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Public Policy in ALS/MND Care: An
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Robert H. Blank
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Public Policy
in ALS/MND Care
An International Perspective
Edited by
Robert H. Blank · Jerome E. Kurent · David Oliver
Public Policy in ALS/MND Care
Robert H. Blank
Jerome E. Kurent • David Oliver
Editors
Public Policy in
ALS/MND Care
An International Perspective
Editors
Robert H. Blank Jerome E. Kurent
University of Canterbury, Medical University of South Carolina
Christchurch, New Zealand Charleston, SC, USA
David Oliver
University of Kent
Canterbury, UK
ISBN 978-981-15-5839-9 ISBN 978-981-15-5840-5 (eBook)

https://doi.org/10.1007/978-981-15-5840-5
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Foreword
When I was a neuromuscular fellow back in 1980, I would observe how

my mentors, Drs. Walter G. Bradley and Theodore L. Munsat, managed
their ALS Clinic and early clinical trials. Because I was concentrating on
basic research, I did not know the factors involved in running clinical pro-
grams. I have to admit I did not pay much attention to administration, as
these issues were neither clinical nor scientific. A few years later when I
wanted to start a multidisciplinary ALS Clinic, I struggled to learn how to
do it successfully. This knowledge was clearly over my head.
When I wanted to conduct my first clinical trial with thyrotropin-
releasing hormone (TRH), I would not have been able to accomplish this
task without the support and assistance of my chairman Dr. John
P. Conomy and his capable administrative assistant. I learned and started
this process from scratch. In those days, public policies and the overall
infrastructure for ALS was still in the early stages of development. I have
to say that since then we have come a long way! We have many multidisci-
plinary ALS Clinics throughout many parts of the world. However, there
are still some areas that lack these clinics. I often use the word, “ALS
Community,” which I believe includes people with ALS (PALS) and their
family caregivers; doctors, clinicians, any scientists who are involved in the
field; hospice and home care agencies; voluntary disease organizations and
advocacy groups; investigator-initiated study groups; clinical trial consor-
tia; the National ALS Registry; ALS-specific annual symposium organiz-
ers; funding agencies for research and patient care; federal health agencies;
policy makers; regulatory agencies; pharmaceutical companies; and new
v
vi FOREWORD
biotech companies. All these stakeholders are working to improve ALS

care and research to help combat this “intractable” disease.
In 2003, I was asked to assemble a new committee to promote excel-
lence in end-of-life care among PALS by the Robert Wood Johnson
Foundation and ALS Association. We published the committee’s recom-
mendations in 2005 and pointed to the need for improvement in public
policies, more overall research in ALS, and the dissemination of key infor-
mation (1). In 2011, I organized an international conference to promote
clinical and patient-oriented research to identify the pathogenesis of ALS
with the support of many funding agencies and pharmaceutical companies
(ALS Community). Prof. Martin Turner and I published a conference
supplement in 2013 in the journal Amyotrophic Lateral Sclerosis and
Frontotemporal Degeneration. There were two articles that were devoted
to discussing infrastructure and resources. Dr. Alexander V. Sherman and
his colleagues reviewed public policies for funding agencies and discussed
overall infrastructure resources for clinical research in ALS (2). Key infra-
structure included ALS Clinics, brain banks, the National ALS Registry,
and study consortia in the United States and Europe. Dr. David A. Chad
also reviewed “Funding agencies and disease organizations: resources and
recommendations to facilitate ALS clinical research” (3). Our efforts are
still largely fragmented, and at times, I wonder if we may need to repeat
our messages over and over again. It has now been ten years since the
international conference. I must admit that management and care for
PALS is much more complicated due to a multitude of health insurance
companies, health care agencies, and palliative care and hospice providers.
Movements such as “right to try” and “right to die” also require a great
deal of knowledge. The number of ALS clinics has really grown in the
United States, Canada, Europe, and Pan-Asian countries. These clinics
have become more comprehensive, as they provide the best care for
patients and conduct extensive clinical research including clinical trials.
Our ultimate goal is to find the cause and pathogenesis of ALS to develop
a cure. In the meantime, we must develop medications to markedly slow
down the disease. We also need to develop interventions to improve diffi-
cult symptoms in ALS, so PALS can maintain their quality of life for as long
as possible. Again, the ALS Community as a whole is working toward these
objectives. As I experienced many years ago, young energetic doctors and
researchers are immediately confronted with a lack of knowledge on public
policies and overall complex infrastructure even within the small world of
ALS. I must admit knowledge of public policies in ALS health care has
FOREWORD vii
grown into a far more complicated web with an overwhelming amount of

information. Some countries are more efficient and have great infrastruc-
ture, whereas other countries may not be as developed. Furthermore, effec-
tive implementation of this information is not easy. Some health agency
workers or officials, government or non- government, may attempt to
improve their policies and infrastructure for the benefits of PALS. Yet, if
there are no data available, any improvement will be challenging. There are
many ALS books on science and clinical care which have been published in
the past, but there is a paucity of information on public policies and beyond,
as well as the infrastructure for ALS care and research.
When I heard the plan for this book and its focus on public policies and
infrastructure for the ALS Community (versus science or patient care), I
was so excited, because I feel it is truly needed. The editors of this book
include Dr. Robert Blank, a world expert on public policies and health
care delivery systems; Dr. Jerome Kurent, a doctor who is most respected
for compassion and humanistic care for PALS and their caregivers; and Dr.
David Oliver, a doctor who introduced the concept of palliative care for
PALS and successfully implemented it. The authors are among the world
experts of ALS across 21 countries and provide commentary on their own
countries’ health policies and infrastructure for ALS. The title of the book
is Public Policies in ALS/MND Care. Yet, I believe this book also covers
broader issues that we need to know that go beyond research when treat-
ing PALS from diagnosis to end-of-life care.
One of the most important goals of this book is to provide the most
comprehensive view possible of ALS/MND care and policy through com-
mentary on different systems across 21 countries and beyond. When we face
the impossible feat of finding the solution for ALS, we need to take a
moment and critically evaluate the field. This book will give us the opportu-
nity to really understand where the field stands and how we can move for-
ward. The impact of this book will be wide-ranging, as it provides the most
thorough and broad view of ALS/MND care and policy—not just locally
but around the world. This is indeed the first book of its kind. I would like
to provide my heartfelt congratulations to the editors and authors, who pro-
vided this crucial information and have addressed gaps in knowledge within
the field. This is an essential book to read for any stakeholder in ALS.
Wesley J Howe Professor of Neurology (at CUIMC) Hiroshi Mitsumoto

Columbia University Irving Medical Center
New York, NY
March, 2020
viii FOREWORD
References
Mitsumoto, H., M. Bromberg, W. Johnston, R. Tandan et al. (2005). Promoting
excellence in end-of-life care in ALS. Amyotrophic Lateral Sclerosis and
Frontotemporal Degeneration 6 (3): 145–54.
Sherman, A.V., A.K. Gubitz, A. Al-Chalabi, R. Bedlack et al. (2013). Infrastructure
resources for clinical research in amyotrophic lateral sclerosis. Amyotrophic
Lateral Sclerosis and Frontotemporal Degeneration 14 (Suppl 1): 53–61.
Chad, D.A., S. Bidichandani, L. Bruijn, J.D. Capra et al. (2013). Funding agencies
and disease organizations: Resources and recommendations to facilitate ALS
clinical research. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
14 (Suppl 1): 62–66.
Preface
This book was first proposed by the three editors to be a co-authored

volume on ALS/MND public policy in the United Kingdom and the
United States. Thanks to an astute reviewer who suggested involving addi-
tional countries, we decided to develop an edited multi-authored volume
which includes a broad range of contributors from across the globe. We
were soon encouraged by the enthusiasm of the many ALS/MND experts
willing to participate in this project, including representation from coun-
tries often overlooked in comparative studies. Authors from twenty-one
countries were requested to describe the current state of ALS/MND pub-
lic policy as well as implications for patient care.
This book focuses on the public policy and ethical dimensions of ALS/
MND care. Policy issues include adequacy of funding for patient care and
research, payment policy and regulatory functions of public and private
insurers, long-term services and caregiver support, access to genetic test-
ing and assistive technologies, and ensuring a competent and adequate
workforce, especially for hands-on caregivers. There also are numerous
challenges related to providing palliative and hospice care for patients with
ALS/MND, as well as use of advance directives and the highly controver-
sial area of assisted suicide. These are matters that confront policy makers
in almost all geographic regions and were addressed whenever possible by
each author.
To provide a starting point for coverage and to facilitate evaluation of
comparative data, chapter contributors were asked to address a series of
questions and issues (these are enumerated in Chapter 1) that embody a
wide range of policy issues. To avoid repetition while maintaining the
ix
x PREFACE
primary focus on policy, authors were requested not to include general

background coverage of ALS/MND since this is provided in the introduc-
tory chapter along with policy themes of the book. A concluding chapter
written by the editors examines the collective findings from the disparate
countries represented in this book. Hopefully, this discussion will serve to
facilitate workable and humane ALS/MND public policy for patient care
and caregiver support in the coming decades. Overall, we argue
for approaches that are both multidisciplinary and international.
We are indebted to the more than fifty authors who provided their
expertise and insights of ALS/MND in producing the chapters that made
this book a reality. We especially thank Professor Hiroshi Mitsumoto of
Columbia Presbyterian Hospital for his invaluable assistance on this proj-
ect and for writing the Foreword. We also thank Mr. Anushangi Weerakoon
at Palgrave Macmillan Publishing for his enthusiastic support of the book’s
concept and for his encouragement over the duration of this project.
We dedicate this book to patients with ALS/MND and their families
across the globe, and to provide hope for the future of eventually conquer-
ing this group of tragic disorders.
Sarasota, FL Robert H. Blank

Charleston, SC Jerome E. Kurent
Canterbury, UK David Oliver
Contents
1 Introduction to Public Policy of ALS/MND 1

Jerome E. Kurent, David Oliver, and Robert H. Blank
2 Public Policy in MND Care: The Australian Perspective 29

Samar Aoun, Carol Birks, Anne Hogden,
and Susan Mathers
3 Public Policy in ALD/MND Care:

The Belgian Perspective 51
Evy Reviers, Ludo Vanopdenbosch, Ludo Van Den Bosch,
and Philip Van Damme
4 Amyotrophic Lateral Sclerosis in Brazil 65

Tauana Bernardes Leoni and Marcondes C. França Jr.
5 Public Policy of ALS in Canada 77

Wendy S. Johnston and Westerly Luth
6 Public Policy for Amyotrophic Lateral Sclerosis

in China 97
Lu Chen and Dongsheng Fan
xi
xii CONTENTS
7 German Perspective on ALS/MND Policy107

Maike F. Dohrn and Roman Rolke
8 Living and Dying with ALS/MND in India:

Public Policy and Private Realities121
Roop Gursahani and Satish Khadilkar
9 The Experience of Amyotrophic Lateral Sclerosis

in Ireland131
Orla Hardiman, Stela Lefter, and Miriam Galvin
10 Public Policy of ALS Care in Israel149

Marc Gotkine and Efrat Carmi
11 Public Policy in ALS Care: The Italian Situation163

Simone Veronese and Andrea Calvo
12 ALS Policy: A Japanese Perspective177

Mieko Ogino and Tamerlan Babayev
13 ALS Policy in Mexico191

Ildefonso Rodriguez-Leyva
14 Public Policy in MND Care: Nigerian Perspective201

Yomi Ogun and Babawale Arabambi
15 Public Policy of ALS: A Pakistani Perspective209

Asfandyar Khan Niazi, Aqdas Kazi, and Arsalan Ahmad
16 Public Policy in ALS Care: The Polish Perspective219

Anna Adamczyk and Wojciech Leppert
CONTENTS xiii
17 ALS Policy: A Russian Perspective227

L. V. Brylev, D. V. Nevzorova, M. N. Zakharova,
and A. B. Guekht
18 Public Policy in ALS/MND Care: South African

Perspective233
Jeannine M. Heckmann and Alexandra S. Amaler
19 Public Policy in ALS Care: South Korea247

Seung Hyun Kim, Kiwook Oh, and Bugyeong Son
20 Amyotrophic Lateral Sclerosis Care in Tunisia263

Imen Kacem, Ikram Sghaier, Amina Nasri,
and Riadh Gouider
21 Public Policy in MND Care: The United Kingdom279

David Oliver and Christopher McDermott
22 ALS Public Policy in the United States301

Benjamin Rix Brooks and Jerome E. Kurent
23 Conclusions: What We Can Learn from the Country

Perspectives327
Robert H. Blank, Jerome E. Kurent, and David Oliver
Notes on Contributors
Anna Adamczyk is a specialist in neurology and in palliative medicine.

