of the following: ◦ 1 or more T2‐hyperintense lesions ^a characteristic of MS in one or more of the following brain regions: periventricular, cortical or juxtacortical or infratentorial ◦ 2 or more T2‐hyperintense lesions ^a in the spinal cord ◦ Presence of CSF‐specific oligoclonal bands

Source

Definition

US NMSS [2]

Progressive disease (primary or secondary)

Clinical: steadily increasing objectively documented neurological dysfunction/disability without unequivocal recovery (fluctuations and phases of stability may occur)

Imaging (MRI): imaging measures of progression are not established or standardised and not (yet) useful as phenotype descriptors for individual patients. Under consideration are increasing number and volume of T1‐hypointense lesions, brain volume loss and changes in magnetic transfer imaging and diffusion tensor imaging

Lorscheider et al. 2016 [14]

SPMS

Three‐strata progression magnitude (an increase in EDSS by 1.5 points if the last EDSS before conversion to SPMS was 0, an increase by 1 point if the EDSS was 1–5.5, or an increase by 0.5 points if the EDSS was >5.5), in the absence of a relapse, and
A minimum EDSS of ≥4, and
A pyramidal FS score of ≥2

confirmed over at least 3 months including within the leading functional system

2017 McDonald criteria [11]

PPMS

1 year of disability progression (retrospectively or prospectively) determined, independent of clinical relapse, plus
Two of the following:
- ◦
  1 or more T2‐hyperintense lesions ^a characteristic of MS in one or more of the following brain regions: periventricular, cortical or juxtacortical or infratentorial
- ◦
  2 or more T2‐hyperintense lesions ^a in the spinal cord
- ◦
  Presence of CSF‐specific oligoclonal bands

Abbreviations: CSF, cerebrospinal fluid; EDSS, Expanded Disability Status Scale; FS, functional status; IgG, immunoglobulin G; MRI, magnetic resonance imaging; MS, multiple sclerosis; PPMS, primary progressive multiple sclerosis; SPMS, secondary progressive multiple sclerosis; US NMSS, United States National Multiple Sclerosis Society.

^{^a}

No distinction is made between symptomatic and asymptomatic MRI lesions.