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a LANGE medical book
Clinic
Clin ical
al Neu
Neurr olog
an d Neu
Neur oan atom
A Local
ocalizat
ization
ion-B
-Base
ased
d Appr oa
Aaron L. Berkowitz, MD, PhD

Assistant Professor of Neurology
Harvard Medical School
Brigham and Women’s Hospital
Bos
oston
ton,, Mass
Ma ssaa chuse
chusett
ttss
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T is book
book is dedic
dedicate
ated
d to:
My mentors Dr. Martin A. Samuels, Dr. Allan H

and Dr. Steven K. Feske, who through their ext
mentorship and teaching trained me not only in
Neurolog
eurology,
y, but in the art
ar t o Medi
Medicine
cine..
T e students
students and resr esidents
idents at Harvard
Harvar d Medical
Medical
Partn
Par tners
ers Neurology
Neurology residency program (Bo (Boston)
ston)
and aculty at Hôpital Universitaire
Universitaire de Mirebal
Nicolas
icolas de St.
St. Marc, and Hôpital St.St. Boni
Boni ace
students and resiresidents
dents at Quee
Queen n Elizabeth
Elizabeth Ce
and Kamu
amuzu zu Central Hospital (Malawi), who who t
brilliant
rilliant ques
questions
tions and insatiable desire
desire to le
how to teach
t each neurolog
neu rology.
y.
T e patients with
with and
an d through whom
whom I learned
learned
o neur
neurolog
ology
y an
andd medicine,
medicine, and whose courage
suf ering inspires us to lear
learnn more about their
t heir d
what we learn to others, and serve them andan d the
the best
best o our abilities.
My wi
wi e Nina , whose
whose boundles
bound lesss support, encour
and companionship have bee
beenn both a sustainin
source
sour ce o great joy.
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Contents
Foreword ix
Pre ace xi
Acknowledgments xiii
P A R I 4 T e Motor and Somatosensory Path

and Approach to Weakness and
NEUROANA OMY AND
NEUROANA Sensory Loss 33
NEUROANA OMIC T e Cortic
Corticospinal
ospinal racts 33
T e Cortic
Corticobulbar
obulbar racts 36
LOCALIZA ION 1 Somat osensor y Path
Pathways
ways or th
thee Body
Locali
ocalization
zation o Motor and Sensory
Sensory De
1 Diagnosti c Re
Diagnostic Reasoning
asoning in Neurology and the
Neurologic History and Examination 1 5 T e Sp
Spinal
inal Cor
Cord
d and App
Approa
roach
ch to
Localiz
ocalization
ation in Neurologic Diagnosis: Determin
Determin ing Myelopathy 41
Where
Whe re T e Prob
Proble
lem
m Is 1 Overview o Spin
pinal
al Cord Anatomy
Anatom y 41
ime Course in in Neurologic Diagnosis:
Diagnosis: Determinin g Lamination o the Long racts in the
What T e Probl
Problem
em Is 2 Spin
pinal
al Cord 41
Associated
As sociated Symptom
Symptomss and Signs in Neurologic Spinal Cord Syndrom
Syndrom es 42
Diagnosis 3 Spinal Cord Pathways For Bowel and
Intr oduction to the Neurologic Ex
Examination
amination 3 Bladder Contr
Control
ol 44
Causes o Myelopath
Myelopathyy 45
2 Introduction to Neuroimaging and
Cerebrospin
Cerebrospinal
al Fluid Analysis 11 6 T e Visua
Visuall Path
Pathwa
way
y and App
Approa
roa
Neuroimaging in Clinical Practice 11 Visual Loss 47
Overview
Overvi ew o Neuroim
Neuroimaging
aging Inter
Interpretation
pretation 12 Anatomy o the Visual
Visual Pathway 47
Interp retation o Brain
Brain C 12 Approach
Appr oach to Visual
Visual Loss
Loss 48
Interp retation o Brain MRI 13 Disorders o Vis
Visual
ual Cognition 50
Contrast-Enhanced
Contr ast-Enhanced Neuroimaging 17
Vascular Imagin
Imaging g 19
7 T e Cer
Cerebra
ebrall Hemispheres and Vas
Nuclear Medicine
Medicine Stud
Studies:
ies: Positron Emission Syndromes 53
omography And Single Photon Emission Cort ical Reg
Cortical Region
ionss 53
Computed omography 21 Subcortic
ubcorticalal Structures:
Structures: T ala
alamus
mus and
Neuroimaging o the Spine 21 Basal Gan
Ganglia
glia 55
Cerebrospin
Cerebr ospin al Fluid Analysis 21 Arterial Supp
upplyly o the Cerebral Hemisph
Clinical Syndrom
Syndrom es Associated
Associated With
3 Overview
Overview o the Anatomy o the Nervous Vascular
Vas cular erritor
erritories
ies 59
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vi Contents
10 Pupillary Control and Approach to 16 Radiculopathy, Plexopathy, and

Anisocoria Cranial Nerves 2 and 3 83 Mononeuropathies o the Upper
Pupillary Constriction: the Parasympathetic Extremity 141
Pathway 83 Neuroan atomy o the Upper Extremity 141
Pupillary Dilation: the Sympathetic Cervical Radiculopat hy 145
Pathway 84 Brachial Plexopathy 147
Approach to Anisocoria and Other Pupillary Monon europathies o the Upper Extremity
Abnormalities 87
17 Radiculopathy, Plexopathy, and
11 Extraocular Movements and Approach to Mononeuropathies o the Lower
Diplopia Cranial nerves 3, 4, and 6 91 Extremity 157
Extraocular Movements I: Muscles and their Neuroan atomy o the Lower Extremity 157
Innervation 91 Lumbosacral Radiculopathy and Lumbar Cana
Extraocular Movements II: Cranial Nerves Stenosis 159
3, 4, and 6 93 Lumbosacral Plexopathy 163
Extraocular Movements III: Supranuclear Cont rol o Monon europathies o the Lower Extremity
Horizontal and Vertical Gaze 96 Approach to Foot Drop 165
Approach to Diplopia 101
12 T e Auditory and Vestibular Pathways

and Approach to Hearing Loss and
P A R II
Dizziness/Vertigo Cranial Nerve 8 105 DISEASES OF HE NERVOUS
T e Auditory System 105
SYS EM 167
Approach to Hearing Loss 105
T e Vestibular System 107
Approach to Dizziness and Vertigo 110 18 Seizures and Epilepsy 167
De nitions and Causes o Seizures
13 Facial Sensation and Movement and Approach
and Epilepsy 167
to Facial Sensory and Motor Def cits Cranial Evaluation o Patients With Seizures 168
nerves 5 and 7 117 Clinical Features o Seizures 169
rigeminal Nerve (Cranial Nerve 5) 117 Electroencephalography (EEG) in the Evaluati
Facial Nerve (Cranial Nerve 7) 120 Seizures 170
14 Cranial Nerves 1, 9, 10, 11, and 12 125 Evaluation and Management o Patients A
Cranial Nerve 1 (Ol actory Nerve) 125 Seizure 171
Cranial Nerve 9 (Glossopharyngeal) and Outpatient Management o Epilepsy 173
Cran ial Nerve 10 (Vagus) 125 Special Scenarios in the Management o Seizur
Cran ial Nerve 11 (Spinal Accessory) 126 Epilepsy 175
Cran ial Nerve 12 (Hypoglossal) 128 Statu s Epilepticus 178
15 T e Peripheral Nervous System and 19 Vascular Diseases o the Brain and Spina
Introduction to Electromyography/Nerve Cord 179
Overview o Ischemic Stroke and In tracerebral
Conduction Studies 129
Hemorrhage 179
Intr oduction to Anatomy and Diseases o the
Ischemic Stroke 180
Peripheral Nervous System 129
Intr acerebral Hemorrhage 192
Anatomy and Diseas Nerve R
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Con
20 In ectious Diseases o the Nervous System 207 26 Headache 275

Meningitis 207 Approach to Headache 275
Viral Encephalitis 212 Second ary Causes o Headache 275
Focal In ectious Brain Lesions 214 Primary Headache Disorders 276
In ections o the Spine 218 Other Causes o Headache 279
In ections o Nerve Roots 219
27 Peripheral Neuropathy 281
In ectious Peripheral Neuropathies 219
Classi cation and Di erential Diagnosis
In ection at the Neuromuscular Junction:
Polyneuropathy 281
Botulism 219
Acute Polyneuropathy 283
In ectious Myositis 220
Chron ic Polyneuropathy 284
Neurologic Mani estations o HIV 220
28 Motor Neuron Disease 289
21 Demyelinating Diseases o the Central Nervous
Overview o Motor Neuron Disease 289
System 223
Amyotrophic Lateral Sclerosis (ALS) and
Multiple Sclerosis 223
Variants 289
Neuromyelitis Optica 227
Spinal Muscular Atrophy (SMA) and Spi
Acute Disseminated Encephalomyelitis 228
Muscular Atrop hy (Kennedy’s Diseas
Optic Neuritis 229
ran sverse Myelitis 229 29 Disorders o the Neuromuscular
Myasthen ia Gravis 293
22 Delirium, Dementia, and Rapidly Progressive
Congenital Myasthenic Syndrom es and N
Dementia 231
Myasthenia Gravis 296
Approach to Altered Cognition 231
Lambert-Eaton Myasthenic Syndrom
Delirium 231
Dementia 233 30 Diseases o Muscle 299
Rapidly Progressive Demen tia 238 Causes o Muscle Disease 299
Clinical Features o Muscle Disease 299
23 Movement Disorders 241
Laboratory esting in Muscle Disease 30
Intr oduction to Movement Disorders 241
In ammatory Myopathies 300
remor 241
Muscular Dystrophies 301
Myoclonus 243
Distal Myopathies (Distal Muscular Dys
Chorea 244
Congenital Muscular Dystrophies and C
Dystonia 246
Myopathies 304
ics, our ette’s Syndr ome, and Sterotypies 246
Metabolic Myopath ies 304
Parkinsonism, Parkinson’s Disease, and Parkinson-
Mitochond rial Myopathies 306
Plus Synd rom es 247
Hyperkalemic and H ypokalemic Periodi
Other Movement Disorders 253
Paralysis 306
24 Neoplastic and Paraneoplastic Disorders o the Muscle Disease Due to Systemic Disease
Nervous System and Neurologic Complications Medications 307
o Chemotherapy and Radiation T erapy 255 31 Leukodystrophies and Mitochondri
Intr acranial umor s 255 Disorders 309
umor s o the Spine 258 Leukodystrophies 309
umor s o the Cranial Nerves 260 Mitochond rial Diseases 309
umor s o the Peripheral Nervous System 261
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Foreword
So much o neurology exists only “in use”. T is is the neu- ar superior to existing books o its size and sc
rology that is practiced in the clinics, wards, and of ces o the thought ulness with which the knowledge
seasoned clinicians and cannot be ound in large encyclopedic and neurological conditions has been assembled
textbooks o neurology or smaller monographs intend ed or down to business, addressing almost every maj
medical students. T e accumulated experience o the neurolo- encountered on the wards and in the clinic.
gist can be distilled to a num ber o action items and thought A book such as this one is more suitable
processes that are challenging to articulate. than or any other branch o medicine. We
Dr. Aaron Berkowitz has written a book that occupies the inter ace between our own re ned clinical
just this position. He has taken the transactional daily work decisions regarding diagnosis and tr eatment. T
o neurology and produced a wonder ully readable, concise, tained here about the meaning o particulars
but by no means super cial book that ts well in the current and examination cannot be ound elsewhere.
pedagogic environment. One might ask whether any book on a seamless tran sit rom th ese data to practical
neurology is needed now that disembodied in ormation is so their application. T e material is clear and av
easily available on the web and algorithms or various signs, ity that clutters most other books. In doing so
symptoms, and diseases abound. But between in ormation that porates the latest thinking rom clinical trials
is as o en misleading as it is use ul, and the storehouse o wis- these eatures provide one o the best modern o
dom accumulated over a long career, sits a great body o neu- pragmatic practice o neurology.
rological knowledge. It is this assembled knowledge that allows It takes a certain outlook on pedagogy a
us to ef ciently move through the workday and can be taught produce such a book. Dr. Berkowitz has more
to students and residents during their rotations. Berkowitz’s and I nd mysel looking at a number o the c
book is m ore than a compendium or teaching guide and is over to reorient mysel to solid teaching and p
Allan H
Executive Vice Chair
Brigham and Wom
Pro es
Harvard
Boston,
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CHAPTER 1 Diagnostic Reasoning in Neurology and the Neuro logic History and Examin
TABLE 1–1 Neuroanato mic Structure s and Pathways Evaluate d in the Neurolog ic Examination .
Structures/Pathways Evaluate d
MENTAL STATUS
Arou sa l Re ticu la r act iva tin g syst em , b ilat eral t halam i, an d ce re bra l h em isp he re s
Attention Frontal and parietal lobes
Memory Temporal lobes
Language Frontal lobe (usually le t)
Praxis Frontal and parietal lobes
Abstract reasoning Frontal lobes
Visuospatial processing Occipital and parietal lobes C
CRANIAL NERVES
Smell CN 1 and ol actory cortex
Pupillary light re ex CNs 2 and 3; midbrain nuclei and pathways C
Visual acuity and f elds Eyes, CN 2, thalamus (lateral geniculate nucleus [LGN]), optic radiations,
occipital corte x
Eye m ove m en ts CNs 3, 4 , a nd 6, b ra in st em p at hwa ys, ro nt al a nd p arie t al e ye f e ld s, ce r-

ebellum or saccades and smooth pursuit
Fa cia l se n sa tio n CN 5, b ra in st em p at hwa ys, t ha la mu s (ve nt ra l p ost e rio r m ed ia l n ucle us