She is Head of the Department of Palliative Medicine at the University
Hospital No. 1 in Clinical Hospital in Bydgoszcz and a lecturer at the
Department of Palliative Care at the Ludwik Rydygier Collegium
Medicum in Bydgoszcz of the Nicolaus Copernicus University in Toruń.
She works within palliative medicine, and practices as a neurologist, with
an interest in caring for patients with diseases of the nervous system, par-
ticularly amyotrophic lateral sclerosis, and in the treatment of neuro-
pathic pain.
Arsalan Ahmad MBBS, MD (Neurology), is Professor of Neurology at
Shifa International Hospital, Shifa Tameer-e-Millat University, Islamabad,
Pakistan. He completed his MBBS from Dow Medical College in 1990
and Doctor of Medicine in Neurology in 2000. He joined Shifa
International Hospital in 2003 and was Head of Neurology from 2005 to
2009 and 2012. He was President of Pakistan Society of Neurology from
2016–2018 and President of Movement Disorders Society Pakistan from
2018–2020. He has an interest in movement disorders, neuromuscular
disorders and neurogenetics. He has over 100 publications and abstracts
including two articles on familial ALS in The Neurogenetics Journal.
Alexandra S. Amaler is a final year postgraduate law student at the
University of Cape Town, who completed her moot on the question of
assisted dying with respect to ALS.
xv
xvi NOTES ON CONTRIBUTORS
Samar Aoun is Professor of Palliative Care Research. She adopts a public

health approach and a focus on under-served population groups such as
people with MND and advocates for person-centred health and social
care. Her research programs on supporting family caregivers at end
of life and the Public Health Model of Bereavement Support have
informed policy and practice at the national and international levels.
She chairs the MND Association in Western Australia, is a director on
the board of MND Australia and a member of the scientific committee of
MND Research Institute Australia.
Babawale Arabambi is a neurologist and physician at the Lagos State
University Teaching Hospital, Nigeria, with a keen interest in stroke neu-
rology, movement disorders, and clinical neurophysiology. He also co-
manages the hospital’s neurophysiology laboratory, where he performs
and interprets the relevant procedures. He is a recipient of the International
Scholarship Award of the American Academy of Neurology for his
work on non-motor symptoms of Parkinson’s disease in Nigerian
outpatients in 2019. He has also authored/co-authored several publica-
tions touching on various fields in neurology.
Tamerlan Babayev graduated from Imperial College London in 2011
before undertaking postgraduate clinical training in the UK. He moved to
Japan in April 2017 to take up an assistant professorship in International
University of Health and Welfare and pursue a passion for medical
education and research. His contribution to this book stems from an
interest in medical ethics and the interplay between culture and health-
care policy.
Carol Birks has worked with people living with MND for over 20 years.
She worked initially as manager of support services at MND NSW before
becoming CEO of MND Australia in 2006. Her work with MND
Australia encompasses influencing government policy to address the
complex care needs of people with MND. Birks was a board member
of the International Alliance of ALS/MND Associations from 2010
to 2017, serving as Treasurer and President. Birks has supported new
and emerging ALS/MND associations globally, particularly in the
Asia Pacific region. Birks is a registered nurse with a graduate diploma in
palliative care.
Robert H. Blank PhD, has been Professor of Public Policy at universities
in New Zealand (Canterbury), Britain (Brunel) and the U.S. (Idaho and
NOTES ON CONTRIBUTORS xvii
Northern Illinois) and a frequent guest professor at Aarhus University

in Denmark and National Taiwan University in Taiwan. Among the
over forty books he published on comparative health policy, genetic
and reproductive policy, and neuroscience policy are Rationing
Medicine (Columbia University Press), Brain Policy (Georgetown
University Press), Intervention in the Brain (MIT Press), Comparative
Health Policy, 1st–5th eds. (Palgrave) and End of Life Decision Making: A
Comparative Study (MIT Press).
Benjamin Rix Brooks MD, is Medical Director of the Carolinas
Neuromuscular/ALS–MDA-Care-Center and the Atrium Health ALS
Association Certified-Treatment-Center-of-Excellence (CTCE) at the
Atrium Health Neurosciences Institute that received the first ever Joint
Commission awarded Disease-Specific Care Certification in ALS, and
Professor, Department of Neurology, University of North Carolina School
of Medicine, Charlotte Campus. Brooks is a founding member of the
World Federation of Neurology Research Group on Motor Neuron
Diseases leading efforts to design the El Escorial criteria for diagno-
sis of ALS and the ALS-Functional-Rating-Scale-Revised
(ALSFRS-R). In 2012, he received the Forbes Norris Award from The
International Alliance of ALS/MND Associations.
L. V. Brylev graduated from the Medical Faculty of Lomonosov Moscow
State University in 2004 and completed residency in the Research Center
of Neurology RAMS in 2006. He is head of the Department of
Neurology at the Buyanov City Hospital, and the Medical Director
of “Live now”, a charity foundation for people with ALS. He is team
leader of ALS Center Moscow, part of the European Network to Cure
ALS Network, and co-author of Russian Clinical Guidelines on ALS.
Andrea Calvo is Associate Professor of Neurology at the Department of
Neurosciences ‘Rita Levi Montalcini’ of the University of Turin. He
has been vice-director at Turin ALS Expert Centre since 1997. He is
the Co-Chair of the EAN Scientific Panel for ALS and FTD from
2019 and Chair of the Italian Motoneuron Diseases Study Group of
the Italian Society of Neurology (SIN). His research interests are in
the neurological field, particularly related to ALS and other neuro-
muscular diseases—epidemiology, clinical genetics, neuroimaging,
palliative care, and clinical trials—with 170 papers in international journals.
xviii NOTES ON CONTRIBUTORS
Efrat Carmi is the CEO of IsrALS—The Israeli ALS Research

Association--and has been with IsrALS since 2007. IsrALS is the primary
organization supporting ALS research and patient care in the country.
Efrat got her Psychology degree from Tel Aviv University and her MBA
from the University of Central Florida. Efrat is an active member in the
ALS/MND International Alliance and has served as a board member of
the Alliance for six years.
Lu Chen MD, is a physician in the Department of Neurology, Peking
University Third Hospital. Chen got her medical degree from Peking
University Health Science Center in 2014. She is focusing on the study
of ALS and has published thirty-six peer-reviewed articles. Her repre-
sentative articles include studies about the natural history and clinical
features of patients with ALS in China (JNNP 2015), the prevalence
and incidence of ALS in China (JNNP 2020), and the difference in
clinical features between German and Chinese ALS patients (J
Neurol 2019).
Maike F. Dohrn is a specialist for neuromuscular disorders with a scien-
tific focus on neurogenetics. She collaborated in the neuromuscular out-
patient clinic of the RWTH Aachen University hospital as a student before
she undertook her resident clinical training in 2015. She has con-
ducted several studies on Charcot-Marie-Tooth disease, hereditary
transthyretin amyloidosis, and small fiber neuropathies. Dohrn
worked as an intern at the Center for Genomics and Transcriptomics
(CeGaT) in Tübingen, Germany, and at the University of Porto,
Portugal. Currently, she has a research fellowship at the John
P. Hussman Institute for Human Genomics at the University of Miami,
Florida.
Dongsheng Fan MD, Professor and Director of the Neurological
Department at PUTH, has focused on the study with the aim of curing
ALS. Fan and his colleagues have made great efforts for Chinese ALS
patients, including the establishment of the biggest follow-up database in
China. Their current works include clinical trial studies, palliative
and hospice care, and genetic investigations to find new genes that
cause ALS in families. The Neurological Department of PUTH has
therefore become a nationally recognized center for research and clinical
care in ALS in China.
NOTES ON CONTRIBUTORS xix
Marcondes C. França Jr. MD, PhD, is Associate Professor in the

Department of Neurology at the School of Medical Sciences at the
University of Campinas (UNICAMP), Campinas, Brazil. He is the direc-
tor of the ALS outpatient clinic at UNICAMP hospital. His research
group has been working in the search for neuroimaging biomarkers and in
the characterization of the genetic epidemiology of ALS in Brazil. França
is the former chair of the Neuromuscular section at the Brazilian Academy
of Neurology. He serves on the board of the Brazilian Association of
Amyotrophic Lateral Sclerosis (ABrELA).
Miriam Galvin is Associate Professor in Intersectional Research
Methodology at the Academic Unit of Neurology, School of Medicine,
TCD and Atlantic Senior Fellow for Equity in Brain Health, Global Brain
Health Institute. She works across a range of health research projects,
mixed methods and qualitative research tracking patient and caregiver
journeys, interaction with the health services and the conceptualisation
and assessment of quality of life and caregiving experiences. She has
a multi-disciplinary academic background in human geography, pop-
ulation health and psychosocial studies, with interests in research method-
ologies, medical anthropology, and discourse.
Marc Gotkine received his medical degree from King’s College, London.
After moving to Israel, he completed his residency in Hadassah University
Hospital, Jerusalem. He set up the Hadassah ALS clinic and he also
heads the neuromuscular service and EMG lab. The Hadassah ALS
clinic serves as an ALS research hub for the whole country both in
basic science and clinical trials. He has a special interest in studying
the genetics of ALS in Israel, with a focus on ALS in consanguineous
families. His other research interests are the genetics and epidemiology of
ALS, ALS biomarkers and the microbiome in ALS.
Riadh Gouider is the head of Department of Neurology at Razi
University Hospital, the Clinical Investigation Centre (CIC), the
Alzheimer’s center and the Multiple Sclerosis Center. He is Trustee of the
World Federation of Neurology and Member in the ALS research project
and related syndromes in tropical zone (TROPALS). He is a fellow of the
European Academy of Neurology as well as the American Academy of
Neurology since 2019. He authored and co-authored many original
papers or book chapters such as Epidemiology of ALS and Clinical Features
of ALS in Africa.
xx NOTES ON CONTRIBUTORS
A. B. Guekht is the director of the Moscow Research and Clinical Center

