C U R R E N T C L I N I C A L P R A C T I C E
P
ARKINSON
’
S
D
ISEASE
AND
M
OVEMENT
D
ISORDERS
Edited by
CHARLES H. ADLER, MD, PhD
J. ERIC AHLSKOG, PhD, MD
HUMANA PRESS
D
IAGNOSIS

AND
T
REATMENT
G
UIDELINES

FOR

THE
P
RACTICING
P

edited by P
HIL LIEBERMAN AND JOHN A. ANDERSON, 1997
Osteoporosis: Diagnostic and Therapeutic Principles,
edited by C
LIFFORD J. ROSEN, 1996
PARKINSON'S DISEASEAND
MOVEMENT DISORDERS
D
IAGNOSIS AND
T
REATMENT
G
UIDELINES
FOR THE
P
RACTICING
P
HYSICIAN
HUMANA PRESS
TOTOWA, NEW JERSEY
Edited by
CHARLES H. ADLER, MD, PHD
Consultant and Co-Director, Parkinson’s Disease and Movement
Disorders Center, Department of Neurology, Mayo Clinic Scottsdale,
Scottsdale, AZ; Associate Professor of Neurology, Mayo Medical
School, Rochester, MN
and
J. ERIC AHLSKOG, PHD, MD
Chair, Division of Movement Disorders, and Consultant,
Department of Neurology, Mayo Clinic Rochester, Rochester, MN;

PREFACE
v
The field of movement disorders is relatively broad, encompassing disorders of in-
creased movement, such as tremors, dystonia, and tics, to disorders characterized by a
paucity of movement, such as Parkinson’s disease. Our understanding of the pathogenic
mechanisms and our treatment options are expanding at a rapid pace. This expansion
ranges from the medical and surgical advances in treating Parkinson’s disease to the flood
of genetic abnormalities that have now been found to cause various movement disorders.
Although many patients are seen by the movement disorders specialist in neurology
clinics around the country, most of these patients receive their follow-up care from a
primary care physician or “general” neurologist who must be versed in the characteristics
and treatment plans of this diverse group of disorders.
The major goal of Parkinson’s Disease and Movement Disorders: Diagnosis and
Treatment Guidelines for the Practicing Physician is to distill this immense amount of
information and to educate the practitioner about the many facets of the movement
disorders field. We believe that this book fills a large void, since most texts on movement
disorders are more detailed and geared toward the specialist. We have asked the chapter
authors to emphasize the clinical characteristics of each disorder, discuss the differential
diagnosis and the diagnostic testing, and then outline the various treatment options, as if
they were teaching during a preceptorship in their clinic. To this end, we have not de-
signed the book to be an exhaustive review of each topic; rather, it takes a general
approach to each subject. We have avoided referencing each statement; a short list of
further recommended reading sources is given at the end of each chapter.
The purpose of this text is to help the practitioner distinguish which disorder is being
encountered, give a basic understanding for test and treatment options that are required,
and synthesize any recommendations made by a consulting specialist. As the movement
disorders specialist becomes busier and insurance regulations limiting specialty referrals
increase, the burden of caring for these patients by the primary care physician will
continue to grow. Thus, we hope that this text will offer the reader full confidence in
approaching patients with movement disorders.

