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 SECOND EDITION

THE INTEGRATED
NERVOUS SYSTEM
A SYSTEMATIC DIAGNOSTIC
CASE- BASED APPROACH
http://taylorandfrancis.com
 SECOND EDITION

THE INTEGRATED
NERVOUS SYSTEM
A SYSTEMATIC DIAGNOSTIC
CASE-BASED APPROACH
WALTER J. HENDELMAN, MD, CM
University of Ottawa
Ontario, Canada

PETER HUMPHREYS, MD, FRCP(C)

Children’s Hospital of Eastern Ontario
Ottawa, Canada

CHRISTOPHER R. SKINNER, MD, FRCP(C)

University of Ottawa
Ontario, Canada
CRC Press
Taylor & Francis Group
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© 2018 by Taylor & Francis Group, LLC

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CONTENTS
Preface................................................................................................................................................................................ vi
Organization of the Book and Integration with the Web Site........................................................................................... viii
Acknowledgements...............................................................................................................................................................x
Authors................................................................................................................................................................................ xi
Introduction....................................................................................................................................................................... xiii

Section 1 The Basics of Neurological Problem Solving

1 Synopsis of the Nervous System.....................................................................................................................................3

2 Neurological Examination........................................................................................................................................... 24

3 Clinical Problem Solving............................................................................................................................................. 44

Section 2 Applying the Basics to Clinical Cases

4 Fifi.................................................................................................................................................................................72

5 Cletus............................................................................................................................................................................92

6 Ernesto........................................................................................................................................................................ 111

7 Bernie..........................................................................................................................................................................131

8 Etienne........................................................................................................................................................................157

9 Cheryl..........................................................................................................................................................................178

10 Patty......................................................................................................................................................................... 200

11 Didi...........................................................................................................................................................................223

12 Armand.....................................................................................................................................................................243

13 Mickey......................................................................................................................................................................261

Section 3 Supplementary Considerations: Rehabilitation and Ethics

14 Neurorehabilitation...................................................................................................................................................285

15 Ethics and Neurology................................................................................................................................................296

Glossary............................................................................................................................................................................307

Annotated Bibliography.................................................................................................................................................... 319

Answers to Chapter Questions..........................................................................................................................................327

Index.................................................................................................................................................................................336
v
PREFACE
You might be wondering why this textbook about neurology and neurologic problem-solving, using a case-based
approach, is called the INTEGRATED Nervous System.
If you have ever attended an orchestral concert playing classical music, perhaps a symphony by Beethoven or
Brahms or Stravinsky, you would have heard, and perhaps seen, the marvelous synchronization of the various musical
instruments. It is incredible how the music is created as each member of the group starts or stops playing according to
the musical score, resulting in the sound that we hear. Perhaps, you are fortunate enough to actually play in an orches-
tra or band and are part of that experience. Next, you might think about yourself, the listener. After all, your brain
receives just sound waves via a complex series of connections. Just where in the brain is ‘the music centre’? Finally,
you might contemplate the genius of the composers whose brains produced this complex combination of sounds, the
music we hear.
So it is with our brains. No part of the brain acts alone. All its component parts participate in a wondrous synchro-
nization of thought/feeling/sensation/motor activity that characterizes our daily behaviour. You almost need to ‘dissect’
behaviour to recognize the contribution of the component parts. The mature nervous system integrates information from
different sensory modalities, amalgamates experiences and information from the past and correlates it with the present
context, resulting in responses (verbal and/or motor) that are (hopefully) appropriate for the situation.
The integration that we the authors are striving to achieve with this book is the approach that you as a non-neurologist
will need in order to weave together your knowledge of neuroanatomy with the art and science of clinical neurology.
We, the coauthors of this book, include a neuroanatomist (WH), an adult neurologist (CS) and a pediatric neurologist
(PH), all of whom have been involved in teaching a combined neuroscience and neurology course to second-year medi-
cal students. This is a five-week course, case-based, with lectures, organized on the principle of adult learning in small
groups, with expert tutors (almost all practicing neurologists). Clinical disease entities (e.g. multiple sclerosis) are used
as the ‘problems’ in the course.
One of the real challenges in a course that includes both neuroscience and neurology is the enormous scope of the
subject matter; this leads to significant information overload when the process is compressed into a short period of block
learning. A by-product of this compressed learning process has been the observation by both clinicians that most of their
students – members of their tutor groups who ‘knew’ their material and passed the course, often with a good grade –
when returning as clinical clerks or while taking a neurology elective, were unable to use the knowledge they once had
mastered to solve clinical problems at the bedside.
This lack of integration of basic science and clinical information has been postulated as the basis of a syndrome
called neurophobia, which apparently can affect one of every two medical students. This lack of ability to reason
through clinical problems results in anxiety and dislike of the subject matter and, eventually, negative sentiments
about and even fear of neurology (Jozefowicz, 1994; Fantaneanu et al., 2014). This book has been created to integrate
these two worlds and to overcome this pedagogical deficit, using a problem-based approach with clinical disease enti-
ties. Our objective is to bridge the gap between the book and the bedside, in other words between the classroom and
the clinic.
The second edition of this book has been revised and edited keeping you, the learner, in mind. We have rewritten and
updated the text, as well as many of the tables. To help you understand the neurological disorders that you will encoun-
ter in this text and the challenges that patients and relatives face when such disorders occur, we have created a fictional
‘person’ for each of the clinical chapters as well as for the cases that are found on the accompanying Web site, called
‘e-cases’, with each of these chapters. The most significant addition is the abbreviated presentation of the history and
­neurologic examination of all the e-cases (now numbering over 50) within the text at the end of each of the clinical chap-
ters; along with this, we have created ‘maps’ – a visual representation of the clinical motor, sensory and reflex findings.
We hope that this inclusion will encourage the student to go to the Web site where each of the cases is presented in detail
and where the ‘expert’ discusses the reasoning for the localization and etiological diagnosis.
We believe that this book and the associated Web site will be of practical value to all the professionals who deal with
people who have neurological conditions, not only medical students and residents. This includes physiatrists (rehabilita-
tion medicine specialists), physiotherapists, occupational therapists and speech therapists, and nurses who specialize in
the care of neurological patients. We think that this text will also be of value for family physicians and specialists in

vi
Preface vii

internal medicine and pediatrics, all of whom must differentiate between organic pathology of the nervous system and
other conditions.

Dr. Walter J. Hendelman

Dr. Peter Humphreys
Dr. Christopher Skinner
Faculty of Medicine
University of Ottawa
Ottawa, Canada