for Neuropsychiatry and Professor of Neurology at the Russian National
Research Medical University. She is the author of more than 200 publica-
tions, including more than 90 papers in international peer-reviewed jour-
nals and chapters in international textbooks. She is the Elected Trustee of
the World Federation of Neurology, Secretary-General of the National
Society of Neurologists and involved in collaborative projects with the
World Health Organisation.
Roop Gursahani is a practicing clinical neurologist at P.D. Hinduja
National Hospital in Mumbai. He finished formal training and certifica-
tion in Internal Medicine and then in Neurology by 1988. His special
interests include epilepsy and palliative neurology. His advocacy efforts
concern end-of-life care in India and include working for appropriate leg-
islation, professional training, and public awareness. He is a member of
the steering committee of ELICIT, the interdisciplinary task force of
intensivists, palliative care physicians and neurologists for End of Life Care
in India.
Orla Hardiman is Professor of Neurology at Trinity College Dublin and
Consultant Neurologist at Beaumont Hospital, where she is Director of
the National Amyotrophic Lateral Sclerosis service. She is also the National
Clinical Lead in Neurology. She is one of only five active clinicians elected
to the Royal Irish Academy. She is Editor-in-Chief of the journal
Amyotrophic Lateral Sclerosis and the Frontotemporal Degenerations. Her
research interests are in clinical phenotyping, epidemiology, genomics,
biomarker development and new therapeutics in ALS and related disorders.
Jeannine M. Heckmann MBChB, PhD, is a neurologist in the Division
of Neurology at Groote Schuur Hospital and the University of Cape
Town, South Africa. Her clinical expertise is in neuromuscular diseases.
She established and heads the multi-disciplinary ALS clinic at Groote
Schuur Hospital, as well as an ALS research group, which collaborates
with international partners.
Anne Hogden is a Senior Lecturer and researcher with the Australian
Institute of Health Service Management. Her research interests are in
healthcare communication, patient-centred care and decision-making and
interprofessional team processes, with an emphasis on people with life-
limiting conditions. Anne’s research includes developing web-based deci-
sion tools to support people living with ALS/MND to make difficult
NOTES ON CONTRIBUTORS xxi
decisions for care and quality of life. Her teaching work is in convening
and delivering healthcare safety and quality and research units for the
Master of Health Service Management, and supervision of PhD students.
Wendy S. Johnston is Professor Medicine in the Division of Neurology
at the Faculty of Medicine and Dentistry at the University of Alberta,
Edmonton, Canada. As Medical Director of the University of Alberta
Amyotrophic Lateral Sclerosis Programme she leads a multidisciplinary
team dedicated to the care of patients with ALS and other motor neuron
diseases. Her research encompasses both qualitative research in ALS as
well as an active investigational drug trial programme. She serves on the
Board of ALS Canada and is past-Chair of the Canadian ALS Clinical
Research network.
Imen Kacem is the director of the ALS center in Department of
Neurology at Razi University Hospital. She is member in the ALS research
project and related syndromes in tropical zone (TROPALS). She won the
International Scholarship Award of the American Academy of
Neurology in 2017. She authored and co-authored many papers and
book chapters about ALS and CMT, such as Epidemiology of ALS and
Clinical Features of ALS in Africa.
Aqdas Kazi MBBS, MRCP, CCST (Palliative Medicine), is a consultant
in Palliative Medicine at Shifa International Hospital, Islamabad. After
MRCP and completion of four years of Specialized Registrar train-
ing, she received the Certificate of Completion of Specialized Training
in Palliative Medicine from the Royal College of the Physicians of the
UK. After working at Singapore General Hospital with the hospital
based palliative care team, she established the Palliative Care Unit at
Shifa and is the lead for Home Based Palliative care. She teaches
medical staff and has been an active advocate for Palliative Care at the
national level.
Satish Khadilkar is one of the first few Indian neurologists to pursue the
subspecialty of neuromuscular disorders, nationally and internationally.
He has been involved with a new clinical sign, sarcoglycanopathies and
dysferlinopathies and the gene in calpainopahties. He has over 130 publi-
cations to his credit and has written six and edited one book on neurology.
He has been trustee and secretary of the Muscular Dystrophy Society and
National Vice President of Multiple Sclerosis Society of India. He was the
xxii NOTES ON CONTRIBUTORS
president of the Indian Academy of Neurology in 2018 and is the trea-

surer of Asian Oceanian Myology Center, where he represents India.
Seung Hyun Kim MD, PhD is Professor of Neurology and Director of
ALS Clinic and Cell Therapy Center at Hanyang University Medical
Center, Seoul. He earned his M.D. degree in medicine and Ph.D. in neu-
roanatomy from Hanyang University. He has pursued the field of ALS and
neurodegenerative disorders. He is conducting translational research in
the development of personalized medicine. He received the Secret of Life
Award, Award from Ministry of Science, ICT and Future Planning. He
serves as a member of the National Academy of Medicine of Korea and
Editorial member of Molecular Neurodegeneration.
Jerome E. Kurent MD, MPH, is Professor of Neurology and Medicine
at the Medical University of South Carolina and director of the ALS
Multidisciplinary Clinic at the Ralph H. Johnson Veterans Affairs Medical
Center. He completed residencies in Neurology and Internal Medicine at
the Johns Hopkins Hospital, followed by fellowships in neuromuscular
diseases and electromyography at the National Institutes of Health.
He was a founding member of the MUSC ALS Multidisciplinary
Clinic and served on the National Hospice and Palliative Care
Organization ALS Work Group. Kurent has served as chair of the
American Academy of Neurology Ethics Section, and the AAN Pain and
Palliative Care Section.
Stela Lefter MD, is a consultant neurologist with subspecialist interest in
neuromuscular disorders. She received her specialist training in Neurology
from the Royal College of Physicians of Ireland. She trained in neu-
romuscular diseases at the National Neuroscience Centres in Dublin
and Cork, Ireland, and MRC Centre for Neuromuscular Diseases,
National Hospital for Neurology and Neurosurgery, Queen Square,
London. Lefter was conferred with an M.D. degree in 2015 for her
thesis ‘A Population-Based Epidemiologic Study of Adult
Neuromuscular Disease in the Republic of Ireland’. Her specialist
research interests include muscle diseases, ALS, and peripheral
neuropathies.
Tauana Bernardes Leoni MD, is a neurologist in the Department of
Neurology at the School of Medical Sciences at the University of Campinas
(UNICAMP), Campinas, Brazil. She supervises the clinical care of patients
NOTES ON CONTRIBUTORS xxiii
at the ALS outpatient clinic at UNICAMP hospital. Leoni is pursuing her

PhD working in a research project devoted to characterizing the gen-
otypes, phenotypes and imaging signatures of familial ALS in Brazil. She
is a fellow of the Brazilian Academy of Neurology.
Wojciech Leppert is involved in the palliative care of patients at out–
patient clinic, home hospice, and in consultations of patients in different
units in hospitals. He has experience in oncology and his main area of
research is pain and quality of life of patients and families in supportive and
palliative care. He is Editor-in-Chief of an international journal Palliative
Medicine in Practice, was elected in 2018 as a President of the Polish
Association for Palliative Care, and was appointed in 2019 as National
Consultant in palliative medicine in Poland to the Ministry of Health.
Westerly Luth is a research associate at the University of Alberta, work-
ing on projects under Wendy Johnston’s qualitative research program.
Her background is in psychology and public health.
Susan Mathers is a neurologist at Monash Health, Adjunct Senior
Lecturer, School of Clinical Sciences, Monash University and Clinical
Director of Neurology at Calvary Health Care Bethlehem, Melbourne.
Her clinical and research interests focus on the management of progressive
neurological diseases and models of care. She is a member of the sci-
entific committee of the Motor Neurone Disease Research Institute
of Australia, the Fight MND CURE Sub-Committee and a founding
member of the Australian Motor Neurone Disease Registry and Victorian
MND research tissue bank.
Christopher McDermott received his medical degree at the University
in Leeds. He undertook the Wellcome Trust Research Training PhD
Fellowship and completed his Specialist Training in Neurology at the
University of Sheffield, becoming a Consultant Neurologist in 2006.
McDermott is now the Professor of Translational Neurology at SITraN
and a Consultant Neurologist at the Sheffield Teaching Hospitals
Foundation NHS Trust, regularly undertaking specialist MND and neu-
romuscular clinics in Sheffield. He aims to develop the evidence base for
delivering supportive and symptomatic care for patients living with
MND. He is chair of the NIHR UK MND Clinical Studies Group.
xxiv NOTES ON CONTRIBUTORS
Hiroshi Mitsumoto MD, DSc, is the Wesley J. Howe Professor of

Neurology at Columbia University at The Neurological Institute of
New York and New York-Presbyterian Hospital/Columbia University
Medical Center. Mitsumoto has devoted his clinical and research effort to
the investigation of ALS in both animal models and clinical trials in patients
with ALS. He has written and lectured extensively on the topic of
ALS. He served as Chair of the ALSA Medical Advisory Board until
1999 and is now a member of the MDA Medical Advisory Board, ALS
CARE Board of Directors, and Neurology editorial board.
Amina Nasri is Assistant Professor of Neurology in the Department of
Neurology, Razi Hospital. She is investigator in the Clinical Investigation
Centre (CIC), member of the Tunisian Neurology Society since 2011 and
fellow of the European Board of Neurology since 2015. She is mem-
ber of the Movement Disorder Society since 2014. She won the
International Scholarship Award of the American Academy of
Neurology in 2020. She authored and co-authored many original
papers or book chapters about movement disorder and Alzheimer disease
as well as ALS.
D. V. Nevzorova graduated from Medical University in Ryazan in 1998.
She entered the First Moscow Hospice in 2004 as head of department and
in 2012 she was appointed to the position of Chief Medical Officer. In
2015 she became the Head of Federal Research and Clinical Center
for Palliative Care at Sechenov First Moscow State Medical University. She
is the chief specialist in palliative medicine in Russia and co-author of
Clinical Guidelines on Chronic Pain in Palliative Care Patients.
Asfandyar Khan Niazi MBBS, MRCP, MPH, is the academic chief resi-
dent (PGY 5) in the Department of Neurology at Shifa International
Hospital, Islamabad, Pakistan. He graduated from Shifa College of
Medicine in 2014. He holds membership of the Royal College of Physicians
(MRCP) of the UK and a Specialty Certificate Examination in neurology.
He also has a background in public health with an MPH from the
University of London. He has a keen interest in neurodegenerative and
neuroinflammatory diseases.
Mieko Ogino MD, PhD, MMA, is board certified Neurologist and
board-certified home care physician. She was a post-doctoral research fel-
low at Columbia University for four years, and was awarded a PhD degree
from Kitasato University in 1994; she received the Master of Medical
NOTES ON CONTRIBUTORS xxv
Administration from Tokyo Medical and Dental University in 2008.

She also finished a course at the Center for Biomedical Ethics and
Law (CBEL) in Tokyo University 2005 and became a specialist in
clinical ethics. She is the advocate for palliative care for non-cancer
patients, especially neurological diseases. She is a director of various aca-
demic societies and member of several national associations.
Yomi Ogun BSc (Hons); MBChB; MPH (London), is Professor of
Internal Medicine/Neurology and Consultant Physician/Neurologist at
Lagos State University College of Medicine Teaching Hospital. With
over 90 publications, he is a leading practicing neurologist in Nigeria
and an authority on NeuroHIV, Stroke and Neurophysiology (EMG/
NCS). Ogun is a Fellow of the Association of British-Council Fellows
and the Royal Society of Tropical Medicine/Hygiene (U.K), and has
been a member of the AAN-International Subcommittee 2019 and
the WHO Stroke Rehabilitation-Program Interventions 2019,
President of the African Academy of Neurology (2017–2019), President
of the NSO, and President of NSNS/PANS.
Kiwook Oh MD, PhD, is an associate professor in the Department of
Neurology, College of Medicine, Hanyang University, Seoul, Korea. He
received his M.D. and Ph.D. degrees from Hanyang University. Since his
early career, he is committed to patients with neurodegenerative diseases.
His work focuses specifically on clinical genetics and clinical trials of ALS
and neurodegenerative disease. His current project is ‘Genetic architec-
ture and clinical characteristics of Korean patients with motor neuron
disease’.
David Oliver has recently retired from his full-time position as Consultant
Physician in Palliative Medicine at the Wisdom Hospice in Rochester,
Kent and is an Honorary Professor at the Tizard Centre at the University
of Kent. He has lectured and published widely on neurological palliative
care, particularly on the care of people with motor neuron disease.
He was the Chair for the UK National Institute for Health and Care
Excellence Guidelines on MND, released in February 2016. He is
principal editor of “Palliative Care of Amyotrophic Lateral Sclerosis—
from diagnosis to bereavement”.
Evy Reviers is the daughter of an ALS patient, which is her motivation to
serve as a representative of pALS at the national and international level.
She is CEO of ALS Liga Belgium, focusing on stimulating and financing
xxvi NOTES ON CONTRIBUTORS
scientific research on ALS, contacts with ALS researchers, collaborations

with university hospitals, and defending the rights of pALS at governmen-
tal institutions and agencies. As Chairperson of the European
Organization for Professionals and Patients with ALS (EUpALS), she is
particularly involved in regulatory aspects of ALS clinical trials and advo-
cates the same rights for access to trials and future medicines for all pALS
worldwide.
Ildefonso Rodriguez-Leyva MD, MSc, PhD, is head of the Neurology
Program of the Faculty of Medicine of the Autonomous University of San
Luis Potosí and is the Head of the Neurology Service of the Central
Hospital “Dr. Ignacio Morones Prieto” from San Luis Potosi, SLP,
Mexico. He has dedicated himself to educate physicians and improve the
quality of Neurology in his country, by advancing the relationship of
Mexican Neurology with the American Academy of Neurology, American
Neurological Association, American Epilepsy Society, Movement Disorders
Society, and the World Federation of Neurology.
Roman Rolke is Director of the Department of Palliative Medicine at
RWTH Aachen University, Germany, since 2014. He is co-speaker of the
Neuropalliative Working Group of the German Neurological Society
and co-speaker of all physicians of the German Palliative Care
Association. His research interests include assessment of pain profiles
using quantitative sensory testing and late-stage neurological disor-
ders. He is editor of several books on pain and palliative medicine. Rolke
is Associate Editor of the German Journal of Palliative Medicine.
Ikram Sghaier has a BSc degree in analytical and experimental biology,
M.Sc. degree in microbiology and molecular epidemiology, Specialist in
immuno-genetics and virology. She won the Award for best participation
in 2012 and 2015 from “STGE” during her PhD. She is Post-doc in the
neuro-genetics laboratory, neurology department in RAZI Hospital
Tunisia and the Clinical investigation centre (CIC) and is focusing in
Amyotrophic lateral sclerosis disease (genetic background and atypical
clinical features)
Bugyeong Son RN, is a nurse coordinator at the Cell Therapy Center,
Hanyang University Medical Center, Seoul, Korea. She earned her bach-
elor’s degree in science of nursing from Hanyang University. She has clini-
cal nursing experience in the oncology department and has been
involved with ALS/MND since joining Cell Therapy Center. She
NOTES ON CONTRIBUTORS xxvii
became a Director of International Cooperation in Korean ALS