We have divided the discussion of the medical treatment of PD into three chapters. The
issue of whether our current drug arsenal includes medications that will slow the progres-
sion of PD is hotly debated, and in Chapter 7, Dr. Ahlskog tackles the theories and
practical issues for the practitioner. Chapter 8, also by Dr. Ahlskog, is devoted to the
various treatment options available for the patient with newly diagnosed PD. This in-
cludes decision-making regarding the use of levodopa, dopamine agonists, and numerous
other agents. The complicated issue of how to treat the patient with more advanced PD
is covered by Dr. Ryan Uitti in Chapter 9.
Patients with PD also have nonmotor manifestations that can be as disabling as the tremor
and bradykinesia. The sleep problems of PD, including insomnia and daytime drowsiness,
are addressed by Dr. Cynthia Comella in Chapter 10. Neurogenic bladder and bowel prob-
lems and symptomatic orthostatic hypotension are common issues in the PD clinic; these
autonomic problems and treatment strategies are covered by Dr. Bradley Hiner in Chapter 11.
Dementia, psychosis, and depression can be overwhelming factors in the patient with more
advanced PD, and Dr. Erwin Montgomery discusses these in Chapter 12.
Currently, the most visible topic concerning PD is surgical treatment, which has made
newspaper and television headlines for the past several years. In Chapter 13, Dr. Kathleen
Shannon discusses neurosurgical intervention, including pallidotomy, thalamotomy, deep
brain stimulation, and cerebral transplantation, and reviews the prospects for the future.
She provides guidelines on which patients may benefit and which of the different proce-
dures may be appropriate for a given patient.
Therapy for patients with PD does not end with medications and surgery; Chapter 14,
written by Drs. Padraig O’Suilleabhain and Susan Murphy, addresses adjunctive treat-
ments. They include nutrition and dietary issues, which are especially important in ad-
vancing disease. They also address the role of physical therapy in management of
parkinsonian motor problems.
All disorders characterized by slowness of movement are not PD, and in Section C we
have devoted six chapters to discussing these other disorders. In Chapter 15, Drs. Eric
Molho and Stewart Factor cover secondary causes of parkinsonism, such as vascular,
toxic, and traumatic etiologies; they also provide a practical strategy for the workup of

confused with primary choreiform syndromes. These iatrogenically induced conditions
are discussed by Dr. Kapil Sethi in Chapter 26.
The lightning-like jerks of myoclonus occasionally cause diagnostic confusion; if
repetitive, they may resemble tremor or the phasic movements seen in some dystonic
conditions. In Chapter 27, Dr. Caviness discusses diagnostic criteria, categorization, and
treatment of myoclonus.
Simple spasm of muscle may have a wide variety of causes, ranging from peripheral
to central nervous system origins. In the most elementary sense, the concept of muscle
spasm should include the sustained muscle contraction state of dystonia. Primary disor-
ders characterized by muscle spasm, however, have their own distinguishing features,
which separate them from primary dystonias. These disorders of muscle spasm, including
the prototypical condition, stiff-man syndrome, are discussed by Dr. Michel Harper in
Chapter 28.
The most common movement disorder of childhood is that of tics. This problem,
however, is not confined to children and occasionally confronts internists with adult
practices. The spectrum of motor and other tics, as well as the constellation of symptoms
that make up Tourette’s syndrome, is the topic of Chapter 29 by Drs. Kathleen Kujawa
and Christopher Goetz.
A disorder that has gained much recognition in the past few years is restless legs
syndrome, discussed by Drs. Virgilio Evidente and Charles Adler in Chapter 30. Dr.
Preface vii
Adler then covers the various uses of botulinum toxin (Chapter 31), an injectable agent
that reduces movement and has application for treating multiple different movement
disorders. This drug has revolutionized the treatment of dystonia and certain other hyper-
kinetic movement disorders.
We conclude this book with Section E, which covers other movement disorders, those
that do not fit well into previous sections. Dr. Katrina Gwinn-Hardy, in Chapter 32,
discusses the autosomal recessively inherited disorder Wilson’s disease, which can
present with hyperkinetic or bradykinetic features. This is critical to diagnose, since
treatment is available; if unrecognized, it can result in irreversible neurologic damage and