REFERENCES
Fantaneanu, T.A. et al. Neurophobia inception. Can J Neurol Sci 2014. 41: 421–429.
Jozefowicz, R.F. Neurophobia: The fear of neurology among medical students. Arch Neurol 1994. 51: 328–329.
ORGANIZATION OF THE BOOK
AND INTEGRATION WITH THE WEB SITE
The aim of this book is to enable you, the learner, to use your knowledge of the nervous system combined with a neuro-
logically based, problem-solving clinical reasoning approach to neurology to help in the diagnosis and treatment, in the
broadest sense, of those who suffer from a neurological disease or injury. We hope that this approach meets with success,
insofar as it leads to an improvement in the diagnosis and care of persons afflicted by neurological problems.
To help you understand the neurological disorders that you will encounter in this text and the challenges that patients
and relatives face when such disorders appear, we have created fictional persons with names. We meet the first of these
people in the Introduction, which lays out the complexity of the nervous system and its capacity to multitask. The intro-
duction exposes the reader to neuroanatomical pathways, which, by the end of the book, you should be quite familiar with
in terms of both their function and their importance in neurology.
The first section is called ‘The Basics of Neurological Problem Solving’. Chapter 1 is a review of clinically relevant
neuroanatomy, enough to set the stage in terms of the basic knowledge of the nervous system needed for this book. The
second chapter is devoted to the clinical neurological examination and the integration of the information garnered in
terms of the normal functioning nervous system, for example, the assessment of reflex activity. Chapter 3 introduces the
student to neurological clinical reasoning for the purpose of localizing the disease or lesion within the nervous system
and determining the aetiology, the pathophysiological mechanism of disease. Patients with neurological problems present
great challenges to their physicians (as well as to their relatives and caregivers) to accurately and completely gather the
information required to make a working diagnosis and plan of investigation based on a single clinic visit. The approach
used in this chapter and its accompanying worksheets is designed to provide students and non-neurologist clinicians with
practical guidelines and tools with respect to diagnosis for the full range of neurological problems seen by a neurological
generalist. This approach is applied throughout the book.
The next section is called ‘Applying the Basics to Clinical Cases’. The succeeding 10 chapters deal with important
specific clinical diseases or syndromes that have afflicted our fictional individuals, each with the focus on a different
component of the nervous system, starting with the peripheral nervous system. The following chapters ‘ascend’ – spinal
cord, brainstem, all the way to the cortex, with the last chapter in this section devoted to behavioural issues. For each of
these cases, the history is presented, followed by the findings of the neurological examination. Additional neuroanatomi-
cal, neurophysiological and neurochemical information is added, where required, as it pertains to the clinical condition
discussed in the chapter. Notwithstanding the information given, it is suggested that students review their knowledge
of neuroanatomy, neurophysiology, neuropharmacology and neuropathology, using other resource books (given in the
suggested readings and references sections at the end of each chapter and in the Annotated Bibliography at the end of
the book). In each of these chapters, there is an application of the process of neurological reasoning to narrow the pos-
sibilities of where the lesion is located. This is followed by a systematic analysis in order to determine what disease (or
diseases) should be considered. Relevant selected investigations are then presented and the results discussed. Finally, the
diagnosis is made, with its prognosis, and an outline of the appropriate management is given, ending with the outcome
of the case.
The text illustrations have been prepared with an emphasis on the functioning nervous system. In addition to neuro­
anatomical drawings related to the cases and tables with relevant clinical data, there are figures illustrating neuro-
physiological concepts, clinical findings (such as radiographic images and electroencephalograms) and microscopic
neuropathological images. Again, the information is described in the context of the disease presented in that chapter. The
glossary of terms also emphasizes clinical terminology.
The third section, named ‘Supplementary Considerations’, presents additional insights written by guest authors,
Dr. Anna McCormick, a physiatrist (a specialist in physical medicine rehabilitation), and Dr. Robert Nelson, a senior neu-
rologist with expertise in ethics. Both discuss other important dimensions of neurological problems: rehabilitation and
ethics. Currently, rehabilitation has much to offer for those afflicted by disease or injury of the nervous system; there is
now a certain air of hopefulness that there can be recovery of function following an insult to the nervous system, in adults
as well as in children. The ethical principles and reasoning on the basis of which decisions (sometimes quite unique) are
taken in neurological cases are presented in the context of an inherited disease of the nervous system.
There is a Web site associated with this textbook: http://www.integratednervoussystem.com.

viii
Organization of the Book and Integration with the Web Site ix

This Web site contains the worksheets that have been developed to apply the clinical reasoning approach to neuro-
logical problem solving and the e-cases that are now part of each of the clinical chapters. The e-cases enlarge the scope of
the book by adding additional commonly seen neurological diseases for each level of the nervous system. These are pre-
sented in a more sequential fashion, although once having learned the analytic approach, it is hoped (expected) that you,
the learner, will work through each clinical case on your own, using the worksheets, before reading the case evolution,
investigations and resolution. It is highly recommended that you, the non-neurologist student/learner, apply this approach
when confronted with a neurological patient. The authors want you to learn how to think like a neurologist. In addition,
the Web site has all the illustrations found in the book, with animation added to assist in the understanding of some reflex
circuits and various pathways. It also includes the glossary. There is also a learning module to assist you, as a non-expert,
in understanding neuroimaging, how the various modes of computed tomography and magnetic resonance imaging assist
in localizing a lesion and defining the likely etiology. The Web site will also be utilized to provide updates on the cases
presented as well as new cases, so it may be wise to check it periodically.
We sincerely hope that our system will work for you and wish you every success in the diagnosis and management
of your neurological patients.
ACKNOWLEDGEMENTS
ILLUSTRATIONS
We wish to express our appreciation and respect for the illustrators who have helped shape this book: Perry Ng, the
principal illustrator and Dr. Tim Willett, who is the principal illustrator for the Atlas of Functional Neuroanatomy (also
published by CRC Press). Without their creative and conscientious efforts, we could not have achieved what is necessary
to convey our message to you, the learner. Several illustrations from the Atlas have been included in this book, with the
permission of the publisher.

CONTRIBUTORS
A special note of gratitude is extended to our chapter contributors, Dr. R. Nelson (Neurology, The Ottawa Hospital, now
retired) and Dr. A. McCormick (Physical Medicine and Rehabilitation, The Children’s Hospital of Eastern Ontario).
Dr. Nelson is a neurologist’s neurologist, highly regarded by his colleagues, with a special interest in ethical issues.
Dr. McCormick, who carries with her an air of enthusiasm and hope, has successfully championed the cause of pediatric
rehabilitation and is one of the few people in her field who actively treats both children and adults.
Many colleagues have contributed collegially and willingly to this book, with illustrations and clinical material. We
are particularly grateful not only for their particular and unique contribution but also for the spirit in which it has been
donated. In many cases, their staff helped with the preparation of this material and we thank them as well.
Dr. D. Grimes: Division of Neurology, The Ottawa Hospital
Dr. R. Grover: Neuroradiology, The Ottawa Hospital (resident-in-training)
Dr. M. Kingstone: Neuroradiology, The Ottawa Hospital
Dr. D. Lelli: Division of Neurology, The Ottawa Hospital
Dr. J. Marsan: Otolaryngology, The Ottawa Hospital
Dr. J. Michaud: Neuropathology, The Ottawa Hospital
Dr. M. O’Connor: Ophthalmology, The Children’s Hospital of Eastern Ontario
Dr. C. Torres: Neuroradiology, The Ottawa Hospital
Dr. S. Whiting: Neurology, The Children’s Hospital of Eastern Ontario, with S. Bulusu (chief technologist, Clinical
Neurophysiology Laboratory, CHEO)
Dr. J. Woulfe: Neuropathology, The Ottawa Hospital

WEB SITE
We would also like to acknowledge the work of David Skinner, who diligently crafted the Web site to emulate the
problem-solving methodology of the text.

SUPPORT
We also wish to thank the secretarial staff that we work with in our various offices, including the Department of Cellular
and Molecular Medicine at the Faculty of Medicine, and particularly Orma Lester, who has assisted us in addition to her
regular duties in a busy hospital office.
The Health Sciences library staff, particularly M. Boutet, is thanked for assistance in creating the Annotated
Bibliography.
Computer support has consistently been available at the Faculty of Medicine at the University of Ottawa from
Medtech (Information Management Services) and from staff at the Children’s Hospital of Eastern Ontario (CHEO).
Photographic services were also provided at CHEO.
Last, but not least, the authors gratefully acknowledge the assistance and cooperation of the production team of CRC
Press, particularly our project editors.

x
AUTHORS
Dr. Walter J. Hendelman is a Canadian born and raised in Montreal. He did his undergraduate studies at McGill
University in science with honours in psychology, where he studied under Dr. Donald Hebb, who is now recognized for
his “cell assembly” theory, explaining how the brain manages information. He then proceeded to do his medical studies
also at McGill. Following a year of internship and a subsequent year of pediatric medicine in Montreal, Dr. Hendelman
chose the path of brain research and academia.
Postdoctoral studies followed in the emerging field of developmental neuroscience, using the “new” techniques
of nerve tissue culture at the Pasadena Foundation for Medical Research. These studies continued, including electron
microscopy, at Columbia University Medical Center in New York City; Dr. Richard Bunge was his research mentor and
his neuroanatomy mentor was Dr. Malcolm Carpenter, author of the well-known textbook Human Neuroanatomy.
Dr. Hendelman then returned to Canada and has made Ottawa his home for his academic career at the Faculty of
Medicine at the University of Ottawa. He began his teaching in gross anatomy and neuroanatomy and then concentrated
on the latter, first assuming the responsibility of coordinator for the course and then becoming co-chair for the teaching
unit on the nervous system in the new curriculum. His research focused on the examination of the development of the
cerebellum and the cerebral cortex in organotypic tissue cultures.
Dr. Hendelman has dedicated his career as a teacher to assisting those who wish to learn functional neuroanatomy
including medical students and trainees, as well as those in the allied health sciences. The first edition of his teaching
ATLAS (1987, published by the University of Ottawa Press) was followed by other editions and subsequently was pub-
lished by CRC Press (2000) with the title Atlas of Functional Neuroanatomy. The second edition of the Atlas (2006) is
accompanied by a Web site (www.atlasbrain.com) with interactive features, including roll-over labelling and animations
of pathways and connections. This edition has been translated into Italian (2009) and also into French (2013), with a
Web site in French. The third edition has now been published (2016, CRC Press), and a Web site for this new edition will
be available. An Italian translation of this edition has already been published and other translations are pending.
Additional learning resources developed by Dr. Hendelman include several narrated teaching videotapes on the skull
and the brain using anatomical specimens; these are now available online on the Web site for the Atlas.
Dr. Hendelman is currently professor emeritus in the Faculty of Medicine, University of Ottawa.

