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Aggressive transformation of secondary progressive MS
Author(s): ,
W.K.W. Fung
Affiliations:
Neurology, Morriston Hospital, Swansea, United Kingdom
,
R. Smith
Affiliations:
Neurology, Morriston Hospital, Swansea, United Kingdom
,
G. Ingram
Affiliations:
Neurology, Morriston Hospital, Swansea, United Kingdom
O.R. Pearson
Affiliations:
Neurology, Morriston Hospital, Swansea, United Kingdom
ECTRIMS Online Library. Fung W. Oct 12, 2018; 228836
Wilson Ka Wai Fung
Wilson Ka Wai Fung
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Abstract: P994

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - Natural course

Introduction: Multiple sclerosis (MS) is a complex inflammatory disorder commonly presenting in young adults with active relapses. The majority of patients develop a more progressive phenotype in their fifth decade at which point active inflammatory relapses are rare.
Methods: Patients were identified from a regional MS centre, all of whom had a slowly progressive disease course but acutely transformed into aggressive/malignant MS. Disease characteristics, imaging, treatment and outcome were reviewed.
Results: Four patients with secondary progressive MS (3 female) of mean age 58y (range 47-72y) with a disease duration of 22.2 years (range 11 to 37y) are described. All patients had a low initial relapse rate and did not receive disease-modifying treatment (DMT) initially before developing a slowly progressive disease course. All patients developed late onset, aggressive reactivation of the inflammatory phase of the disease with an annualised relapse rate over 3.0, multiple new T2 and gad-enhancing lesions. Despite acute and rescue treatments (steroids/plasma exchange) rapid disability (EDSS > 8.0) or death occurred. The case histories and imaging are presented.
Conclusions: Fulminant demyelinating disease remains a challenge to physicians. This case series highlights a challenging group of older patients who begin their MS journey with relatively mild disease followed by a initial typical secondary progressive disease course. Unexpected acute transformation to an inflammatory phenotype occurred, with rapid disability progression over several months. The importance of early aggressive treatment of the inflammatory component of MS, in spite of age, is demonstrated.
Disclosure: Wilson KW Fung underwent a Movement Disorders Fellowship in Melbourne, Australia (2017-2018, Ipsen). Gillian Ingram received honoraria and travel expenses from Biogen, Genzyme and Merck and served on advisory board for Merck. Owen R Pearson received honoraria and travel expenses from Biogen, Bayer, Genzyme, Merck, Novartis, Roche and Teva and served on advisory boards for Biogen, Novartis, Merck and Roche.

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