Electromyography and Neuromuscular Disorders Clinical

Electrophysiologic Ultrasound Correlations, 4th Edition

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xii Preface to the Fourth Edition

supervision. The continued goal of this text is to present text, one can sit down with a patient, take a history, perform
material in an easily understandable and logical manner. We a physical examination, and use the appropriate electrodi-
have often commented to our students that with knowl- agnostic studies, with ultrasound if indicated, to reach the
edge of anatomy, physiology, and neurologic localization, the most accurate and complete diagnosis.
practice of electrodiagnostic studies makes sense. With the
addition of ultrasound correlation, it now makes even more David C. Preston
sense. We hope that with the information contained in this Barbara E. Shapiro
 xiii

Preface to the Third Edition

Since the publication of this book in 1997, followed by the of them. Most of the photographs are now in color. Being
second edition in 2005, we have been profoundly gratified strong believers in “a picture is worth a thousand words,”
by the continued overwhelmingly positive reception it has we have added over 100 new figures and photos, and others
received from physicians, both in training and in practice. It have been updated. In addition, one of the major improve-
has become one of the key resources for residents and fel- ments in the third edition has been the inclusion of cross-­
lows who are learning electrodiagnostic testing and clinical sectional anatomy of the muscles used for needle EMG.
neuromuscular disorders. The goal of the first edition was to To really master the needle EMG, one needs to be able to
create a textbook that integrated electrodiagnostic studies think three dimensionally – not only where the muscle is,
and neuromuscular disorders in a practical and concise man- but what other muscles are nearby and, even more impor-
ner, always remembering the important principle that nerve tant, what other important vascular structures and nerves
conduction studies and electromyography (EMG) are an are nearby that one needs to avoid. To this end, we adapted
extension of the clinical examination. In the second edition, cross-­sectional line drawings from the outstanding work of
the companion CD of EMG waveforms was added so that Eycleshymer and Schoemaker published in 1911. Each indi-
the reader could have the benefit of being able to see and vidual drawing was scanned and then oriented to the posi-
hear examples of classic EMG waveforms. The second edi- tion used for EMG. The muscle of interest was shaded red
tion was also expanded in the number of chapters and the and, likewise, all major nerves, veins, arteries and tendons
breadth of information, adding chapters on Pediatric EMG, were color coded. Finally, a life size image of a conventional
Electricity and Electronics, EMG in the ICU, Iatrogenic needle EMG was placed in the correct orientation used for
Electrodiagnosis, and Statistics for EMG studies. EMG. Thus, each muscle used for needle EMG now has a
As the intention of this text was, and remains, to convey photo showing its correct insertion point along with its rel-
basic and essential information, and the vast majority of the evant cross-­sectional anatomy at that location.
basic and essential information has not changed, the ques- Like the first and second editions, this text is meant to
tion again arises, “why do a third edition?” The reasons are provide a single resource for those physicians training in
multifactorial. or practicing electrodiagnostic studies. From our perspec-
First, the authors now read many of our neurology jour- tive of teaching for over 20 years, on a post-­graduate, resi-
nals and an increasing number of books on our iPads and dency, and fellowship level, we feel that if one can master
other similar devices. We also now use these devices to the fundamentals in this book, one should have all the basic
connect to our electronic medical record, and look up drug concepts and information one needs to competently under-
information and a host of medical information. It therefore stand and interpret electrodiagnostic studies. Although a
makes sense for a third edition to be both in print and com- great deal of information is presented regarding the perfor-
pletely electronic. Although it is difficult for many of us mance of studies, there remains no substitute for hands-­on
to think about replacing books with electronic media, this experience under supervision. However, for the recognition
is clearly where the world is moving, led by our students, and interpretation of EMG waveforms, the videos now pub-
residents, and fellows in training. lished on the web should make this much easier to master.
Second, writing a third edition gave us the opportunity The continued goal of this text is to present material in
to review the medical literature since 2005 on all the topics an easily understandable and logical manner. The authors
in the text, especially the chapters that deal with clinical have often commented to their students that with knowl-
disorders. Since the publication of the second edition there edge of anatomy, physiology, and neurologic localization,
have been significant advances in understanding the genet- the practice of electrodiagnostic studies makes sense. We
ics, pathophysiology, and treatment of many neuromuscu- hope that with the information contained in this text, one
lar conditions, and these are included in the third edition. can sit down with a patient, take a history, perform a physi-
In addition, some electrodiagnostic techniques have been cal examination, and use the appropriate electrodiagnostic
improved and other new ones described that are included in studies to reach a diagnosis.
this third edition of the book.
Third, with advances in publishing, we have now greatly DCP
improved the figures and color has been added to many BES
 xv