[VPM]), somat ose nsor y corte x
Fa cia l m ove me nt s CN 7, m ot or p at hwa y ro m p re ce nt ra l g yru s t o CN 7 n ucle us in p on s
He arin g In ne r e ar, CN 8, b rain st em a ud it ory p at hways, t hala mus (m ed ia l g en iculat e

nucleus [MGN]), auditory cortex in supe rior temp oral gyrus
Ve st ib ula r sys te m In ne r e ar, CN 8 , b ra in st e m p at hwa ys a nd t he ir co nn ec tio ns wit h n ucle i o

CNs 3, 4, 6, and cereb ellum
Palate elevation and gag re ex CNs 9 and 10, their pathways in the medulla, and motor control rom the
prec en tral gyrus
Sterno clediomastoid and trape zius CN 11 and motor cont rol rom the prece ntral gyrus
strength
To ng ue m ove me nt s CN 12 a nd m ot or co nt ro l ro m t he p re ce nt ra l g yru s
MOTOR
St re ng th Cort icosp ina l t ract (p re ce nt ra l g yrus t hro ug h su bco rt ica l wh it e m at te r,

brains te m, and sp inal cord), vent ral roots, perip he ral nerves, neu romu scu-
lar junct ion, and mu scle
Initiation o movements and assessment Basal ganglia

o abnormal involuntary movement s
SENSORY Periphe ral nerves, dorsal root g ang lia, dorsal roots, spinal cord an d brain-
stem pat hways, thalamus (ventral po sterior lateral nu cleus [VPL]), postce n-
tral gyrus
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C H A P
Introduction to
Neuroimaging and
Cerebrospinal Fluid
Analysis
2
C HA P T ER CO N T EN T S
NEUROIMAGING IN CLINICAL PRACTICE CONTRAST-ENHANCED NEUROIMAGING

OVERVIEW OF NEUROIMAGING INTERPRETATION VASCULAR IMAGING
INTERPRETATION OF BRAIN CT NUCLEAR MEDICINE STUDIES: POSITRON EM
Cause s o Hype rden sity on Brain CT TOMOGRAPHY AND SINGLE PHOTON EMISS
COMPUTED TOMOGRAPHY
Cause s o Hypo de nsity on Brain CT
NEUROIMAGING OF THE SPINE
INTERPRETATION OF BRAIN MRI
CEREBROSPINAL FLUID ANALYSIS
T1-weighted, T2-weighted, and FLAIR MRI
Sequences CSF Press ure
Di usio n-Weig hte d Imag ing and Apparent CSF Che mist ry: Gluco se and Prot ein
Di usion Coe f cient MRI Sequences CSF Cell Cou nts and Cell Type s
Susceptibility-Weighted Imaging and Gradient CSF Microbio log y: Cultu res, PCR, an d Antib
Echo MRI Seq ue nce s Patterns o CSF Abnormalities
MR Spectroscopy Additio nal tes ts o CSF
Neurodiagnostic tests aid in determining both localization to particular symptoms and diseases, and how
and diagnosis. T e main neurodiagnostic tests are: neuro- diagnosis o various neurologic condition s.
imaging, cerebrospinal uid (CSF) analysis, electroencepha- In clinical practice, many patients have u
lography (EEG), and electromyography/nerve conduction roimaging studies be ore a neurologist has ev
studies (EMG/NCS). EEG is discussed in the context o the and some patients may be re erred or neur
diagnosis o seizures and epilepsy (Ch. 18) and EMG/NCS because o neuroimaging ndin gs r ather than
in the context o the diagnosis o neuromuscular disease (Ch. 15 ings. In such scenarios, part o the clinical
or th e principles; Chs. 16–17 and 27–30 or clinical use). is interpreting the neuroimaging in the conte
Neuroimaging and CSF analysis are discussed throughout this cal ndin gs: Do the neuroimaging ndings
book, bu t an introduction to their use an d interpr etation is the patient’s clinical presentation? Are there
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40 PART I Neuroan atomy and Neuroanatom ic Localization
Hemispheric Brainstem Spinal cord Root Ne rve Poly- Myopa

lesion lesion lesion lesion lesion neuropathy
FIGURE 4–5 Schematic showing patterns o weakness and/or senso ry disturbances caused by lesions at dif erent levels o
nervous system. Note tha t a myopa thy will not cause se nsory de cits. Adap ted with permission rom Aminof M, Green be rg D, Simon
cal Neurology, 9th ed. New York: McGraw-Hill Education; 2015.
In sum mary (Fig. 4–5): Isolated bilateral sensory changes generally su

process a ecting the spin al cord, dor sal roots, do
• I there are sensory and/ or motor changes in the ace (with
ganglia, or peripheral nerves.
or without de cits in the extremities), this requires a lesion
at the level o the brainstem or in the cerebral hemisphere. With respect to time course o symptom on
(Evaluation o acial weakness and sensory changes is dis- evolution:
cussed in urther detail in Chapter 13.)
• Sudden-onset weakness and/or sensory change
• I the arm and leg are a ected on one side without the ace mon causes o sudden-onset weakness and/or
being involved, the lesion is most likely in the medulla or changes localizing to the brain include stroke, se
cervical spinal cord . migraine. T e most common cause o sudden-ons
• I one limb is a ected in isolation, a lesion at nearly any ness and/or sensory changes localizing to the brai
level o the n ervous system is possible: a small cortical stroke. Common causes o sudden- onset weaknes
or subcortical lesion, a spinal cord lesion, or a lesion sensory changes localizing to the spinal cord inclu
at the level o the nerve roots, plexus, or one or more disc prolapse or vertebral collapse (e.g., due to tr
periph eral ner ves. A brain stem lesion causin g u nilat eral metastatic malignancy to the vertebrae), which
involvement o one limb is un likely since the ibers o cally pain ul. Spinal cord in arct occurs r arely (se
the motor and sensory pathways are packed so closely in Sudden-onset weakness and/or sensory changes lo
the brainstem. to a particular nerve may be caused by a nerve in
can be seen in vasculitic neuropathies; see Ch. 15)
• Isolated bilateral weakness suggests a process a ecting the
spinal cord, peripheral nerves, muscles, or neuromuscu- • Acute- to subacute-onset (over hours to days
lar junction . o have bilateral weakness rom a brain or ness and/or sensory changes. Acute- to subacu
brainstem lesion would requir e bilateral lesions, which will weakness and/or sensory changes at any level
usually cause symptoms/signs beyond just motor problems vous system can be due to inf ammatory conditio
(e.g., changes in mental state i there are bilateral cere- Guillain-Barré syndr ome (see Ch. 27), transverse
bral lesi cranial de cits i ther bilateral or an acute demyelinating lesion in multiple scler
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C H A
T e Spinal Cord and
Approach to Myelopathy
5
OVERVIEW OF SPINAL CORD ANATOMY Cen tral Cord Synd rome
LAMINATION OF THE LONG TRACTS IN THE SPINAL CORD Subacute Combined Dege neration
SPINAL CORD SYNDROMES SPINAL CORD PATHWAYS FOR BOWEL AND
Brown-Séquard (Hemico rd) Syndrome CONTROL
Anterior Cord Synd rome CAUSES OF MYELOPATHY
OVERVIEW OF SP INAL CORD ANATOMY T e lateral corticospinal tracts are lateral a

the spinal cord, the dorsal columns are posteri
T e spinal cord begins where the medulla ends, running rom and the anterolateral (spinothalamic) tracts ar
the oramen magnu m at the base o the skull to about the level lateral (as their name suggests) (Fig. 5–2).
o the rst lumbar vertebra (L1) (Fig. 5–1). T e spinal cord is
divided into cervical, thor acic, lumbar, and sacral regions. T e
cervical and thoracic regions o the spinal cord corresponds to LAMINATION OF THE LONG TR
the cervical and th oracic regions o the spinal column . How- THE SPINAL CORD (FIG. 5 –3)
ever, the spinal cord is shorter th an the spinal column, and so
the lumbar region o the spinal cord actually corresponds to Lamination re ers to the arrangement o bers
the lower thor acic spine, and the sacral region o the cord is For the corticospinal, anterolateral, and dorsal col
housed in a short region called the conus medullarisa t about this re ers to where the arm, leg, and torso bers
the level o the L1-L2 vertebrae. T roughout the spine, dorsal no bers or the head in the spinal cord since
roots enter and ventral roots exit through the neural oramina to/ rom the brainstem by the cranial nerves).
o the vertebrae that correspond to th eir spinal cord level o As the corticospinal tracts descend rom
origin/exit. At cervical and thor acic levels, the corr esponding arm bers synapse on lower motor neur ons
oramina are essentially adjacent to th e spinal cord levels with cord, but leg bers must wait until the lumbar
which they are associated. Since the spinal cord ends at L1, T ere ore, arm bers must be most medial to h
below L1, the lumbosacral nerve roots (cauda equina) must to the alpha motor neurons, and leg bers a
descend to reach their corresponding exiting oramina gaining exposure to the anterior hor n cells onl
(discussed urther in Chapters 15 and 17). bers h ave done so and departed as th e tr
T e lateral corticospinal tracts, dorsal column path ways, corticospinal tracts are thus laminated with
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Spinal Vertebral Dorsal columns

Sacral
cord bo dy
segment
Cervical
C1
C2
C3
C4 Cervical roots
Cerv
C5
C6
C7
C7
C8 Corti
T1 T1
T2
T3 Sacral
Spinothalamic tr
T4 Cervical
T5 FIGURE 5–2 Schematic o axial sectio n o the spinal
T6
This schema tic demonstrat es the locations o the t hree m
T7 Thoracic roots relevant pa thways and the ir lamination: corticospinal tracts,
T8
columns, and a nte rolateral (spinothalamic) tracts. Reprod
T9
pe rmission rom Waxman S: Clinical Neuroa na tomy, 27th
T10 New York: McGraw-Hill Educa tion; 2013.
T11
T12 o central cord syndrome( e.g., as can be caused by a

(See “Centr al Cord Syndrom e” later).
L1
L2 SPINAL CORD SYNDROMES

L3 Lumbar roots T ere are our important clinical patterns to recogniz
L4 ni y localization to particular parts o the spinal cord
L4 a particular di erential diagnosis (Fig. 5-4 and able
L5
Brown-Séquard (Hemicord) Syndro
S1 (Fig. 5 4A)
S2
S 3 Sa cral roots Hemicord syndrom e a ects all pathways on one sid
S4 spinal cord. T e corticospinal tracts cross in the
S5
Coccygeal ne rve which is just above their entry into the cervical spin
Posterior Anterior T ere ore, motor de cits caused by a unilateral
lesion will cause ipsilateral weakness below the lev
FIGURE 5–1 Schematic o lateral view o the spinal cord and lesion. T e dorsal column pathways remain ipsil
nerve roots in relation to th e spinal column. Reprod uced with they cross in the medulla, so unilateral spinal cord
pe rmission rom Amino M, Green be rg D, Simon R: Clinical Neurology,
will cause ipsilateral de cits o proprioception and v
9th ed . New York: McGraw-Hill Edu cat ion; 2015.
sensation below the level o the lesion. Since the a
eral (spinothalamic) tracts cross as they enter th e spi
lesions o one side o the spinal cord will ect already
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CHAPTER 5 T e Spinal Cord and Approach to Mye
Leg area
Leg area Leg area S
M S e
o n s
c o t o r o r
y
r te c
x o
r t
e x
Arm area
Arm are a
Thalamus
Internal
capsule
Medial
lemniscus
Dorsal column nuclei

Decussation Fasciculus cuneatus
To a rm From arm From arm
muscles
Lower motor
Fasciculus gracilis
neurons
To leg From leg

muscles
From leg
Dorsal horn
A Ventra l horn B C
FIGURE 5–3 Schematic o the three long tracts. A:C orticospinal tracts. B: Dorsal column path ways. C: Spinothalamic tracts. R
pe rmission rom Waxman S: Clinical Neuroa na tomy, 27th e d. New York: McGraw-Hill Edu cation ; 2013.
Dorsal columns Dorsal columns
Corticos pinal trac t Corticos pinal tract
A Anterolatera l (spinotha lamic) trac t B Anterolateral (spinothalamic) tract
Dorsal columns Dorsal columns

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TABLE 5–1 Spinal Cord Syndrome s.