Dr. Peter Humphreys, a graduate of the McGill University Faculty of Medicine in 1966, trained in pediatrics at Boston
Children’s Hospital and at St. Mary’s Hospital in London, followed by training in neurology at the Montreal Neurological
Institute. After a six-year stint on the neurology staff of the Montreal Children’s Hospital, he became the founding head
of the Neurology Division at the Children’s Hospital of Eastern Ontario, Ottawa, a position he held for 23 years. Although
now semi-retired, Dr. Humphreys continues an active outpatient clinical practice. His principal area of interest is in
disorders of brain development. Ten years ago, he started the first Canadian hospital-based clinic devoted to the compre-
hensive care of girls and women with Rett syndrome. In this role, he is involved in clinical research related to movement
disorders in Rett syndrome as well as collaborating with researchers in the University of Ottawa Faculty of Medicine in
the investigation of the enteric nervous system of mouse models of Rett syndrome.
A full professor in the Department of Pediatrics at the University of Ottawa, Dr. Humphreys has been active at all
levels of the medical curriculum. For many years, he was a tutor in small-group learning sessions for second-year medi-
cal students doing a problem-based introductory course on the nervous system. During the same course, he presently
conducts a full-class lecture on brain developmental disorders as well as a live patient demonstration devoted to the
pediatric neurological examination. He also does clinic instruction for residents in neurology and pediatrics. Finally, he
participates in a teaching role in refresher courses for paediatric neurology residents at the University of Ottawa and for
pediatricians in courses offered by the Canadian Paediatric Society.

Dr. Christopher R. Skinner earned his BEng (electrical) from the Royal Military College in 1970. He worked as a systems
engineer with the Department of National Defence, implementing nationwide information systems until 1975. He earned
his medical degree from Queen’s University in 1979. He received his specialist certification in general internal medicine
in 1986, in neurology in 1987, and qualified as a Diplomat of the American Board of Sleep Medicine in 2005. He was
chief information officer at the Ottawa Hospital from 1996 to 1998.

xi
xii Authors

He has been a clinical teacher and lecturer in the Faculty of Medicine at the University of Ottawa since 1993. He has
taught clinical neurology, occupational neurology and sleep medicine to all levels of study, including medical students,
residents, physician assistants and military flight surgeons. He was also involved in the design and implementation of the
problem-based digital curriculum portal used for the teaching of medical students.
In 2011, Dr. Skinner did a three-month sabbatical with the Russian Space Agency in Moscow, studying the effects of
long-duration space flight on the nervous system and its effect on sleep in space.
Dr. Skinner currently practices general neurology and sleep medicine at the Ottawa Hospital. He also has a practice
in rural Quebec as well as patients in inner city shelters in Ottawa.
INTRODUCTION
Crash (as he is known to his colleagues from his call sign) had applied and been accepted into the international astronaut
training program.
This was a gruelling four-year training program learning how to control both Russian and American spacecraft in
order to go into space for near-earth and deep-space exploration.
He was selected to be the commander of a mission, which consisted of a three-person crew to rendezvous, land on
and redirect a near-earth asteroid.
He had over 800 days of spaceflight under his belt. After his last mission, he had noticed a decrease in his vision and
the flight surgeon had noted some papilledema. This could be a sign of visual impairment and increased intracranial
pressure, a condition seen in astronauts with long duration in space.
His latest post-flight magnetic resonance imaging scan of the brain also had shown some new changes in the white
matter of the brain. He wondered if this could be damage related to long exposure to cosmic radiation, which damages
the white matter.
Learning the command and control systems of the Russian spacecraft was particularly difficult, not only because the
instructions and instruments were all in Russian but also due to the fact that the controls required a much more direct
physical touch than the fly-by-wire American systems. He welcomed having to learn a new language as he had heard that
it prevents dementia.
Crash was due to go on a test flight of eight days in orbit to evaluate the systems required for the asteroid mission.
As he entered the elevator to the preparation room at the launch site in Kazakhstan prior to launch, his olfactory sys-
tem sensed the odour of kerosene in the rocket fuel outside and transmitted these impulses to his mesial temporal cortex.
He says to himself, ‘I love the smell of rocket fuel in the morning’. It is amazing how smells can bring back memories,
thinking back to his early flying career.
As he sat with his pressure suit on the pad in the Baikonur Cosmodrome, he could feel the weight of the hand control
at 1G of gravity. The sensation of the position of his fingers travelled up through the posterior columns of the spinal cord,
relaying once in the brain stem and again in the contralateral thalamus to terminate in the parietal cortex. This informa-
tion would be relayed to areas of the prefrontal cortex to allow Crash’s corticospinal tract to send messages to the spinal
cord and then spinal motor units to make small adjustments in his hand muscles to control the spacecraft. The motor
sequences that had been practiced thousands of times were stored in a network involving the prefrontal cortex and basal
ganglia modulated by the cerebellum. His memories of the procedures and instructions had been encoded through the
mammillothalamohippocampal system and then exported to populations of cortical neurons for long-term storage.
As he waited on the pad with his two crewmates, his amygdala kicked in, causing him to feel anxious; he could feel
that there was spasm of his anal sphincter through the spinothalamic tract passing pain and temperature information to
the thalamus and contralateral cortex. He says to himself, ‘Come on – Let’s light up the old vodka burner’.
Time for launch: 5, 4, 3, 2, 1! The five large rocket motors of the booster assisted by two solid rockets light up under
him and the vehicle clears the tower heading down range at a velocity faster than the speed of sound. At 65 seconds into
the flight, an alarm sounds, there is a problem with the left hand solid rocket booster!
Crash has to make a decision: abort or not to abort; he had exactly 3 seconds to make this decision. The executive
function of his frontal cortex is in overdrive. He presses the abort button; the solid rockets and upper stages fall away.
He mutters to himself, ‘another bunch of guys at the rocket factory are going to go to jail for this’. The spacecraft is now
60 km in altitude going 10,000 km per hour in freefall. Although he had practiced for such a ‘forward abort’ situation, this
requires Crash to make some hard decisions about where to land. He coolly turns to his crewmates and asks, ‘Вода или
Земля’ (‘Water or Land’)? They both answer ‘land’ knowing that water meant the Arctic Ocean in January.
With his visual and auditory system fully engaged, Crash calmly takes hold of the controls, commands the spacecraft
a series of S turns to bleed off the speed and heads for the frozen, snow-bound taiga. The parachute then deploys and the
capsule lands with a thud in the Siberian taiga, 500 km from any habitation.
The crew members crawl out, pull out the survival kits and light a big fire with wood that they have gathered. Crash
says casually to his crewmates: ‘I guess we will have to cross-country ski out like they did in Voskhod 2’.
He calls Fifi on his satellite cell phone, ‘Good news dear, we will not miss for our anniversary this year’. His Russian
­colleague asks: ‘What are we going to call this mess?’
Crash answers: ‘Сибирский Лебединое погружение’ (‘The Siberian Swan Dive’).

