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PARKINSON’S DISEASE


A Johns Hopkins Press Health Book


Parkinson’s Disease
A Complete Guide for
Patients and Families
  
William J. Weiner, M.D.
Lisa M. Shulman, M.D.
Anthony E. Lang, M.D., F.R.C.P.

The Johns Hopkins University Press
Baltimore & London


N   R. This book is not meant to substitute for medical care of
people with Parkinson’s disease, and treatment should not be based solely on its
contents. Instead, treatment must be developed in a dialogue between the individual and his or her physician. Our book has been written to help that dialogue.
© 2001 The Johns Hopkins University Press
All rights reserved. First edition 2001
Large print edition 2002

Printed in the United States of America on acid-free paper
987654321
The Johns Hopkins University Press
2715 North Charles Street
Baltimore, Maryland 21218-4363
www.press.jhu.edu
Library of Congress Cataloging-in-Publication Data
Weiner, William J.
Parkinson’s disease / William J. Weiner, Lisa M. Shulman,
Anthony E. Lang. — Large print ed.
p. cm.
ISBN 0-8018-6880-7 (lg. print)
1. Parkinson’s disease — Popular works. 2. Large type books.
I. Shulman, Lisa M. II. Lang, Anthony E. III. Title.
RC382. W45 2002
616.8Ј33—dc21
2001038915
A catalog record for this book is available from the British Library.
Illustrations on pages 7, 9, 38, 187, 189, 194, 234, and 292 by Jacqueline
Schaffer.


To Monica and Miriam Weiner. May you continue to
pursue your dreams and goals.
WJW
To Joshua and Corey Shulman. We continue to learn
together.
LMS
To Matthew, Stephen, and Kathryn Lang. Your happiness
and joy in life are always in my thoughts and prayers.

And in memory of the remarkable inspiration and love of
their grandfather Thomas Lang.
AEL



Contents
Preface ix
Acknowledgments xiii
PART I

Introduction

1. What Is Parkinson’s Disease? 3
2. Who Gets Parkinson’s Disease? 25
PART II
3.
4.
5.
6.
7.

Signs and Symptoms of Parkinson’s
Disease

Early Symptoms 43
Moderate Parkinson’s Disease 59
Advanced Parkinson’s Disease 82
Behavioral Changes and Psychiatric Symptoms 104
Young-Onset Parkinson’s Disease 121

PART III Diagnosing Parkinson’s Disease
8. How a Diagnosis Is Made 135
9. Types of Parkinsonism 152
10. Diagnosing Other Neurologic Problems 165


viii / CONTENTS

PART IV Treatment of Parkinson’s Disease
11.
12.
13.
14.

How the Brain Works and How Treatment Works 185
Choosing the Correct Medications 202
Drug Therapies 221
Diet, Exercise, and Complementary and Alternative
Therapies 266
15. Surgical Treatments 282
PART V

Other Issues

16. Illness, Hospitalization, and Parkinson’s Disease 305
17. Parkinson’s Disease Research 313
18. Questions and Answers 328

Resources 349

Index 355


Preface
This book has been in the planning stage for a considerable period of time. Each of us has been involved in the
care of patients with Parkinson’s disease for many years,
and we have become keenly aware of the need for a book
that will help patients and their families develop a fuller
understanding of what living with Parkinson’s is like.
When people first learn of the diagnosis of Parkinson’s
disease, they generally know very little about this illness.
Understandably, a myriad of questions arise. Many patients and families become alarmed as they translate
their limited experience and knowledge to themselves.
The fact is that the effect of Parkinson’s disease on people’s lives has been fundamentally altered in recent years,
as new and better treatments have become available.
Indeed, in the last few decades, Parkinson’s disease has
become a disorder that does not prevent people from living productive and satisfying lives for many years. Understanding the symptoms of Parkinson’s disease and
the management of the disease is the key to each person’s
ability to preserve his or her sense of stability and control. There are many examples of medical conditions
that are chronic illnesses, including arthritis, asthma, diabetes, and Parkinson’s disease. In every one of these examples, the recipe for living well with the disorder in-