Brown -Séquard Syndrome
Anterior Cord Subacute Com
Ipsilateral Contralateral Syndrome Central Cord Syndrome Degeneration
Motor A ected Spared A ected Spared bilaterally (until very A ect ed bilate
bilatera lly advanced)
Vibration/ A ected Sp ared Sp ared b ilaterally Sp ared b ilaterally A ecte d b i
proprioception
Pain/ Spared A ected A ected A ected bilatera lly (in Spared bilater
temperature bilatera lly hand s/upp er extremities
rst)
For example, in a lower thoracic Brown-Séquard syndrome spinothalamic (and, in some cases, corticospinal) tra
a ecting the right hemicord, there will be right-sided (ipsilateral) be compressed rom medially to laterally. Since upper
weakness and vibration sense/proprioception loss with spared ity bers are medial in both pathways, these are a
pain/temperature sensation, and the opposite pattern on the leading to involvement o the upper extremities be o
lef (contralateral) side: preserved strength an d vibration ing the lower extremities i the syrinx progresses.
sense/ pr oprioception but impaired pain/temperature sensation. Syrinx can occur in the setting o a Chiari mal
Hemicord syndrome is most commonly caused by pen- (see “Chiari Mal ormation” in Ch. 26), or any le
etrating trauma (e.g., stab or gunshot wound), but can also spinal cord leading to obstruction o the central ca
be caused by a neoplasm compressing the cord rom on e side tumor, prior trauma, demyelination, hemorrhage).
(e.g., meningioma) or a un ilateral demyelinating lesion (e.g.,
tran sverse myelitis). Subacute Combined Degeneration
(Fig. 5 4D)
Ante rior Cord Syndrome (Fig. 5 4B)
Selective involvement o the dorsal columns and
Anterior cord syndrome involves nearly the entire cross sec- nal tracts together occurs in subacute combined dege
tional area o the spinal cord with the exception o the dorsal Subacute combined degeneration is most commonl
columns. T ere ore, motor unction and pain and tempera- by vitamin B12 de ciency, but can also be caused
ture sensation are impaired below the level o the lesion but de ciency. Vitamin B12 de ciency can be caused b
proprioception and vibration are spared. Both upper and sorption (e.g., pernicious anemia, small intestine pat
lower motor neuron signs may be seen: upper motor neu- surgery, gastric bypass) or a vegetarian or vegan di
ron signs due to interruption o the descending corticospinal per de ciency can occur in the setting o excess zinc
tracts and lower motor neuron signs due to involvement o (which can be caused by zinc-containing denture
the gray matter at the a ected level(s) o the spinal cord. Ante- a er gastric bypass surgery, or due to malabsorpti
rior cord syndrome occurs most commonly due to in arction vitamin B12 de ciency and copper de ciency can
in the territor y o the anterior spinal artery o the spinal cord, concurrent myelopathy and n europathy (myeloneur
which is most o en caused by abdomin al aortic aneurysm which can cause mixed upper and lower motor neu
(AAA), AAA rupture, or in the setting o surgery or AAA tures on examination (e.g., absent an kle ref exes
repair. T e reasons why the anterior spinal cord is more vul- knee ref exes, or aref exia with Babinski signs).
nerable to ischemia than the posterior cord are discussed in T e dorsal columns and corticospinal tracts
Chapter 19 (see “Ischemic stroke o the Spinal Cord”). selectively a ected in the vacuolar myelopat hy
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CHAPTER 5 T e Spinal Cord and Approach to Mye
TABLE 5–2 Ef ects o Spinal Lesio ns on Bowe l and Bladder Function

Acute Spinal Cord Injury Chronic Spinal Cord Injury Cauda Equina/Conus M
Bowel Constipation Constipation Constipation
Decreased rectal tone Increased rectal tone Decreased rectal tone
Incontinence Incontinence
Bladder Retention Spastic bladder Retention
Overf ow incontinence Frequency and incontinence Overf ow incontinence
generally f accid acutely, and upper motor n euron signs (e.g., • Inf ammator y disease (see Ch. 21): transve
hyperref exia, spasticity) develop over time (see “Upper Motor o multiple sclerosis, f are o neur omyeli
Neuron Lesions versus Lower Motor Neuron Lesions” in Ch. 4). Sjögren’s syndrome, sarcoidosis
Similarly, acute spinal cord pathology causes f accidity o the
Subacute to chronic-onset (days to mo
bowel and bladder, and spasticity develops over time.
can be caused by:
For the bladder, this means that in the acute setting o
spinal cord injury, the bladder is f accid and does not cont ract, • Stru ctur al disease o the spine (see Ch.
leading to urinary retention with overf ow incontinence. Over stenosis
time, an upper motor neuron pattern emerges whereby the • umor (see Ch 24): primary or metastatic
bladder is spastic/hyperref exic: it contracts too much, leading • In ections (see Ch. 20): tuberculosis o the sp
to urgency and incontinence.
• Vascular causes (see Ch. 19): spinal dural
For the bowel, acute spinal cord lesions lead to bowel
stula
and r ectal f accidity, causing constipation du e to decreased
• Metabolic causes: vitamin B12 de cien
bowel motility and incon tinence due to decreased rectal
de ciency
tone. Chronic spinal cord lesions lead to increased bowel
and rectal tone, which results in constipation (generally Chronic-onset (months to years) myelo
requiring physical stimulation o the rectum or a bowel caused by:
movement to occur).
• In ections (see Ch. 20): hu man -cell lym
Lower motor n euron lesions or both bowel and blad-
1 (H LV-1) and AIDS
der (sacral roots 2—4 o the cauda equina) lead to f accidity,
leading to retention o both stool and ur ine, with overf ow • Neurodegenerative myelopathies: hereditary
incontinence o urine and bowel incontinence due to f accid plegia (see below), spinocerebellar ataxia (se
sphincter tone. nomyeloneuropathy (see Ch. 31)
• Radiation-indu ced myelopathy (see Ch. 24)
T ese conditions are discussed in Part
CAUSES OF MYELOPATHY tary spastic paraplegia, which is there ore discu
Common causes o myelopathy are classi ed here by time

Hereditary Spastic Paraplegia
course o onset and evolution. Details o individual disorders
Hereditary spastic paraplegia (HSP) is an in
are discussed in Part 2 o the book.
erative disorder that pr edominan tly a ects
Hyperacute-onset (minutes to hours) myelopathy can
tracts in the spinal cord leading to p rogressiv
be caused by:
extremity weakness and spasticity. More tha
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C H A
T e Cerebral
Hemispheres and
Vascular Syndromes
7
CORTICAL REGIONS Wate rshed (Borderzone ) Territories
Parieta l Lob es: Spatial Atten tion and Praxis CLINICAL SYNDROMES ASSOCIATED WITH
Temp oral Lob es : Reco gnit ion Memo ry CEREBRAL VASCULAR TERRITORIES
Front al and Temp oral Lob es : Lang uag e MCA Territo ry Infarction
SUBCORTICAL STRUCTURES: THALAMUS AND ACA Territo ry Infarctio n

BASAL GANGLIA Anterio r Cho roidal Arte ry Territory Infarcti
The Thalam us PCA Territo ry Infarctio n
The Basal Ganglia Lacun ar Strokes
ARTERIAL SUPPLY OF THE CEREBRAL HEMISPHERES Infarction in the Wate rshe d (Borderzone
The Vascular Territo ries of th e ACA, MCA, and PCA
Chapters 4 and 6 mapped th e primary motor cortex (precen- situated to combine visual and spatial in orm
tral gyrus o the rontal lobe), primar y somatosensory cortex roles in awareness o the body in space, spatial
(postcentral gyrus o the parietal lobe), and the primary visual mathematical processing. T e projection
cortex (calcarine cortex o the posterior occipital lobe) onto the lobe superiorly to the parietal lobe (the dor
cerebral hemispheres. T e primary auditory cortex is housed re erred to as the “where” pathway: Visual i
in the superior tempor al gyrus o the temporal lobe. Knowing processed h ere to determ ine where th ings are
the locations o the motor cortex and these three primary sen- respect to the body. Lesions here can cause
sory cortices allows or a logical deduction o the unction s o patient is unaware o one hal o the world. N
the rest o the cortical sur ace as is discussed below. common with lesions in the nondominant
T e hemisphere contralateral to the side o handedness which is most commonly the right parietal lob
is considered the dominant hemisphere (e.g., the le hemi- sided neglect. Examination ndings in patien
sphere in a right-handed patient), and the hemisphere ipsilat- may include extinction to double simultaneo
eral to the side o hand edness is considered the nondominant (see Ch. 4), lack o awareness o de cits ( ano
hemisphere (e.g., the right hemisphere in a right-handed not acknowledging that a paretic limb is weak
patient). Most patients are right-han ded, so t heir le hemi- ity to move it), and in severe cases, inability to
sphere is the dominant hemisphere. Language dys unction is neglected body par ts as one’s own.
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CHAPTER 7 T e Cerebral Hemispheres and Vascular Syndr
TABLE 7–1 Aphasias.