Preface to the Second Edition

Since the publication of the first edition of this book in Indeed, we have added or updated more than 175 figures to
1997, we have been gratified by the overwhelmingly posi- the first edition of the book.
tive reception it has received from physicians, both in train- We have improved the book in several other ways. First,
ing and in practice. The goal of the first edition was to create we have expanded many of the clinical chapters, and in
a textbook that integrated electrodiagnostic studies and some cases separated them into new chapters, including
neuromuscular disorders in a practical and concise manner, median neuropathy at the wrist, proximal median neuropa-
always remembering the important principle that nerve con- thy, ulnar neuropathy at the wrist, ulnar neuropathy at the
duction studies and electromyography (EMG) are an exten- elbow, amyotrophic lateral sclerosis, and atypical motor
sion of the clinical examination. As this text is intended to neuron disorders. All of the clinical chapters follow the
convey basic and essential information, the question arises, same format that was used in the first edition, first pre-
“why do a second edition?” The authors acknowledge that senting the important anatomic and clinical aspects of the
no new muscles have been discovered in the human body disorder, followed by a discussion of the relevant electro-
over the past six years. Likewise, PCR and genetic tests diagnostic studies. Each chapter ends with example cases
have failed to identify any new nerves. Although much of based on actual patients, illustrating many important clinical
the basic information in the fields of electrodiagnostic stud- and electrodiagnostic teaching points. In addition, in section
ies and neuromuscular disorders has not changed, we have three we have added a new chapter on basic statistics for
written this second edition to improve and expand on many electrodiagnostic studies, discussing several basic statistical
topics. concepts that every electromyographer needs to know in
First and most important, needle electromyography order to properly interpret a study.
relies upon the proper interpretation of waveforms in real-­ The first edition was divided into six separate sections.
time. While one can read about waveforms until they are This new edition has been expanded to eight. The first new
blue in the face, it is very difficult to appreciate the audio section deals with EMG in Special Clinical Settings, includ-
and visual qualities of a waveform unless one can see and ing the approach to electrodiagnostic studies in the inten-
hear it. For the last fifteen years, we have collected video sive care unit, and the approach to electrodiagnostic studies
examples of classic EMG waveforms from a variety of in the pediatric patient. In the last several years, electro-
patients. Two years after the publication of the first edi- myographers are called upon more frequently to perform
tion of this book, we introduced a companion videotape of EMG studies in the intensive care unit to evaluate patients
common EMG waveforms. With new digital technology, we with profound weakness. New clinical disorders and elec-
have now been able to digitize these video waveforms and trodiagnostic techniques to evaluate these disorders have
put them on a companion CD which accompanies this book. been extensively reported over the last several years and are
Thus, in this second edition, the reader can view the CD on reflected in this new edition. We have included a discussion
any computer, and watch and hear every common and clas- of pediatric EMG because of its own unique set of chal-
sic EMG waveform. Because all the waveforms are digital, lenges and techniques that differ from adult studies.
the reader can freeze or replay any waveform at any time. The other new section deals with the basics of elec-
The textbook description and discussion of each waveform tricity and electronics, in addition to the potential risks
are now greatly enhanced by the companion CD. and complications of electrodiagnostic studies. Some
Since the publication of the first edition there have been knowledge of electricity and electronics is extremely
significant advances in some neuromuscular conditions, and helpful in understanding electrodiagnostic studies. From
these are included in the second edition. Some new disor- a practical point of view, this knowledge is also very help-
ders have been described, among them paralytic poliomyeli- ful in understanding and correcting many of the technical
tis caused by the West Nile virus. In addition, several new problems that arise in the everyday practice of electrodi-
techniques that have been described and validated in the agnostic medicine. The latter chapter arose from a con-
electrodiagnosis of neuromuscular conditions are included tinuing medical education course which we were asked to
in this second edition of the book. For instance, the elec- give at an annual meeting of the American Association of
trodiagnosis of ulnar neuropathy at Guyon’s canal has sig- Electrodiagnostic Medicine, which was followed up as a
nificantly improved over the past few years, and several review article in the journal Muscle and Nerve. Although
new techniques that are useful in making this diagnosis are nerve conduction studies and EMG are usually well toler-
included in this second edition. ated and in most patients have minimal side effects, there
We spent a considerable amount of time thinking of bet- are potential risks and complications, especially in certain
ter ways to present complex material in a logical and concise patient populations. It is essential that all physicians per-
manner for this second edition of the book. Being strong forming these studies are aware of these potential risks and
believers in “a picture is worth a thousand words,” we have complications, albeit rare, and follow simple protocols to
added many new figures, and others have been updated. minimize them.
xvi Preface to the Second Edition

Like the first edition, this text is meant to provide a Finally, the goal of this text is to present material in an
single resource for those physicians training in or practicing easily understandable and logical manner. The authors have
electrodiagnostic studies. From our perspective of teaching often commented to their students that with knowledge of
for many years, both on a post-­graduate and residency level, anatomy, physiology, and neurologic localization, the prac-
we feel that if one can master the fundamentals in this book, tice of electrodiagnostic studies makes sense. We hope that
one should have all the basic concepts and information one with the information contained in this text, one can sit down
needs to competently understand and interpret electro- with a patient, take a history, perform a physical examina-
diagnostic studies. Although a great deal of information is tion, and use the appropriate electrodiagnostic studies to
presented regarding the performance of studies, there is no reach a diagnosis.
substitute for hands-­on experience under supervision. How-
ever, it is our hope that with the companion CD as part of DCP
the textbook, the recognition and interpretation of EMG BES
waveforms will be easier to master.
 xvii

Preface to the First Edition

This text is written primarily for clinicians who perform reflexes, and repetitive nerve stimulation studies. In Sec-
and interpret nerve conduction studies and electromyogra- tion Three, important technical factors and artifacts are
phy (EMG), as well as for physicians who use the results discussed, including the anomalous innervations. Sec-
of these electrodiagnostic studies to evaluate patients with tion Four discusses the practical details of performing the
peripheral nervous system disorders. Nerve conduction most commonly used nerve conduction studies. In Section
studies and EMG are best considered an extension of the Five, the focus changes to needle EMG. After discussing
clinical examination. Indeed, these studies cannot be prop- the overall approach to the needle EMG, the anatomy of
erly planned, performed, or interpreted without knowing the bulbar, upper extremity, and lower extremity muscles
the patient’s symptoms and findings on the clinical exami- is reviewed in detail. The last two chapters in this section
nation. Numerous nerves and literally hundreds of muscles cover the approach to the needle EMG examination, includ-
can be studied. To study them all would be neither practical ing the assessment of spontaneous activity and the analysis
for the electromyographer, nor desirable for the patient. In of motor unit action potentials.
every case, the study must be individually planned, based Section Six, Clinical–Electrophysiologic Correlations,
on the clinical differential diagnosis, and then modified as forms the core of the text. After an overview of the impor-
the study progresses and further information is gained. The tant patterns, all of the major peripheral nervous system
electromyographer needs to perform the studies necessary conditions are discussed, from both the clinical and the
to both confirm and exclude certain diagnoses while mini- electrophysiologic points of view. Included are the mono-
mizing the amount of patient discomfort. Most often, nerve neuropathies, polyneuropathies, motor neuron diseases,
conduction studies and EMG can successfully localize the radiculopathies, plexopathies, disorders of the neuromus-
lesion, provide further information about the underlying cular junction and muscle, and the myotonic and periodic
pathophysiology, and assist in assessing the disorder’s sever- paralysis disorders. At all times, the text integrates the
ity and temporal course. important basic clinical and electrophysiologic points. In
Although there are many excellent textbooks on electro- Chapters 16–32, clinical cases and their respective nerve
diagnosis and several superb references on clinical neuro- conduction and EMG data are presented. Each case exam-
muscular disorders, few integrate the two in a practical and ple is that of an actual patient taken from our EMG teaching
concise manner. The approach we take in this text parallels file during the past 10 years.
our teaching program developed for the EMG fellowships The authors appreciate that some specific techniques
and neurology residencies at the Brigham and Women’s and normal values may vary from laboratory to laboratory.
Hospital and the Massachusetts General Hospital in Boston. Nevertheless, the goal of this book is to present a logical
The book is divided into six fundamental sections. Sec- approach in the EMG laboratory that combines the clinical
tion One covers the overall practical approach to a patient and electrophysiologic evaluations of a patient with a disor-
in the EMG laboratory, followed by a review of the basic der of the peripheral nervous system.
anatomy and neurophysiology that every electromyogra-
pher needs to understand. Section Two discusses the fun- DCP
damentals of nerve conductions, including motor, sensory, BES
and mixed nerve studies, as well as late responses, blink
 xix

