Fast Facts: Multiple Sclerosis, Fifth Edition

166 Pages·2021·10.913 MB·English

by  Hughes, Stella E. and Macaron, Gabrielle

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Multiple Sclerosis
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Multiple Sclerosis p
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9 Epidemiology and genetics le
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18 Pathology i
s Stella E Hughes and Gabrielle Macaron
28 The clinical picture
63 Treatment of relapses and symptoms
85 Disease-modifying treatment
120 Emerging therapies
128 Special MS populations
145 Lifestyle considerations and the multidisciplinary team
154 Advanced MS
ISBN 978-3-318-06797-2
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© S. Karger Publishers Ltd 2021
5
FAST FACTS
FIFTH
EDITION
Multiple Sclerosis
Stella E Hughes MB BCh BAO MD FRCP
Consultant Neurologist
Department of Neurology
Royal Victoria Hospital
Belfast Health and Social Care Trust
Belfast, UK
Gabrielle Macaron MD
Neurologist
Neurology Department, Hotel-Dieu de France Hospital
Université Saint Joseph de Beyrouth
Beirut, Lebanon
Adjunct staff, Mellen Center for Multiple Sclerosis
Cleveland Clinic, Cleveland, Ohio, USA
Declaration of Independence
This book is as balanced and practical as we can make it.
Ideas for improvement are always welcome: [email protected]
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Fast Facts: Multiple Sclerosis
First published 2000; second edition 2006; third edition 2014; fourth edition 2016
Fifth edition 2021
Text © 2021 Stella E Hughes, Gabrielle Macaron
© 2021 in this edition S. Karger Publishers Ltd
S. Karger Publishers Ltd, Elizabeth House, Queen Street,
Abingdon, Oxford OX14 3LN, UK
Tel: [phone]
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Fast Facts is a trademark of S. Karger Publishers Ltd.
All rights reserved. No part of this publication may be reproduced,
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otherwise, without the express permission of the publisher.
The rights of Stella E Hughes and Gabrielle Macaron to be identified as
the authors of this work have been asserted in accordance with the
Copyright, Designs & Patents Act 1988 Sections 77 and 78.
The publisher and the authors have made every effort to ensure
the accuracy of this book, but cannot accept responsibility for any
errors or omissions.
For all drugs, please consult the product labeling approved in your
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Registered names, trademarks, etc. used in this book, even when not marked
as such, are not to be considered unprotected by law.
A CIP record for this title is available from the British Library.
ISBN 978-3-318-06797-2
Hughes SE (Stella)
Fast Facts: Multiple Sclerosis/
Stella E Hughes, Gabrielle Macaron
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List of abbreviations 5
Introduction 7
Epidemiology and genetics 9
Pathology 18
The clinical picture 28
Treatment of relapses and symptoms 63
Disease-modifying treatment 85
Emerging therapies 120
Special MS populations 128
Lifestyle considerations and the multidisciplinary team 145
Advanced MS 154
Useful resources 160
Index 161
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List of abbreviations
9-HPT: 9-hole peg test FDA: (US) Food and Drug
Administration
AAN: American Academy of
Neurology FLAIR: fluid-attenuated inversion
recovery
ACTH: adrenocorticotropic hormone
HLA: human leukocyte antigen
ADEM: acute disseminated
encephalomyelitis IFN-β: interferon-beta
ADL: activities of daily living IgG: immunoglobulin G
AHSCT: autologous hematopoietic IL: interleukin
stem cell transplantation
INO: internuclear ophthalmoplegia
AQP-4: aquaporin 4
IPMSSG: International Pediatric
bd: twice daily Multiple Sclerosis Study Group
CIS: clinically isolated syndrome i.v.: intravenous
CNS: central nervous system JC: John Cunningham (virus)
CSF: cerebrospinal fluid MHC: major histocompatibility
complex
CT: computed tomography
MOG: myelin oligodendrocyte
DHODH: dihydroorotate
glycoprotein
dehydrogenase
MRI: magnetic resonance imaging
DIS: dissemination in space
MS: multiple sclerosis
DIT: dissemination in time
MSFC: Multiple Sclerosis Functional
DMT: disease-modifying therapy
Composite
EAN: European Academy of
MSN: multiple sclerosis specialist
Neurology
nurse
EBV: Epstein–Barr virus
NEDA: no evidence of disease activity
ECTRIMS: European Committee of
NMO: neuromyelitis optica
Treatment and Research in Multiple
Sclerosis NMOSD: neuromyelitis optica
spectrum disorder
EDSS: Expanded Disability Status
Scale Nrf2: nuclear factor (erythroid-