Speech Speech
Production Comprehension Repetition Lesion Location (Most Commonly in L
Broca’s apha sia Im pa ire d Pre se rve d Im p aire d In e rio r ro nt al g yru s (Bro ca ’s a re a)
Transcortical mot or aphas ia Im pa ire d Pre se rve d Pre se rve d An te rio r/ su pe rio r t o Bro ca’s a re a
Wernicke’s aphasia Preserved Impaired Impaired Posterior superior temporal gyrus (Werni
Transcortical sens ory aphas ia Preserved Impaired Preserved Parietal, posterior to Wernicke’s area
Global aphasia Im pa ire d Im pa ire d Im p aire d Bro ca’s a nd We rn icke ’s a re as
Mixed transcortical aphasia Impaired Impaired Preserved Extensive lesions o ten involving middl
arte ry–anterior cereb ral art ey (MCA-ACA
Conductio n aphasia Pre se rve d Pre se rve d Im paire d Arcua te ascicu lus
comprehend. However, patients with Broca’s aphasia originates in or spreads to language regions. M
may have di iculty with compr ehension o gramm atically development o aphasia can be seen with a
complex phr ases (e.g., “ he tiger was eaten by the lion. subdural hematoma or a tu mor a ecting l
Who survived?”). In the most severe Broca’s aphasias, the Aphasia can also develop even more insid
pat ient is mu te. When less severe, the pat ient may have neurodegenerative diseases such as primar
e ort ul speech with requent error s. Since comprehen- aphasia (see Ch. 22).
sion is generally largely preserved in Broca’s aphasia, the In addition to regions involved in langua
pat ient is aware o and ru strated by th e inability t o speak. control, the rontal lobes suppor t executive
In a pur e Broca’s aphasia, the patient cannot repeat phr ases ing working memory, decision making, abstract
stated by the examiner but can compr ehend (i.e., can ol- emotional processing. Frontal lobe lesions can
low comman ds). I a patient has an expressive aphasia with (decreased initiative, motivation, speech, a
pr eserved rep etition , th is is called a transcortical mo tor response), behavioral disinhibition, and/or im
aphasia. any o the above executive unctions.
In pure Wernicke’s aphasia, comprehension is impaired he higher order unctions o the oc
(receptive aphasia), and although the prosody (melody and discussed in Chapter 6.
rhythm ) o speech is preserved (f uent aphasia), the content
is nonsensical. T e patient cannot un derstand his or her own
nonsensical speech, and so may not appear concerned by the SUBCORTICAL STRUCTURES:
de cit. In pure Wernicke’s aphasia, a patient cannot r epeat THALAMUS AND BASAL GANG
phrases. I repetition is preserved in a receptive aphasia, this is
(FIG. 7–2)
called a transcortical sensory aphasia.
I both production and comprehension are impaired, this T e thalamus and basal ganglia are “islands”
is called a global aphasia. Rarely, patients with both produc- in th e subcortical white matter. Both ar e nodes
tive and receptive aphasia are still able to repeat what they circuits that begin and/or end in the cortex, b
hear; a scenario called mixed transcortical aphasia. or cerebellum.
Note that all o the transcortical aphasias are char acter-
ized by preserved repetition , and nam ed or the primary
The Thalamus
language def cit: transcortical motor aphasia is characterized by
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56 PART I Neuroan atomy and Neuro

Neuroanatom
anatom ic Loc
Localiz
alization
ation
Caudate nucleus
Putamen
Corpus
callosum
Caudate nucleus
Lateral
Putamen
ventricle
Globus pa ll
llidus:
idus:
Thalamus External segment
Basal
Internal segme nt
Internal ganglia
capsule Subthalamic
nucleus
Claustrum
Substantia nigra
Amygdala
FIGURE 7–2 Schematic of a coronal section de monstra

Schematic monstrating
ting the inter
internal
nal anatomy of the cerebr al hemispheres . Reprod
cerebral Reprod uced
pe rmission rom Rop
Rop pe r A,
A, Samu
Samu els M,
M, Klein J: Ada
Ada ms and Victor’
Victor’ss Princi
Principles
ples of Neurology
eurology,, 10th e d. New
New York:
York: McGr
cGraw-Hi
aw-Hill
ll Edu cation
tran smits smell

smell in ormation to
t o the thalamus (dorsomedial consciousness due to thalamic involvement). Given
nucleus)). use connections
connection s with
with diverse cort
cortical
ical regions, lesion
lesion
2. Motor control pathways. T e ventralventral anterior
anterior (VA)
(VA) and thalamus are said to be able to “do anything” (i.e., c
ventral lateral (VL
(VL)) nuclei o the thalamus
thalamu s participate in type o de cit), includin
includin g causing “cortical”
“cortical” signs
cortical–basal ganglia–cortical loops and cerebellar–cortical sia, neglect, cognitive de cits) and eye
eye movement ab
pathways. ties (in part due to e ects on nearby midbrain path
3. Consciousness/arousal pathways. T es esee pathways
pathways beg
begin
in eye movements).
in the
th e brainstem reticular activating sys
system
tem and project to
both thalami, which in turturn
n project di usely throughout
throu ghout
the cortex. The Bas
asal
al Gang
Gang lia
T e basal gangli
gangliaa include the caudate, putamen, gl
4. Cognition/emotion pathways. Corticocortical loops
lidus, and subthalamic nucleus (see(see Fig.
Fig. 7–2). T
pass th
throu
rou gh the thalam
thalamus,
us, playing roles in diverse
and putamen together are re erred to as the striat
cognitive ununctions.
ctions. One such
such loop is the circuit o
the putamen and globus pallidus together are re
Papez which participates in memory and emotion:
as the lenticular nuclei . T e basal
basal ga
gangli
ngliaa are
hippocampus" o rnix "mamillary bodies"anterior
cuits that initiate and coordinate movements, and
nucle
nu cleus
us o the thalamus anterior cingulate entorhinal
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CHAPTER 7 T e Cerebral
Cerebral Hemisphere
Hemispheress and Vascul
Vascular
ar Syndr
Syndr
TABLE
TABLE 7 -2 Thala
halamic
mic Nuclei.
Fu nct io n In p u t Ou tp ut
Senso ry nuclei
Ventral posterior lateral (VPL) Somatosensory Dorsal column path way
Dorsal Postcentral gyrus
or body Ante rolateral (spinot
(spinot halamic) tracts (superior-medial)
Vent ral pos terior me dial (VPM) Somatosensory Trig e mi
min al
al p at
at hw
hw ay
ays Po st
st ce
ce nt
nt ra
ra l g yr
yr
or ace
Late ral ge niculate nucle us (LG
(LGN) Visu a l p a t h wa y Op t ic t ra ct s Occip it a l lo b e s
Medial geniculate nucleus (MGN) Au d itit or
ory p at
at hw
hwa y In e ri
rio r co llicu lu s Su pe
pe ri
rio r t em
em po
po r
Pulvinar Visu al
al a t te
te nt
nt io
io n Su p er
erio r co llicu lu
lu s Occip itit al
al a nd
nd p a
Motor nuclei
Ven tral ant erio
eriorr (VA) Mo t o r circu it s Ba sa l g a n g lia Mo t o r/ p re m o t o
motor cortex
Ven tral lat eral (VL
( VL) Mo to
to r circu itit s Ba sa
sa l g an
an gl
glia Motor/premotor/s
Cerebe ll llum
um (v
(via
ia sup erior motor cortex
cerebellar
cerebell ar p eduncle)
Cog nition, consciousne ss, and arousal nuclei

Anterior Memory Mamill
amilloth
oth alamic tract s (Papez cir- Ante rior cingulat
and e moti
motion
on cuit originates in hippocampus) ultimate ly projects
(Papez circuit) hippocampus)
Mediodorsal Cognition Cortex Corte x (except
(except
Centromedian Arousal Basal ganglia which
which project to
Reticular Consciousness Reticular act iv
ivating
ating system nuclei)
Intralaminar Other thalamic nuclei
ARTERIAL SUP P LY OF THE

ARTERIAL [SCAs],
[SCAs ], anterior in erior cerebellar
cerebellar arteries
art eries [A
terior in erior cerebellar arteries [PICAs]), w
CEREBRAL
CE REBRAL HEMIS
HEMISPP HERES
cussed in relation to the brainstem in Chapter 9
(FIGS.
(FIGS. 7–3 AN
AND
D 7–4 ) T e anterior circul
circulation
ation and posterior c
linked by the posterior communicating art
T e brain, brainstem, and cerebellum
cerebellum are supplied
supplied by arteries
ACAs are linked by the anterior communi
arising rom the paired internal carotid
carotid arteries (the anterior
T es
esee connections
connections orm the circl circlee o Will
circulation) and the paired vertebral arteries (the posterior
sur ace o the brain, which
which provides routrouteses
circulation). T e internal
internal carotid
carotid arterie
arteriess arise
arise rom the com-
com-
Not all patients
pat ients have a complete circle o
mon carotid arteries
art eries,, which
which themselves
themselves arise
arise rom th e aortic
patients have
h ave anatomic variants, the m ost com
arch ( r om the brachiocephalic trun k on the right and an d directly
are introduced
introduced here. T e clinic
clinical
al impli
implicati
cations
ons o
rom th e aortic arch on the le ). Each
Each carotid artery ultimately
ultimately
variants are discussed later
later in this chapter.
givess rise to a middle cerebral artery( MCA) and an anterior
give
cerebral artery( ACA),
ACA), and these arteries together supply the • Hypoplastic vertebral artery. Many patie
majority o the cerebral
cerebral hemispheres including the ront rontal
al dominant vertebral artery
artery and a smaller
smaller
lobes, parietal lobes, and superior and lateral temporal lobes. dominant vertebral artery. When this occu
Each internal carotid artery also gives rise to an oph- artery appears
appears to swi
swing
ng to the side
side o the non
thalmic artery (which supplies the retina) and an anterior tebral artery, and the vertebral canal is smal
choroidal artery (which
(which supplie
suppliess the p osterior thalamus and o the congenital
congenitally
ly small
smaller
er vertebral
vertebral artery.
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Neuroanatom
anatom ic Loc
Localiz
alization
ation
Left anterior
cerebral artery
Left pos terior

Left
cerebral artery
Anterior
Anterior communicating arte ry
Left middle
cerebral artery Internal carotid arter
Anterior ce rebra
Carotid siphon artery
Basilar
Basilar a rtery
Middl
iddlee ce
Left vertebra l
Left artery
artery
Posterior
Left interna l communicat
carotid artery artery
Left commo n
Left Posterior
carotid
carotid artery cerebral art
Superior
cerebe llar a
Basilar
Basilar
with
with p ontin
bra nc he
Left s ubclavi
Left ubclavian
an
Anterior inferi
artery
cerebe llar arte
Posterior inferior
Aorta
cerebe ll
llar
ar artery
Left vertebral artery
Anterior spinal arte ry
A B
FIGURE 7–3 Schematic of the b lood su pply to the brai brain. A:F
n. A: rontal view
view o the ce rebral circul
circulation
ation (as i look
looking
ing at the p atient; n
the circum ere ntial branche s o the verte broba si
silar
lar sys
system
tem are n ot shown in this diagram).
diagram). B: View o the in erior sur ace o the brain (an
rior
rior sur ace o the b rainstem) showing
showing the arte ri
rial
al supp
supp ly to the ce rebral hemispheres, brainstem, and cerebe ll llum.
um. Reprod
Reprod uced with per
rom Waxman S: Clinical Neuroanatomy, 27th ed. New York: McGraw-Hill Education; 2013.
• Azygous ACA. In this variant, both ACAs

ACAs emerge
emerge rom a plane—anteriorly
lane—anter iorly and medially to this trapezoid are
common trunk. by the ACA
ACAs,s, and poster iorly and in erior
eriorly
ly are sup
• Fetal PCA. In this variant,
variant, the PCA arises rom th e internal the PCAs (including the th alamus, which
which is supplied
carotid
carotid artery rather than the top o the basila
basilar.
r. T is va
variant
riant etrating vessel
vesselss arising
arising rom th e PCAs
PCAs and posteri
may occur unilaterally or bilaterally (Fig. 7–5). mun icating arteries).
arteries).
• Artery o Percheron. In this variant, a single artery rom
one o the PCAs
PCAs supplies both thalami (rather than
th an an indi-
vidual supply on each side).
Wate rshe d (B
(Bo
o rde rzo ne
ne)) Te
Te rrito
itorrie
(Fig.
(Fig. 7 7)
T e watershed
watershed (borderzone)
(borderzone) territories are the regi
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Cerebral Hemisphere
Vascular
ar Syndr
Syndr
Middl
iddlee c ere bral arteries Interna
Interna l caroti
carotid
d a rteri
rteries
es
(MCAs) (ICAs)
Middle cerebral
arteries
(MCAs)
Anterior cerebral
arteries Anterior
(ACAs) cerebral
arteries
(ACAs)
Basilar artery
Vertebral arteries
Posterior
Posterior cerebral arteries
(PCAs)
Interna l carotid arteries
(ICAs) Vertebral arteries
An te rio r v ie w In fe rio r v ie w
A B
FIGURE 7–4 MR ang iog ram (MR (MRA) of t he intracranial arterial circulation . A: F
ronta
ronta l view
view (as
(as i looki
looking
ng at the pat ient).
(as i looki
looking
ng up at the base o the brain rom below).
below).
to hypoper usion, this is not always

always the cause, and
and an embolic superior ronrontal
tal lobes
lobes and the medial and su
etiology should also be considered
considered as the etiology o border- lobes (supplied by the ACA), and the occipit
zone in arction ( or an interesting discussion
discussion o this topic, see temporal lobes
lobes (PCA territory). T e unctiona
Caplan and H ennerici, 1998).
1998). plied by
b y th e MCA there ore
o re include the moto
tor regions, somatosensory cortex, the ron
language areas ( ound on the le in the majori
parietal r egions respon sible or spatial atten
CLINICAL SY
CLINICAL S YNDRO
NDROMES
MES etal lesions
lesions may cause le -sided neglect),
neglect), and th
ASSOCIATED WITH CEREBRAL in erior radiations
radiation s o the visual
visual pathways
pathways as
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Cerebral Hemisphere
Vascular
ar Syndr
Syndr
Cortical branches of:

Anterior
Ant erior cere bral artery
Arteries:
Anterior cere
cere bra l
Middle ce rebral
Middl
iddlee cere bral arter
Posterior cerebral
Cortical branches
of middle cerebral
artery in late
late ral
sulcus Poster
cerebr
artery
Se gments of internal
internal
ca roti
rotid
d a rtery:
Cerebral
Carotid
Intracavernous
siphon
Intrapetrosal
Cervical
Branche s of poster
Branche
cerebral artery:
Parieto-occipital
Branches of anterior
cerebral artery:
Callosomarginal
Calcarine
Pericallosal
Frontopolar
and medial
orbitofrontal
Anterior cerebra l
Superior
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Neuroanatom
anatom ic Loc
Localiz
alization
ation
Lateral ventricle Arteries:

(ante ri
rior
or horn)
Anterior cerebral
Striatum
Middle cerebral
Internal caps ul
ule:
e: Pos teri
terior
or cerebral
Anterior limb Anterior
Anterior ch oroi
oroida
da l
Genu
Pos terior li
limb
Globus pa ll
llidus
idus
Thalamus
Lateral ventricle
(atrium)
Ant.. ce rebra l A
Ant
Int. capsule
Middle
Body of caudate cerebral A
Claustrum
Thalamus
Putamen
Pos t. cerebral A
Globus palli
pallidus
dus
Red nucleus
Subthalamic body
Cerebral pe duncl
dunclee
Penetrating
Uncus bra nc he s of
middle
Post. cerebral A
Ant. choroidal A
cerebral A (Putamen,
(Lower
(Lower 2/3 of int.
int. ca psu le, upper int.
int. caps ul
ule,
e,
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Cerebral Hemisphere
Vascular
ar Syndr
Syndr
Anterior/middle to have
have arise
arisen
n rom the anterior
anterior circulati
circulation.
on.
cerebral watershed tant to r ecog
ecognize
nize in patient s with
with PCA stroke
stroke
carotid
carot id stenosis: A etal PCA
PCA on the
th e side
side o
carotid stenosis suggests that the stenotic ca
tomatic (see Ch. 19).
In some patients, the le t and right tha
supplied by a single arter y that ar ises
re erred to as the artery o Percheron . Occ
artery can lead to bithalamic in arction ca
altered ment al status, a rare global, rath er tha
syndro me (Fig. 7–12).
7–12).
Middle/posterior
cerebral watershed Lacun
acunar
ar Stroke s
st rokes are caused
Lacunar strokes caused by occlusion
occlusion o
ing arteries a ecting the subcortical
subcortical white
white m
capsule), subcortical gray matter (basal gan
Anterior/middle [Fig. 7–13]), or anterior pons. Lacunar stro
cerebral watershed include:
• Pure motor stroke: unilateral hemiparesis/h
to involvement
involvement o the posterior limb
limb o the in
or the anterior pons.
• Pure sensory stroke: unilateral hemisensor
involvement
involvement o the VPL
VPL/VPM nuclei
nuclei o the t
• Ataxia-hemiparesis: unilateral hemipares
(due to involvement
involvement o the corticospinal tra
in the weak
weak limb(s) due to interru ption o
pont ocerebel
ocerebellar
lar bers destined or the
pedun cle
cless (see Ch. 8). T is can occur due to
in either the internal capsule or the anterior
which are places where the corticospinal tra
Middle/posterior pont ocerebel
ocerebellar
lar bers run together.
cerebral watershed
• Dysarthria–clumsy hand: dysarthria and
FIGURE
FIGURE 7–7 Schematic of ACA/MCA and MCA/PCA limb ataxia; locali
localization
zation is the same as or atax
watershed (borderzone) territories . Reprod
Reprod uced with pe rmis
rmissi
sion
on (internal capsule or anterior pons).
rom Aminof M, Gree
Gree nb erg D, Simon
Simon R: Cli
Clinical
nical Neurology,
Neurology, 9th e d.
New York: McGraw-H
cGraw-Hil
illl Edu cation ; 2015.
Infarc
nfarctio
tionn in the Wate rshe d (Bo
(Bo
Territories (Fig. 7 14)
ability to write ( alexia without agraphia) (i there is le T e MCA-ACA
MCA-ACA watershed regions span th
in erior temporal involvement), decreased ability to recog- the border o the two territo ries. Re
Recall
calling
ing
nize aces (prosopagnosia) (i there is right in erior tempo- lus (Fig
(Fig.. 4–1), the part o the motor hom
ral involvement), and/or changes in cognition and/or level o by th e MCA-ACA water shed region inclu
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Neuroanatom
anatom ic Loc
Localiz
alization
ation
A B
FIGURE 7–8 MCA infa rct. A

xi
xial
al CT
CT scan d em onst rating ull-
ull-te
te rritory le
le t MC
MCA in arct . Note
Note spared are as including: A:c audat e (supp
recu rrent arte ry o Huebn er), thalamu s (supplied b y PCAPCA), ACAand PC PCAA te rritories, as well as th e me dial tem po ral lob
lob e ( B). Note also hy
le t MCA in B (see Ch. 19 or explanat ion o this sign).
sign).
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CHAPTER 7 T e Cerebral Hemispheres and Vascular Syndr
A B
FIGURE 7–11 PCA infarct. Axial dif usion-we ighte d MRI (DWI) de mon strat ing ischemic in arct in the t erritory o the le t PC
territory involves not only the po sterior occipital lobe but also the t halamus ( A) and med ial temp oral lobe ( B).
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A B C
FIGURE 7–14 Watershed (borderzone) infarcts. Axial dif usion-weigh te d MRI (DWI) de mon strat ing ischemic in arction in th e bo
zones. A:B
ilateral MCA-ACA borderzone in arctions. B:B
ilateral dee p b orderzone in arctions. C: Bilateral MCA-PCA borderzone in arctio
visual attent ion that can include some or all o the elements REFERENCES
o Balint’s syndrome: optic ataxia, ocular apr axia, and simul-
Caplan LR, Henn erici M. Impaired clearance o emboli (w
tanagnosia (see Ch. 6).
is an important link between hypoper usion, embolism,
T e evaluation and management o patients with cerebral ischemic stroke. Arch Neurol 1998;55:1475–1482.
in arction is discussed in detail in Chapt er 19.
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TABLE 8–1 Disting uishing Cerebe llar Ataxia From Di erential Diag nosis o Cerebe llar
Sens ory Ataxia. As with any neurologic problem, once localized, the
Cerebellar Ataxia Sensory Ataxia tial diagnosis arises rom an understanding o the tim
o symptom onset and evolution.
Finger nose Intention tremor “Searching/
Hyperacute-onset (over seconds to hours) o
testing meandering”
movements pathologyc an be caused by:
Worsens with e yes • Vascular causes: ischemic stroke, cerebellar hem
closed (see Ch. 19)
Accompanying Nysta gm us Decreased • Acute toxicity: alcohol, cytarabine (see Ch. 24)
eatures prop riocep tion
Dysarthria Acute- to subacute-onset (over hours to days) o
Romb erg sign
pathologyc an be caused by:
Pseudoathetosis
• In ection: progressive multi ocal leukoencepha
(Hyporef exia or
(see Ch. 20)
aref exia i due
to neuropat hy or • Inf ammatory causes:
ganglionopathy)
• Postin ectious cerebellitis (m ost common
Gait Wide based Wide based children a er a viral illness, most comm only
in ection)
• Flare o multiple sclerosis (see Ch. 21)
eyes open, let alone closed. Patients with a de cit in prop rio-
ception can stand with their eet together when using vision Subacute to chronic-onset (over weeks to mo
to compensate, but closing the eyes removes this cue and cerebellar pathologyc an be caused by:
requires the p atient to rely exclusively on propr ioception, so • Paraneoplastic cerebellar degeneration , which can
the patient may lose her/h is balance (Romberg sign). ciated with anti-Yo (ovarian and breast cancer),
On nger–nose testing, cerebellar and sensory ataxia (small cell lung cancer), anti- r (Hodgkin’s ly
have di erent app earances. Cerebellar ataxia appears as an anti-Ma2 (testicular cancer), and anti-GAD (o
oscillatory movement perpendicular to the plane o move- ciated with a malignancy) antibodies (see Ch. 24)
ment (i.e., side-to-side when the patient approaches the • umor: medulloblastoma (in children), metastat
target in the nger-nose task) and worsens as the patient (in adults)
approaches the target. Sensory ataxia causes what resembles
• Metabolic causes: vitamin E de ciency
a “searching” movement in which the a ected limb looks
as i it is approaching the target with meandering, circular Chronic-onset (over months to years) o
movements. With slow movements under visual guidance, a pathologyc an be caused by:
patient with sensory ataxia may be able t o gain reasonable • Chronic drug/toxin exposure: phenytoin, alcohol
accuracy with nger–n ose testing. However, i the examiner • Degenerative etiologies
leaves the target nger in the same place and asks the patient
• Acquired: multiple systems atrophy cerebel
to continue going back and orth rom nose to nger with
(MSA-C) (see Ch. 22)
the eyes closed, the patient will become increasingly inaccu-
rate. T is is because removing the patient’s visual compensa- • Inherited:
tion requires complete reliance on proprioception, which is • Friedreich’s ataxia (autosomal recessive)
impaired in sensory ataxia. • Spinocerebellar ataxias (autosomal dominan
An additional subtle sign o diminished proprioception • Fragile X–associated tremor ataxia syndrome
that may be seen is pseudoathetosis. Athetosis is a movement
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CHAPTER 8 T e Cerebellum and Approach to A
Inhe rite d Cause s o Cerebe llar Ataxia may be accompanied by parkinsonism and/o
Friedr eich’s at a xia—T is autosomal recessively inherited characteristic MRI ndin g is 2/FLAIR hype
ataxia a ects the spinocerebellar tr acts as well as the dor sal the bilateral middle cerebellar pedun cles (F
columns, corticospinal tracts, and peripheral nerves. In addi-
tion to ataxia and sensory loss developing in young adult-
hood, most patients develop cardiomyopathy. T e causative
mutation is in the frataxin gene (caused by GAA repeat).
Spinocereb ellar at axia—T is term is applied to a growing

number (more than 30) o autosomal dominantly inherited
ataxias that are all characterized by adult-onset ataxia, but
which can have a variety o additional eatures such as pyra-
midal, extrapyramidal, or cognitive dys unction, and/or neu-
ropath y. T e most common spinocerebellar ataxia (SCA) is
SCA3 ( Machado-Joseph disease), which causes cerebellar
dys unction, cognitive impairment, neuropathy, and, in some
cases, extrapyramid al eatures (e.g., parkinsonism; see Ch. 23).
T e highest prevalence o SCA3 is in the Azores, and the caus-
ative mutation is in the ATXN3 gene (caused by CAG repeat).
Fra gile X–as socia te d tre mo r/a ta xia syndr ome (FXTAS)—
An adult-onset progressive ataxia called ragile X–associated
tremor/ ataxia syndrom e (FX AS) can be caused by muta-
tions in the same gene (FMR1) that causes ragile X syndrom e
(a common cause o mental retardation in boys, accom-
panied by dysmorp hic acial eatures and large testicles).
FX AS occurs in patients with a ewer number o trin ucel-
otide (CGG) repeats than ar e necessary to prod uce ragile X
syndr ome (e.g., in the parent or grandparent o a child with
ragile X syndrome), re erred to as a premutation. As an
X-linked condition, the disorder most commonly occurs in FIGURE 8–5. Axial FLAIR MRI in ragile X-ass
men, but can rarely occur in women in a milder orm. Onset ataxia synd rome (FXTAS) de monst rating bilateral
o cerebellar ataxia begins most common ly a er age 50, and ties in the middle cerebellar peduncles .
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86 PAR I Neuroan atomy and Neuroanatom ic Localization
Hypothalamus
Ophthalmic division
of trigeminal nerve
Long cilia ry ne rve
To sweat glands of forehead
To s mooth mus cle of eye lid
To pupil
Interna l carotid a rtery To sweat glands of face
External carotid artery

C2
Third ne uron
Sup erior cervica l ganglion

First neuron
T1
Second ne uron
Spinal cord
FIGURE 10 –3 The sympath etic pathway or pupillary dilation . Rep rod uce d with pe rmission rom Amino M, Gree nb erg D,
Clinical Neurology, 9th ed . New York: McGraw-Hill Edu cat ion; 2015.
• At the level o the third-order neuron hydroxyamphetamine eye drops all have e ects on
• Internal carotid artery dissection (see Ch. 19) nephrine transmission, and the responses o anisocor
• Neck surgery to these eye drops can help to determin e localization o
along the oculosympathetic pathway ( able 10–1).
• Cavernous sinus pathology, usually accompanied by
Cocaine eye drops decrease norepinephrine r
multiple de icits in ocular movements and/or numb-
allowing more norepinephrine to stay in the sy
ness in the upper ace, since CNs 3, 4, and 6 and th e
dilates a normal pupil. However, in Horn er’s syndr ome
V1 and V 2 branches o CN 5 also travel through the
epinephrine is being released at this synapse, so there i
cavernous sinus.
epinephrine reuptake to block, and the constricted pup
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CHAP ER 10 Pupillary Contr ol and Approach to Anis
TABLE 10 –1 Pharmacologic Localization in Horner’s Syndrome.