xiii
http://taylorandfrancis.com
 Section 1

THE BASICS OF NEUROLOGICAL

PROBLEM SOLVING

1
http://taylorandfrancis.com
 Chapter 1

Synopsis of the Nervous System

Objectives disease process (e.g. infectious, vascular, neoplastic) is
occurring and its pathophysiology, and to identify dis-
• To review the basic histological knowledge of eases that most likely account for the patient’s signs and
the nervous system from a functional (neuro- symptoms.
logical) perspective Laboratory investigations, including blood work, special
• To organize the nerves, nuclei and tracts of the tests (e.g. disease-specific antibody levels) and particu-
nervous system into functional systems larly neuroimaging, usually provide additional informa-
tion to help pinpoint the localization of the disease and
In the Introduction, Crash, pilot and astronaut, executes a often limit the possible list of most likely diseases.
number of intricate tasks in response to input from several Lastly, the neurologist will synthesize the patient’s
sensory systems: tactile, muscle and joint, visual, vestibu- history and the symptoms with the signs found on neuro-
lar and auditory. He reacts to all of these stimuli appro- logical examination as well as the additional information
priately and performs highly accurate and skilled motor provided by the investigations to come up with the defini-
movements. Pathways were sketched for the sensory input tive diagnosis. This diagnosis allows for a therapeutic plan
and for executing the motor movements. and some idea of the likely outcome: the prognosis. All
How does the brain process all this information? of this must be communicated sensitively to the patient
Which parts of the nervous system are involved in the and family in a way that can be readily understood. (A
exquisite motor control required to fly a jet aircraft or pilot sample case exemplifying this approach is presented in
a space craft? Where in the ‘brain’ are the integration and Chapter 3.)
decision-making functions carried out?
A neurologist’s view of the nervous system is one of
functionality – are all the components operational in order
to receive information, analyze and assess its significance
1.1 NEUROBASICS
and produce the appropriate action? If not, the task of the The nervous system is designed to receive information,
physician is to determine where the problem is occurring analyse the significance of this input and respond appro-
and what is its most likely cause – the localization of the priately (the output), usually by performing a movement
lesion and its possible etiology. or by communicating ideas through spoken language. In
In order to determine where, the localization, one its simplest form, this process would require a minimum
needs to have knowledge of the anatomy and physiol- of three neurons, but as we come up through the animal
ogy of the nervous system. This chapter will provide that kingdom, the complexity of analysis increases incredibly.
information from a functional perspective, but the stu- This evolutionary development culminates in the human
dent should expect to consult other resources – details of central nervous system (CNS), with all its multifaceted
neuroanatomy, neurophysiology and neuropathology – to functions.
supplement this presentation (see Suggested Readings and The nervous system consists of two divisions (Figure 1.1),
the Annotated Bibliography). One needs this knowledge a peripheral component, called the peripheral nervous
to understand the significance of the findings of the neuro- system, the PNS, and a central set of structures, called
logical examination, which is outlined in the next chapter. the central nervous system, the CNS. The PNS consists
Determining the likely cause, the etiology, requires of sensory neurons and their fibers, which convey mes-
knowledge of disease processes. This determination is sages that originate from the skin, muscles and joints and
based initially on the nature of the symptoms and the his- from special sensory organs such as the cochlea (hear-
tory of the illness – how long the problem has been occur- ing); it also carries the motor nerve fibers that activate
ring (acute, subacute, chronic) and how the symptoms the muscles. The autonomic nervous system, the ANS, is
have evolved over time. The task of the p­ ractitioner – also considered part of the PNS; it is involved with the
physician, resident or student – will be to determine what regulation of the cardiac pacemaker system and of smooth

3
4 The Integrated Nervous System

Sensory PNS Motor PNS

Myelinated
motor neuron
Myelinated DRG
sensory neuron
Unmyelinated
Unmyelinated DRG autonomic
sensory neuron AG neuron

Muscle spindle receptor

Skeletal muscle
(voluntary)

Joint position receptor

Sweat gland
(autonomic)
Skin receptor
(fine touch)

Skin receptor Gut

(pain) (autonomic)

PNS – Peripheral nervous system

DRG – Dorsal root ganglion
AG – Autonomic ganglion

FIGURE 1.1: Overview of the nervous system – CNS, PNS and ANS. Sensory fibers, coloured purple, convey information towards
the CNS (afferent) from receptors in skin and muscle. Motor fibers, coloured green, carry instructions away from the CNS (efferent)
to muscle and via autonomic ganglia to glands and viscera.

muscle and glands, including some control of bowel and cells, the neuron has a cell body (the soma or peri-
bladder functions. The CNS consists of the spinal cord, karyon) with a nucleus and the cellular machinery to be
the brainstem and the brain hemispheres. The CNS adds its nutritive centre. Morphologically, it is the cellular
analytic functionality and varying levels of motor control, processes – dendrites and axon – that distinguish a neu-
culminating in a remarkably intricate capacity for ‘think- ron from other cells. The electrochemical nature of its
ing’ forward and backward in time, consciousness, lan- membrane, whereby the interior of the cell and its pro-
guage and executive functions, processes performed in cesses have a negative charge, is a characteristic fea-
different areas of the cerebral cortex. ture of the neuron (see Figures 11.1, 11.2 and 11.3). The
synapse, the electrochemical communication between
neurons, is the other unique feature of nervous tissue
1.1.1 THE NEURON (discussed in Section 1.1.4).
A neuron is the basic cellular element of the nervous sys- The typical neuron in the CNS (Figure 1.2a) has den­
tem. In the most simplistic language of today’s electronic drites that extend from the cell body for several microns.
world, each of the billions of neurons in the human Dendrites receive information from other neurons at
CNS is equivalent to a unique microchip, possessing specialized receptor areas, the synapses, some of which
a specific information processing capacity. Like other form small excrescences on the dendrites, called synaptic
Synopsis of the Nervous System 5

Axon

Cell body

Myelin sheath

(a) Dendrites

Dorsal root ganglion

Spinal nerve/peripheral process

Central process/dorsal root

(b)

Myelin sheath Node of Ranvier

(c)

Presynaptic Postsynaptic
terminal site

(d)

Synaptic vesicle Ligand-gated chloride channel Cl− ion

Ligand-gated sodium channel Na+ ion

FIGURE 1.2: (a) A ‘typical’ CNS neuron has several dendrites and a single (myelinated) axon; its terminal branches communicate
with other neurons. (b) A PNS neuron has both central and peripheral (myelinated) processes. The sensory endings of the peripheral
process are located in the skin, joints and muscles. The cell body of the PNS neuron is located within the dorsal root ganglion (DRG).
The central process enters the CNS via the dorsal root. (c) An axon of a nerve with its myelin sheath: each internode segment is the
territory of a single Schwann cell in the PNS or an oligodendrocyte process in the CNS. A node of Ranvier separates each segment
and the impulse ‘jumps’ from node to node, a process called saltatory conduction. (d) A ‘generic’ synapse is illustrated, including
the presynaptic ending with the synaptic vesicles containing a neurotransmitter, the synaptic ‘gap’ and the postsynaptic site with
its Na and Cl ion channels.