1 Approach to the Patient With a Movement Disorder:
Basic Principles of Neurologic Diagnosis 3
J. Eric Ahlskog, PhD, MD
2 Motor Speech Disorders: Clues to Neurologic Diagnosis 35
Joseph R. Duffy, PhD
B. PARKINSON’S DISEASE 55
3 What Is Parkinson’s Disease? Neuropathology, Neurochemistry,
and Pathophysiology 57
Howard Hurtig, MD
4 Clinical Features of Parkinson’s Disease 71
Richard B. Dewey, Jr., MD
5 Epidemiology and Genetics of Parkinson’s Disease 85
Demetrius M. (Jim) Maraganore, MD
6 Parkinson’s Disease: Etiologic Considerations 91
Peter A. LeWitt, MD
7 Medication Strategies for Slowing the Progression
of Parkinson’s Disease 101
J. Eric Ahlskog, PhD, MD
8 Initial Symptomatic Treatment of Parkinson’s Disease 115
J. Eric Ahlskog, PhD, MD
9 Advancing Parkinson’s Disease and Treatment
of Motor Complications 129
Ryan J. Uitti, MD
10 Sleep and Parkinson’s Disease 151
Cynthia L. Comella, MD, ABSM
11 Autonomic Complications of Parkinson’s Disease 161
Bradley C. Hiner, MD
12 Treatment of Cognitive Disorders and Depression
Associated With Parkinson’s Disease 175
Erwin B. Montgomery, Jr., MD

Daniel Tarsy, MD
24 Hemifacial Spasm 313
Mark F. Lew, MD
25 Huntington’s Disease and Other Choreas 321
John N. Caviness, MD
26 Tardive Dyskinesias 331
Kapil D. Sethi, MD, FRCP
27 Myoclonus 339
John N. Caviness, MD
28 Spasms and Stiff-man Syndrome 351
C. Michel Harper, Jr., MD
x Contents
29 Gilles de la Tourette’s Syndrome and Tic Disorders 365
Kathleen A. Kujawa, MD, PhD, and Christopher G. Goetz, MD
30 Restless Legs Syndrome 373
Virgilio Gerald H. Evidente, MD, and Charles H. Adler, MD, PhD
31 Botulinum Toxin Treatment of Movement Disorders 385
Charles H. Adler, MD, PhD
E. OTHER MOVEMENT DISORDERS 395
32 Wilson’s Disease 397
Katrina A. Gwinn-Hardy, MD
33 Gait Disorders: Recognition of Classic Types 411
Frank A. Rubino, MD
34 Post-traumatic Movement Disorders 427
Sotirios A. Parashos, MD, PhD
35 Psychogenic Movement Disorders: Theoretical and Clinical
Considerations 435
David S. Glosser, ScD, and Matthew B. Stern, MD
Appendix 453
Index 459

YNTHIA L. COMELLA, M.D., A.B.S.M. • Associate Professor, Department of Neurological
Sciences, Department of Psychology, Rush Medical College, Rush-Presbyterian-St. Luke’s
Medical Center, Chicago, Illinois
R
ICHARD B. DEWEY, JR., M.D. • Director, Clinical Center for Movement Disorders,
Department of Neurology; Assistant Professor, University of Texas Southwestern
Medical School, Dallas, Texas
J
OSEPH R. DUFFY, PH.D. • Chair, Division of Speech Pathology, Mayo Clinic Rochester,
Rochester, Minnesota; Professor of Speech Pathology, Mayo Medical School,
Rochester, Minnesota
V
IRGILIO GERALD H. EVIDENTE, M.D. • Director, Movement Disorders Center, St. Luke’s
Medical Center, Quezon City, Philippines
S
TEWART A. FACTOR, D.O. • Riley Family Chair, Parkinson’s Disease and Movement
Disorders Center; Professor, Department of Neurology, Albany Medical College,
Albany, New York
D
AVID S. GLOSSER, SC.D. • Clinical Assistant Professor, Department of Neurology,
Jefferson Medical College, Philadelphia, Pennsylvania
C
HRISTOPHER G. GOETZ, M.D. • Professor, Department of Neurology, Rush Medical
College, Rush-Presbyterian-St. Luke’s Medical Center, Chicago, Illinois
K
ATRINA A. GWINN-HARDY, M.D. • Research Associate, Mayo Clinic Jacksonville,
Jacksonville, Florida; Assistant Professor of Neurology, Mayo Medical School,
Rochester, Minnesota
C. M
ICHEL HARPER, JR., M.D. • Consultant, Division of Neuroimmunology, Department