Dedication

To our daughters, Hannah and Abigail.

 xxi

Acknowledgments
The authors are indebted to their mentors in clinical neuro- the early parts of our academic and publishing careers. Then
physiology: Drs. John J. Kelly, Jr., Eric L. Logigian, and Bhag- there are a host of individuals whose books, presentations,
wan T. Shahani. Dr. Bashar Katirji, our friend and colleague courses, and research ignited our passion in neuromuscular
for more than 20 years, has been an inspiration and a great ultrasound. Among them are Drs. Francis O. Walker, Michael
partner in teaching, research, and patient care in clinical neu- S. Cartwright, Lisa D. Hobson-­Webb, Jeff Strakowski, Lucia
romuscular disorders and electrophysiology. In addition, the Padua, Stefano Bianchi, Carlo Martinoli, Andrea J. Boon,
authors wish to thank their colleagues, technologists, and Leo Visser, Craig M. Zaidman, Antonios Kerasnoudis, H.
present and former Neuromuscular and EMG fellows at the Stephan Goedee, James B. Caress, and Joon Shik Yoon. At
University Hospitals Cleveland Medical Center, the Brigham Elsevier, Melanie Tucker, Lisa Barnes, and Doug Turner were
and Women’s Hospital, and the Massachusetts General Hos- instrumental in bringing the fourth edition into print and
pital. The contributions of Dale Preston, Thayer Preston, electronic forms. And of course we will always be grateful
and Richard (Zack) Zydek to the photography are greatly to our dear friend, Susan Pioli, who has been there since the
appreciated. A special thanks to our friend, colleague, and inception of this book and was instrumental in bringing the
mentor, Dr. Martin A. Samuels, who was instrumental in first and second editions to publication.
 SECTION I • Overview of Nerve Conduction Studies and Electromyography

Approach to Nerve Conduction

Studies, Electromyography, and
Neuromuscular Ultrasound
1
Electrodiagnostic (EDX) studies play a key role in the eval- peripheral nerves, neuromuscular junctions (NMJs), and
uation of patients with neuromuscular disorders. In selected muscles. In addition, these studies may provide useful diag-
cases, the additional use of neuromuscular ultrasound (U/S) nostic information when the disorder arises in the central
can add key complementary anatomical and diagnostic nervous system (CNS) (e.g., tremor or upper motor neu-
information. ron weakness). Occasionally, information from the EDX
EDX studies include nerve conduction studies (NCSs), study is so specific that it suggests a precise etiology. In
repetitive nerve stimulation, late responses, blink reflexes, most cases, however, the exact etiology cannot be defined
and needle electromyography (EMG), in addition to a vari- based on EDX studies alone. It is in some of these cases that
ety of other specialized examinations. NCSs and needle neuromuscular U/S is most useful. Neuromuscular U/S,
EMG form the core of the EDX study. They are performed the use of U/S to study the peripheral nerves and muscles,
first and usually yield the greatest diagnostic information. is often able to suggest the precise etiology of the disease,
NCSs and needle EMG are complementary and, therefore, such as chronic inflammatory demyelinating polyneuropa-
are always performed together and in the same setting. thy (CIDP) when a particular pattern of nerve enlargement
Performed and interpreted correctly, EDX studies yield is seen or inclusion body myositis when a particular pattern
critical information about the underlying neuromuscular of muscle involvement is present. EDX studies provide an
disorder and allow other laboratory and diagnostic tests, initial aid in defining the disorder, giving several key pieces
including neuromuscular U/S, to be used in an appropriate of information, which in some cases can be further refined
and efficient manner. Sometimes, the information gained with neuromuscular U/S.
from EDX studies leads to specific medical or surgical ther-
apy. For example, a patient with a peripheral neuropathy
clinically, who is subsequently found to have an acquired
demyelinating neuropathy with conduction blocks on EDX 6HQVRU\QHUYH
studies, most often has a potentially treatable condition. 'RUVDOURRW URRW
Indeed, the value of EDX studies has been validated in JDQJOLRQ
several large case studies. In the most recent study by Lind-
strom and colleagues, they analyzed data from 1414 consec-
utive patients and found that EDX studies either changed
or confirmed the diagnosis in 52% and 47% of cases, respec-
tively. Even more impressive was that EDX studies resulted
in a change in patient management in 63% of patients.
In practice, EDX studies serve as an extension of the clini- 0RWRUQHXURQ
cal examination and should always be considered as such. 0RWRUQHUYH
3HULSKHUDO
Accordingly, a directed neurologic examination should always QHUYH URRW
be performed before EDX studies to identify key clinical
abnormalities and establish a differential diagnosis. With 6HQVRU\ 0RWRU
numerous nerves and literally hundreds of muscles available, QHUYH QHUYH
it is neither desirable for the patient nor practical for the elec-
1HXURPXVFXODU
tromyographer to study them all. In each case, the study must MXQFWLRQ
be individualized, based on the neurologic examination and
differential diagnosis, and modified in real time as the study
progresses and further information is gained. 0XVFOH
NCSs and EMG are most often used to diagnose disor- Fig. 1.1 Elements of the peripheral nervous system. Note that the
ders of the peripheral nervous system (Fig. 1.1, Box 1.1). primary motor neuron resides within the spinal cord, whereas the
primary sensory neuron, the dorsal root ganglion, lies outside the
These include disorders affecting the primary motor neu- spinal cord. The dorsal root ganglion is a bipolar cell. Its proximal
rons (anterior horn cells), primary sensory neurons (dorsal process forms the sensory nerve root; the distal process becomes the
root ganglia), nerve roots, brachial and lumbosacral plexuses, peripheral sensory nerve.