Association in 2019 and is now a second-year master’s student at Hanyang
University.
Ludo Van Den Bosch is a scientist investigating the molecular mecha-
nisms involved in different neurodegenerative diseases, including ALS. In
addition, he develops new therapeutic strategies for these diseases. He is
full professor of Neuroscience at the University of Leuven and group
leader at the VIB-Center for Brain & Disease Research. He is head of the
Laboratory of Neurobiology and scientific founder of Augustine
Therapeutics, a spin-off of VIB developing therapeutic strategies for
neurodegenerative disorders.
Philip Van Damme is a neurologist at the University Hospital Leuven.
During his neurology training, he undertook a PhD on the role of excito-
toxicity in ALS. Afterwards he continued to focus on the neuromuscular
disorder ALS. He is Professor of Neuroscience at the University of Leuven
and is currently directing the Neuromuscular Reference Center,
which coordinates the care of ALS patients. His research activities
focus on translational ALS research including fundamental studies to
understand the causes and disease mechanisms of ALS, clinical stud-
ies on biomarkers of the disease and prodromal disease stages, and
clinical trials with novel treatments for ALS.
Ludo Vanopdenbosch is a private neurologist with subspecialty interests
in neuro-oncology and multiple sclerosis. He is also director of palliative
care and head of the ethics committee at AZ Sint Jan Brugge. His research
interest is palliative care in neurology. At present he is co-chair of the sci-
entific panel on palliative care of the European Academy of neurology. He
is guest professor at the University of Leuven and the Free University of
Brussels.
Simone Veronese MD, PhD is a consultant in palliative care, head of
research at Fondazione FARO, Turin, Italy. He is a member of the Italian
Society for Palliative Care (SICP), of the Group of Study for Bioethics and
Palliative Care of the Italian Society of Neurology (SIN), and of the
Reference Group for Neurology of the European Association of
Palliative Care (EAPC). He is the author of several articles on the
role of palliative care in neurodegenerative conditions, and on quality of
life and outcome measurements.
xxviii NOTES ON CONTRIBUTORS
M. N. Zakharova graduated from Sechenov Medical Institute in 1979.

She started to work in the Research Center of Neurology as a resident and
now she is head of the department. She is a professor on the Medical
Faculty of Moscow State University and author of more than 200 articles
and book chapters. She is co-author of the Clinical Guidelines on Multiple
Sclerosis and Amyotrophic Lateral Sclerosis.
List of Figures
Fig. 3.1 Kaplan-Meier curve of patients seen at the University Hospitals

Leuven before 2009 (n = 474) and after 2009 (n = 726)53
Fig. 5.1 CALS sites. (Adapted from Zinman et al. 2013) 84
Fig. 9.1 Management structure of the ALS/MND services in Ireland 135
Fig. 22.1 USA Annual ALS Research Budgets 2012–2020. Annual ALS
Research Budgets for Fiscal Years 2012–2020 for National
Institutes of Health (NIH), Department of Defense ALS
Research Program (DoD ALSRP), Center for Disease Control
and Prevention—Agency for Toxic Substances Disease Registry
National Amyotrophic Lateral Sclerosis Registry (CDC ALS
Registry), Department of Veterans Affairs (VA) and
Amyotrophic Lateral Sclerosis Association (ALSA). Original
source material available at NIH = https://report.nih.gov/
categorical_spending.aspx; DoD ALSRP = http://cdmrp.army.
mil/about/fundinghistory; CDC ALS Registry = https://
www.atsdr.cdc.gov/features/alsregistryanniversary/index.
html; VA = https://cdmrp.army.mil/alsrp/pdfs/ALSRP%20
Strategic%20Plan.pdf; ALSA = http://www.alsa.org/
about-us/financial-information.html306
xxix
List of Tables
Table 1.1 Conditions that can mimic ALS/MND 5

Table 1.2 Symptom management 10
Table 1.3 Selected ethical issues and controversies 12
Table 1.4 Triggers for initiating discussion about end-of-life issues
in ALS/MND 14
Table 1.5 Country characteristics 22
Table 2.1 Proportions in each place of death (percent) 2017–18 34
Table 4.1 Phenotypic characterization of Brazilian patients with ALS 67
Table 4.2 Items provided by Public Healthcare in Brazil for patients
with ALS 70
Table 7.1 Overview of palliative care and hospice services
across Germany 113
Table 20.1 Multidisciplinary team disease management in Tunisia 270
Table 22.1 ALS deaths per year with location of death 307
Table 23.1 Incidence and prevalence of ALS/MND 330
Table 23.2 Support for caregivers of patients with ALS/MND 336
Table 23.3 Access to multidisciplinary clinics 339
Table 23.4 Access to palliative care/hospice 340
xxxi
CHAPTER 1
Introduction to Public Policy of ALS/MND
Jerome E. Kurent, David Oliver, and Robert H. Blank
Abstract This chapter outlines the framework and goals of this cross-
national study of amyotrophic lateral sclerosis/motor neuron disease
(ALS/MND) policy. It also describes the key features of ALS/MND and
discusses criteria and associated challenges in making the diagnosis and the
importance of excluding ALS/MND mimics. Epidemiology, genetics and
possible environmental risk factors for ALS/MND are reviewed.
Management of symptoms, challenges confronting caregivers, including
access to palliative care and hospice during the end-of-life, and the broad
range of ethical issues surrounding ALS/MND care are highlighted. This
chapter examines the often-ignored public policy context and the impor-
tance of cultural and religious factors in understanding policy differences
which exist across countries. Finally, this chapter introduces the countries
J. E. Kurent
Medical University of South Carolina, Charleston, SC, USA
e-mail: kurentje@musc.edu
D. Oliver
University of Kent, Canterbury, UK
e-mail: drdjoliver@gmail.com
R. H. Blank (*)
University of Canterbury, Christchurch, New Zealand
e-mail: rblank24601@hotmail.com
© The Author(s) 2021 1

R. H. Blank et al. (eds.), Public Policy in ALS/MND Care,
https://doi.org/10.1007/978-981-15-5840-5_1
2 J. E. KURENT ET AL.
selected for this study and summarizes the key topics of importance the
authors were asked to address in their chapters.
Keywords ALS • MND • Public policy • Cross-national studies

• Multidisciplinary teams • Palliative care • End-of-life decisions
Introduction
Incurable progressive neurological disorders cause immense pain and suf-
fering for their victims and their families. The associated huge economic
and indirect costs to society are often difficult to calculate. A rapidly grow-
ing aging population in many regions of the world is strongly associated
with the increased incidence of neurodegenerative disease and has major
public policy implications. Amyotrophic lateral sclerosis/motor neuron
disease (ALS/MND) may be the prime example of a devastating rapidly
progressive neurodegenerative disease for which there is no cure or signifi-
cant disease-modifying therapy. Although there are three approved drugs
used for the treatment of ALS/MND, their benefits are limited, and most
other treatment options are limited primarily to symptom management.
There is a clear need for effective public policy and research focused on
ensuring optimal clinical care of patients with ALS/MND and provision
of adequate supportive services. Increased research funding focused on an
enhanced understanding of the etiology(ies) and pathophysiology of
ALS/MND and mechanisms of motor neuron cell death is expected to
lead to development of additional disease-modifying agents and ultimately
to a cure. There is also the potential that insights gained from the study of
ALS/MND may provide clues to other enigmatic neurodegenerative dis-
eases, including dementia, that plague our societies.
A primary goal of this book is to develop a compendium of public
policy summaries representative of many different countries across the
globe. It discusses a range of ALS/MND policy elements and perspectives
while providing a framework for understanding ALS/MND as it affects
patients and their families, professional caregivers and researchers. The
book should be of interest to those involved in health care policy and pub-
lic health as well as for ALS clinicians including neurologists, nurses, nurse
practitioners, allied health professionals and physicians’ assistants involved
in the complex range of issues related to ALS/MND. Additional stake-
holders who should find this book of interest include social workers, ALS
clinic coordinators and administrators and members of ALS and MND
1 INTRODUCTION TO PUBLIC POLICY OF ALS/MND 3
private foundations and public agencies dedicated to the optimum treat-

ment of patients with ALS/MND—including the goal of its ultimate
defeat. The book may also serve as a useful reference for organizations
providing durable medical equipment such as wheelchairs, communica-
tion devices, beds and other assistive equipment utilized in the manage-
ment of patients with progressive disability.
What Is ALS/MND?
ALS is a progressive fatal neuromuscular disease first described by Charcot
in 1869 which primarily affects spinal cord and brainstem motor neurons
and the upper motor neuron system. ALS may be regarded as a syndrome
rather than a specific disease entity. There is increasing evidence of wider
neuronal involvement, including the frontal lobes, and cortical and sub-
cortical regions (Westeneng et al. 2016). ALS is also known as Lou
Gehrig’s disease in the United States, named after the famous major league
baseball player who succumbed to this illness in 1941, whereas in the
United Kingdom, motor neuron disease is used to designate ALS. Within
the classification ICD-9 (code 335.2), the term motor neuron disease is
also indistinguishable from ALS. It has been suggested that the term
motor neuron diseases be used to include both ALS and its clinical sub-
types. Thus, ALS/MND as used here encompasses:
• ALS/MND, with both upper and lower motor neuron involvement,

leading to a mixed picture of weakness associated with spasticity and
flaccidity, muscle wasting, fasciculation and extensor plantar
responses; average longevity three to five years; more than 90 per-
cent of people with limb onset develop bulbar symptoms;
• Progressive bulbar palsy affects about 20 percent of patients in which
there is involvement of the bulbar motor neurons, affecting speech
and swallowing;
• Progressive muscular atrophy (PMA) affects about 5 percent, with
predominantly lower motor neuron involvement leading to muscu-
lar weakness and wasting, particularly of the legs and arms. The
prognosis is longer and may be five to ten years;
• Primary lateral sclerosis (PLS) which affects 1–2 percent with pre-
dominantly upper motor neuron involvement, including spasticity,
stiffness, increased deep tendon reflexes and extensor plantar

response. It has a longer prognosis often greater than ten years
The most common clinical presentation of ALS/MND is spinal-onset

and involves slowly progressive painless weakness and atrophy of a distal
upper or lower extremity. Weakness typically progresses to involve the
contralateral limb and then to other areas of the body including bulbar
and respiratory muscles. Rarely—less than 5 percent—do patients first
present with respiratory failure that requires ventilator support. The aver-
age age of onset is 55 to 57 years, with a male to female ratio of 1.7:1,
except 1:1 having onset after 65 to 70 years of age. Death typically occurs
within three to five years from respiratory muscle failure, although 25
percent of patients survive for five years and 10 percent are alive at ten
years (Shaw et al. 2014).
Frontotemporal dementia (FTD) occurs in up to 15 percent of
patients (McKhann et al. 2001). More subtle frontal lobe dysfunction may
occur in another 20 to 25 percent of patients (Lomen-Hoerth et al. 2003;
Strong et al. 2009; Phukan 2012). There are patients with FTD who later
develop ALS/MND with a spectrum from pure ALS to pure FTD—often
related to the C9orf72 genetic abnormality (Couratier et al. 2017). An
estimated 90 to 95 percent of patients have sporadic ALS (sALS) which
occurs in the absence of a clear family history. Five to 10 percent of ALS
patients have familial ALS (fALS) usually inherited as a Mendelian autoso-
mal dominant gene. Reduced penetrance is not uncommon.
Making the Diagnosis of ALS/MND: A Diagnosis