x / PREFACE

volves having a clear understanding of both the medical
condition and the pivotal role that a prepared individual
can assume in the management of his or her own health.
For example, in making clinical decisions, a physician relies on the history and feedback that the patient and
caregivers provide. There is no one formula for the successful management of Parkinson’s disease. Instead, success relies upon an individualized and comprehensive
plan of care based on shared decision-making between

the experienced physician and the prepared patient.
We have written a book that explores Parkinson’s disease in straightforward and honest terms. We have explained what goes wrong in the brain which leads to the
disorder, and we have also explained how a physician
goes about making the diagnosis of Parkinson’s disease.
We have discussed the subtleties involved in diagnosis,
so people with Parkinson’s disease and their families understand the challenges that sometimes arise in arriving
at a correct diagnosis.
Parkinson’s disease is a progressive problem, which
gradually worsens with time. In separate chapters we
have delineated the common problems associated with
the early, middle, and advanced stages of Parkinson’s
disease. We not only discuss these problems in considerable detail but also offer numerous suggestions based on
our experiences regarding how to better live with these
problems.
Most people think that the major problems of Parkinson’s disease relate to problems with movement, including tremor and walking. However, patients with many


PREFACE / xi

years of experience with Parkinson’s are also familiar
with a variety of nonmotor symptoms. Therefore, we
have extensively reviewed the many common nonmotor
symptoms that may occur, including depression, apathy,
anxiety, sweating, sexual dysfunction, memory problems, sleep disturbance, bladder problems, and constipation.
The role of drug therapy in the treatment of Parkinson’s disease is thoroughly discussed in this book. In the
last thirty years, impressive advances in the therapy of
Parkinson’s disease have been made. We outline how
Parkinson’s drugs work, why they are helpful, the side effects associated with them, and what patients can realistically expect of each of them. How to use medications
safely and effectively, and the various drug combinations, are also thoroughly reviewed, as is drug therapy
for the various non-motor symptoms.

The role of surgery in the treatment of Parkinson’s
disease is rapidly evolving. We review the different types
of surgery that can be of value in Parkinson’s disease.
Importantly, we present the key questions that patients
and families should ask themselves and their physician
before considering surgery as a therapeutic option.
The book closes with a long chapter of commonly
asked questions and clear and concise answers to these
many difficult questions that often arise during an office
visit. (The subject of each question and answer is covered in much greater detail in the many chapters within
the book.)
Our experience with taking care of patients with Par-


xii / PREFACE

kinson’s disease teaches us that well-informed, knowledgeable patients do better over time. Our goal in writing
this book is to contribute to a foundation of knowledge
for persons who are living with Parkinson’s disease in
order to help them make healthy adjustments to these
changes, to develop competency in self-monitoring and
self-management, and to become effective partners in
shared decision-making with their physician. The chapters of the book can be read in sequence but they can
also be read separately. To permit this approach we have
incorporated a certain amount of duplication in the
information provided. For those reading chapters in sequence this can serve to reinforce the important issues
covered.


Acknowledgments

We wish to thank all the patients and families whom we
have taken care of over the years, for sharing with us
their insights and responses to Parkinson’s disease. The
spirit that the Parkinson’s community exhibits is an enduring inspiration. We hope that we successfully communicate the world conveyed by our patients to the
larger community of persons and families affected by
Parkinson’s disease.
We thank Maria Macias, who has diligently supported
us with fine administrative assistant skills during the
preparation of this book. We thank our editors at the
Johns Hopkins University Press, Jacqueline Wehmueller,
Alice Lium, and Linda Strange, who helped us transform
our initial text into an easily readable and patientfriendly format. We also thank special benefactors who
have generously supported our research on this project
as well as many other Parkinson’s disease related projects: Rosalyn Newman, Morton Shulman, and Jack and
Mary Clark.



