New Multiple Sclerosis Phenotypic Classification

Новая фенотипическая классификация рассеянного склероза
Fred Lublin
2014-01-01

clinically isolated syndromedisease activity and progressionmultiple sclerosis phenotypic classificationprogressive MSrelapsing-remitting
BACKGROUND: In 1996, the clinical course of multiple sclerosis (MS) was characterized as relapsing-remitting, primary progressive, secondary progressive or progressive relapsing. Since then, an increased understanding of MS and its pathology prompted a re-examination of these clinical phenotypes. Main recommendations of the 2013 revisions are provided herein. SUMMARY: Clinically isolated syndrome has been added, and progressive relapsing MS has been eliminated, from the clinical course descriptions. All forms of MS should be further subcategorized as either active or non-active. Active MS is defined as the occurrence of clinical relapse or the presence of new T2 or gadolinium-enhancing lesions over a specified period of time, preferably at least one year. An additional subcategory for patients with progressive MS differentiates between those who have shown signs of disability progression over a given time period and those who have remained stable. The term 'worsening' is recommended to describe patients whose disease is advancing for any reason, whereas 'disease progression' should be reserved for those with progressive disease who are truly progressing (as opposed to worsening from a relapse). The term 'benign' should be used with caution as the course of MS can worsen at any time, even after many years of apparent stability. Key Messages: Newer characterizations of MS phenotypes include a consideration of disease activity (based on the clinical relapse rate and imaging findings) and disease progression. Accurate clinical course descriptions are useful for communication, prognostication, clinical trial design and to guide everyday clinical decision-making.
1
All forms of MS should be subcategorized as either active or non-active based on clinical relapses or new T2/gadolinium-enhancing lesions over a specified period (preferably ≥1 year).
2
Clinically isolated syndrome (CIS) has been added to MS clinical course descriptions, and progressive relapsing MS has been eliminated.
3
Progressive MS should include an additional subcategory distinguishing patients with documented disability progression over a given time period from those who remain stable.
4
The term 'benign' MS should be used cautiously because the disease can worsen after many years of apparent stability.
5
The term 'worsening' is recommended for any advancing disease, while 'disease progression' should be reserved for true progression in progressive MS distinct from relapse-related worsening.

Clinical phenotypic classification of multiple sclerosis

Revised phenotype categories and subcategories focusing on disease activity (clinical relapses and MRI lesions) and disease progression/worsening, including addition/removal of specific phenotypes and terminology recommendations

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2014-01-01
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Fred Lublin
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