1
2 SECTION I Overview of Nerve Conduction Studies and Electromyography

Box 1.1 Peripheral Nervous System Disorders: LOCALIZATION OF THE DISORDER

Localization and Major Categories
IS THE MAJOR AIM OF THE
Motor Neuronopathy Neuropathy ELECTRODIAGNOSTIC STUDY
Amyotrophic lateral Pattern The principal goal of every EDX study is to localize the
sclerosis and its variants Mononeuropathy disorder. The differential diagnosis is often dramatically
Spinal muscular atrophy • Entrapment
narrowed once the disorder has been localized. Broadly
Infectious (poliomyelitis, • Trauma
West Nile virus) Mononeuritis multiplex speaking, the first order of localization is whether the dis-
Focal motor neuron disease Polyneuropathy order is neuropathic, myopathic, a disorder of neuromuscu-
(monomelic amyotrophy) Primary nerve pathology lar transmission, or a disorder of the CNS. For example, in
Demyelinating patients with pure weakness, EDX studies can be used to
Sensory Neuronopathy Axonal localize whether the disorder is caused by dysfunction of
Autoimmune Primary fiber type
Paraneoplastic involvement
the motor neurons/axons, NMJs, or muscles or if it has a
Toxic Sensorimotor central etiology. The pattern of nerve conduction and espe-
Infectious Motor cially EMG abnormalities usually can differentiate among
Sensory these possibilities and guide subsequent laboratory investi-
Radiculopathy gations. For example, a patient with proximal muscle weak-
Macroscopic Neuromuscular
­Junction Disorders ness may have spinal muscular atrophy (i.e., a motor neuron
Disk herniation
Spondylosis Postsynaptic disorder), myasthenic syndrome (i.e., a NMJ disorder), or
Neoplasia Myasthenia gravis polymyositis (i.e., a muscle disorder) among other condi-
Hemorrhage Toxic tions, including those with central etiologies (e.g., a para-
Abscess Congenital sagittal frontal lesion). EDX studies can easily differentiate
Microscopic Presynaptic among these conditions, providing key information to guide
Infarction Lambert-­Eaton
subsequent evaluation and treatment, which differ mark-
Infectious myasthenic syndrome
Inflammatory Botulism edly among these diseases.
Neoplastic Toxic Once the localization is determined to be neuropathic,
Demyelinating Congenital myopathic, or a disorder of the NMJ or of the CNS, EDX
studies can usually add other important pieces of informa-
Plexopathy Myopathy
tion to localize the problem further (Fig. 1.2). For instance,
Radiation induced Inherited
Neoplastic Muscular dystrophy
the differential diagnosis of a patient with weakness of the
Entrapment Congenital hand and numbness of the fourth and fifth fingers includes
Diabetic Metabolic lesions affecting the ulnar nerve, lower brachial plexus, or
Hemorrhagic Acquired C8-­T1 nerve roots. If EDX studies demonstrate an ulnar
Inflammatory Inflammatory neuropathy at the elbow, the differential diagnosis is limited
Toxic
to a few conditions, and further diagnostic studies can be
Endocrine
Infectious directed in a more intelligent manner. In this situation, for
instance, there is no need to obtain a magnetic resonance

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Fig. 1.2 Possible localizations determined from PRQRQHXURSDWKLHV
the electrodiagnostic study. CNS, Central nervous
system; NMJ, neuromuscular junction. 3RO\QHXURSDWK\
 Chapter 1 • Approach to Nerve Conduction Studies, Electromyography, and Neuromuscular Ultrasound 3

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&KURQLF pathic localization.

imaging (MRI) scan of the cervical spine to assess a pos- Conversely, predominantly motor or predominantly sensory
sible cervical radiculopathy because the EDX studies dem- neuropathies are rare and suggest a much more limited set of
onstrated an ulnar neuropathy at the elbow as the source disorders. For instance, a patient with numbness in the hands
of the patient’s symptoms. This is a situation where neuro- and feet and diminished reflexes may be diagnosed with a
muscular U/S may be particularly useful, able to precisely peripheral neuropathy. However, if EDX studies demon-
localize the lesion and help assess for anatomic etiologies. strate abnormal sensory nerve conductions with completely
In a patient with a CNS disorder who is mistaken as hav- normal motor nerve conductions and needle EMG, then the
ing a peripheral disorder, the EDX study often correctly sug- differential diagnosis changes from a peripheral neuropathy
gests that the localization is central. For example, transverse to a pure sensory neuropathy or neuronopathy, which has a
myelitis may mimic Guillain-­Barré syndrome, or a small much more limited differential diagnosis.
acute cortical stroke may occasionally mimic the pattern of Second, EDX studies often can define whether the
a brachial plexopathy or mononeuropathy. In settings such underlying pathophysiology is demyelination or axonal loss.
as these, the EDX study is often the first test to suggest that Although most demyelinating neuropathies have some sec-
the correct localization is central rather than peripheral. ondary axonal loss and many axonal loss neuropathies have
some secondary demyelination, EDX studies usually can
differentiate between a primary demyelinating and a pri-
Neuropathic Localization mary axonal neuropathy. Because EDX studies usually can
Neuropathic is probably the most common localization made make this differentiation quickly and noninvasively, nerve
on EDX studies. Neuropathic literally means a disorder of biopsy is essentially never required to make this determi-
the peripheral nerves. However, in common usage, it includes nation. Furthermore, the differentiation between primary
the primary sensory and motor neurons as well. EDX stud- axonal and primary demyelinating pathology is of consid-
ies are particularly helpful in neuropathic conditions. First, in erable diagnostic and prognostic importance, especially in
conjunction with the history and examination, they can usu- the case of polyneuropathies. Most polyneuropathies are
ally further localize the disorder to the neurons, roots, plexus, associated with primary axonal degeneration, which has an
or peripheral nerve. In the case of peripheral nerves, further extensive differential diagnosis. In contrast, the number
localization is usually possible to a single nerve (mononeu- of true electrophysiologic primary demyelinating neuropa-
ropathy), multiple individual nerves (mononeuropathy mul- thies is extremely small. They are generally subdivided into
tiplex), or all nerves (polyneuropathy). In the case of a single those that are inherited and those that are acquired (e.g.,
nerve, the exact segment of nerve responsible for the prob- Charcot Marie Tooth [CMT] vs. CIDP). EDX studies can
lem may be localized in some cases. typically make that determination as well. The finding of
In the case of neuropathic lesions, EDX studies often an unequivocal primary demyelinating polyneuropathy on
yield further key information, including the fiber types EDX studies often leads quickly to the correct diagnosis
involved, the underlying pathophysiology, and the temporal and, in the case of an acquired demyelinating polyneuropa-
course of the disorder (Fig. 1.3). thy, often suggests a potentially treatable disorder.

