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Refining the Criteria for Diagnosing Secondary Progressive Multiple Sclerosis
Investigators established a definition that identified secondary progression 3 years earlier than treating clinicians.
The diagnosis of secondary progressive multiple sclerosis (SPMS) is challenging and often made in retrospect. Investigators used the MSBase dataset to develop an objective measure that reflected long-term disease worsening. MSBase includes clinical data from more than 34,154 patients and 113 MS centers in 34 countries. SPMS definitions were based on the following criteria: amount of Expanded Disability Status Scale (EDSS) worsening, minimum EDSS at progression, minimum pyramidal Functional System (FS) score at progression, time to confirmed progression, confirmation within the same FS that triggered the progression, relapse activity prior to progression event, and relapse activity after.
Of 17,356 patients with sufficient follow-up and complete data, SPMS was diagnosed in 2360 (14%). The best definition included:
A 1-point EDSS worsening with EDSS ≤5.5 or a 0.5-point EDSS worsening with EDSS ≥6.0, in the absence of a relapse
A minimum EDSS score of 4
Pyramidal FS score of ≥2
Confirmed worsening over ≥3 months, and
Confirmation of worsening within the same FS as worsening onset.
This definition identified 18% of the patients as converting to SPMS. Among those with ≥5 years of follow-up after SPMS diagnosis by this definition, 78% had continuing disability worsening and 70% needed to use at least a cane to walk 100 m. This definition also enabled diagnosis 3 years earlier than a clinical diagnosis of SPMS by the treating physician.
This refinement of the SPMS research definition will increase the sensitivity and speed of the diagnosis compared with current clinical practice. Most patients who met these criteria continued worsening in subsequent years. Although the criteria may not be 100% specific (86% vs. 95% for clinician diagnosis), they help define a particular high-risk population. With possible therapies for progressive MS on the horizon, making an accurate diagnosis of primary and secondary MS will be increasingly important.
Robert T. Naismith, MD reviewing Lorscheider J et al. Brain 2016 Jul 7.
Lorscheider J et al. Defining secondary progressive multiple sclerosis. Brain 2016 Jul 7; [e-pub].
[Free full-text Brain article PDF | PubMed® abstract]
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