of Neurology, Mayo Medical School, Rochester, Minnesota
E
RIC S. MOLHO, M.D. • Assistant Professor, Department of Neurology, Albany Medical
College, Albany, New York
E
RWIN B. MONTGOMERY, JR., M.D. • Head, Movement Disorders Program, Department
of Neurology, Cleveland Clinic Foundation, Cleveland, Ohio
S
USAN M. MURPHY, M.D. • Assistant Professor, Department of Physical Medicine
and Rehabilitation, University of Texas Southwestern Medical Center, Dallas, Texas
P
ADRAIG E. O’SUILLEABHAIN, M.B. • Assistant Professor, Department of Neurology,
University of Texas Southwestern Medical Center, Dallas, Texas
S
OTIRIOS A. PARASHOS, M.D., PH.D. • Minneapolis Clinic of Neurology Ltd.; Director
of Research, Struthers Parkinson’s Center; Clinical Instructor of Neurology,
University of Minnesota, Minneapolis, Minnesota
F
RANK A. RUBINO, M.D. • Consultant, Department of Neurology, Mayo Clinic
Jacksonville, Jacksonville, Florida; Professor of Neurology, Mayo Medical School,
Rochester, Minnesota
K
APIL D. SETHI, M.D., F.R.C.P. • Professor, Department of Neurology, Medical College
of Georgia, Augusta, Georgia
K
ATHLEEN M. SHANNON, M.D. • Associate Professor, Department of Neurological
Sciences, Rush Medical College, Rush-Presbyterian-St. Luke’s Medical Center,
Chicago, Illinois
M
ARK STACY, M.D. • Director, Muhammad Ali Parkinson Research Center, Barrow

on nuances of the neurologic examination as it pertains to movement disorders as well
as provide a conceptual framework and definition of terms.
Approach to the Patient
With a Movement Disorder:
Basic Principles of Neurologic Diagnosis
J. Eric Ahlskog, PhD, MD
Contents
BACKGROUND
THE NEUROLOGIC EXAMINATION: OVERVIEW
PERIPHERAL NEUROLOGIC SIGNS RELEVANT TO MOVEMENT DISORDERS
CENTRAL NERVOUS SYSTEM SIGNS
HYPER KINETIC DISORDERS
FINAL COMMENTS
SELECTED READING
1
3
From: Parkinson’s Disease and Movement Disorders:
Diagnosis and Treatment Guidelines for the Practicing Physician
Edited by: C. H. Adler and J. E. Ahlskog © Mayo Foundation
for Medical Education and Research, Rochester, MN
4 Part A/ Basic Diagnostic Principles
In a simplistic sense, movement disorders can be divided into conditions in which
the problem is primarily reduced movement (hypokinesis) or excessive movement
(hyperkinesis). The hypokinetic category is predominantly parkinsonism and its vari-
ous subgroups, whereas problems characterized by excessive (hyperkinetic) move-
ment include dystonia, chorea, tics, myoclonus, and tardive syndromes. Tremor is
obviously a form of excessive movement; however, tremor with the limb at rest is
typically associated with hypokinetic signs, and hence resting tremor is usually clas-
sified in the hypokinetic (parkinsonian) category.
The distinction between patients who move too much and those who move too little

or with proximal lower extremity weakness). Once the patient is standing, observe the
first step. This step should be done without hesitation; in contrast, some patients with
parkinsonism may have transiently frozen feet. Observe the feet while the patient walks.
Normally with each step, the patient plants the heel with the foot dorsiflexed and then
rocks the foot forward to push off with the ball of the foot. Weakness of the feet may
Chapter 1 / Patients With Movement Disorders 5
Fig. 1-1. Origins of the corticospinal tract. A, Origins. B, Pathways to spinal cord anterior horn
cells (i.e., to lower motor neurons).
6 Part A/ Basic Diagnostic Principles
Table 1-1
Motor System Neuroanatomical Distinctions
a
Primary distinctions Component circuits
Central nervous system • Corticospinal tract
(brain and spinal cord) • Extrapyramidal system, basal ganglia
•Cerebellum
• Praxis circuits
Peripheral nervous system • Anterior horn cells (in spinal cord,
but origin of motor nerves)
• Nerve
• Neuromuscular junction
• Muscle
a
The neurologic examination should provide signs to identify the involved systems.
Fig. 1-2. View of cerebellum showing the two hemispheres and selected circuits. The midline
cerebellum (vermis), critical for normal gait, is not shown. Lesions of the superior cerebellar
peduncle result in severe tremor. Palatal tremor (also known as palatal myoclonus or palatal
myorhythmia) is associated with lesions of Mollaret’s triangle, which consists of the cerebellar
dentate nucleus, the red nucleus, and the inferior olive.
Chapter 1 / Patients With Movement Disorders 7