Information About the Fiber Types Involved and the Assessing the Degree of Axonal Loss Versus
Underlying Nerve Pathophysiology can be Gained, Demyelination has Implications for Severity and
Which Then Further Narrows the Differential Diagnosis Prognosis
In the case of neuropathic disorders, the involved fiber types A nerve that has sustained a demyelinating injury often can
and the underlying pathology can usually be determined. First, remyelinate in a very short time, usually weeks. However, if
EDX studies are more sensitive than the clinical examination there has been substantial axonal loss, whether primary or
in determining which fiber types are involved: motor, sen- secondary, the prognosis is much more guarded. The rate of
sory, or a combination of the two. Sensorimotor polyneurop- axonal regrowth is limited by the rate of slow axonal trans-
athies are common and suggest a large differential diagnosis. port, approximately 1 mm per day. Clinically, axonal loss
4 SECTION I Overview of Nerve Conduction Studies and Electromyography

lesions can rarely be differentiated from demyelinating ones, 0\RSDWKLF

especially in the acute setting. For example, in a patient who
awakens with a complete wrist and finger drop, the etiology
usually is compression of the radial nerve against the spiral 'LVWULEXWLRQ 3DWKRORJ\ 7HPSRUDOFRXUVH
groove of the humerus. However, the paralysis could result
from either conduction block (i.e., demyelination) or axonal
loss, depending on the severity and duration of the compres- 3UR[LPDO %ODQG $FXWH
sion. Clinically, both conditions appear the same. Neverthe-
less, if the injury is due to axonal loss, it has a much worse 'LVWDO $FWLYHGHQHUYDWLRQ 6XEDFXWH
prognosis and a longer rehabilitation time to recovery than a
*HQHUDOL]HG 0\RWRQLFGLVFKDUJHV &KURQLF
similarly placed lesion that is predominantly demyelinating
in nature. EDX studies can readily differentiate axonal loss &UDQLREXOEDU
from demyelinating lesions.
6\PPHWULF$V\PPHWULF
Assessment of the Temporal Course can Often be Made
For neuropathic conditions, there is an orderly, temporal 6HYHULW\RILQYROYHPHQW
progression of abnormalities that occurs in NCSs and nee- Fig. 1.4 Key electrodiagnostic findings in a myopathic localization.
dle EMG. A combination of findings often allows differ-
entiation among hyperacute (less than 1 week), acute (up
to a few weeks), subacute (weeks to a few months), and
chronic (more than a few months) lesions. The time course 10-
suggested by the EDX findings may alter the impression
and differential diagnosis. For example, it is not uncommon
for a patient to report an acute time course to his or her 'LVWULEXWLRQ 3DWKRORJ\ (WLRORJ\
symptoms, whereas the EDX studies clearly indicate that
the process has been present for a longer period of time than 3UR[LPDO
the patient has been aware of.
Conversely, the temporal course described by the patient *HQHUDOL]HG 3UHV\QDSWLF $FTXLUHG
may impact the interpretation of the EDX findings. For
instance, the finding of a normal ulnar sensory nerve action &UDQLREXOEDU 3RVWV\QDSWLF ,QKHULWHG
potential recording the little finger, in a patient with numb- Fig. 1.5 Key electrodiagnostic findings in a neuromuscular junction
ness of the little finger, has very different implications localization. NMJ, Neuromuscular junction.
depending on the time course of the symptoms. If the symp-
toms are truly less than 1 week in duration, the normal ulnar can be very helpful in narrowing the differential diagnosis. For
sensory response could indicate an ulnar neuropathy (with example, inclusion body myositis may present asymmetrically,
wallerian degeneration yet to have been completed), a proxi- whereas polymyositis and dermatomyositis do not.
mal demyelinating lesion, or a lesion at the level of the nerve Second, the presence of spontaneous activity on needle
root or above. On the other hand, if the symptoms have EMG is helpful in limiting the differential diagnosis and
been present for several weeks or longer, the same finding suggesting certain underlying pathologies. Most myopathies
would indicate either a proximal demyelinating lesion or a are bland, with little or no spontaneous activity. However,
lesion at the level of the nerve root or above. These temporal myopathies that are inflammatory, necrotic, and some that
changes underscore the electromyographer’s need to know the are toxic or dystrophic may be associated with active dener-
clinical time course of symptoms and signs to ensure an accu- vation. In addition, other myopathies may have prominent
rate interpretation of any electrophysiologic abnormalities. myotonic discharges at rest. The presence of myotonic dis-
charges in a myopathy markedly narrows the differential
diagnosis to only a few possible disorders.
Myopathic Localization Last is the issue of the temporal course. Although this
In the case of myopathic (i.e., muscle) disease, EDX studies can determination is more challenging than with neuropathic
also add key information to further define the condition (Fig. lesions, in some myopathies, a determination can be made if
1.4). First, the distribution of the abnormalities may suggest a the myopathy is acute, subacute, or chronic, a finding that
diagnosis: are they proximal, distal, or generalized? Most myopa- again narrows the differential diagnosis.
thies preferentially affect the proximal muscles. Few myopathies,
such as myotonic dystrophy type I, affect distal muscles. Some
very severe myopathies (e.g., critical illness myopathy) can be Neuromuscular Junction Localization
generalized. In rare myopathies, there is prominent bulbar weak- Disorders of the NMJ are distinctly uncommon. However,
ness; accordingly, EDX abnormalities may be most prominent in when they occur, EDX studies not only help in identifying
the bulbar muscles. Most myopathies are fairly symmetric; the them but can also add other key pieces of information (Fig.
finding of asymmetry either clinically and/or on EDX studies 1.5). First is the distribution of the abnormalities on EDX
 Chapter 1 • Approach to Nerve Conduction Studies, Electromyography, and Neuromuscular Ultrasound 5