spines (discussed in Section 1.1.4). More complex neurons The neurons in the PNS include both sensory neurons
have an extensive arborization of dendrites and receive and those associated with the ANS. The cell body of a
information from perhaps hundreds of other neurons. sensory neuron is displaced off to the side of its two pro-
Neurons of a certain functional type tend to have a typi- cesses: a peripheral process (e.g. to the skin) and a cen-
cal configuration of their dendrites and group together tral process, which will form synapses within a nucleus
to form a nucleus (somewhat confusing terminology!) in in the spinal cord or brainstem (Figure 1.2b). The distal
the CNS, or a layer of cortex (e.g. the cerebral or cerebel- endings of sensory neurons (e.g. in the skin) are sensitized
lar cortex). Brain tissue is traditionally fixed in formalin to receive information of a certain type and hence behave
for the purpose of study, and neuronal areas (e.g. the cor- functionally as dendrites. Often, they are enveloped by
tex) become grayish in appearance when the brain is cut; specialized receptors (e.g. specialized touch and tempera-
hence, the term gray matter is used for areas of neurons, ture receptors in the skin). Receptors for pain sensation
their processes and synapses. are ‘naked’ nerve endings in the skin and are located
6 The Integrated Nervous System

within all tissues (except brain tissue of the CNS). For the membrane. Impulse conduction along a myelinated axon
special senses such as hearing, there are highly developed ‘jumps’ from node to node, a process called saltatory
receptor cells (e.g. hair cells of the cochlea), which are in conduction, which thus speeds the transmission of the
contact with the sensory neurons. impulse; the membrane potential is recharged at each
The cell bodies of sensory neurons congregate in a node (discussed further in Chapter 4).
specific location, forming a peripheral ganglion ­(plural, Myelin is formed and maintained by glia, support-
­ganglia), typically located along the dorsal root (the dor- ing cells of the nervous system. In the PNS, a single cell,
sal root ganglia; see Figure 1.1). Neurons of the ANS also known as the Schwann cell, is responsible for each inter-
form ganglia (see Figure 1.1), located alongside the ver- node segment of myelin (see Figures 1.2c and 4.6). In the
tebra (sympathetic)­and within or closer to the end organ CNS, the equivalent glial cell is the oligodendrocyte, and
(parasympathetic; see Section 1.2.2.4). each cell is responsible for several segments of myelin, on
a number of axons. Areas of the CNS containing myelin-
ated tracts have a whitish appearance with formalin fixa-
1.1.2 AXONS (NERVE FIBERS)
tion and are hence called the white matter.
Each CNS neuron has (with rare exception) a single axon,
also called a nerve fiber, which is the efferent process of
the neuron, acting like an electric wire to convey infor- 1.1.4 SYNAPSE AND NEUROTRANSMISSION
mation from a neuron to other neurons, muscle or other In the CNS, the terminal end of each axon and each of its
tissue, with the possibility of many branches (collaterals) collaterals is a synapse, a specialized junction, the conduit
en route. Millions of axons course within the CNS provid- by which one neuron communicates electrochemically
ing extensive intercommunication within and between the with another. Synapses abut on the dendrites of other neu-
CNS neuronal nuclei and cortical areas. The axons of func- rons, typically at synaptic spines; they are also located on
tionally linked sensory and motor neurons usually bundle the cell body of neurons, on the initial segment of the axon
together and are called tracts or pathways. A neuron within and sometimes on other synapses.
the brainstem or spinal cord that sends its axon (via the Synapses can be seen with light microscopic tech-
PNS) to skeletal muscle fibers is called a motor neuron, niques but are best visualized with electron microscopy.
also known clinically as the lower motor neuron (LMN). A synapse (Figure 1.2d) consists of an enlargement of the
A typical (observable, dissectible) nerve in the PNS terminal end of the presynaptic axon containing small
usually has both motor and sensory nerve fiber bundles (synaptic) vesicles, the presynaptic membrane and a post-
and, often, autonomic fibers. The postganglionic fibers of synaptic receptor site (e.g. a dendritic spine), where the
the ANS (sympathetic and parasympathetic) are distrib- membrane is specialized for neurotransmission; in some
uted to smooth muscle and glands. cases, the postsynaptic membrane is thickened. In between
is a space or cleft, the synaptic gap, which is sometimes
1.1.3 MYELIN widened compared to the usual space between adjacent
cells in the CNS.
Myelin is the biological (lipid–protein) insulation surround- Biologic agents that can alter the membrane proper-
ing axons; its function is to increase the speed of axonal ties of neurons at the postsynaptic site are called neuro­
conduction (Figure 1.2c). Since axonal conduction velocity transmitters. These are synthesized in the cell body,
increases in proportion to axonal diameter, an alternative transported down the axon and stored in the synaptic end-
to a myelin sheath would be a marked increase in axonal ing in packets, the synaptic vesicles. In some cases, the
diameter; this, if present in most CNS axons, would cause neurotransmitter may be synthesized within the nerve ter-
the nervous system to be extremely bulky. Such an arrange- minal. These endings are activated when the axonal elec-
ment would require much larger axons that would be eas- trical impulse, the action potential, invades the terminal,
ily susceptible to transmission degradation, rendering the setting off a process whereby the transmitter is released
nervous system more inefficient. In the human brain, axons into the synaptic cleft (see Figure 11.4).
may travel for long distances, and the longer the distance, Synaptic transmission is therefore both an electrical
the more likely it is that the fibers are myelinated; faster and a chemical event. In some instances, synapses have
transmission such as that required for certain sensory and built-in mechanisms for recapturing the neurotransmitter
motor functions requires axons with larger diameters, and (recycling); alternatively, enzymes in the synaptic cleft may
these axons have thicker myelin sheaths. destroy the active neurotransmitter. Glial cells (astrocytes)
Myelin is composed of segments, and between each may be involved in this process, for example by removing
segment is a very short ‘naked’ section of the axon, called the neurotransmitter from the synaptic site.
the node of Ranvier; the segments are therefore called Receptors on the postsynaptic neuron are activated
internodes. At each nodal section of an axon, there is a by the neurotransmitter, causing a shift of ions and a net
concentration of sodium ion channels within the axonal change in the membrane potential of the postsynaptic
Synopsis of the Nervous System 7

neuron, leading to either depolarization or hyperpolar- • To integrate this information with ongoing brain
ization of the membrane. This contributes to an increase activity and, if possible, relate it to previous
in the likelihood of the neuron either to discharge more experience
frequently (depolarization, excitatory) or to discharge less • To act or react in an appropriate fashion, in order
often (hyperpolarization, inhibitory). to accommodate to the new situation or per-
haps to alter it

1.1.5 NEUROTRANSMITTERS We detect changes in the external environment via

A neuron may synthesize one or more neurotransmitters, and the PNS, including particularly the special senses. The
these are released at all of its synaptic endings. The action of CNS is the integrative centre for analyzing the incoming
any single neurotransmitter may differ at each site depending information and organizing the output. The CNS consists
on the receptor type or subtype in the postsynaptic neuron. of several distinct areas, each contributing a piece to this
Typical examples of neurotransmitter chemicals are sim- operation; all parts must function harmoniously in order
ple amino acids, such as the inhibitory-acting gamma-amino to carry out complex tasks. Most of our responses include
butyric acid (GABA) or the excitatory-acting glutamate; movements to adapt to these changes via the nerve fibers
these have an immediate but short-lasting (millisecond) (of the PNS) that activate the skeletomuscular system.
effect on the postsynaptic membrane. More complex mol- One can discuss the nervous system as consisting of a
ecules may act long-term (seconds or minutes) to change set of modules:
the nature of the response (an effect called neuromodula- • The periphery – sensory and motor nerves;
tion) or to alter the properties of the membrane of the post- neuromuscular junctions and muscles
synaptic neuron, thereby strengthening or diminishing the • The spinal cord – the location of the LMNs and
synaptic relationship, a process known as long-term poten- the site of sensorimotor reflex activity; path-
tiation (see Figure 13.7). Other neurotransmitters may ways ascending and descending
cause the release of messengers, which enter the nucleus • The brainstem – three divisions each with cra-
and bring about the activation or deactivation of genes in nial nerve nuclei; reticular formation; pathways
the nucleus, thereby producing a long-lasting effect on the ascending and descending
cell and its synaptic relationships. (Neurotransmitters are • The diencephalon – hypothalamus for vegeta-
discussed further in Chapter 11.) tive functions, and the thalamus for amalgama-
tion with the cerebral cortex
• The cerebellum – a major modulator of the
1.1.6 MUSCLE motor system in particular, but also systems
Although voluntary skeletal muscle is not part of the ner- involved in cognition
vous system, the output of the nervous system most often • The basal ganglia – several nuclei with both
includes some form of muscular activity. The neuromus- motor and nonmotor functions
cular junction between the motor neuron and its associ- • The cerebral cortex – integration, visuospatial
ated muscle fibers is an essential link in the chain, part of orientation, language, memory, and executive
the PNS. Neurologists must therefore assess patients for function
muscle diseases (such as muscular dystrophy) and need • The limbic system – involved in the develop-
to distinguish these entities from diseases that affect the ment and expression of emotional reactions
synapse at the neuromuscular junction (e.g. myasthenia
gravis), from diseases of the peripheral nerves, or from When disease or injury affects the nervous system, there
lesions of the spinal cord. is a disruption of function. It is the physician’s (neurolo-
gist’s) task to use his or her knowledge to diagnose where
the nervous system is malfunctioning and the nature of
the problem. It is the characteristic contribution of each
1.2 NERVOUS SYSTEM part that permits the physician to determine the localiza-
OVERVIEW tion of any damage or lesion.
In order for the CNS to function collaboratively, path-
The perspective of this book is an understanding of the ways (tracts) are needed to carry information from the
functioning nervous system as it goes about achieving its special senses, skin, muscle, joints, and viscera to higher
three essential tasks: ‘centres’ in the brain, including the cerebral cortex, as
well as from these coordinating areas back down to the
• To detect what is happening in the external (e.g. (lower) motor neurons producing actual movements. At the
vision, hearing) or internal (e.g. within muscles same time, there is a need for the various CNS modules to
and joints) environment exchange information about the task that each is performing
8 The Integrated Nervous System

