ECTRIMS eLearning

Rethinking the importance of paroxysmal and unusual symptoms as first clinical manifestation of multiple sclerosis: they do matter!
Author(s):
R. Ehling
,
R. Ehling
Affiliations:
G. Bsteh
,
G. Bsteh
Affiliations:
F. Di Pauli
,
F. Di Pauli
Affiliations:
H. Hegen
,
H. Hegen
Affiliations:
K. Roetzer
,
K. Roetzer
Affiliations:
M. Auer
,
M. Auer
Affiliations:
F. Deisenhammer
,
F. Deisenhammer
Affiliations:
M. Reindl
,
M. Reindl
Affiliations:
T. Berger
T. Berger
Affiliations:
ECTRIMS Learn. Bsteh G. 09/16/16; 146678; P838
Gabriel Bsteh
Gabriel Bsteh
Contributions
Abstract

Abstract: P838

Type: Poster

Abstract Category: Clinical aspects of MS - Natural course

Background: Paroxysmal (PS) and unusual symptoms (US) as initial manifestation of multiple sclerosis (MS) are rare and have been reported only anecdotally. Due to their mostly self-limiting nature they are often thought to indicate “benign” MS. However, there is a lack of data on further disease courses of MS patients initially presenting with PS or US.

Objective: To investigate the prevalence and long-term clinical disease course of patients who experienced PS or US as a first clinical manifestation of MS.

Methods: In this retrospective, observational study, clinical and cerebrospinal fluid data of MS patients with a minimum follow-up of five years were obtained in a large representative MS centre. Patients presenting with PS and US were compared to patients with classical bout onset regarding gender, age at disease onset, time to first relapse, annualized relapse rate (ARR) and disability (EDSS).

Results: In a cohort of 1,396 patients with relapsing onset MS, 15 patients (1.1%) were identified having presented with PS (trigeminal neuralgia, dysarthria and ataxia, tonic spasms, paroxysmal sensory symptoms, diplopia, akinesia) and 7 patients (0.5%) with US (epileptic seizure or status epilepticus). Despite anticonvulsive and/or corticosteroid treatment mean duration of symptoms was 77 days. At symptom onset, groups were comparable with respect to gender, age at onset and intrathecal immunoglobulin synthesis (p>0.05). During a mean follow-up period of 13.6 years, all patients initially presenting with PS or US converted to CDMS after a mean duration of 3.4 years (95% CI 1.9 - 4.8) as compared to 1,295 patients (94%) presenting with classical bout onset who converted to CDMS after a mean disease duration of 2.7 years (95% CI 3.0 - 3.4; p=0.759). However, compared to patients presenting with US or classical bout onset, patients with PS had a significantly lower ARR and EDSS less clinical disability (EDSS) 10 years after disease onset (p=0.008 and p=0.001, respectively).

Conclusion: In a large cohort of 1,396 MS patients, 1.1% of individuals presented with PS and 0.5% with US at disease onset. Irrespective of the transient nature of symptoms, patients presenting with PS and US were at the same risk of developing CDMS as classical bout onset patients. Consequently, awareness of identifying PS or US as a possible first clinical symptom of MS is critical and close clinical monitoring and consequent evaluation for early DMT initiation mandatory.

Disclosure: RE has participated in meetings sponsored by and received honoraria (lectures and consultations) from Biogen, Merck Serono and Teva Ratiopharm and has received grants for educational purposes from Biogen, Böhringer Ingelheim, Merck Serono, Novartis Pharma GmbH, Ottobock and Teva Ratiopharm. GB has received speaking honoraria from Biogen-Idec, Roche Austria, Novartis Pharma and Merck Serono. FDP has received speaking honoraria from Biogen-Idec and Sanofi-Aventis Austria. HH has received speaking honoraria from Bayer Schering, Biogen Idec, Merck Serono and Novartis. KR has nothing to declare. FD has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Bayer Healthcare, Biogen Idec, Genzyme-Sanofi, Merck, Novartis Pharma, and Teva-Ratiopharm. His institution has received financial support for participation in randomized controlled trials of INFb-1b (Betaferon, Bayer Schering Pharma), INFb-1a (Avonex, Biogen Idec; Rebif, Merck Serono), glatiramer acetate (Copaxone, Teva Pharmaceuticals), Natalizumab (Tysabri, Biogen Idec), in multiple sclerosis. He is section editor of the Multiple Sclerosis and Related Disorders Journal. MR and Innsbruck Medical University receive payments for antibody assays (AQP4 and antineuronal antibodies) and for AQP4 antibody validation experiments organized by Euroimmun. MR acts as an academic editor of PLOS ONE. TB has participated in meetings sponsored by and received honoraria (lectures, advisory boards, consultations) from pharmaceutical companies marketing treatments for MS: Almirall, Astra, Bayer, Biogen Idec, Genzyme, Merck, Novartis, Octapharma, ratiopharm, Sanofi Aventis, TEVA. His institution has received financial support in the past 12 months by unrestricted research grants (Biogen Idec, Bayer, Merck, Novartis, ratiopharm, Sanofi Aventis) and for participation in clinical trials in multiple sclerosis sponsored by Alexion, Bayer, Biogen Idec, Merck, Novartis, Octapharma, Sanofi Aventis, TEVA.