testing: are they proximal, bulbar, or generalized? For instance, Box 1.2 Patient Encounter
myasthenia gravis preferentially affects oculobulbar muscles
1. Take a brief history and perform a directed physical
and then proximal muscles on EDX studies, whereas myas- examination.
thenic syndrome is a generalized disorder on EDX studies, 2. Formulate a differential diagnosis.
although clinically it has a predilection for proximal muscles. 3. Formulate a study based on the differential diagnosis.
Broadly speaking, the underlying pathology can be 4. Explain the test to the patient.
divided into presynaptic and postsynaptic disorders. EDX 5. Perform the nerve conduction studies and modify which
nerve conduction studies to add, based on the findings as
studies are usually very good at making this determination. the test proceeds.
Myasthenia gravis is the prototypic postsynaptic disorder, 6. Perform the needle electromyography study and modify
whereas myasthenic syndrome and botulism target the pre- which additional muscles to sample, based on the findings
synaptic junction. as the test proceeds.
Last is the issue of the etiology of the NMJ disorder,
whether it is acquired or inherited. Almost all NMJ disor-
ders are acquired. However, there are rare inherited NMJ always be amended as the testing proceeds. Before begin-
disorders. In some of these, there may be unique findings on ning, however, one should first explain to the patient in
EDX testing that suggest one of these rare disorders. simple terms what the test involves. Many patients are very
anxious about the examination and may have slept poorly or
not at all the night before the EDX study. Simple explana-
PATIENT ENCOUNTER tions, both before the test begins and while it is ongoing, can
Every EDX study begins with a brief history and directed greatly reduce a patient’s anxiety.
physical examination (Box 1.2). This point cannot be overem- After the test is explained to the patient, the NCSs are
phasized! Some may (incorrectly) argue that the history and performed first, followed by the needle EMG. Indeed, one
clinical examinations are not part of the EDX examination and needs the findings on the NCSs to adapt the needle EMG
that the EDX study needs to stand on its own. Nothing could strategy accordingly and to interpret the needle EMG find-
be further from the truth. One is not expected to perform the ings correctly. For instance, active denervation in the abduc-
same detailed history and physical examination that is done in tor digiti minimi (an ulnar, C8–T1 innervated muscle) has a
the office consultation setting. However, before starting every completely different interpretation depending on whether
study, the EDX physician must know some basic facts: the ulnar motor and sensory NCSs are abnormal or not
  
(ulnar neuropathy in the former, a radiculopathy or motor
• What are the patient’s symptoms?
neuron disease in the latter).
• How long have they been going on?
A proper balance must be maintained among obtain-
• Is there any important past medical history (e.g., diabe-
ing a thorough study, collecting the necessary information
tes, history of chemotherapy, etc.)?
to answer the clinical question, and minimizing patient dis-
• Is there muscle atrophy?
comfort. If performed correctly, nearly all NCSs and needle
• What is the muscle tone (normal, decreased, or in-
EMG can be completed within 1–1.5 hours. Rarely, a longer
creased)?
study is needed if specialized tests such as repetitive nerve
• Is there weakness, and, if so, where is it and how severe
stimulation are performed in addition to the standard stud-
is it?
ies. There clearly is a limit to what most patients can tolerate.
• What do the reflexes show (normal, decreased, or
The electromyographer should always remember the Willy
increased)?
Sutton rule concerning robbing banks: “Go where the money
• Is there any loss of sensation, and, if so, what is the dis-
is.” If there is any question as to whether a patient will toler-
tribution; what modalities are disturbed (e.g., tempera-
ate the entire examination, the study should begin with the
ture, pain, vibration, etc.)?
   area of interest. For instance, in the patient with numbness
The duration, type, and distribution of symptoms, along and tingling of the fourth and fifth fingers, ulnar motor and
with the physical examination, help determine the differen- sensory studies should be done first. Likewise, needle EMG
tial diagnosis, which in turn is used to plan the EDX stud- examination of the ulnar-­innervated muscles, as well as the
ies. The EDX study is planned only after the differential C8–T1 non-­ulnar–innervated muscles, are of most interest
diagnosis is determined. For instance, the EDX evaluation in such a patient. Plan and consider which NCSs and needle
of a patient with slowly progressive proximal weakness is examination of which muscles should be performed first, in
very different from that of a patient with numbness and case the patient can tolerate only one or two nerve conduc-
tingling of the fourth and fifth fingers. In the former case, tions or examination of only a few muscles by EMG.
the differential diagnosis includes disorders of the anterior
horn cell, motor nerve, NMJ, or muscle. In the latter case, CARDINAL RULES OF NERVE
the differential diagnosis includes an ulnar neuropathy at its
various entrapment sites, a lower brachial plexus lesion, or CONDUCTION STUDIES AND
cervical radiculopathy. The EDX plan includes which nerves ELECTROMYOGRAPHY
and muscles to study and whether specialized tests, such as EDX studies rely on the physician’s ability to pay meticu-
repetitive nerve stimulation, may be helpful. The study can lous attention to technical details during the study while
6 SECTION I Overview of Nerve Conduction Studies and Electromyography

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Fig. 1.6 Cardinal rules of nerve conduction studies

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keeping in mind the bigger picture of why the study is being abnormality when none is present) and type II errors
performed. As more data are obtained, the study must be (i.e., failing to recognize an abnormality when one is
analyzed in real time and the test altered as needed. Analy- present). Although both are important, type I errors
sis of online results gives the electromyographer the oppor- are potentially more serious (e.g., the patient is labeled
tunity to modify the strategy as the testing proceeds, an with an abnormal EDX study result, such as neuropathy,
opportunity that is lost once the patient has left the labo- when the “abnormality” on the EDX testing is simply
ratory. The following cardinal rules of EDX studies should due to unrecognized technical errors). Such faulty
always be kept in mind while an EDX study is being per- diagnoses can lead to further inappropriate testing
formed (Fig. 1.6): and treatment. If there is an unexpected abnormal
  