of Exclusion
The neurologist must take a careful history and perform a meticulous

neurological examination to determine if an alternative cause of the
patient’s symptoms can be identified. Depending on localizing signs
and symptoms, MRI imaging of the spine is often necessary to rule out
structural lesions, such as cervical or lumbar stenosis, herniated disc
with foraminal encroachment and spinal cord compression, meningi-
oma and other spinal cord pathology. Although there are no specific
biomarkers currently available to confirm the diagnosis or to monitor
the clinical course of ALS/MND, efforts are underway to identify
biomarkers.
Table 1.1 Conditions that can mimic ALS/MND
Spinal cord pathology that causes spinal cord and/or nerve root compression
(myeloradiculopathy) includes but is not limited to cervical spondylosis and meningioma
Autoimmune neuropathies—including multifocal motor neuropathy
Spinobulbar muscular atrophy (Kennedy’s disease)
Multiple sclerosis
Hereditary spastic paraplegia
Benign fasciculation syndrome
Myopathies—inclusion body myositis
Diabetic amyotrophy
Thyrotoxicosis
Hyperparathyroidism
As illustrated in Table 1.1, there are many conditions that can mimic
ALS/MND. A second independent opinion from a neuromuscular spe-
cialist having special expertise in ALS/MND is often requested by the first
neurologist suspecting ALS/MND in order to confirm the diagnosis of
what many consider to be a “death sentence.” Muscle biopsy and spinal
fluid examination are not considered necessary for routine evaluation of
patients suspected of having ALS/MND, but they may be helpful in diag-
nosing a suspected alternative condition or ALS mimic.
Criteria have been developed to assist with the diagnosis and manage-
ment of ALS. These include the modified El Escorial criteria (Brooks et al.
2000), which are particularly relevant when considering clinical drug tri-
als. The criteria suggest that for a diagnosis of ALS/MND, there should
be progressive upper and lower motor neuron deficits in at least one limb
or region of the human body (meeting the revised El Escorial criteria for
possible ALS) or lower motor neuron deficits as defined by clinical exami-
nation (one region) and/or by EMG in two body regions (defined as
bulbar, cervical, thoracic, lumbosacral). There must be progressive
changes, fasciculations and near normal motor nerve conduction and no
sensory abnormalities. There should be no abnormalities of the sensory
nervous system, no impairment of the visual or autonomic nervous system
and no sphincter involvement.
The Awaji criteria were subsequently developed and are based on the
expanded role of the electrophysiological diagnosis of ALS/MND (Chen
et al. 2010; Costa et al. 2012). Electromyography is a critical component
of diagnosing ALS/MND. Diffuse denervation should be present in three

different anatomical regions to meet electrophysiological criteria for the
diagnosis of ALS/MND. Fibrillations and positive waves consistent with
active denervation are typically present and are usually seen in association
with high amplitude, prolonged duration motor unit potentials consistent
with chronic denervation/re-innervation. Fasciculation potentials are
often present. It is imperative that ALS/MND mimics have been ruled out.
Epidemiology of ALS/MND
The incidence of ALS/MND across the world is unclear. A systematic
review suggests that the incidence in Europe is 2–3/100,000 with a prev-
alence of 5–7/100,000 population (Chio et al. 2013), while the incidence
of ALS in the United States is approximately 3–5/100,000. It is unclear if
the differences across countries relate to the design of studies, the ability
of patients to receive medical attention or differences in defining
ALS/MND.
Although considered a rare disease, the incidence of ALS/MND is
approximately one-half that of multiple sclerosis (MS). However, public
perception and general awareness of MS is much greater than that of
ALS/MND primarily due to the marked contrast in life expectancy for
victims of these two disorders. Although patients with MS face a reduction
in life expectancy of seven to twelve years (Magyari and Sorensen 2019),
most patients with ALS/MND will not survive more than three to five
years after diagnosis. The incidence of ALS/MND in the non-Caucasian
population and individuals of mixed ethnicity is considered less than
whites of European ancestry.
In the Western Pacific the incidence of ALS among the indigenous
Chamorros in Guam had occurred at rates 50 to 100 times that noted
elsewhere around the world (Armon 2013). ALS often co-existed with
Parkinson-dementia complex. The incidence of Guamanian ALS has dra-
matically dropped since World War II, and it is suspected that dietary tox-
ins present in cycad seed, which were used as a primary source of dietary
flour, along with a possible genetic predisposition, may have been at least
in part responsible for this phenomenon. Genetic susceptibility may have
contributed to an increased incidence of ALS in the Kii Peninsula of Japan,
which also represents a well-known, but poorly understood, ALS cluster.
Genetics of ALS/MND
The genetics of ALS/MND has become increasingly complex. The first
ALS gene mutation—SOD1—was reported in 1993 (Rosen et al. 1993).
There are now more than twenty different genes known to cause ALS. The
majority of familial ALS is transmitted as a Mendelian autosomal domi-
nant gene, but penetrance may be incomplete. The most common
genes are:
• SOD1—accounting for 10–20 percent of fALS and 1 percent of sALS

• C9orf72 hexanucleotide repeat—accounting for 40 percent of fALS
in many countries, but less than 10 percent in Asia, found in 10–20
percent of sporadic ALS in Europe
• TDP-43—accounting for 4 percent of fALS and less than 1 per-
cent of sALS
• FUS/TLS—accounting for 4 percent of fALS and more rare in sALS
• Ataxin 2—accounting for 4 percent of fALS and 1 percent of sALS
• VCP—accounting for 1–2 percent of fALS (Corcia et al. 2017)
Reviews have described in detail the rapidly evolving body of knowl-

edge related to the genetics of ALS/MND and their contribution to
advancing our understanding of the underlying basis for this disorder and
means to develop effective therapies (Andersen and Al-Chalabi 2011;
Brown and Al-Chalabi 2017). The investigation of these genetic muta-
tions is providing some insights into mechanisms of motor neuron cell
damage and death. Until recently, numerous clinical trials with humans
affected with ALS/MND have not been able to duplicate positive results
reported in animal studies (Benatar et al. 2009). However, the develop-
ment of gene-specific therapies for ALS/MND may indeed be realized in
the case of the SOD1 mutant gene and C9orf72. The development of anti-
sense oligonucleotides is well underway along with early-stage clinical tri-
als for individuals having the SOD1 genetic variant and C9orf72 (Ly and
Miller 2018; Miller et al. 2020). SOD1 suppression with adeno-associated
virus and microRNA was also recently reported (Mueller et al. 2020).
There is the underlying assumption that patients with sALS have a
genetic predisposition for developing ALS/MND. The presumed genetic
predisposition alone is considered insufficient, but in conjunction with
other factors including environmental influences, it can trigger a process

resulting in clinical ALS/MND. It has been suggested that a six-step pro-
cess occurring over the life of an individual may converge with a genetic
predisposition to initiate the cascade of motor neuron damage and cell
death, resulting in ALS/MND (Al-Chalabi et al. 2014; Chio et al. 2018).
Identical twin studies have failed to demonstrate an increased risk of devel-
oping ALS in the unaffected sibling (Al Chalabi et al. 2010; Xi et al. 2014;
Tarr et al. 2019), perhaps supporting the suspected role of environmental
factors and epigenetics.
Environmental Risk Factors

The suspected role of environmental factors as they relate to genetics in
ALS has been discussed in detail (Al-Chalabi and Hardiman 2013).
Numerous toxins and other insults as well as nutritional deficiencies have
been considered. The role of physical trauma and emotional stress has also
been studied, but without demonstrating a clear relationship to causality.
More recent observations related to a suspected increased risk of ALS and
other neurodegenerative diseases in US National Football League (NFL)
professional athletes have been highlighted. The possible role of chronic
traumatic encephalopathy (CTE) in a subgroup of patients with ALS has
been suggested (McKee et al. 2010), although other reports have ques-
tioned this relationship (Bedlack et al. 2011; Armon and Miller 2011).
Toxins and other environmental agents which have been investigated
include smoking, alcohol, heavy metals like lead and mercury, pesticides
and electric shock. Smoking has been associated with ALS/MND risk in
several studies, although its precise role in susceptibility has not been
defined. Inhalation of other environmental toxins associated with urban
pollution is also being investigated (Malek et al. 2013; Seelen et al. 2017).
There is also renewed interest in toxins associated with cyanobacteria,
which have ubiquitous presence in the environment. Viral infections
including reverse transcriptase agents have been studied, but without
definitive proof of causality. Patients with HIV/AIDS have developed
ALS, but their neurological disability has not responded to antiviral therapy
In the United States, ALS has been declared a military service-connected
disability based on several epidemiological studies (Horner et al. 2003;
Weisskopf et al. 2005), suggesting a two-fold increase in risk for develop-
ing ALS by deployed veterans. An increased risk was first described for
veterans deployed to Iraq, and subsequently for those involved in earlier
conflicts and wars dating back to World War II. Numerous factors related
to military service have been considered, but without demonstrating a
direct causal link to ALS. These factors include burning oil fields, as in
Iraq, multiple immunizations, concussion and other physical and emo-
tional stressors, as well as nerve toxin antidotes, such as pyridostigmine.
Italian soccer players have also experienced a significantly increased risk of
developing ALS, but for unknown reasons. Occasional clustering of
patients with ALS/MND has been reported, suggesting possible common
environmental exposures, but with inconclusive findings.
Management of Symptoms
Multidisciplinary ALS clinics are considered the gold standard for provid-
ing comprehensive care (NICE 2016; Miller et al. 2009) and are widely
accessible through most of North America, the United Kingdom, Europe
and many countries around the globe. Their implementation has been
associated with improved quality of life and possibly extension of the natu-
ral history of ALS/MND (Aridegbe et al. 2013; Rooney et al. 2015). The
ALS Functional Rating Scale-Revised (ALS-FRS-R) is a tool routinely uti-
lized in multidisciplinary clinics to monitor overall clinical status of the
patient and is a reliable measure of clinical progression and loss of function
(Cedarbaum et al. 1999). Numerous excellent resources are available to
guide the clinical management of patients with ALS/MND (Miller et al.
1999; Miller et al. 2009; Bedlack and Mitsumoto 2013).
Most patients with ALS/MND will die of respiratory failure secondary
to weakness and atrophy of diaphragmatic and accessory muscles of respi-
ration. Patients will experience a wide range of disabling symptoms over
the course of their illness including dyspnea, weakness and atrophy, muscle
cramps, dysarthria, dysphagia, weight loss, pathological laughter and cry-
ing (pseudobulbar affect), drooling, pain, sleep disturbance and constipa-
tion (see Table 1.2). Multidisciplinary ALS clinics provide a wide range of
professional expertise required to assist with management of these symp-
toms while providing comprehensive care for the patient and support for
family caregivers.
Table 1.2 Symptom management

Symptom Management
Pain Muscle spasm Muscle relaxants

Musculoskeletal Nonsteroidal
Skin pressure anti-inflammatory
Analgesics/opioids
Cramps Fasciculations Quinine sulfate (not available
Spastic muscles in US)
Baclofen
Gabapentin
Tizanidine
Spasticity Muscle spasm Baclofen
Tizanidine
Gabapentin
Physiotherapy
Sialorrhea Reduced swallowing Atropine/scopolamine
Botulinum toxin injections
Radiotherapy to salivary glands
Thick secretions N-acetylcysteine
Grape fruit juices
Nebulized water
Laryngospasm Sublingual lorazepam
Insomnia Anxiety Anxiolytics
Respiratory failure Noninvasive ventilation
Depression Antidepressants
Pseudobulbar affect Uncontrolled laughter/crying Amitriptyline,
Dextromethorphan/quinidine
sulfate
Dyspnea Respiratory muscle weakness Noninvasive ventilation
and respiratory failure Opioids
Tracheostomy ventilation
Dysarthria Speech and language therapy Communication aids
assessment
Dysphagia Speech and language therapy Modified consistency of food
and dietitian assessment Consider gastrostomy
Cognitive issues Frontal lobe dysfunction Psychological assessment
Frontotemporal dementia Advice and support for
Language/loss of empathy/ caregivers
executive function loss/
memory loss
Constipation Poor diet/anticholinergic Fluid and fiber
medication side effects Laxatives
Gastroesophageal Lower esophageal sphincter Meals while upright
reflux disease weakness H-2 blockers
Proton pump inhibitors
Palliative Care, Hospice and End-of-Life Care