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Fast Facts: Multiple Sclerosis
PEG: percutaneous enteral SDMT: Symbol Digit Modalities Test
gastrostomy
SLE: systemic lupus erythematosus
PLEX: plasma exchange
SNRI: serotonin–norepinephrine
PML: progressive multifocal reuptake inhibitor
leukoencephalopathy
SPMS: secondary progressive multiple
POMS: pediatric-onset multiple sclerosis
sclerosis
STIR: short time inversion recovery
PPMS: primary progressive multiple
T25FW: timed 25-foot walk
sclerosis
TKI: tyrosine kinase inhibitor
PRMS: progressive relapsing multiple
sclerosis TNF: tumor necrosis factor
RCT: randomized controlled trial UTI: urinary tract infection
RIS: radiologically isolated syndrome UV: ultraviolet (light)
RRMS: relapsing remitting multiple VCAM1: vascular cell adhesion
sclerosis molecule-1
S1P: sphingosine 1-phosphate WHO: World Health Organization
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Introduction
Multiple sclerosis (MS) is a leading cause of disability in young adults,
and it has a significant effect on patients’ quality of life, family plans
and careers. By causing varying degrees of physical and cognitive
disability, MS carries a considerable individual and societal economic
burden.
Fortunately, we are entering a new era in the management of MS.
Several recent studies support initiating treatment early in the disease
course with higher-efficacy disease-modifying therapies in patients with
relapsing-remitting MS. This approach has been associated with better
long-term outcomes, specifically a lower risk of conversion to secondary
progressive MS. Moreover, new molecules with the potential to induce
myelin repair or halt the neurodegenerative process are in the pipeline
for the treatment of progressive MS. Coupled with a recent update to the
diagnostic criteria, clinicians can now diagnose and treat patients in the
earliest phases of their disease.
In this new edition of Fast Facts: Multiple Sclerosis, we present the
latest evidence on disease pathogenesis and all clinical aspects of the
condition, as well as the latest on disease-modifying therapies and
other potential treatments.
Given the need for multidisciplinary management of MS, we have
written this resource for the benefit of all health professionals involved
in the care of patients with this complex disease.
Acknowledgments. The authors wish to thank the authors of the third
and fourth editions of this resource, Drs Michael Barnett, Omar Malik,
Ann Donnelly, Mary Rensel and Orla Gray, for the strong foundation
on which this new edition is based.
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1 Epidemiology and genetics
Multiple sclerosis (MS) is a neurological condition resulting from
inflammation and degeneration within the central nervous system
(CNS). This inflammation can affect different sites at different times,
producing a variety of symptoms and signs. Periods of relapse and
remission occur in the early stages of the disease, and in most patients
a slowly progressive course ensues within one to two decades of
disease onset. The cause of MS is unknown, but dysregulation of the
immune system is central to the pathogenesis of the disease.
Epidemiology
MS is the leading cause of neurological disability in the young and
middle-aged populations of the developed world. Survey figures from
2020 suggest that it affects 2.8 million people worldwide.1 Given the
lack of complete surveillance data in some countries, this is likely to
be an underestimate.
Prevalence. The worldwide prevalence of MS appears to be increasing.
This is related to many factors, including improved counting methods,
better diagnosis, global population growth, people with MS living
longer (through improved treatment and support) and a true increase
in disease incidence, especially in females.
The number of people with MS in a given population at any one
time is usually expressed as cases per 100 000 population. Prevalence
varies worldwide, but MS is most prevalent in northern European
populations, especially in individuals of Nordic descent, and is notably
more prevalent in temperate than equatorial regions. While, globally,
the median estimated prevalence of MS is 36 per 100 000,1 in some
countries, prevalence exceeds 300 per 100 000.
Regionally, the median estimated prevalence of MS is greatest in
Europe and the Americas (133 and 112 per 100 000, respectively).1
However, prevalence varies significantly both within regions and PM
within countries. For© e xSa. mKaprglee,r iPnu bE liushroerpse L, tSda 2n0 2M1arino and Germany 9 Downloaded by: Consortium Luxembourg185.106.24.55 - 1/19/2023 1:57:36