and what it is accomplishing. In fact, much of the substance The spinal cord is an elongated mass of nervous tissue
of the hemispheres consists of nerve fibers interconnecting with attached spinal roots that is located in the vertebral
various parts of the brain and the two hemispheres with each column; it ends normally at L2 (second lumbar vertebral
other; these nerve fibers constitute the CNS white matter. level) in the adult (Figure 1.3a). Although the spinal cord
is uninterrupted structurally (Figure 1.3b), it is organized
segmentally, with each segment responsible for a portion of
1.2.1 THE PERIPHERY/PNS the body peripherally: a sensory area supplied by a segment
is called a dermatome, and muscle supplied by a segment
Strictly speaking, the PNS is the nervous system outside is called a myotome. The spinal cord segments are named
the brainstem and spinal cord. It includes the peripheral according to the level at which their spinal nerves exit
nerves, both sensory and motor, as well as the neuromuscu- the vertebral column. There are 8 pairs of cervical spinal
lar junctions. Muscle diseases are within the sphere of neu- nerves (and spinal cord segments; C1–C8), 12 thoracic (T1–
rology; examples of muscle disease will be introduced in T12), 5 lumbar (L1–L5), 5 sacral (S1–S5) and 1 coccygeal.
the online cases associated with Chapter 4, called e-cases. Because the spinal cord is shorter than the vertebral
Information from the skin and from receptors in column, a spinal cord segment responsible for a patch of
muscles and joints is constantly needed for adaptation to a skin and certain muscles in the limbs and the periphery
changing environment. Sensory information from the skin does not correspond exactly with the vertebral level, with
is detected by nonspecialized and specialized receptors the exception of the upper cervical spinal cord. A lesion of
for two main categories of sensation, called modalities: the spinal cord is described as the level of the cord that has
been damaged, not the vertebral level; therefore, knowing
1. Highly discriminative information such as fine which part of the body is supplied by a spinal cord seg-
touch and texture. Discriminative touch sensa- ment has clinical importance (see Table 2.1).
tion is carried to the spinal cord by larger fibers Each spinal cord segment has a collection of sensory
with thicker myelin; therefore, the information and motor nerve rootlets that coalesce to form a single sen-
is conveyed more rapidly (Figure 1.1). sory (dorsal) and motor (ventral) nerve root on each side of
2. Pain, temperature and non-discriminative the cord; the dorsal and sensory roots combine to form a spi-
(crude) touch. Fibers are mostly smaller and nal nerve (Figure 1.3c). Transverse sections (cross-sections)
tend to be thinly myelinated or unmyelinated; of the spinal cord reveal a core of gray matter (neurons and
impulse conduction along these fibers is there- synapses) in a butterfly-like configuration, surrounded by
fore slower (Figure 1.1). white matter, consisting of tracts, also called pathways. The
dorsal aspect of the spinal cord gray matter has sensory-
Motor nerve fibers, originating from motor neurons associated functions; the sensory input is carried via the
in the spinal cord, project to the muscles in order to initi- dorsal root. The ventral portion of the spinal cord gray mat-
ate and control movements. Again, these nerves are well ter has the motor neurons, known from a functional per-
myelinated and carry information rapidly. The synapse on spective as the LMNs (also called the alpha motor neurons).
muscle cells is specialized as the neuromuscular junction, The axons of these motor cells leave the spinal cord (and
where the neurotransmitter acetylcholine is stored and change the nature of their myelin sheath as they do so) to be
released when the action potential invades the synapse. distributed via the ventral root to the muscles.
Finally, intact muscle is required to produce the intended
movements, either intentional (voluntary) or procedural, as
1.2.2.1 Sensory A spects
well as for postural adjustments in response to changes in
position or to the force of gravity (discussed further in the Peripheral nerves carry the sensory information from the
motor section in Chapter 2). two modalities to the spinal cord where the two systems
follow quite different pathways (tracts). The modalities
of discriminative (fine) touch along with proprioception
1.2.2 THE SPINAL CORD
and the ‘sense’ of vibration ascend and stay on the same
The spinal cord is intimately connected with the PNS but side throughout the spinal cord but cross in the brainstem
is part of the CNS. Functionally, the spinal cord is respon- (Figure 1.4a), whereas crude touch, pain and temperature
sible for receiving sensory (including muscular) input fibers synapse and cross to the other side in the spinal cord
from the limbs and the body wall and for sending out the (Figure 1.4b) and then ascend. After the thalamic relay both
motor instructions to the muscles. It adds the functional reach the cortex, where further elaboration and identifica-
capability for reflex activity, in response to information tion of the sensory information occur, most of this being
from both the muscles and the skin, and can organize in the realm of consciousness (discussed in further detail
some basic movements (e.g. walking). In addition, it car- in Chapter 2 and also in Chapter 5). Other information
ries ascending and descending axonal pathways (tracts). (known as proprioception), derived from special sensory
Synopsis of the Nervous System 9

Vertebral Sk Spinal cord

levels levels
C2

Cervical Cervical

Cervical
C7
T1 SAS D and
A

Thoracic

Thoracic Thoracic

Lumbar
T12
L1 Sacral
Lumbar
DRG
LC
Lumbar ES
PLL Sacral
LFl
L5 Cauda CM
equina
S1
Cauda
Sacral equina

Co (b)
(a)
Sk – Skull PLL – Posterior longitudinal ligament D and A – Dura and arachnoid
SAS – Subarachnoid space Co – Coccyx (opened)
(cerebrospinal fluid) Meninges: DRG – Dorsal root ganglion
LC – Lumbar cistern Dura CM – Conus medullaris
ES – Epidural space Arachnoid
LFl – Ligamentum flavum Pia

FIGURE 1.3: (a) The spinal cord, with its meninges, is situated within the vertebral canal. The vertebral levels are indicated on the left
side of the illustration and the spinal cord levels on the right side. The cord terminates at the level of L2, the second lumbar vertebra (in
the adult). The subarachnoid space (SAS) and lumbar cistern (LC) with CSF is shown (pale blue). Note the ligamentum flavum (LFl) and
the epidural space (ES). (b) A photographic view of a (human) spinal cord with attached roots (dorsal and ventral) demonstrating its
unsegmented appearance. The meninges (dura and arachnoid) have been opened and are displayed; note the dorsal root ganglion (DRG).
 (Continued)

units responsive to stretch in the muscles (muscle spindles) the upper motor neurons (UMNs); the spinal cord neurons
and movement detectors in the joints, is also conveyed to are called the lower motor neurons. The control of muscle
the CNS via the discriminative touch pathway; much of activity is funnelled through the LMN, which is function-
this information does not reach the level of consciousness. ally the final common pathway for motor activity. The
LMN, its motor axon and the muscle fibers it supplies are
collectively known as the motor unit (see Figure 4.3).
1.2.2.2 Motor A spects
Neurons in the cerebral cortex (and brainstem) control the 1.2.2.3 Reflexes
activity of the motor neurons in the spinal cord. One major
pathway descends from the ‘upper’ levels of the nervous One of the most interesting aspects of muscle activity is
system (the cerebral cortex; see Section 1.2.7.2) to the spinal a feedback mechanism whereby the muscle informs the
cord and is termed the corticospinal tract (Figure 1.5a; also nervous system about its degree of stretch. Receptors that
discussed in Chapter 7); note that this is a crossed pathway gauge the degree of stretch are located among the regu-
(in the lower brainstem). The cortical neurons are known as lar muscle fibers; they are known appropriately as stretch
10 The Integrated Nervous System