As there is no curative treatment for ALS/MND, the approach is often
palliative. The World Health Organization (WHO) defines palliative care
as “the total active care of patients whose disease is not responsive to cura-
tive treatment. The goal of palliative care is the achievement of the best
quality of life for patients and their families.” The involvement of specialist
palliative care/hospice care for ALS/MND started first at St Christopher’s
Hospice in London, UK, in 1967 and has spread across the world. Many
guidelines have recommended palliative care including the American
Academy of Neurology Ethics and Humanities Committee Position
Statement (Carver 1996) which highlighted the urgency that neurologists
understand and apply principles of palliative care in the management of
their patients. The American Academy of Neurology Practice Parameter
(Miller et al. 2009) and the EFNS Guidelines (Andersen et al. 2012) fur-
ther supported this approach.
A palliative care approach considers the whole patient in the context of
their family and caregivers—assessing the physical, psychological, social
and spiritual aspects of care. It encompasses a multidisciplinary approach,
and it has been recommended that the multidisciplinary team (MDT) for
ALS/MND should include palliative care expertise (NICE 2016; Miller
et al. 2009; Robert Wood Johnson Foundation 2004).
Ethical Issues in ALS/MND Care

Numerous challenging ethical issues often arise while caring for the patient
with progressive neurodegenerative diseases, including ALS/
MND. Table 1.3 illustrates some of these issues related to the manage-
ment of patients with ALS/MND, beginning with communicating the
bad news of the recent diagnosis of ALS/MND to a patient. A compre-
hensive description of the ethical issues and their resolution as they occur
in neurology practice has been provided by Bernat (2008).
The subject of breaking the news to the patient with newly diagnosed
ALS/MND is a matter of compassion and empathy and should include
discussion of the possible available treatments, anticipated future needs,
avoidances and preferences of the patient. The National Institute for
Health and Care Excellence (NICE) Guidance gives clear recommenda-
tions (NICE 2016). The newly diagnosed patient may, or may not, sus-
pect the seriousness of his neurological condition at the time of meeting
Table 1.3 Selected

• Breaking bad news
ethical issues and
• Abandonment
controversies • Enrollment in clinical trials
• Genetic testing of patients
• Genetic testing of family members
• Use of feeding tube and option to
withdraw once initiated
• Use of tracheostomy and mechanical
ventilation
• Palliative sedation
• Physician-assisted suicide
• Euthanasia
• Organ donation by living patient with
ALS
• Organ donation by deceased patient
with ALS
• “Right to Try” legislation
• Treatment with unproven and
potentially dangerous agents
• Charlatans and quacks offering
ineffective or unproven therapies
• Role of public policy in addressing
these issues
to summarize results of diagnostic testing with his or her neurologist. It is

vitally important for the physician to break the bad news in-person and to
allow adequate time for the patient to begin to process this life-changing
information. Excellent resources are available to help guide this discussion
(Johnston et al. 1996; Sloan and Borasio 2014).
The expression “Diagnose and Adios” has been used to characterize
some neurologists’ interactions with patients and suggests the lack of ade-
quate empathic connection. This suggests that the neurologist may pos-
sibly be more intent on making the correct diagnosis than on caring for
the patient. This approach is changing as the neurologist provides ongo-
ing care, often as part of a multidisciplinary team (Gursahani 2016; NICE
2016). In the past, physicians sometimes tried to protect the patient from
the bad news of a life-limiting diagnosis by using terms such as a “disease
of the motor system” or simply delaying the diagnosis. There is no justifi-
cation for withholding or downplaying the significance of diagnostic
information considered essential to prepare for advance planning and for
getting one’s personal and business affairs in order.
The ethical principle of truth-telling requires that patients be provided

honest and accurate information related to their illness. Most patients
expect full disclosure of facts related to the diagnosis of serious illness.
Prognosis and related discussions may be provided over a series of meet-
ings at a pace comfortable to the patient since some patients may be over-
whelmed with too much information provided at one time (Seeber et al.
2019). However, it is important to allow these discussions whenever the
patient and family wish to facilitate discussion and to plan for the future
(NICE 2016). The responses of patients with newly diagnosed ALS/
MND can be variable (Johnston et al. 1996; Sloan and Borasio 2014).
Some patients have a relatively positive response, “At least I know what I
have,” while others can express feelings of despair. There is a delicate bal-
ance between the neurologist providing immediate and full disclosure and
communicating in a caring and empathic manner. Communication should
be tailored to the unique needs of the patient to the extent this can be
determined. Cultures vary to the extent to which critical information is
expected to be communicated either directly to the patient, or sometimes
preferably to family members. Many patients explore the Internet to seek
out information related to their symptoms and suspected diagnosis.
It is usually helpful to ask the patient what he/she understands about
their condition. Most patients have little knowledge about ALS/MND,
although this is changing with the increased use of the Internet. A blunt
approach used to provide the diagnosis in an abrupt manner is strongly
discouraged and can be devastating to the patient and family. It is also
reasonable to leave some room for hope, although this may be challeng-
ing. Clinical trials may offer optimism for some patients.
Medical paternalism, in which the patient is “protected” from bad
news, is no longer accepted in most Western cultures. A direct and forth-
right discussion with the patient is expected. However, some cultures
around the world expect that unfavorable information pertaining to a seri-
ous diagnosis be provided to family members of the patient rather than to
the patient. These cultural norms should be respected by the physician and
should be known in advance of the discussion if possible.
Patients with ALS have sometimes expressed a sense of physical or emo-
tional abandonment by their physicians as their illness progressed. Non-
abandonment of the patient is a key ethical obligation shared by all
professionals involved in the care of patients with ALS/MND. Abandonment
of the patient by a physician can be either physical or emotional. Following
the diagnosis of ALS/MND, professional caregivers including physicians
Table 1.4 Triggers for initiating discussion about end-of-life issues in ALS/MND
• The patient or family asks, or “opens the door,” for end-of-life information and/or
possible interventions
• Severe psychological and/or social or spiritual distress or suffering
• Pain or other symptoms requiring higher than normal dosages of medications
• Dysphagia requiring a feeding tube
• Dyspnea or symptoms of hypoventilation, usually with reduced forced vital capacity
of 50 percent or less
• Loss of function in two body regions (bulbar, arms or legs)
Modified from Robert Wood Johnson Foundation (2004)
may feel uncomfortable in following up the patient with an incurable dis-

ease. Increasingly, the ongoing care is shared within a multidisciplinary team,
which shares the load and allows all aspects of care to be considered, often
with an improvement of quality and even length of life (Aridegbe et al. 2013).
Professional caregivers may have a basic discomfort with death and
dying, including fear of death, a sense of failure as well as challenges in
confronting one’s own mortality. However, there remains an obligation to
discuss end-of-life care issues with the patient having advanced ALS/
MND including the need for advance care planning. Table 1.4 lists six
triggers for initiating a discussion about end-of-life care decision-making
for the patient with ALS.
Clinical Trials
ALS/MND clinical trials offer hope for patients, and many are eager to
participate. Clinical trial participation should be strongly encouraged.
Although most patients appear to fully understand that participation in
clinical trials will most likely not be of direct benefit to them, it is impor-
tant that they have this clear understanding prior to enrollment in the
interest of truth-telling and informed consent.
Genetic Testing
The genetics of ALS/MND have provided critical insights into molecular
mechanisms of motor neuron cell damage and death and offer hope that
meaningful therapies focused on specific gene defects can be developed.
Availability of genetic testing for ALS/MND varies greatly around the
world. There are numerous medical-ethical dilemmas arising from the
availability of genetic testing for ALS/MND. Continued acquisition of
new knowledge related to understanding and ultimately curing ALS/

MND will depend to a great extent on identifying additional genes. A
recommendation for routine genetic testing of patients with sporadic ALS
has been made, but this raises the challenging issue of the testing of unaf-
fected individuals who are at potential risk of developing ALS/
MND. There also may be issues of payment by insurance companies and
restricted resources and availability of genetic counseling and testing in
many parts of the world.
Genetic testing for clinically unaffected family members at-risk and
related to a patient with clinical ALS/MND having a strong family history
has been a subject of discussion. Testing of individuals at-risk for develop-
ing ALS/MND can provide the opportunity to arrange for family plan-
ning in addition to making other key decisions involving employment and
the acquisition of medical and life insurance. Genetic counseling prior to
and following testing is obligatory whether the individual tests positive or
negative for the gene. It has been questioned whether there is an ethical
obligation to disclose positive results of gene testing to other family mem-
bers even though they are not actively participating. Clinically unaffected
individuals, although not suspecting that they might be carrying a mutant
gene, could potentially benefit from this information. However, even if
legislation exists to protect individuals having a genetic mutation, discrim-
ination by insurance companies and employers might still occur, posing
serious implications for the economic well-being of clinically unaffected
patients who test positive for a deleterious ALS/MND gene.
Withholding and Withdrawal of Care

There are many ethical and legal issues at the end of life particularly con-
cerning the withholding and withdrawing of care. In many countries there
is no ethical or legal distinction between withholding and withdrawing of
care, such as the placement or use of a feeding tube or the use of invasive
ventilation. Such interventions can be either withheld altogether, or with-
drawn once in place. These decisions are typically at the direction of a
patient having decision-making capacity or his/her legal surrogate
decision-maker. In some countries it is not legally permissible to withdraw
treatment, such as invasive ventilation, once it has been initiated. These
approaches vary by country and often have strong cultural underpinnings,
which form the basis for law and public policy. Therefore, careful discus-
sion is necessary before any consideration of these interventions, so that a
clear decision is made; the implications of the intervention, such as the

continued deterioration of the disease after ventilatory support has started,
are discussed; and plans for future care, including the possible withdrawal
of treatment (NICE 2016; Oliver and Turner 2010), are considered. If
withdrawal is not possible within a country, these discussions are even
more crucial so that a truly autonomous decision can be made.
There is increasing awareness of frontotemporal dementia and related
cognitive change which can occur in ALS/MND, and a consideration of
mental capacity to make decisions and advance care planning is important.
There may be opportunities earlier in the disease progression when the
person can make decisions and make their wishes for their future care
known. If these are recorded clearly, as advance directives or defining a
proxy to make decisions on their behalf, the person’s wishes can be
respected. However, it may be difficult to have these discussions, and
patients and families may be resistant to looking ahead, but this may be
the only opportunity as cognition and communication may be lost in the
future (Goldstein 2014).
Palliative Sedation and Assisted Dying

At the end of life some patients experience significant distress. The aim of
palliative care is to minimize suffering, and with good symptom manage-
ment, a distressing death is considered rare (Neudert et al. 2001).
However, on some occasions the patient may remain distressed—physi-
cally, emotionally or spiritually. In certain circumstances therapeutic/pal-
liative sedation may be necessary. The European Association for Palliative
Care framework has suggested that “Therapeutic/palliative sedation in
the context of palliative medicine is the monitored use of medications
intended to induce a state of decreased or absent awareness (unconscious-
ness) in order to relieve the burden of otherwise intractable suffering in a
manner that is ethically acceptable to the patient, family and health-care
providers” (Cherny et al. 2009). Morphine and benzodiazepine medica-
tions are typically used in a closely monitored clinical environment to
sedate in order to relieve suffering.
The National Hospice and Palliative Care Organization (2010) has
provided guidelines for the implementation of palliative sedation in the
rare instance that it may be required. This includes the recommendation
that patient caregivers as well as the patient, if capable, participate in a
detailed discussion with hospice or palliative care professionals caring for
Another random document with
no related content on Scribd:
The Project Gutenberg eBook of Tea, its
history and mystery
This ebook is for the use of anyone anywhere in the United
States and most other parts of the world at no cost and with
almost no restrictions whatsoever. You may copy it, give it away
or re-use it under the terms of the Project Gutenberg License
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laws of the country where you are located before using this
eBook.
Title: Tea, its history and mystery
Author: Joseph M. Walsh
Release date: December 4, 2023 [eBook #72306]
Language: English
Original publication: United States: Henry T. Coates & Co, 1892
Credits: Steve Mattern, Gísli Valgeirsson and the Online

Distributed Proofreading Team at https://www.pgdp.net
(This file was produced from images generously made
available by The Internet Archive)
*** START OF THE PROJECT GUTENBERG EBOOK TEA, ITS

HISTORY AND MYSTERY ***
TEA
—IT S—
HISTORY AND MYSTERY

—BY—
JOSEPH M. WALSH,
AUTHOR OF
“COFFEE, ITS HISTORY, CLASSIFICATION AND

DESCRIPTION.”
PHILADELPHIA:
HENRY T. COATES & CO.
COPYRIGHTED, 1892,
BY
JOSEPH M. WALSH.
All Rights Reserved.

