Abstract
BackgroundParoxysmal (PS) and unusual symptoms (US) account for approximately 1.6% of initial manifestations of multiple sclerosis (MS) and have comparable conversion rates to clinically definite MS (CDMS) as classical bout onset symptoms (CS). However, long-term prognosis and clinical outcome of patients experiencing PS or US as first clinical manifestation are unclear.MethodsClinical, MRI and cerebrospinal fluid data were obtained retrospectively and patients presenting with PS or US were compared to patients with CS presentation.ResultsIn a cohort of 532 relapsing onset MS patients followed for a mean period of 11.4 years (SD 3.6), 10 (1.9%) patients initially presented with PS/US. PS/US patients received disease modifying treatment (DMT) in a significantly smaller proportion immediately after the first clinical symptom (30% vs. 61.7%; p = 0.021) and during the observation period (60% vs. 83.5%; p = 0.033). In multivariate models correcting for sex, age at initial symptoms, complete remission of initial symptoms, total number of T2 and contrast-enhancing lesions, presence of oligoclonal bands and DMT exposure, PS/US were not associated with lower annualized relapse rate or lower EDSS over time.ConclusionIn addition to a similar conversion rate to CDMS, patients presenting with PS/US at disease onset display very similar relapse and disability rates as patients with CS onset. Consequently, initial presentation with PS/US does not indicate benign or atypical MS, but requires DMT initiation based on the same criteria as in CS patients.
Highlights
Paroxysmal symptoms (PS) are brief symptoms occurring suddenly and many times a day
In multivariate models correcting for sex, age at initial symptoms, complete remission of initial symptoms, total number of T2 and contrast-enhancing lesions, presence of oligoclonal bands and disease modifying treatments (DMT) exposure, PS/unusual symptoms (US) were not associated with lower annualized relapse rate or lower EDSS over time
RE has participated in meetings sponsored by and received honoraria from Biogen, Merck Serono and Teva Ratiopharm, has received grants for educational purposes from Biogen, Bohringer Ingelheim, Merck Serono, Novartis Pharma GmbH, Ottobock and Teva Ratiopharm, and has received honoraria for acting as consultant for Teva Pharmaceuticals Europe
Summary
Paroxysmal symptoms (PS) are brief (lasting seconds to minutes) symptoms occurring suddenly and many times a day. They are often stereotyped and continue in clusters with great intensity for days up to a few months[1]. While their pathophysiology is well characterised, they are among the most frequently misinterpreted manifestations of multiple sclerosis (MS) (1). Paroxysmal (PS) and unusual symptoms (US) account for approximately 1.6% of initial manifestations of multiple sclerosis (MS) and have comparable conversion rates to clinically definite MS (CDMS) as classical bout onset symptoms (CS). Long-term prognosis and clinical outcome of patients experiencing PS or US as first clinical manifestation are unclear
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