Ef ect on Both
Pupil With Sympa the tic Pupils in Patie nt With
Normal Pupil Dys untion Horner’s Syndrome Localizing
Cocaine Dilates Does not dilate Anisocoria worsens Conf rms H
Apraclonidine No e ect or Dilates Anisocoria reverses Conf rms H

mild dilation
Hydroxyamphetamine Dilates Does not dilate i (Not tested in both eyes) Distinguishes
third-order lesion lesion rom f
second - order
Dilates i f rst-order or second-
order lesion
the nal synapse, and so the postsynaptic receptors become I pupillary asymmetr y is more pron ounced i
hypersensitive over time since they have been deprived o suggests that the smaller pupil is the abnorm
norepinephrine. In a normal pupil, there is no (or very little) not dilated adequately in darkness as it should
change in pupil size when apraclonidine is administered, but normal pupil has dilated, exaggerating the di
in a chr onically denervated Horn er’s pupil, the sup ersensitive them. I pupillary asymmetr y is more pron oun
receptors are easily excitable by stimulation, so ap raclonidine this suggests that the larger pupil is the abnorm
causes the pupil to dilate. In Horner’s syndrome, i apracloni- not constricted adequately in response to ligh
dine drops are administered to both eyes, the Horner’s pupil have and the normal pupil has constricted, ex
dilates more than the normal pupil, leading to reversal o di erence between them (Fig. 10-7).
the anisocoria (i.e., the ormerly smaller pup il is now larger) I ptosis is present on th e side o the small
(Fig. 10–4D–F). Note that since supersensitivity takes time to gests Horner’s syndrome. I ptosis is present on
develop, there will be no pu pillary dilation with apraclonidine large pupil, this suggests CN 3 path ology (
in a recently developed Ho rner’s syndrom e. Like cocaine eye Anisocoria can be due to:
drops, apraclonidine con rms that th ere is a Horner’s syn-
drom e present but d oes not localize it. • CN 3 lesion (larger pupil abnormal); or ex
Hydroxyamphetamine eye dropsc ause norepinephrine compression rom posterior communicatin
release rom third-or der oculosympathetic neurons. I the rysm or uncal herniation (see Ch. 24).
third -order neuron s are the site o the lesion, there will be no • Lesion along the sympath etic pathway (small
norepinephrine released since the third-order neurons are mal; see “Impaired Pupillary Dilation” above
not working. However, i the third-or der neuron s are intact • Pharmacologic e ect: ipratropium n ebulize
but are n ot receiving stimulation due to a lesion at the level one eye and scopolamine patch (i patient
o the rst-order or second-order neurons, the third-order then touches eye) are both common pharma
neurons will have a large amoun t o norepinephr ine waiting o pupillary dilation.
to be released, and hydroxyamphetamine will cause the pu pil • Local iris pathology (e.g., prior ophthalmolo
to dilate (Fig 10–4G–I). T ere ore, hydroxyamphetamine eye trauma)
drops can determine whether a Horner’s syndrome is due to
• Migraine.
third-order pathology or not, but cannot distinguish between
• Seizure and postictal state.
rst-order and second-order lesions.
In sum, cocaine and apraclonidine eye drops can con- • Physiologic anisocoria: a benign ndin
rm the presence o Horn er’s syndrome but do not localize anisocoria, both pupils usually react symm
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88 PAR I Neuroan atomy and Neuroanatom ic Localization
Cocaine
A B C
No
Signal
NE NE
NE NE
NE NE
NE NE
NE NE
Cocaine
Cocaine Cocaine
Cocaine
Cocaine
NE NE
NE
NE
NE NE NE
P P P

u u u
p p p
i l la
r i l la
r i l la
r
y d y d y d
i la
t o r i la
t o r i la
t o r
Norm a l e ye Preganglionic Postganglionic

Horner syndrome Horner syndrome
D E F
G H I
FIGURE 10 –4 The ph ysiolog ic basis o pharmacologic localization in Horner’s syndrome . A C:C ocaine eye drops in Horner’s
syndrome. A:A
normal pup il dilate s when cocaine eye drop s are administered b ecause n orepinep hrine reupt ake is blocked. B C:I n Ho
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90 PAR I Neuroan atomy and Neuro

Neuroanatom
anatom ic Loc
Localiz
alization
ation
becomes supersensitive (just as with the pup pupill

ill
muscle in Horn er’
er’ss syndrome that is deprived o nor
rine; see
see discussion
discussion o apraclonidine eyedrops abov
carpine eyedrops stimulate the supersensitive toni
leading to constriction more so than would occur i
A mal pupil. T ere ore, pilocarpine
pilocarpine administere
administered d to bo
will cause the tonic pupil to constrict more than the
pupil, reversing the an isocoria (i.e.,
(i.e., the larger pup
the smaller
smaller pupil).
pup il). I the pupil
pup il is large
large due to pharm
e ect as opposed to it being a tonic pupil, pilocarpine
pilocarpine
3 mm 3 mm 6 mm 4 mm have this e ect.
B
Bilateral Pupillary Abnormalities

Bilateral pupillary dilation can be seen with:
• Bilateral CN 3 lesions
5 mm 3 mm 6 mm 6 mm
• Midbrain lesions
C • Sympathom
ympathomimetic
imetic medications (e.g
(e.g.,
., amphetamines,
amphetam ines,
Bilateral pupillary constriction can be seen with:
• Pontine pathology
3 mm 2 .5 m m 6 mm 5.5 m m
• Opiate medications
• Old age
D
• Syphilis. In syphilis, the pupils constrict to acco
FIGURE
FIGU RE 10 –7 Examining aniso coria in light and dark dark..
tion but not to light, which is called Argyll-R
A:A nisocoriaa with t he larger pup il on the patient ’s right. B:I th e
nisocori pupils
u pils (mn emon ic: Accommodates— yes, R
anisocoriaa is worse in darkness, this
anisocori this sugge sts that the small
smaller
er pu pil ArgYll-Robertso N).
(on the pat ient’
ient’ss le
le t) has ail
ailed
ed to d il
ilate
ate an d is the ab normal pupil.
C:I the anisocori
anisocoriaa is worse
worse in light
light , this sugge sts that the larger pupil
Dilated or constricted pupils can also be seen
(on the pat ient’
ient’ss right)
right) has ail
ailed
ed to con stri
strict
ct and is the abn ormal ally with widespread sympathetic and/or parasym
pu pil. D:I n physiologic anisocoria, subtle anisocoria is generally dys unction as can be caused by autonomic neu
pre sen t in both light a nd d ark. Rep rodu ced with p ermission rom (see Ch. 27).
Martin T, Corbett J: Practical Neuroophthalmology. New York:
McGraw-Hil
cGraw-Hilll EduEdu cat ion; 2013.
2013 .
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C H A P
Ext r aocular Mov
oveem ent
entss
and
an d Appr
Approac
Diplopia
Cranial
Cr
oach
anial ner
n erv
h to
to
ves 3, 4,
4, and 6
1
EXTRAOCULAR MOVEMENTS I: MUSCLES Horizontal Gaze

AND THEIR INNERVATION Vertical Gaze
EXTRAOCULAR MOVEMENTS II: CRANIAL Supranuclear Versus Nuclear/In ranuclear
NERVES 3, 4, AND 6 Les ions A ecting Eye
Eye Mov
Mov eme nts
Cranial Nerve 3 : The Oculomo to r Nerve APPROACH TO DIPLOPIA
Cranial Ne rve 4 : The
The Trochle ar Nerve Alternate Cover Tes
Tes t in the Diagno sis o
Cranial Nerve 6 : The Abduce ns Ne rve Diplopia
EXTRAOCULAR MOVEMENTS III: SUPRANUCLEAR The Maddox Rod
Rod in the Diagno sis o
CONTROL OF HORIZONTAL AND VERTICAL GAZE Diplopia
Opto kinetic Re ex
Each eye
eye is moved by six mumuscles
scles:: our r ectus muscles and EXTRAOCULAR
EXTRAOCUL AR MOVEMENTS
two oblique muscles.
muscles. T ese muscles
muscles are controlled by three
nerves: cranial nerves (CNs)
(CNs) 3, 4, and 6. T ese cranial
cranial nerves
MUSCL
MUS CLES
ES AND
AN D THEIR INNERV
IN NERV
all originate rom brainstem nuclei that commun icate with (FIG. 11 –1 AND TAB
ABL
LE 11–1
11 –1))
one another through the medial longi longitudinal
tudinal asciculus
T e six
six muscl
muscles
es that control each
each eye
eye are the
(MLF)
(ML F) to coordinate movements betwe betweenen the le
le and right
cles (superior, in erior, medial, lateral) and th
eyes.
eye s. T ese nu cle
cleii are controlled by brainstem gaze
gaze centers
muscles (superior and in erior). CN 4 contro
that coordinate the eyes to move together horizontally or
oblique, CN 6 controls the lateral rectus, and
vertically, and these gaze centers are stimulated by cortical
the rest (superior, in erior, and medial rect
eye elds. From the top down, the cort cortical
ical eye elds stimu
stimu--
oblique).
oblique). T e principal ey
eyee movements
movements per
late the gaze centers in the brainstem, the brainstem gaze
tus muscl
mu scles
es are easy
easy to un derstand :
centers communicate with with the cranial nerve nuclei o CN
3, CN 4, and CN 6, and CN3, CN 4, and CN 6 activate the • Lateral rectus (CN 6) moves the eye laterally
extraocular muscles. • Medial rectus (CN 3) m oves the eye mediall
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Neuroanatom
anatom ic Loc
Localiz
alization
ation
Trochlea Superior Inferior