PART I

Introduction



CHAPTER

1

What Is Parkinson’s Disease?
• What are the symptoms of Parkinson’s disease?

• What causes these symptoms?
• What is the difference between Parkinson’s disease
and parkinsonism?
• When should I tell other people that I have Parkinson’s?

Whenever a diagnosis of Parkinson’s disease is made, patients and families naturally ask, “What is Parkinson’s
disease?” As doctors treating people with Parkinson’s, of
course, we discuss this question with many patients. But
we—and they—are aware that the answer is both straightforward and elusive.
Parkinson’s disease is a degenerative neurologic disease. Degenerative means “declining in quality”—thus,
the disease increases in severity over time; neurologic
refers to the nervous system. One could therefore say
that Parkinson’s disease is a disease of the nervous system that gets worse over time.
We also describe Parkinson’s disease as a chronic, progressive neurologic disease. Chronic means “of long duration”; progressive means “proceeding in steps” or “ad-


4 / INTRODUCTION

vancing.” Parkinson’s disease does not go away, and it
gradually gets worse.
Parkinson’s disease is named after the English physician James Parkinson, who first described the illness. His
original paper describing this disorder, published in
1817, was entitled “Essay on the Shaking Palsy,” and to
this day Parkinson’s disease is still sometimes called the
“shaking palsy.” Another name for this illness is paralysis agitans, which is simply the Latin translation of
“shaking palsy.” The names Parkinson’s disease, shaking
palsy, and paralysis agitans all refer to the same illness.
There is some fairly good news. True Parkinson’s disease progresses slowly. Even after the symptoms have become serious enough and clear enough to allow a definitive diagnosis, it is usually years, maybe a decade or
more, before a person suffers from a serious disability.
Further, treatments are available that can relieve symptoms, so that years, sometimes a decade or more, can go

by before symptoms have a significant impact on a person’s quality of life.
In people with Parkinson’s disease, specific groups of
brain cells called neurons are slowly and progressively injured, then selectively degenerate or die. This process
causes the typical symptoms of Parkinson’s disease,
which doctors call “characteristic symptoms” because
they are the major features of Parkinson’s. (In this book,
when we say symptoms are “characteristic” of or “characterize” a disorder, we mean they are typical of that particular disorder and distinguish it from others.)
These are the characteristic symptoms of Parkinson’s


WHAT IS PARKINSON’S DISEASE? / 5

disease: People who have Parkinson’s may tremble involuntarily. They find their muscles become rigid and
stiff, and they lose their ability to make rapid, spontaneous movements. They walk in a recognizable manner,
with a typical gait in which the body is bent or flexed,
and they may have difficulty maintaining their balance.
The characteristic symptoms of moderate Parkinson’s
disease can be remembered with the acronym TRAP: T
is for tremor and R for rigidity. A is for akinesia (meaning, literally, “lack of movement”), referring to the loss
of spontaneous or voluntary movement and loss of fluid
motion (the slowing down, rather than complete loss, of
movement is called bradykinesia). P is for postural instability, which involves difficulties with balance and the
risk of falling (Table 1.1). Parkinson’s has neither a cure
nor any treatment to slow down its progression.
Furthermore, the signs and symptoms* of early Parkinson’s are only subtly different from those of other diseases, some more serious and some less serious than
Parkinson’s. The similarities among these diseases can
make diagnosis difficult, and as frustrating as it may be
for a person with Parkinson’s symptoms, often the only
way to identify Parkinson’s disease for sure is to wait and
see (see Chapter 8).

*

Symptoms are what the patient complains of; signs are what the
doctor finds on examination. Parkinson’s has both signs and symptoms that are typical of the disease. We discuss them in depth in
later chapters.


6 / INTRODUCTION

Table 1.1
Characteristic Signs and Symptoms of Parkinson’s
Disease

Image not available.