Abstract: P838

Type: Poster

Abstract Category: Clinical aspects of MS - Natural course

Background: Paroxysmal (PS) and unusual symptoms (US) as initial manifestation of multiple sclerosis (MS) are rare and have been reported only anecdotally. Due to their mostly self-limiting nature they are often thought to indicate “benign” MS. However, there is a lack of data on further disease courses of MS patients initially presenting with PS or US.

Objective: To investigate the prevalence and long-term clinical disease course of patients who experienced PS or US as a first clinical manifestation of MS.

Methods: In this retrospective, observational study, clinical and cerebrospinal fluid data of MS patients with a minimum follow-up of five years were obtained in a large representative MS centre. Patients presenting with PS and US were compared to patients with classical bout onset regarding gender, age at disease onset, time to first relapse, annualized relapse rate (ARR) and disability (EDSS).

Results: In a cohort of 1,396 patients with relapsing onset MS, 15 patients (1.1%) were identified having presented with PS (trigeminal neuralgia, dysarthria and ataxia, tonic spasms, paroxysmal sensory symptoms, diplopia, akinesia) and 7 patients (0.5%) with US (epileptic seizure or status epilepticus). Despite anticonvulsive and/or corticosteroid treatment mean duration of symptoms was 77 days. At symptom onset, groups were comparable with respect to gender, age at onset and intrathecal immunoglobulin synthesis (p>0.05). During a mean follow-up period of 13.6 years, all patients initially presenting with PS or US converted to CDMS after a mean duration of 3.4 years (95% CI 1.9 - 4.8) as compared to 1,295 patients (94%) presenting with classical bout onset who converted to CDMS after a mean disease duration of 2.7 years (95% CI 3.0 - 3.4; p=0.759). However, compared to patients presenting with US or classical bout onset, patients with PS had a significantly lower ARR and EDSS less clinical disability (EDSS) 10 years after disease onset (p=0.008 and p=0.001, respectively).

Conclusion: In a large cohort of 1,396 MS patients, 1.1% of individuals presented with PS and 0.5% with US at disease onset. Irrespective of the transient nature of symptoms, patients presenting with PS and US were at the same risk of developing CDMS as classical bout onset patients. Consequently, awareness of identifying PS or US as a possible first clinical symptom of MS is critical and close clinical monitoring and consequent evaluation for early DMT initiation mandatory.

Disclosure: RE has participated in meetings sponsored by and received honoraria (lectures and consultations) from Biogen, Merck Serono and Teva Ratiopharm and has received grants for educational purposes from Biogen, Böhringer Ingelheim, Merck Serono, Novartis Pharma GmbH, Ottobock and Teva Ratiopharm. GB has received speaking honoraria from Biogen-Idec, Roche Austria, Novartis Pharma and Merck Serono. FDP has received speaking honoraria from Biogen-Idec and Sanofi-Aventis Austria. HH has received speaking honoraria from Bayer Schering, Biogen Idec, Merck Serono and Novartis. KR has nothing to declare. FD has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Bayer Healthcare, Biogen Idec, Genzyme-Sanofi, Merck, Novartis Pharma, and Teva-Ratiopharm. His institution has received financial support for participation in randomized controlled trials of INFb-1b (Betaferon, Bayer Schering Pharma), INFb-1a (Avonex, Biogen Idec; Rebif, Merck Serono), glatiramer acetate (Copaxone, Teva Pharmaceuticals), Natalizumab (Tysabri, Biogen Idec), in multiple sclerosis. He is section editor of the Multiple Sclerosis and Related Disorders Journal. MR and Innsbruck Medical University receive payments for antibody assays (AQP4 and antineuronal antibodies) and for AQP4 antibody validation experiments organized by Euroimmun. MR acts as an academic editor of PLOS ONE. TB has participated in meetings sponsored by and received honoraria (lectures, advisory boards, consultations) from pharmaceutical companies marketing treatments for MS: Almirall, Astra, Bayer, Biogen Idec, Genzyme, Merck, Novartis, Octapharma, ratiopharm, Sanofi Aventis, TEVA. His institution has received financial support in the past 12 months by unrestricted research grants (Biogen Idec, Bayer, Merck, Novartis, ratiopharm, Sanofi Aventis) and for participation in clinical trials in multiple sclerosis sponsored by Alexion, Bayer, Biogen Idec, Merck, Novartis, Octapharma, Sanofi Aventis, TEVA.

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