PREFATORY.
Utility, not originality, has been aimed at in the compilation of this

work. The obstacles and difficulties its author had met with in his
endeavors to learn something of the article he was commissioned to
sell when he first entered the Tea trade, the almost total lack of
knowledge displayed by the average dealer in the commodity, allied
to the numerous inquiries for a work containing “all about tea,” first
prompted the undertaking.
The material was collated at intervals, in a fragmentary manner,
covering a period of over twenty years, and arranged amid the many
interruptions incident to an active business life, subjected to constant
revisions, repeated prunings and innumerable corrections, due
mainly to the varying statements and conflicting opinions of admitted
authorities in every branch of the subject. Still, as careful and
judicious an arrangement of the data has been given as possible, a
faithful effort being made to omit nothing that may prove useful,
instructive or profitable to the expert, the dealer or general reader.
Aware that many facts have been omitted, and many errors
committed in its preparation, he still trusts that the pains he has
taken to avoid both have not been in vain, that the former may be
few, and the latter of no great importance. The work was compiled
under impulse, not under inducement, a single line not being
intended originally for the market, and is now being published solely
for the benefit of those “whom it may concern.”
Philadelphia, December, 1892.
CONTENTS.
Chapter Page
I. Early History 9-28
II. Geographical Distribution 29-35
III. Botanical Characteristics 37-49

and Form
IV. Cultivation and 51-68

Preparation
V. Classification and 69-132

Description
VI. Adulteration and Detection 133-157
VII. Testing, Blending and 159-204

Preparing
VIII. Chemical, Medical and 205-235

Dietical Properties
IX. World’s Production and 237-252

Consumption
X. Tea Culture, a Probable 253-265

American Industry
(Branch of Tea Plant.)
CHAPTER I.
E A R LY H I S TO RY.
The history of Tea is intimately bound up with that of China, that is,
so far as the Western world is concerned, its production and
consumption being for centuries confined to that country. But, having
within the past two centuries become known and almost
indispensable as an article of diet in every civilized country of the
globe, it cannot but prove interesting to inquire into the progress,
properties and effects of a commodity which could have induced so
large a portion of mankind to abandon so many other articles of diet
in its favor, as well as the results of its present enormous
consumption.
Although now to be found in a wild state in the mountain-ranges of
Assam, and in a state of cultivation through a wide range from India
to Japan, the original country of Tea is not definitely known, but from
the fact of its being in use in China from the earliest times it is
commonly attributed to that country. Yet though claimed to have
been known in China long anterior to the Christian era, and even
said to have been mentioned in the Sao-Pao, published 2700 B. C.,
and also in the Rye, 600 B. C., the exact date or manner of its first
discovery and use in that country is still in doubt. One writer claims
that the famous herb was cultivated and classified in China 2000 B.
C., almost as completely as it is to-day, and that it was used as a
means of promoting amity between Eastern monarchs and
potentates at this early period. Chin-Nung, a celebrated scholar and
philosopher, who existed long before Confucius, is claimed to have
said of it: “Tea is better than wine, for it leadeth not to intoxication,
neither does it cause a man to say foolish things and repent thereof
in his sober moments. It is better than water, for it doth not carry
disease; neither doth it act as a poison, as doth water when the wells
contain foul and rotten matter,” and Confucius admonishes his
followers to: “Be good and courteous to all, even to the stranger from
other lands. If he say unto thee that he thirsteth give unto him a cup
of warm Tea without money and without price.”
A Chinese legend ascribes its first discovery to one Darma, a
missionary, famed throughout the East for his religious zeal, who, in
order to set an example of piety to his followers, imposed on himself
various privations, among which was that of forswearing sleep. After
some days and nights passed in this austere manner, he was
overcome and involuntarily fell into a deep slumber, on awakening
from which he was so distressed at having violated his vow, and in
order to prevent a repetition of allowing “tired eyelids to rest on tired
eyes,” he cut off the offending portions and flung them to the ground.
On returning the next day, he discovered that they had undergone a
strange metamorphosis, becoming changed into a shrub, the like of
which had never been seen before. Plucking some of the leaves and
chewing them he found his spirits singularly exhilarated, and his
former vigor so much restored that he immediately recommended
the newly discovered boon to his disciples.
Tradition, on the other hand, never at a loss for some marvelous
story, but with more plausibility, claims that the use of Tea was first
discovered accidentally in China by some Buddhist priests, who,
unable to use the brackish water near their temple, steeped in it the
leaves of a shrub, growing in the vicinity, with the intention of
correcting its unpleasant properties. The experiment was so
successful that they informed the inhabitants of their discovery,
subsequently cultivating the plant extensively for that express
purpose. While another record attributes its first discovery about
2737 B. C. to the aforementioned Chin-Nung, to whom all agricultural
and medicinal knowledge is traced in China. In replenishing a fire
made of the branches of the Tea plant, some of the leaves fell into
the vessel in which he was boiling water for his evening meal. Upon
using it he found it to be so exciting and exhilarating in its effects that
he continued to use it; imparting the knowledge thus gained to
others, its use soon spread throughout the country.
These accounts connected with the first discovery of the Tea plant in
China are purely fabulous, and it is not until we come down to the
fourth century of the Christian era that we can trace any positive
allusion to it by a Chinese writer. But, as the early history of nearly
every other ancient discovery is more or less vitiated by fable, we
ought not to be any more fastidious or less indulgent towards the
marvelous in the discovery of Tea than we are towards that of fire,
iron, glass or coffee. The main facts may be true, though the details
be incorrect; and, though the accidental discovery of fire may not
have been made by Suy-Jin in the manner claimed, yet it probably
was communicated originally by the friction of two sticks. Nor may it
be strictly correct to state that Fuh-he made the accidental discovery
of iron by the burning of wood on brown earth any more than the
Phœnicians discovered the making of glass by burning green wood
on sand, yet it is not improbable that some such accidental
processes first led to these discoveries. Thus, also, considerable
allowances are to be deducted from the scientific discoveries of
Chin-Nung in botany, when we read of his having, in one day,
discovered no less than seventy different species of plants that were
poisonous and seventy others that were antidotes against their
baneful effects.
According to some Chinese authorities, the Tea plant was first
introduced into their country from Corea as late as the fourth century
of the present era, from whence it is said to have been carried to
Japan in the ninth. Others again maintaining that it is undoubtedly
indigenous to China, being originally discovered on the hills of those
provinces, where it now grows so abundantly, no date, however,
being named. While the Japanese, to whom the plant is as valuable
as it is to the Chinese, state that both countries obtained it
simultaneously from Corea, about A. D. 828. This latter claim not
being sustained by any proof whatever—Von Siebold, to the contrary
—who, relying on the statements of certain Japanese writers to this
effect, argues in support of their assertions, the improbability of
which is unconsciously admitted by Von Siebold himself when he
observes “that in the southern provinces of Japan the tea plant is
abundant on the plains, but as the traveler advances towards the
mountains it disappears,” hence inferring that it is an exotic. The
converse of this theory holding good of China, a like inference tends
to but confirm their claim that with them the plant is indigenous. That
the Japanese did not originally obtain the plant from Corea but from
China is abundantly proven by the Japanese themselves, many of
whom admit that it was first introduced to their country from China
about the middle of the ninth century. In support of this
acknowledgment it is interesting to note, as confirming the Chinese
origin of tea, that there is still standing at Uji, not far from Osaka, a
temple erected on what is said to have been the first tea plantation
established in Japan, sacred to the traditions of the Japanese and in
honor of the Chinese who first introduced the tea plant into the Island
empire. Another more authentic account states that the Tea-seed
was brought to Japan from China by the Buddhist priest Mi-yoye,
about the beginning of the thirteenth century, and first planted in the
southern island of Kiusiu, from whence its cultivation soon spread
throughout that country.
Some English writers go so far as to claim that Assam, in India, is
the original country of tea, from the fact that a species has been
discovered there in a wild state as well as in the slopes of the
Himalaya mountains. But though found in both a wild and cultivated
state in many countries of the East at the present time, all its
Western traditions point to China, and to China only, as the original
country of Tea, and that the plant is native and indigenous to that
country is indisputably beyond question.
It was not known to the Greeks or Romans in any form; and that it
could not have been known in India in very early times is inferred
from the fact that no reference to the plant or its product is to be
found in the Sanscrit. But that the plant and its use, not only as an
agreeable and exhilarating beverage, but as an article of traffic
worthy of other nations, must have been known to the Chinese as
early as the first century of the Christian era, the following extract
from an ancient work entitled the “Periplous of the Erythræan Sea,”
may serve to prove. The author, usually supposed to be Arryan, after
describing “a city called Thinæ,” proceeds to narrate a yearly
mercantile journey to the vicinity of “a certain people called Sesatæi,
of short stature, broad faces, and flat noses”—evidently natives of
China—adds “that the articles they bring for traffic outwardly
resemble vine leaves, being wrapped in mats, which they leave
behind them on their departure to their own country in the interior.
From these mats the Thinæ pick out a haulm, called petros, from
which they draw the fibre and stalks; spreading out the leaves, they
double and make them up into balls, passing the fibre through them,
in which form they take the name of Malabathrum, and under this
name they are brought into India by those who so prepare them.”
Under any interpretation this account sounds like a remote, obscure
and confused story. Still one of the authors of the able “Historical
Account of China,” published in 1836, has ventured to identify this
Malabathrum of the Thinæ with the Tea of the Chinese. Vossius
Vincent and other authors, while admitting the difficulty of
understanding why it should be carried from Arracan to China, and
from China back to India, unhesitatingly assert that Malabathrum
was nothing more than the Betel-leaf, so widely used in the East at
the time as a masticatory. Horace mentions Malabathrum, but only
as an ointment. Pliny refers to it both in that sense and as a
medicine. Dioscorides describing it as a masticatory only. While the
author of the “Historical Account” prefers to consider the passage in
the Periplous as a very clumsy description of a process not
intelligently understood by the describer, but as agreeing far better
with the manipulation of Tea than with that of the Betel-leaf, and his
conjecture, unsupported as it is, merits citation if only for its
originality.
The first positive reference to Tea is that by Kieu-lung in the fourth
century, who not only describes the plant, but also the process of
preparing it, of which the following is a free and condensed
translation: “On a slow fire set a tripod, whose color and texture
show its long use, and fill it with clear snow-water. Boil it as long as
would be sufficient to turn cray-fish red, and throw it upon the
delicate leaves of choice Tea. Let it remain as long as the vapor
arises in a cloud and only a thin mist floats on the surface. Then at
your ease drink the precious liquor so prepared, which will chase
away the five causes of sorrow. You can taste and feel, but not
describe the state of repose produced by a beverage thus prepared.”
It is again mentioned by Lo-yu, a learned Chinese, who lived during
the dynasty of Tang, in 618, who became quite enthusiastic in its
praise, claiming that “It tempers the spirits, harmonizes the mind,
dispels lassitude and relieves fatigue, awakens thought and clears
the perceptive faculties,” and according to the Kiang-moo, an
historical epitome, an impost duty was levied on Tea as early as 782
by the Emperor Te-Tsing, and continued to the present day.
McPherson, in his “History of European Commerce with India,”
states that Tea is mentioned as the usual beverage of the Chinese
by Solieman, an Arabian merchant, who wrote an account of his
travels in the East about the year 850. By the close of the ninth
century, however, Tea was found in general use among the Chinese,
the tax upon it at that time being a source of considerable revenue
as recorded by Abuzeid-el-Hazen, an Arabian traveler cited by
Renaudot in a translation of his work. There is also independent
evidence furnished by two other Arabian travelers in a narrative of
their wanderings during the latter half of the ninth century, admitting
their statements to be trustworthy as to the general use of Tea as a
beverage among the Chinese at that period. Moorish travelers
appear to have introduced it into Mohammedan countries early in the
tenth century, and other travelers in China in the seventeenth give
most extravagant accounts of its virtues, which appears to have
been in very general use throughout the greater part of Asia at that
time.
Father de Rhodes, a Jesuit missionary, who entered China in 1633,
states that “the use of Tea is common throughout the East, and
begins, I perceive, to be known in Europe. It is in all the world to be
found only in two provinces of China, where the gathering of it
occupies the people as the vintage does us.” Adding that he found it
in his own case to be an instantaneous remedy for headache, and
when compelled to sit up all night to hear confessions its use saved
him from drowsiness and fatigue. Adam Olearius, describing the
travels of an embassy to Persia in 1631, says of the Persians: “They
are great frequenters of taverns, called Tzai Chattai, where they
drink Thea or Cha, which the Tartars bring from China, and to which
they assign extravagant qualities, imagining that it alone will keep a
man in perfect health, and are sure to treat all who visit them to this
drink at all hours.” These strong expressions as to the use of Tea,
applying as they do to a period not later than 1640, are sufficient to
prove that the ordinary accounts place the introduction of that
beverage as regards Europe, particularly the Continent, as too late.
INTRODUCTION INTO EUROPE.