Sup erior oblique
oblique rectus oblique
Superior rectus
Levator
Lateral Medial
rectus rectus
Inferior Superior
rectus oblique
Lateral rectus
Inferior rectus
C
A Inferior oblique
FIGURE
FIGU RE 11 –1 Schemati
Schematicc o the e xtr xtraocular
aocular muscl
muscleses and e ye move ments. A:
A: The atta chmen ts o the extraocular muscles
muscles on th
eye, viewed laterally rom the le t side. B: The actions o the sup erior oblique as shown rom above on the right eye (see text).
viewed laterally text). C:Princi
tions o the extraocular muscles
muscles demo nstrate d or the right eye. Note th at the sup erior oblique and in erior oblique
oblique act maximally
maximally to de
and e levate th e eye in the a dd ucte d position. Rep
Rep rod uce d with permission rom Amino
Amino M, Gr Gree
ee nb erg D, Si
Simon
mon R:
R: Cli
linical
nical Neurology,
New York: McG cGraw-Hi
raw-Hill
ll Edu cation ; 2015.
T e principal ey eyee movements

movements per ormed by the oblique
oblique head tilts to one side, the eye on the side to wh
muscles are slightly more complicated. In addition to mov- head is tilted (bottom eye) intorts and the oth
ing up, down, le , and right, the eyes can can also rotate when eye) exto rt s (Fig
(Fig 11
11–2).
–2). T is is important in under
the head is tilted
tilted to either side. Rotation o the eye toward the symptoms
symptom s and signs o a CN 4 palsy,palsy, which
which is d
the nose/midline is called intorsion and rotation toward the urther below.
ear is called extorsion. As the head is tilted to the le and Intor sion o the eye
eye is the main role o the
the eyes
eyes attempt to maintain xation straight
straight ahead, the le oblique muscle. Howeve
However, r, when the
th e eye
eye is ully addu
eyee must intort (turn towa
ey toward
rd th e nose) and the right eye must superior oblique depresses the eye.
eye. o understand th
extort (turn toward the ear). As the head is tilted to the right, o the superior
superior oblique, take your le
le hand and place
the right eye
eye intor
intor ts and the le eye extorts. In sum, when
when the crown o your head with your your elbow sticking out to
TABL
ABLE
E 11 –1 Inne rvation and Action s o the Extraocular
Extraocular Muscles .
Cranial Nerve
In ne rv
rvat io n Princip al Actio n Se co n da ry Act io n E e ct o We akn
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CHAPTER 11 Extraocular Movements

Movements and Approach to Di
Diaphragma
sella
Hypophysis
Se ll
llaa turci
turcica
ca
Internal carotid
artery
Dura
mater
FIGURE
FIGU RE 11 –2 Schemati
Schematicc o intor
intorsion
sion and
and extorsion
extorsion o the Sphenoid Na s op ha ryn x
tilting .
eyes with head tilting sinus
FIGURE
FIGU RE 11 –3 Sc Schematic
hematic o a coronal
coronal view o
sinus. Rep rodu ced with pe rmissi
rmission
on rom RopRop pe r A
Look straight ahead. Your head now represents the right eye Klein J: Adam s and
a nd Victo
ictor’
r’ss Principles of Neurology,
Neurology, 10t
and your arm represents the right superior oblique muscle, McGraw-Hill
McG raw-Hill Edu cat ion; 2014 .
bent at the elbow to represent the bend o the superior oblique oblique
muscle as as it passes
passes through the th e pulley
pulley (trochlea) or which 2. CNs 3, 4, or 6. rauma and nerve in arct
CN 4 is named (i.e., trochlear nerve). I you pull with with your common
comm on causes o isolated 3, 4, or 6 pals
hand,, this will
hand will tilt the head inward— this is intorsion. I you a ect CNs 3,3, 4, and
and 6 because
because their leng
turn
tur n your head all the way to the le so you you are looking at render th em susceptible
susceptible to traum a. Ne
your elbow crease (addu(adducting
cting the r ight eye), now pulling with
with 3, 4, or 6 is most commonly caused by
your han d causes the head to look down (depr essi essing
ng the eye). 3, 4, and 6 can also be a ected by skul
T e angle
angle o the superior
superior oblique
oblique allow
allowss or it to intort
intort the eye
eye aneurysms, subarachnoid hemorrhage, m
when the eye is midline or abducted, and to depress the eye Guillain-Barré syndrome (especially the
when
whe n th e eye
eye is adducted.
adducted. T e in erior
erior oblique per orms
orms an variant; see “Guillain-Barré syndrome” in
equal but opposite unction : extorsion o the eye eye when
when the eye
eye
3. Cavernous sinus. CNs 3, 4, and 6 pass thr
is midline
midline or abducted,
abdu cted, and elevation
elevation o the eye when the eye
ernous sinus along with the V1 and V
is adducted.
trigeminal nerve (Fig. 11–3). Potential p
T e primary
primary actions
actions o the superior
superior rectus
rectus and in in erior
erior
includes cavernous sinus thrombosis, car
rectus are what would be expected based on their names:
stula, pituitar
pituitaryy tum
tumors
ors or pituitary apoplex
superior rectus elevates the eye, in erior depresses it. How-
Hunt syndrome (an idiopathic inf ammato
ever,
eve r, these two
two muscles also per orm rotatorotatory
ry un
unctions.
ctions. Just
Just
the cavernous sinus).
as the superior oblique int orts th e eye,
eye, the superior rectu s also
also
contributes to intorsion; just as the in erior oblique extorts 4. Orbit. When CNs 3, 4, and/ or 6 are a ecte
the eye,
eye, the in erior rectus also cont
contributes
ributes to extorsion (mn e- the optic nerve is also o en a ected (this
monic to recall that in erior muscles extort and superior with cavern
cavernous
ous sinus pathology
pat hology since
since the op
muscles intort: In EXions will leave you SupINe). Just as the not pass through the cavernous sinus). Po
superior and in erior oblique per orm their secondary actions pathology in cludes tumors,
tu mors, in ections
ection s (or
(depression and elevation) in the addu cted position, the supe- and orbital pseudotumor
pseudotumor (an idiopathic
rior and in erior recti also per orm their secondary actions condition o the orbit).
(intorsion and ex extorsion)
torsion) in t he adducted position.
Crania
aniall Nerve 3: The
The Oculo mo to
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Neuroanatom
anatom ic Loc
Localiz
alization
ation
A B C
D E F
G H I
FIGURE
FIGU RE 11 –4 Right p upil-spar
upil-sparing CN 3 in arct) . The pat ient has ptosis o the right eye wi
ing third nerve p alsy (due to diabet ic CN
“down a nd o ut” (E). There is impaired
impaired add uction ( C, F, I), impaired elevation (A, B, C), and impaired dep ressi
ression
on ( G, H, I) o the right eye.
tion is spared (A, D, G). Spari
Sparingng o superior oblique unction is is di cult to observe in
in the setting o impaired adduction. Reprod uced wit
sion rom Martin
Mart in T,
T, Corb
Corb et t J: Pract
Practical
ical Neuroo
Neuroo pht ha lmology. New York: McGraw-Hi
McGraw-Hillll Edu cat ion; 2013.
A complete third nerve palsy (Fig. 11–4) causes: Each CN 3 nuclear complex in the dorsal midb
several
seve ral subn
subn uclei: one or each extraocular muscle mu scle
• Weakness o the our supplie
supp lied
d muscles,
muscles, leavi
leaving
ng the eye
eye
rectus, in erior rectus, medial rectus, in erior obliq
down and out: down due to the unopposed action action o the
Edinger-Westphal nuclei provide parasympathetic
superior oblique (CN 4) and out due to the unopposed
pupillary constriction. T e levator palpebrae m uscle
action o the lateral rectus (CN 6)
elevate the eyelid) are supplied bilaterally by a single
• Weakness o the levator
levator palpebrae, causing
causing ptosis called the central caudal nucleus. T e central central cauda
cauda
• Decreased parasympathetic input to the pupil, leading to projects bilateral
bilaterallyly to allow or symmetric blinking.
blinking. T
pupillary dilation
dilation (m ydriasis
ydriasis)) rior rectus subnucleus o each third nerve nucleus
Due to the way the di erent bers run in the third nerve, contralatera
cont ralaterallylly,, and the crossing
crossing bers pass in close
close
partial lesions
lesions o the third n erve can a ect the pupillary bers ity to the contralateral CN 3 nucleus.nucleus. T ere ore, a v
in isola
isolation
tion or the ocul
ocular
ar motor bers in isolati
isolation.
on. T e pupil- ocal les
lesion
ion o the entire third nerve nuclear
nuclear comple
lary bers run on the medial exterior part o the nerve, whereas side wil
willl cause ipsilateral impair
impairmen
mentt o all thir
third
d ner
the oculomotor
oculomotor bers run on the inside o the nerve. A les lesion
ion tions and bilate
bilateralral involvement
involvement o the superior r ectus
compressing the third nerve a ects the outerm ost bers rst, o the third n erve nucleus cause cause bilateral
bilateral superior rect
which can lead to impaired pupillary constriction with no ness because
because the a ecte ected
d superior rectus subnucleus
extraocular muscle dys unction (or preceding the develop- contralaterally
contralaterall y (causing
(causing contralateral impairment o
ment o extraocular muscle
muscle dys unction). On the other hand,han d, and the crossi
crossingng bers projecting rom the una ecte
an ischemic
ischemic insult to the nerve wil
willl a ect the innermost
innermo st bers lateral superior rectus subnucleus pass in close prox
supplied by small penetr ating vessels
vessels,, and can cause extraoc- the a ected nucleus, causing involvement
involvement o the eye
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CHAPTER 11 Extraocular Movements

Movements and Approach to Di
contralateral tremor and/or contralateral ataxia ( Claude’s Patients with CN 4 palsy have verticalvertical dou
syndrome) due to involve
involvement
ment o the red nucleus and crossed is worst in in downgaze when lookinglooking away rom
superior cerebellar
cerebellar pedun cle (coming rom the contralateral a ected eye (e.g (e.g.,
., looking le i the right eye
eye
cerebellar hemisphere; see Ch. 8). because looking away rom the side side o the a
a ected eye eye in adduction , the position
position in whic
Crania
aniall Ner
Ne rve 4 : The
The Tro chle ar Nerve oblique un unctions
ctions to depress the eye.
eye. When pla
CN 4 originates in the dorsal midbrain and is the only cra- tion th at most needs the superior oblique (down
nial nerve to exit posteriorly and the only cranial nerve that dys u nction wil willl be most evident,
evident, leading to dou
crosses to project contralaterally. It innervates one muscle, is worst in th is position (Fig. 11–5).
11–5).
the superior oblique. Like CN 3 and CN 6, it is susceptible In summar y or superior oblique palsies, palsies,
to trauma and diabetic nerve in arct (although diabetic nerve the head away rom the side o the palsy, palsy,
in arct occurs lessless commonly
common ly in CN 4 than in CN 3 or CN 6). worsens with downgaze away rom the side
CN 4 can also be compressed by dorsal midbrain pathology (i.e.,, a ected eye in adducted position). T
(i.e.
(e.g., pineal mass). is the only cranial nerve that crosses, and as a
When the head is tilted to one side, the eye that intorts is de cits can also also be thou ght o as “cross
“crossed”:
ed”: T
the one on the side o the head to which the patient patient is tilting the rom th e sideside o the superior oblique palsy,
palsy, and
head ( or example, i the patient tilts the head to the le , the le le ens when
when looking away rom the side o the su
eyee must intort,
ey intort , rotating equal and opposite
opp osite to the direction
direction that palsy (i.e.
(i.e.,, adducting the a ected eye).
eye).
the head is tilting).
tilting). When intorsion is impaired due to a CN 4
palsy,
alsy, double visi vision
on (diplopia) occurs when the head is tilted
Crania
aniall Nerve 6: The
The Abd
bduce
uce ns
toward
tow ard the
th e a ected side since that eye cannot intort int ort to maintain
xation.
xation. T ere ore, the patient’s
patient’s pre erred head position
position is to tilt
tilt T e abducens
abducens nuclei resi
reside
de in the dorsomedia
the head away rom the th e a ected side
side to keep
keep the eyes
eyes alig
aligned.
ned. In bilateral CN 6 run rom their nuclei thr throug
oug
a le CN 4 palsy, a patient’
patient ’s double vision
vision will worsen when tilt- pons,
on s, exit anteriorly,
ant eriorly, and then pass over t he c
ing the head to the le le , and so the patient will
will pre er to keep the the cavernous sinus, to the orb its.
head tilted to the right.
r ight. In a right
r ight CN 4 palsy, a patient’s
patient’s double An abducens palsy
palsy leads to ailure to a
vision will worsen when tilting the head to the right, and so the eye (Fig.
(Fig. 11–6). Abduction weakness causes ho
patient
atient wil
willl pre er to keekeepp the head tilte
tilted
d to the le
le . pia that worsens
worsens when looking toward the side
B
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A B C
FIGURE 11 –6 Right s ixth nerve p alsy ( due to diab et ic CN 6 in arct). The p atien t’s right e ye is sligh tly medially de viated a t ba selin
the p atient is unable to abdu ct the right eye (A). Le tward g aze is preserved (C). Rep rodu ced with pe rmission rom Martin T, Corbe tt J:
Neuroophth almology. New York: McGraw-Hill Edu cation ; 2013.
de cit (e.g., a right-sided CN 6 palsy will cause diplopia when by asking the patient to ollow the examiner’s nger.
looking to the right, which requires right eye abduction). I may occur voluntar ily (a conscious decision to look
there are no other cran ial nerve or extraocular muscle de - thing), or can occur in voluntarily (e.g., eyes move re
cits, looking away rom the side o the de cit should lead to in the direction o a loud noise). T e rontal eye
complete resolution o double vision, since adduction an d intentional saccades, and the parietal eye eldsa re
contralateral eye abduction are spared. I a CN 6 palsy causes in ref ex saccades and smooth pursuit. T is is log
complete paralysis o the lateral rectus, the a ected eye may recalls that intentional actions originate in the ron
be misaligned medially at rest with no lateral movement o the whereas spatial attention is supported by the parie
eye on attempted gaze toward the a ected side. With partial (see Ch. 7).
weakness, the eye may be able to abduct only partially, allow-
ing some o the lateral sclera to remain visible on attempted Opto kinetic Re ex (Fig. 11 7)
lateral gaze (called inability to “bury the sclera”).
T e saccadic ( rontal) and smooth pur suit (parie
CN 6 has a long and tortuous intracranial course that
tems can be tested by evaluating the optokinetic r
takes it over the clivus. Like CNs 3 and 4, the length o CN
6 makes it susceptible to trauma. CN 6 in arct, most com-
monly due to diabetes, is anoth er common cause o unilateral
CN 6 palsy. Unilateral or bilateral CN 6 palsy can also occur
when intracranial pr essure is elevated since this pressure leads
to stretching o the nerve(s) (see Ch. 25). T is is sometimes
re erred to as a “ alse localizing” sign since it is a ocal de cit
that may not necessarily be caused by a ocal lesion.
I CN 6 and CN 7 are a ected on the same side, this sug-
gests pon tine localization since th e CN 6 and CN 7 nu clei are
adjacent in the pons. With a larger unilateral pontine lesion,
contralateral hemiparesis may accompany CN 6 and CN 7
lesions (due to involvement o the not- yet-crossed corticospi-
nal tract). I CN 6 is a ected with CN 3 and/ or CN 4 without
involvement o CN 2, localization o the lesion in the cavern-
ous sinus should be considered. Involvement o CNs 3, 4, and/
or 6 an d CN 2 suggests localization in the orbit. Smo oth pursuit
EXTRAOCULAR MOVEMENTS III: Saccade