EDX finding that does not fit the clinical examination,
1. NCSs and EMG are an extension of the clinical the lack of a clinical-­electrophysiologic correlation
examination. NCSs and EMG cannot be performed should suggest a technical problem. For instance, if a
without a good directed clinical examination. Every routine sural nerve sensory conduction study shows an
examination must be individualized based on the absent potential but the patient has a normal sensory
patient’s symptoms and signs and the resulting examination of the lateral foot (i.e., sural territory),
differential diagnosis. If marked abnormalities are found one should suspect a technical problem (e.g., improper
on electrophysiologic testing in the same distribution electrode or stimulator placement or too low of a
where the clinical examination is normal, either the stimulus intensity). If the data are not technically
clinical examination or the electrophysiologic testing accurate, then correct data interpretation can never
must be called into question. One usually finds that occur, either at the time of the study or later by the
the better the clinical examination, the better the treating physician.
differential diagnosis, and thus, the more clearly 3. When in doubt, reexamine the patient. This is essentially
directed the EDX studies will be. an extension of cardinal rule number 1. In the example
2. When in doubt, always think about technical factors. given with rule number 2, if the sural sensory response
EDX studies rely upon collecting and amplifying is absent after all possible technical factors have been
very small bioelectric signals in the millivolt and corrected, the clinician should reexamine the patient.
microvolt range. Accomplishing this is technically If the patient has clear loss of vibration at the ankles,
demanding; many physiologic and nonphysiologic there is less concern about an absent sural sensory
factors can significantly interfere with the accuracy response. If the patient’s sensory examination is normal
of the data. Accurate NCSs and EMG depend on on reexamination, the absent sensory response does not
intact equipment (e.g., EMG machine, electrodes, fit the clinical findings, and technical factors should be
and stimulator), and on correct performance of the investigated further.
study by the electromyographer. Technical problems 4. EDX findings should be reported in the context of
can easily lead to absent or abnormal findings. Failure the clinical symptoms and the referring diagnosis. In
to recognize technical factors that influence the EDX every study, electrophysiologic abnormalities must be
study can result in type I errors (i.e., diagnosing an correlated with the clinical deficit.
 Chapter 1 • Approach to Nerve Conduction Studies, Electromyography, and Neuromuscular Ultrasound 7

Because electrophysiologic studies are quite sensitive, it If all three results fit together, the diagnosis is secure.
is not uncommon for the electromyographer to discover However, if the NCSs and EMG findings do not fit together
mild, subclinical deficits of which the patient may not and, more importantly, if they do not correlate with the
be aware. For example, a diabetic patient referred to clinical findings, the significance of any electrical abnormali-
the EMG laboratory for polyneuropathy may show ties should be seriously questioned. Consider a patient with
electrophysiologic evidence of a superimposed ulnar pain in the arm who has an otherwise normal history and
neuropathy but have no symptoms of such. Accordingly, examination. If the NCSs are normal except for a low ulnar
the electromyographer should always report any sensory potential and the EMG demonstrates only mild rein-
electrophysiologic abnormality in the context of its nervation of the biceps, one should be reluctant to interpret
clinical relevance so that it can be properly interpreted. the study as showing a combination of an ulnar neuropathy
5. When in doubt, do not overcall a diagnosis. Because and a C5 radiculopathy. These mild abnormalities, which
electrophysiologic tests are very sensitive, mild, are not substantiated by other electrophysiologic findings
subclinical, and sometimes clinically insignificant and do not have clear clinical correlates, may have little to
findings often appear on EDX testing. This occurs do with the patient’s pain. In such a case, the patient should
partly because of the wide range of normal values, be reexamined. If no clinical correlate is found, the studies
which vary with the nerve and muscle being tested. should be rechecked. If the abnormalities persist, they may
In addition, there are a variety of physiologic and be noted as part of the impression but interpreted as being
nonphysiologic factors that may alter the results of both of uncertain clinical significance.
NCSs and EMG, despite attempts to control for them. When performed properly, NCSs and EMG can be very
These factors, often when combined, may create minor helpful to the referring physician. However, the limitations
abnormalities. Such minor abnormalities should not of EDX studies must be appreciated, technical factors well
be deemed relevant unless they correlate with other controlled, and a good differential diagnosis established
electrophysiologic findings and, most importantly, with before each study. Otherwise, the study may actually do a
the clinical history and examination. It is a mistake to disservice to the patient and to the referring physician by
overcall an electrophysiologic diagnosis based on minor leading them astray by way of minor, irrelevant, or techni-
abnormalities or on findings that do not fit together cally induced “abnormalities.” If the cardinal rules of NCSs
well. Sometimes, the clinical or electrophysiologic and EMG are kept in mind, EDX studies are far more likely
diagnosis is not clear-­cut and a definite diagnosis to be of help to the referring clinician and the patient with
cannot be reached. Occasionally, NCSs and EMG are a neuromuscular disorder.
clearly and definitely abnormal, but a precise diagnosis
still cannot be determined. For example, consider
the patient whose clinical history and examination NEUROMUSCULAR ULTRASOUND
suggest an ulnar neuropathy at the elbow. The EDX Over the last several years, neuromuscular U/S has increas-
study often demonstrates abnormalities of the ulnar ingly been used along with EDX studies in the evaluation
nerve in the absence of any localizing findings, such as of patients with various neuromuscular conditions. The use
conduction block or slowing across the elbow. Although of neuromuscular U/S has grown due to a combination of
the referring surgeon usually wants to know whether several factors, including the marked improvement of the
the ulnar neuropathy is at the elbow, often, the only resolution and software of U/S machines while the physi-
accurate impression that the electromyographer can cal size and cost of the machines have gone down. These
give is one of a non-­localizable ulnar neuropathy that is smaller, portable machines can easily be used and shared
at, or proximal to, the most proximal abnormal ulnar-­ among EDX laboratory rooms. Indeed, some manufacturers
innervated muscle found on EMG. are working on combined EDX and U/S machines housed in
6. Always think about the clinical-­electrophysiologic one unit. Hundreds of peer-­reviewed articles on the useful-
correlation. This rule combines all of the earlier rules. ness of neuromuscular U/S are published each year. Thus,
One usually can be certain of a diagnosis when the neuromuscular U/S has become a validated, reliable, and
clinical findings, NCSs, and EMG abnormalities all important tool in the evaluation of many neuromuscular
correlate well. Consider again the example of the disorders.
patient with weakness of the hand and tingling and Physicians who perform EDX studies are best suited to
numbness of the fourth and fifth fingers. If NCSs learn and perform neuromuscular U/S, which is the study
demonstrate abnormal ulnar motor and sensory of peripheral nerves and muscles. Neuromuscular U/S dif-
potentials associated with slowing across the elbow, fers from vascular U/S and musculoskeletal U/S, although
and the needle EMG shows denervation and reduced there is some overlap. It is essential to emphasize that neuro-
numbers of motor unit potentials in all ulnar-­ muscular U/S is complementary to EDX studies; it does not
innervated muscles and a normal EMG of all non-­ replace EDX studies. The situation is similar to the role that
ulnar–innervated muscles, there is a high degree of EDX studies and MRI have in the evaluation of radiculopa-
certainty that the patient truly has an ulnar neuropathy thy. EDX studies are physiologic tests and, as such, yield
at the elbow, and the electrophysiologic abnormalities information about the function of the nerves, nerve roots,
are indeed relevant. and muscles. In contrast, imaging studies show a picture of
  