Dorsal root (sensory)

Dorsal root ganglion
Sensory neurons
Spinal nerve (mixed)

Ventral root (motor)

Motor neurons

(c)

FIGURE 1.3 (CONTINUED): (c) A spinal cord segment in cross-section (an axial view) shows the configuration of the gray matter
(neurons and synapses); sensory neurons are located in the dorsal horn and motor neurons in the ventral (anterior) horn. Ascending
and descending tracts (pathways) are found in the surrounding white matter. The dorsal root with the dorsal root (sensory) ganglion
and ventral (motor) root are also seen, forming the (mixed) spinal nerve.

receptors. These receptors are spindle shaped and are thus • The spinal cord, where afferent interfaces with
called muscle spindles. The afferent information from efferent
the muscle spindles is carried by a peripheral myelinated • A motor neuron (a generic LMN) at the appro-
nerve fiber, which gives off a collateral branch in the spi- priate (lumbar) level of the spinal cord, with
nal cord (see Figure 1.5b). This fiber synapses directly, only a single synapse
by way of only a single synapse (i.e. monosynaptic), with • A (myelinated) motor axon travelling as a
an LMN supplying the muscle from which it originated. peripheral myelinated nerve, returning to the
Activation of these receptors will normally lead to a reflex same muscle (with a neuromuscular synapse)
contraction of that very same muscle. to effect a reflex contraction
This reflex circuit, known as the (muscle) stretch
reflex, the deep tendon reflex or myotatic reflex, is tested
clinically by tapping on a tendon (e.g. the patellar tendon Reflex contraction of the muscle is graded in a standard
at the knee), which stretches the muscle and thereby acti- way (see Table 2.3).
vates the muscle spindles (Figure 1.5b) The muscle con- The sensitivity of this reflex circuit is influenced by neu-
tracts (in this instance producing extension of the knee). rons located in the brainstem reticular formation (see Section
This monosynaptic reflex requires the following elements: 1.2.4). Therefore, the assessment of reflex activity in the clini-
cal setting not only is one of the most significant tests for
• The muscle spindle with an intact function- motor functionality as well as spinal cord integrity but also
ing peripheral (myelinated) nerve carrying the is extremely important for overall assessment of the nervous
afferent information system. Note that intact myelinated peripheral nerve fibers
Synopsis of the Nervous System 11

Cerebral hemisphere
Th

Brainstem

Th – Thalamus

Muscle spindle
receptor

Joint position
receptor

Posterior
column

Peripheral Skin receptors (fine touch)

sensory
nerve

(’Vibration sense’)

(a)

FIGURE 1.4: (a) Sensory information from muscle spindles and joint receptors and for discriminative touch and vibration sensation
enters the spinal cord and ascends on the same side, in the posterior (dorsal) column, crosses and is distributed via the thalamus to the
postcentral gyrus. (Continued)

and functional neuromuscular junctions are also required for • Two sensory pathways that ascend to higher
the reflex arc. Finally, healthy intact muscle, appropriate for levels – the posterior (dorsal) column (for dis-
the size and age of the individual, is needed for a response. criminative touch, proprioception and vibra-
One of the remarkable features of the muscle spindles tion) and the spinothalamic pathway (for pain,
is their capability for resetting their sensitivity to the temperature and crude [or non-discriminative]
stretch stimulus; each spindle has within it a few muscle touch; Figure 1.4a and b)
fibers that will reset the length of the spindle and thus alter • One major motor tract, the corticospinal (for
its responsiveness. Specific neurons (called gamma motor voluntary motor actions), that descends from
neurons) located in the spinal cord supply the muscle the cortex (Figure 1.5a)
fibers within each spindle.
Note that other reflexes that have a protective function, 1.2.2.4 The ANS
such as the response to touching a hot surface or stepping The ANS has two functional divisions, the sympathetic
on a sharp object, involve more than one synapse. and parasympathetic. The sympathetic portion functions
To recapitulate, although there are many pathways in in circumstances of stress, for example, those requiring
the spinal cord, both ascending (sensory) and descending ‘fight or flight’ reactions (increased adrenaline, sweating,
(motor), from the clinical perspective, there are three that mobilization of glucose). The parasympathetic division is
are highly relevant (note that each half of the cord has all
concerned with restoring energy and functions in quiet
three pathways):
periods (such as after a big meal).
12 The Integrated Nervous System

Skin receptor
(pain, temperature,
crude touch)

Spinothalamic
tract
Peripheral
sensory
nerve

(b)

FIGURE 1.4 (CONTINUED): (b) Sensory information for crude touch, pain and temperature enters the spinal cord, crosses and
ascends, as the (lateral) spinothalamic tract and is distributed via the thalamus to the postcentral gyrus, as well as to other areas of
the cortex.

Sympathetic outflow begins in the hypothalamus and preganglionic neurons are found in the region of the conus
descends through the brainstem (its fibers are located in medullaris, the lowest portion of the spinal cord (see
the lateral aspect of the medulla). These fibers synapse Figures 1.3b and 5.3). Its ganglia are situated mostly nearer
with the preganglionic sympathetic neurons located in the target organ, including the gut, glands and the bladder
the ‘lateral horn’ of the spinal cord, a small gray matter wall (see Chapter 14).
excrescence, located from T1 to L2, between the dorsal
(sensory) and ventral (motor) horns. Their axons exit
1.2.3 THE BRAINSTEM
with the ventral root and the fibers synapse next in the
paraspinal sympathetic chain, located for the most part The brainstem, situated above the spinal cord and within
alongside the vertebral column. The sympathetic supply the lower region of the skull, adds control mechanisms
to the head region (particularly the pupil and the eyelid) for basic movements, particularly in response to changes
is somewhat circuitous and will be discussed in Chapter 2 in position and to the effects of gravity. Most important,
(with Figure 2.3). the nuclei controlling the vital functions of respiration
Parasympathetic outflow starts in the lateral hypothal- and heart rate are found in the lower brainstem. In addi-
amus and descends to the brainstem for the control of heart tion, there are groups of neurons located in the core of the
rate, vasoconstriction, respiration, digestion and sweating. brainstem, collectively known as the reticular formation;
The parasympathetic outflow from the CNS is from the some of these neuronal groups have both a general effect
brainstem (see Section 1.2.3; with cranial nerves III, VII, on the level of activation of motor neurons of the spinal
IX and particularly with X, the vagus) and from the sacral cord while others modulate the level of consciousness. The
division of the spinal cord (S2, 3, 4); the parasympathetic three major pathways continue through the brainstem – two
Synopsis of the Nervous System 13

Upper motor neuron

Basal ganglia Th

Th – Thalamus

Corticospinal tract

Skeletal muscle
(voluntary)

Peripheral
motor nerve

Lower motor neuron

(a)