What Happens in Parkinson’s Disease?
In Parkinson’s disease, neurons (nerve cells) of the brain
area known as the substantia nigra (Latin for “black substance”) are primarily affected (Figure 1.1). When neurons in the substantia nigra degenerate, the brain’s ability to generate body movements is disrupted and this
disruption produces signs and symptoms characteristic
of Parkinson’s disease—tremor; rigidity; akinesia (lack
of movement or loss of spontaneous movement) and
bradykinesia (slowness of movement); and problems
with walking and posture.


Image not available.

FIGURE 1.1 This figure shows the location of the substantia nigra (the area of the brain which contains dopamine
cells), deep within the brain. The large cerebral hemispheres enclose and cover the substantia nigra as well as

other deep midbrain structures.


8 / INTRODUCTION

The symptoms of any brain disease are determined in
part by the location of the neurons that degenerate. For
example, Alzheimer’s disease involves the degeneration
of neurons of the cerebral cortex and results in memory
loss and mental deterioration. In amyotrophic lateral
sclerosis (ALS, or Lou Gehrig’s disease), the selective
deaths of motor neurons in the spinal cord and brain
cause profound motor weakness. Again, in Parkinson’s
disease, the affected neurons are located in the substantia nigra, an area of the brain that is important for control and regulation of motor activity (movement).

What Causes the Symptoms?
The substantia nigra is a very small area located deep
within the brain. There is one substantia nigra on the
right side of the brain and one on the left, but for ease of
discussion, the medical literature refers to them as if they
were a single structure. The symptoms of Parkinson’s
disease do not become noticeable until about 80 percent
of the cells of the substantia nigra have died, because the
human nervous system has multiple safety factors and
redundancies built into it. For a long time, these safety
factors are able to take over the activities of the dying
cells.
In autopsies of persons with Parkinson’s disease, the
brain appears to be relatively normal except that the substantia nigra has lost its usual black pigment (Figure
1.2). Under the microscope we can see substantially

fewer cells in this substantia nigra than in that of healthy


Image not available.

FIGURE 1.2 The top panel shows the normal substantia nigra with its dark pigment which gives it the characteristic black color. This is the actual appearance that is seen
by the naked eye when this part of the brain is examined.
The bottom panel with the faded pale area in the region
of the substantia nigra is the characteristic finding seen
with the naked eye in the brain of a patient with Parkinson’s disease. The loss of the dopamine-containing pigmented black cells within the substantia nigra is the
landmark pathologic feature of Parkinson’s disease.


10 / INTRODUCTION

brains, and the remaining cells often show signs of abnormality. One hallmark of Parkinson’s disease is the
presence of small bodies known as Lewy bodies within
the remaining substantia nigra cells.
The substantia nigra accounts for an extremely small
percentage of the brain’s weight, but because of its important electrochemical connections with motor centers
(brain centers that control movement), it is a vital component in how we move. Specifically, a series of complicated electrical and chemical events within the brain
transmits information from neuron to neuron. The
chemicals that brain cells use to communicate with one
another are called neurotransmitters or, more generally,
neurochemicals. The specific neurotransmitter produced
and used by the substantia nigra is dopamine. When the
cells of the substantia nigra degenerate and die, dopamine is lost and dopamine-relayed messages to other
motor centers cannot go through. This is the primary
cause of the motor symptoms in Parkinson’s disease.
There is more. While the loss of dopamine-producing

cells is the primary neurochemical disturbance in Parkinson’s, the neurochemical disturbances are not limited
to the cells of the substantia nigra and to the loss of dopamine. Other small nuclear centers within the brain
(e.g., the regions called the dorsal motor nucleus of the vagus and the locus ceruleus) also are affected by the degeneration. In Parkinson’s disease, as the concentrations
of dopamine in the brain decline, so do concentrations
of other neurotransmitters such as norepinephrine and
serotonin, although changes in these other neurotrans-