The earliest European notice of Tea is that found in a work by
Ramusio, first printed in 1550, though written several years prior to
that year. In it he quotes Hazzi Mohamed in effect, “And these
people of Cathay (China) do say that if these in our parts of the world
only knew of Tea, there is no doubt that our merchants would cease
altogether to use Ravino Cini, as they call rhubarb.” Yet no accounts
at present accessible establish the date of its first introduction into
Europe, and it is also a difficult matter to determine to which of the
two nations—Portugal or Holland—the credit of first introducing it
belongs. Some writers claiming that the Dutch East India Company
brought Tea to Amsterdam in 1600, while the Portuguese claim the
honor of its first introduction prior to that year. An indisputable
argument in favor of the latter is the notice given of it by Giovani
Maffei in his “History of India,” published in 1559. “The inhabitants of
China, like those of Japan,” he writes, “extract from an herb called
Chia a beverage which they drink warm, and which is extremely
wholesome, being a remedy against phlegm, languor and a promoter
of longevity.” While Giovani Botero, another Portuguese, in a work
published in the same year, states that “the Chinese have an herb
from which they press a delicate juice, which they use instead of
wine, finding it to be a preservative against these diseases which are
produced by the use of wine amongst us.” Taxiera, also a native of
Portugal, states that he saw the dried leaves of Tea at Malacca some
years prior to 1600, and the article is also mentioned in one of the
earliest privileges accorded to the Portuguese for trading in 1558; yet
it was not until nearly a century from the beginning of that trade that
we find the first distinct account from a European pen of the use of
Tea as a beverage.
In a “Dissertation upon Tea, by Thomas Short,” printed in London, in
1730, the author gives the following account of its first introduction
into Europe: “The Dutch East India Company on their second voyage
to China carried thither a good store of Sage and exchanged it with
the Chinese for Tea, receiving three to four pounds of the last for one
pound of the first, by calling it a wonderful European herb possessed
of as many virtues as the Indians could ascribe to their shrub-leaf.
But because they exported not such large quantities of Sage as they
imported of Tea they also bought a great deal of the latter, giving
eight- to tenpence a pound for it in China. And when they first
brought it to Paris they sold it for thirty livres the pound; but thirty
years ago the Chinese sold it at threepence, and never above
ninepence a pound at any time, frequently mixing it with other herbs
to increase the quantity.” Macaulay also states in the history of his
embassy to China that “early in the seventeenth century some Dutch
adventurers, seeking for such objects as might fetch a price in
China, and hearing of a general use there of a beverage produced
from a plant of the country, bethought themselves of trying how far a
European plant of supposed great virtues might also be appreciated
by the Chinese; they accordingly introduced to them the herb Sage,
the Dutch accepting in exchange the Chinese Tea, which they
brought back with them to Holland.” These statements but tend to
confirm the Portuguese claim, the efforts of the Dutch to open up
trade with the Chinese in Tea being evidently made many years
subsequent to its introduction by the former; in still further support of
which the following may be noted:—
In 1662 Charles II. married the Portuguese princess, Catharine of
Braganza, who, it is said, was very fond of Tea, having been
accustomed to it in her own country. Waller, in a poem celebrating
the event, ascribes its first introduction to her country in the
appended lines:—
“Venus her myrtle has—Phœbus her bays;

Tea both excels, which she vouchsafes to praise.
The best of Queens and best of herbs we owe
To that proud nation which the way did show.”
The earliest mention made of Tea by an Englishman is that

contained in a letter from a Mr. Wickham, agent of the East India
Company at Firando, Japan, and dated June 27, 1615, to a Mr.
Eaton, another officer of the Company, resident at Macao, China,
asking for “a pot of the best Cha.” How the commission was
executed does not appear, but in Mr. Eaton’s subsequent account of
expenditures occurs this item, “Three silver porringers to drink Tea
in.” The first person, however, to advocate the use of Tea in Europe
was Cornelius Bottrekoe, a professor of the Leyden University, who,
in a treatise on “Tea, Coffee and Chocolate,” published in 1649,
strongly pronounces in favor of the former, denying the possibility of
its being injurious even when taken in immoderate quantities.
Tea was evidently known in England previous to its direct importation
there, small quantities having been brought from Holland as early as
1640, but used only on rare occasions. The earliest mention made of
it, however, is that contained in a copy of the “Mercurius Politicus,” at
present in the British Museum, and dated September, 1658, in which
attention is called to “that excellent, and by all Physitians approved,
China drink, called by the Chineans Tcha, by other nations Tay, sold
at the Sultaness Head, a Cophee-house by the Royal Exchange,
London.” The most famous house for Tea at this early period,
however, was Garway’s, more popularly known for upwards of two
centuries as “Garraway’s,” being swept away only a few years ago
by the march of improvement. Defoe refers to it as being “frequented
only by people of quality, who had business in the city and the
wealthier citizens”; but later it became the resort of speculators, and
here it was that the numerous schemes which surrounded and
accompanied the “Great South Sea Bubble” had their centre, and,
appropriately enough, “Garraway’s” was also the headquarters of
that most remarkable but disastrous Tea speculation of 1842.
A singular handbill issued by its founder is still extant, being
discovered by accident in a volume of pamphlets found in the British
Museum, where it may still be inspected. Although the document
bears no date, there is ample internal evidence to prove that it must
have been printed about 1660. It is a quaint and extraordinary
production, purporting to be “An exact description of the leaf Tea,
made according to the directions of the most knowing merchants and
travelers in those Eastern countries, by Thomas Garway,” setting
forth that:—
“Tea is generally brought from China, growing there on little shrubs, the
branches whereof are garnished with white flowers of the bigness and
fashion of sweetbriar, but smell unlike, and bearing green leaves of the
bigness of myrtle or sumac, which leaves are gathered every day, the
best being gathered by virgins who are destined for the work, the said
leaves being of such known virtues that those nations famous for
antiquity, knowledge and wisdom do frequently sell it among themselves
for twice its weight in silver. That it hath been used only as a regalia in
high treatments and entertainments, presents being made thereof to
grandees.”
Proceeding at considerable length to enumerate its “virtues,” many

of which are decidedly apocryphal, and attributing to the beverage,
among its other properties, that of—
“Making the body active and lusty, helping the headache, giddiness and
heaviness, removing the difficulty of breathing, clearing the sight,
banishing lassitude, strengthening the stomach, causing good appetite
and digestion, vanishing heavy dreams, easing the frame, strengthening
the memory, and finally preventing consumption, particularly when drank
with milk.”
Many other remarkable properties being credited to this wonderful

“Chinese herb,” the advertiser closes his great encomiums by
suggesting—
“That all persons of eminence and quality, gentlemen, and others who
have occasion for tea in the leaf may be supplied. These are to give

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Public Policy in ALS/MND Care: An

International Perspective 1st ed. Edition

ISBN 978-981-15-5839-9 ISBN 978-981-15-5840-5 (eBook)

Cover illustration: © Ed Reschke/Getty Images

When I was a neuromuscular fellow back in 1980, I would observe how

biotech companies. All these stakeholders are working to improve ALS

grown into a far more complicated web with an overwhelming amount of

Wesley J Howe Professor of Neurology (at CUIMC) Hiroshi Mitsumoto

This book was first proposed by the three editors to be a co-authored

primary focus on policy, authors were requested not to include general

Sarasota, FL Robert H. Blank

1 Introduction to Public Policy of ALS/MND 1

2 Public Policy in MND Care: The Australian Perspective 29

3 Public Policy in ALD/MND Care:

4 Amyotrophic Lateral Sclerosis in Brazil 65

5 Public Policy of ALS in Canada 77

6 Public Policy for Amyotrophic Lateral Sclerosis

7 German Perspective on ALS/MND Policy107

8 Living and Dying with ALS/MND in India:

9 The Experience of Amyotrophic Lateral Sclerosis

10 Public Policy of ALS Care in Israel149

11 Public Policy in ALS Care: The Italian Situation163

12 ALS Policy: A Japanese Perspective177

13 ALS Policy in Mexico191

14 Public Policy in MND Care: Nigerian Perspective201

15 Public Policy of ALS: A Pakistani Perspective209

16 Public Policy in ALS Care: The Polish Perspective219

17 ALS Policy: A Russian Perspective227

18 Public Policy in ALS/MND Care: South African

19 Public Policy in ALS Care: South Korea247

20 Amyotrophic Lateral Sclerosis Care in Tunisia263

21 Public Policy in MND Care: The United Kingdom279

22 ALS Public Policy in the United States301

23 Conclusions: What We Can Learn from the Country

Anna Adamczyk is a specialist in neurology and in palliative medicine.

Samar Aoun is Professor of Palliative Care Research. She adopts a public

Northern Illinois) and a frequent guest professor at Aarhus University

Efrat Carmi is the CEO of IsrALS—The Israeli ALS Research

Marcondes C. França Jr. MD, PhD, is Associate Professor in the

A. B. Guekht is the director of the Moscow Research and Clinical Center

president of the Indian Academy of Neurology in 2018 and is the trea-

at the ALS outpatient clinic at UNICAMP hospital. Leoni is pursuing her

Hiroshi Mitsumoto MD, DSc, is the Wesley J. Howe Professor of

Administration from Tokyo Medical and Dental University in 2008.

scientific research on ALS, contacts with ALS researchers, collaborations

became a Director of International Cooperation in Korean ALS

M. N. Zakharova graduated from Sechenov Medical Institute in 1979.

Fig. 3.1 Kaplan-Meier curve of patients seen at the University Hospitals

Table 1.1 Conditions that can mimic ALS/MND 5

Introduction to Public Policy of ALS/MND

Jerome E. Kurent, David Oliver, and Robert H. Blank

© The Author(s) 2021 1

Keywords ALS • MND • Public policy • Cross-national studies

private foundations and public agencies dedicated to the optimum treat-

• ALS/MND, with both upper and lower motor neuron involvement,

stiffness, increased deep tendon reflexes and extensor plantar

The most common clinical presentation of ALS/MND is spinal-onset

Making the Diagnosis of ALS/MND: A Diagnosis

The neurologist must take a careful history and perform a meticulous

Sarasota, FL Robert H. Blank

1 Introduction to Public Policy of ALS/MND 1

2 Public Policy in MND Care: The Australian Perspective 29

3 Public Policy in ALD/MND Care:

4 Amyotrophic Lateral Sclerosis in Brazil 65

5 Public Policy of ALS in Canada 77

6 Public Policy for Amyotrophic Lateral Sclerosis

7 German Perspective on ALS/MND Policy107

8 Living and Dying with ALS/MND in India:

9 The Experience of Amyotrophic Lateral Sclerosis

10 Public Policy of ALS Care in Israel149

11 Public Policy in ALS Care: The Italian Situation163

12 ALS Policy: A Japanese Perspective177

13 ALS Policy in Mexico191

14 Public Policy in MND Care: Nigerian Perspective201

15 Public Policy of ALS: A Pakistani Perspective209

16 Public Policy in ALS Care: The Polish Perspective219

17 ALS Policy: A Russian Perspective227

18 Public Policy in ALS/MND Care: South African

19 Public Policy in ALS Care: South Korea247

20 Amyotrophic Lateral Sclerosis Care in Tunisia263

21 Public Policy in MND Care: The United Kingdom279

22 ALS Public Policy in the United States301

23 Conclusions: What We Can Learn from the Country

Table 1.1 Conditions that can mimic ALS/MND 5