FIGURE 1.5: (a) The motor system is organized at several levels, including the spinal cord, brainstem and cerebral cortex. The
motor neurons of the cerebral cortex are the UMNs, while spinal cord neurons are the LMNs. The pathway descending from
the cortical neurons to the spinal cord is the corticospinal tract. The axon of the LMN innervates skeletal (voluntary) muscle.
 (Continued)

sensory (ascending) and one motor (descending). Finally, sensory and motor, of these cranial nerves (Figure 1.6b
the brainstem is connected to the cerebellum. and c):
The brainstem is divided into three parts, from above
downward; each part is morphologically quite distinct • The nuclei of CN III (oculomotor) and IV
(Figure 1.6a): (trochlear) are found in the midbrain; both of
these nerves are involved with eye movements.
• The midbrain, demarcated by the cerebral In addition, the parasympathetic supply for
peduncles, situated below the diencephalon constriction of the pupil runs with the CN III
• The pons, distinguished by its prominent completing the connections for the pupillary
(anterior) bulge light reflex (see Figure 2.2).
• The medulla, with the pyramids on either side of • The nuclei of CN V (trigeminal), VI (abducens),
the midline, which continues as the spinal cord VII (facial) and VIII (vestibulo-cochlear) are
all found within the pons. The trigeminal nerve
Attached to the brainstem are the sensory and motor supplies sensation to the skin of the face and
nerves supplying the skin and muscles of the head and is motor to the chewing muscles (mastication).
neck, known as the cranial nerves (usually abbreviated The abducens nerve is responsible for lateral
CN, Figure 1.6a). Within the brainstem are the nuclei, eye movement. The facial nerve supplies the
14 The Integrated Nervous System

3. Peripheral
sensory
2. Muscle spindle stretches nerve

1. Tap 4. Synapse within

spinal cord

6. Peripheral
motor
nerve

8. Muscle
contraction
7. Neuromuscular
junction

5. Lower motor neuron

9. Leg
movement

(b)

FIGURE 1.5 (CONTINUED): (b) The stretch reflex, a deep tendon monosynaptic reflex, is shown in this case as the patellar reflex.
Upper figure: tapping on the tendon below the patella (1) causes a stretching of the muscle and firing of the muscle spindles (2); the
afferents (3) enter via the dorsal root and synapse with motor neurons (4) in the spinal cord. This is a monosynaptic connection.
Lower figure: the motor neurons (5) send axons via the ventral root (6) to the same muscle, and assuming a normal neuromuscular
junction (7), the muscle contracts (8), causing the leg to extend at the knee (9). Note: This reflex circuit is animated on the text Web site.

muscles of facial expression (around the eyes the chest and abdomen. The spinal accessory
and the lips); parasympathetic fibers supply nerve, CN XI, is responsible for raising the
some of the salivary glands and the lacrimal shoulder and turning the head. (Its nucleus is
gland. The special senses of hearing and body actually in the spinal cord, but as it enters the
motion are carried in the VIIIth nerve. skull, it has been included as one of the cra-
• The nuclei of CN IX (glossopharyngeal), X nial nerves.) Movements of the tongue are con-
(vagus) and XII (hypoglossal) are all found trolled by the hypoglossal nerve.
in the medulla. The mucosa of the pharynx is
supplied by the glossopharyngeal nerve, and Each cranial nerve emerges from the brainstem at
the muscles of the pharynx and larynx, by the approximately the level where its nucleus is located,
vagus. The vagus nerve provides the major except for CN V. Examination of these cranial nerves and
parasympathetic innervation to the organs of the reflexes associated with each part of the brainstem is
Another random document with
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civilised world, and surprise can hardly be felt when we remember
that a sentence of mutilation was carried out in England little more
than 300 years ago. Camden’s Annals for the year 1581 contain
an account of the mutilation of one Stubbs, for publishing an
attack upon Queen Elizabeth’s proposed marriage with the Duke
of Alençon. The historian was an eyewitness of the scene, which
has been utilised by Sir Walter Scott in the Fortunes of Nigel,
chap. xiii.
[38]A Moorish beehive is made from the bark of the cork-tree.
In the summer months, when the sap rises, a vertical incision
about four feet long is made through the cork to the inner bark,
and the part to be removed, having been cut above and below, is
hammered with a heavy mallet. The cork is separated from the
stem of the tree, and being elastic, is taken off entire. Two circular
pieces of cork are inserted in the orifices at each end and
fastened with wooden pegs. The bees close with wax the cracks
which may appear. The hive is warm, and keeps out both wet and
sun.
[39]Mr. Reade was Consul, Mr. Green Private Secretary. The
latter, as Sir William Kirby Green, succeeded Sir John Hay as
Minister to the Court of Morocco in 1886.
[40]Jebel Kebír, now known as ‘The Hill.’
[41]These were troops from the seat of war not yet disbanded.
The Sultan evidently desired to impress Mr. Hay with the strength
of his army.
[42]The duties on the export of wheat and barley were never
added to those noted above, in spite of Sir John’s constant and
unceasing endeavours.
[43]In allusion to the manner in which, in ancient times, Jews
and Christians in Morocco were put to death. The victims were
suspended by large iron hooks through the flesh of their backs;
one of these hooks was still to be seen on a gate of the city of
Marákesh in 1846; or a spit was run through their bodies, and
they remained transfixed till death put an end to their tortures.
 [44]The late Sultan Sid Mohammed, the descendant of Sultan
Mulai Ahmed, was a good mathematician, and also very clever as
a mechanist. He mended and cleaned his own watches. When I
presented H.M. with a breech-loading gun, and at his request
took it and the lock to pieces, I bungled in putting them together.
H.M., taking the gun from me, at once re-adjusted it.—J. H. D. H.
[45]On the site now occupied by the chief mosque.
[46]Pauper, or holy man.
[47]A delicate paste, partaking of the nature of Italian paste,
but round in form, the best being no larger than dust shot.
[48]Ovis musimon.
[49]Cedrus Atlantica and Callitris quadrivalvis.
[50]Yet, according to Marmol, it may be inferred that by this
pass the ‘Almoravides’ entered Western Barbary from Numidia.
[51]On this, as on all his other Missions, the members of Sir
John Hay’s family and his ‘private friends’ were his personal
guests, the ‘officials’ travelled at the expense of Government.
[52]Sultan Mulai Hassan.
[53]A white but much sunburnt Moorish servant of Sir J. H. D.
H.
[54]Though this permission was then granted, the laying of the
cable was delayed until 1886-87.
[55]His son, then Consul at Mogador.
[56]The ‘arum arisarum,’ called ‘yerna’ by the Moors, is used
by the inhabitants of Western Barbary as an article of food in
times of great scarcity, though it is held by them to be poisonous
without careful preparation. The tubers when collected are cut up
in small pieces, which they wash in many waters and then steam,
as they do their ‘siksu,’ after which they pound them into meal, of
which they make cakes, mixed if possible with a little ‘dra’ (millet)
meal. They also make this arum meal into a kind of porridge. This
food appears to contain few nourishing qualities, and those who
are reduced to live on it suffer much in health.
[57]Journal of Society for Psychical Research, March, 1891, p.
40.
[58]Mashallah.
[59]The loan referred to was that raised in England in 1862 to
enable the Sultan to pay the Spanish war indemnity. See chapter
xv. p. 218.
[60]Then Spanish Minister in Morocco.
[61]Sheríf of Wazan.
[62]Though Ordega acknowledged that the dead Moor had
received two hundred lashes.
[63]Moorish Minister for Foreign Affairs.
[64]German Minister.
[65]‘Mellah,’ the Jewish quarter in all Moorish towns.
[66]Baba, father.
[67]A large covert a short distance off.
[68]Evil Genius.
[69]Moors have a superstition that in hunting the lion the man
who first reports having seen the ‘S’ba’ (lion), and mentions the
word, will be the first victim.
 Transcriber's note:

pg x Added comma after: flourished in the environs of Tangier

pg 87 Changed: the Luxis of the ancients to: Lixus
pg 103 Paragraphs starting with "On March 28" and the following one formatted as
being from the Editors' perspective
pg 180 Paragraph starting with "Ten days later" formatted as not belonging to the
surrounding quoted correspondence
pg 270 Changed: admit the cortége to: cortège
pg 344 Changed: formidable porportions to: proportions
pg 356 Changed: she decares she detests to: declares
Minor changes in punctuation and quotation mark placement have been done
silently.
Other spelling errors or inconsistencies have been left unchanged.
New original cover art included with this eBook is granted to the public domain.
*** END OF THE PROJECT GUTENBERG EBOOK A MEMOIR OF
SIR JOHN DRUMMOND HAY, P.C., K.C.B., G.C.M.G., SOMETIME
MINISTER AT THE COURT OF MORROCCO ***

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