8 SECTION I Overview of Nerve Conduction Studies and Electromyography

the nerves, nerve roots, and muscles, but yield no informa- its origin in the lower brachial plexus and assess for struc-
tion on the functioning. Thus, for example, whereas EDX tural abnormalities to localize the lesion.
studies give information about how well the nerve root is Regardless of whether the EDX study can definitely
functioning, an MRI can show the nerve roots and whether localize the mononeuropathy or not, it is important to know
they appear impinged by a disk, spondylosis, or other struc- what is causing it. Is it an entrapment from wear and tear?
tural causes. Likewise, whereas EDX studies may be able Or from tenosynovitis? Or from compression from a gan-
to identify an ulnar neuropathy at the elbow, they cannot glion cyst? Or from a nerve sheath tumor? And the list con-
discern what is causing it. This is where neuromuscular U/S tinues. This is also where U/S may add critical anatomic
may add critical complementary information to the EDX information about what is causing the mononeuropathy.
studies. For example, it may reveal bony spurs and excess Performing U/S after EDX studies for a mononeuropa-
callus in the ulnar groove from tardy ulnar palsy, compres- thy may result in three different outcomes:
  
sion of the ulnar nerve in the true cubital tunnel under
1. It may add no useful information, or
the humeral-­ulnar aponeurosis, or in some cases a synovial
2. It may add important complementary information, or
cyst compressing the ulnar nerve, among many possible
3. It may be the key to the case.
etiologies.   
Best used, neuromuscular U/S serves as an extension of Unfortunately, one does not know before doing the U/S
the clinical examination and most often of the EDX studies, study which of these three outcomes will be the result.
and should be used as such. Chapters 17, 18, and 19 discuss Thus, one can make a strong argument that U/S is a reason-
neuromuscular U/S in greater detail. In addition, many of able adjunct to EDX studies for most mononeuropathies.
the later clinical chapters expand on the usefulness of neu-
romuscular U/S in specific conditions.
Similar to EDX studies, there are several general princi- Peripheral Nerve: Polyneuropathy
ples of neuromuscular U/S. Each neuromuscular U/S study The role of neuromuscular U/S in polyneuropathy is most
must be individualized, based on the neurologic examina- useful when looking for evidence of a hypertrophic (usu-
tion, the EDX study, and the differential diagnosis, and ally meaning demyelinating) polyneuropathy. As will be dis-
modified as the study progresses and further information cussed in Chapter 29 on Polyneuropathy, the vast majority
is gained. Its primary use is to add anatomic and pathologic of polyneuropathies display an axonal loss pattern on EDX
information that complements the EDX study. Among neu- studies. Very few are primarily demyelinating. However,
romuscular U/S’s many potential uses, the major ones are the presence of demyelination on EDX studies of a poly-
described in the following sections. neuropathy is a critical piece of information, as it mark-
edly narrows the differential diagnosis. Furthermore, in the
case of acquired demyelinating polyneuropathies, most are
Peripheral Nerve: Mononeuropathy inflammatory (autoimmune) and potentially very treatable.
In general, U/S is particularly helpful in many mononeu- The U/S picture of a chronic demyelinating polyneu-
ropathies. Mononeuropathies are commonly caused by ropathy is usually that of a hypertrophic neuropathy (from
entrapment, trauma, and in some cases other structural repeated demyelination and remyelination, Schwann cell
causes. When the EDX study has localized the problem to proliferation, and onion blub formation). EDX studies may
one segment of one peripheral nerve (e.g., the median nerve be indeterminate in some chronic demyelinating neuropa-
at the carpal tunnel), U/S can confirm the localization by thies. Sometimes, this is due to responses being absent. In
revealing structural changes in the nerve at the involved site. others, the EDX criteria for demyelination are not fully
In the case of carpal tunnel syndrome, U/S usually simply met, or the conduction velocities are in the borderline range
adds additional confirmatory evidence of the nerve loca- between axon loss and demyelination. In these cases, pre-
tion, although as we will see later in Chapter 20 on Median sumably the demyelination is not marked enough to call,
Neuropathy at the Wrist, U/S may add more information, or the segments of nerve that are demyelinated are not eas-
especially in postoperative cases or when symptoms recur. ily assessed by standard EDX studies (e.g., very proximal
In other cases of mononeuropathy, when the EDX study nerves, plexus, and/or roots). Hence, U/S can be extremely
localizes the problem to one nerve but is not able to local- useful in establishing a polyneuropathy as demyelinating.
ize a specific segment, U/S can be particularly useful. This
occurs when the pathophysiology is that of axonal loss. In
this situation, U/S is particularly helpful as it can often Motor Neuron Disease
localize the lesion more precisely than EDX studies. Take EDX studies in conjunction with the history and physi-
the example of the patient with a weak grip and numbness cal examination are the cornerstones of diagnosing motor
involving digit 5. An ulnar neuropathy is considered most neuron disease. U/S has a limited role in the diagnosis of
likely. The EDX study might show abnormalities limited to motor neuron disease, with a couple of notable exceptions.
the ulnar nerve, but there is no slowing or conduction block First, U/S is extremely good at detecting fasciculations—
across the elbow. Is the lesion at the elbow? Or is it as the better than the clinical examination and needle EMG. The
wrist, in the forearm, upper arm, or in the lower brachial research criteria for the diagnosis of motor neuron disease
plexus? U/S can visualize the ulnar nerve from the wrist to (and qualification for clinical studies) requires evidence of