term was initially introduced to include all motor pathways that
influence the lower motor neuron, except those of the
pyramidal (corticospinal) tract. More recently, the term has
been used somewhat interchangeably with the term “basal
ganglia.”
Lenticular nuclei • Refers to the putamen and globus pallidus. The name was
chosen because of the macroscopic lens shape.
Lower motor neuron • Spinal cord anterior horn neurons, which are the source of motor
nerves to muscles.
Motor neuron • Cortical neurons concerned with movement that project to the
spinal cord (upper motor neuron), plus anterior horn spinal
motor neurons (lower motor neuron).
Neuromuscular junction • Interface between the lower motor neuron synaptic terminal and
the corresponding cholinergic receptor on the muscle cell
membrane.
Parkinsonism • Clinical signs of slowness (bradykinesia), rigidity, resting
tremor, imbalance, and attenuated automatic movements that
develop after damage to certain components of the
extrapyramidal motor system.
Pallidal • Refers to the globus pallidus of the basal ganglia.
Pyramidal tract • Corticospinal projections passing through the medullary
pyramid.
Striatum • Caudate and putamen, also known as “neostriatum.” In the older
literature, the term “corpus striatum” was used to refer to
caudate, putamen, and globus pallidus.
a
Also, see figures.
8
Chapter 1 / Patients With Movement Disorders 9
change this pattern, as does parkinsonism, in which the foot moves parallel to the ground

relax. Tested regions are wrists, elbows, shoulders, knees, and sometimes hips. Assess-
ment of tone in the neck and trunk can be similarly done with nonrapid movement.
• Rigidity is characterized by resistance to movement that is relatively uniform across the
entire range of motion. If present, this characteristic suggests involvement of extrapyra-
midal systems within the brain. If tremor is superimposed, the examiner feels a ratchety
resistance to passive movement, termed “cogwheel rigidity.”
• Spasticity is assessed by more rapid limb excursions and is characterized by detection of
a catch during this movement. To assess this in the lower limbs, have the patient lie supine,
as relaxed as possible, with your hand under the distal thigh, just proximal to the popliteal
fossa. Rapid elevation of a spastic lower limb elicits an initial partial flexion at the knee (to
gravity) followed by an interruption of the movement (catch). If mild, this catch is over-
come, with gravity pulling the leg through the rest of the downward movement. This hitch
in the movement of the limb occurs with quick but not slow movements. The similarities
to the mechanics of a pocket knife (initial resistance and then relaxation) have led to the term
10 Part A/ Basic Diagnostic Principles
“clasp-knife reflex.” In the upper limbs, quick flexion-extension movements of the wrist
or elbow, or pronation-supination of the forearm, may elicit a similar sign.
• Dystonia implies an abnormal posture (see below) and is apparent by observation. Some
forms of dystonia are present only in certain positions or with certain tasks. A dystonic limb,
trunk, or neck is resistant to movement by the examiner.
• Gegenhalten, or paratonia, characterized by an inability to relax the limb, is associated with
confusional, demented, or frontal lobe states. This effect exceeds the mildly increased tone
sometimes detected in a tense but normal person. Characteristically, patients with
gegenhalten hamper the assessment by activating the limb while the examiner is
moving it.
• Hypotonia is the opposite of all the above, with the examiner appreciating flaccidity or
floppiness as the patient’s limb is moved. This is often difficult to distinguish from
normal tone. It may occur in patients with cerebellar syndromes, chorea, or extreme
neuromuscular weakness of peripheral origin.
REFLEXES

finger on the upper lip provokes a brisk contraction in patients with anterior cerebral
hemisphere dysfunction. This response does not substantially diminish with repeated