SUPRANUCLEAR CONTROL OF
HORIZONTAL AND VERTICAL GAZE Left Right
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CHAPTER 11 Extraocular Movements and Approach to Di
optokinetic nystagmus (OKN) strip typically has vertical T e saccades in the opposite direction rom
alternating white and red stripes, and an OKN drum typi- o motion o the OKN strip/d rum (le in
cally has vertical alternating white and black stripes. When an supported by the ron tal lobe ipsilateral to th
OKN strip is moved across the visual eld in one direction (or movement o the OKN strip/drum (in this exa
an OKN drum is rotated in one direction ), the eyes ollow it rontal lobe generates le ward saccades when
in the direction it is moving. However, in order or the patient is moving to the right).
to continu e ollowing it, the patient must make saccades in In addition to utilizing optokinetic nystag
the direction opposite the direction o movement o the strip/ ize rontal versus parietal lesions, the optokinet
dru m (like when watching trees pass by out o the window o hard to inhibit and, there or e, may be used to d
a train). For example, when moving the OKN strip rom le to chogenic blindn ess rom tr ue visual loss.
right (or spinnin g the OKN drum rom le to right), the eyes
ollow smoothly to the right with interrupting saccades back
to the le . T e pursuit in the direction that the OKN strip is Horizontal Gaze (Fig 11 8)
moving/dr um is turn ing is supported by the parietal lobe ipsi- T e signal to voluntarily move the eyes comes
lateral to the direction that th e strip is moving/drum is turn- tal eye elds. Just as each hemisphere controls
ing (in this example, the right parietal lobe supports rightward eral side o the body and sees the contralateral
smooth pursuit when the OKN strip is moving to the right). rontal eye elds send the eyes to the contr
Right Frontal
Eye Field
Lateral Medial
rectus rectus
IIIrd nerve
Midbrain
IIIrd n ucle us
VIth
nerve
IVth nucleus
MLF
P Pons
P
R
F
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le ron tal eye eld sends the eyes to the right, and the right eye elds"contralateral PPRF"CN 6 nucleus"con
rontal eye eld sends the eyes to the le . CN 3 nucleus (via MLF).
Horizont al gaze requires synchron izing the eyes or For example, to look to the le , the le eye mus
conjugate movements. For example, to look to the le , the (le lateral rectus controlled by le CN 6) and the
le eye must abduct (le lateral rectus cont rolled by le CN must adduct (right medial rectus controlled by righ
6) and the right eye must adduct (right medial rectus con- T e initial signal to intentionally move the eyes co
trolled by right CN 3). o achieve conjugate hor izontal gaze, the right rontal eye eld and crosses to connect w
there must be a communication between the CN 6 nucleus PPRF, which is adjacent to the le CN 6 nucleus.
on one side and the CN 3 nucleus on the other. T is com- PPRF signals the le CN 6 nucleus to activate the l
munication is the medial longitudinal asciculus (MLF), a rectus. T e le CN 6 nucleus simultaneously commun
tract th at connects each CN 6 nucleus with th e contralateral the contr alateral (right) CN 3 nucleus by way o the ri
CN 3 nu cleus. to signal the right CN 3 to activate the right medial r
T e MLF crosses rom the CN 6 nucleus en route to the lesion o the right rontal eye eld, the le PPRF
contralateral CN 3 nucleus almost immediately, spending CN 6 nucleus would, there ore, all lead to impaired le
most o its course contr alateral to its point o origin. For this both eyes. Both eyes are a ected because the problem
reason, the MLF is named or the side o the CN 3 nucleus gaze in a particular direction rather than a problem
with which it conn ects rather than the CN 6 nucleus rom individual nerve or muscle. In contrast, a lesion o
which it originates: T e lef MLF travels rom the right CN cens nerve (CN 6) itsel would preclude lateral mov
6 nucleus to the le CN 3 nucleus, and the right MLF travels that eye, but on attempted lateral gaze, the contrala
rom the le CN 6 nucleus to the right CN 3 nucleus. would still be able to add uct.
T e rontal eye elds do not communicate directly with the
cranial nerve nuclei but rather th rough hor izontal and vertical Conjugate Horizontal Gaze Abnormalities
gaze centers. T ese are the centers that commun icate with th e (Fig. 11 9 and Table 11 2)
cranial nerve nuclei, which in turn communicate with each A patient with a large middle cerebral artery (MCA) st
other to synchronize conjugate eye movements. T e horizon- a ects the rontal eye eld will have gaze deviation
tal gaze center is the paramedian pontine reticular orma- hemisphere o the stroke, which is away rom the si
tion(PPRF). T ere is a le PPRF in the le pons or le ward hemiparesis. For example, a large right MCA stroke c
gaze and a right PPRF in the right pon s or rightward gaze. le hemiparesis and right gaze deviation with inabilit
T e f ow o in ormation or horizontal gaze is rom rontal to the le . In contrast, patients with unilateral pon
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T is leaves only one horizontal moveme

tion o the eye contr alateral to the lesion on cont
gaze (because the contralateral PPRF and CN 6 nuc
spared). In the above example, out o all o the horiz
movements, only le eye abduction is preserved.
drome is called one-and-a-hal syndrome since thre
movements” are impaired, where each “hal ” is one d
A o horizont al gaze (le eye abduction + le eye add
right eye abduction + right eye adduction).
WEBINO (Fig. 11 –12 )—I a lesion a ects both ML

will be no addu ction o either eye on hor izontal gaze
direction (bilateral INO). In some such cases, the
abducted in primary gaze (exotropic), causing a “wa
appearance. T is constellation o ndin gs is called
bilateral INO, or WEBINO . As with INO, multiple
B
and stroke are the most common causes.
FIGURE 11 –11 One-and-a-hal syndrome in a patient with
multiple scle rosis. A:O n attemp ted right ga ze, neithe r eye moves. Vertical Gaze
B. On atte mpted le t ga ze, the le t eye abducts, but the right eye doe s
not add uct. This is consistent with a lesion in the right PPRF and/o r Vertical eye movements are controlled by two b
CN 6 nucle us as we ll as th e right MLF. Rep rod uce d with p erm ission nuclei, the rostral interstitial nucleus o the M
rom Martin T, Corbett J: Practical Neuroophthalmology. New York: interstitial nucleus o Cajal , both located in the dor
McGraw-Hill Edu cat ion; 2013. brain close to the CN 3 and CN 4 nuclei, as would be
(CN 6 does not participate in vertical gaze). Like th
stem horizontal gaze centers, these nuclei are under
and/ or CN 6 nucleus and the already-crossed MLF are a ected trol o the cortical eye elds.
together on one side, this has two consequences: Impaired conjugate vertical gaze can be caused b
o the dorsal midbrain or in the region o the
1. Ipsilateral conjugate gaze is impaired du e to the lesion o tricle (e.g., pineal pathology, tectal glioma, hydro
the PPRF and/or CN 6 nucleus. with expansion o the ourt h ventricle) and neurod
2. Adduction o the ipsilateral eye on contralateral gaze is tive diseases (e.g., progressive supranuclear palsy; see
impaired since the MLF that has crossed over rom th e Upgaze limitations m ay be seen in otherwise norm
contralateral CN 6 nucleus en route to the CN 3 nucleus patients. Pathology that compresses the dorsal midb
on the side o the lesion is unable to signal CN 3 to adduct. cause a constellation o ocular motor ndin gs
For example, a lesion o the right PPRF and/ or CN 6 Parinaud’s syndrome:
nucleus and the right MLF (i.e., the MLF that crossed over • Impaired u pgaze with downgaze pre erence
rom the le CN 6 nucleus en route to the right CN 3 nucleus) • Light-near dissociation o pupillary reactions (con
will cause: on accommodation but n ot in r esponse to light)
• Loss o conjugate horizontal gaze to the right (du e to the • Eyelid retraction (called Collier’s sign ), causing
lesion o the right PPRF and/ or right CN 6 nucleus) eyed appearan ce to the eyes
• Loss o right eye adduction on le gaze (due to the lesion o • Convergence-retraction nystagmus, in which the
the right MLF, which conn ects the le CN 6 nucleus to the pulled medially and retracted inward. T is can be
right CN 3 nucleus) out in upward gaze
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TABLE 11 –3 Summary o f ndings in conjugate and dysconjugate gaze abnormalities.

E ects o lesion Le t-sided lesion Right-sided lesion
Frontal eye f eld Conjugat e movement s away rom lesion Le t gaze de viat ion Right gaze de viation
impaired; deviation t oward lesion
PPRF Conjugate movements impaired toward Right gaze d eviation Le t gaze de viat ion
side o lesion; deviation awa y from lesion
Abducens Conjugate movements impaired toward No le t ga ze; righ t ga ze de viation No right gaze ; le t g aze de
Nucleus side o lesion; deviation awa y from lesion
Abducens Nerve Dysconjugat e with ailure to abd uct On le t gaze, right e ye adduct s, On right g aze, le t eye add
a ected eye le t eye does not abduct right eye doe s not abduct
MLF Internuclear ophthalmoplegia: On right g aze, right eye abd ucts, On le t gaze, le t e ye abdu
le t e ye does not addu ct; right right eye does not adduct
•##
Dysconjugate with failure to adduct one (abducting) eye o t en has right- (abducting) eye o ten has
o the eyes on contralateral gaze; all othe r be atin g nysta gmu s be atin g nysta gm us
horizontal movements preserved
• Convergence spared
PPRFor CN 6 One-and-a-hal syndrome: Cont ralateral No moveme nt o eith er eye on No moveme nt o eith er ey
nucleus a nd MLF abd uction is the only unct ioning horizontal attemp ted le t gaze; on right gaze, attemp ted right gaze;
movement right eye abdu cts, but le t eye does gaze, le t eye abd ucts, bu
not adduct eye does not adduct
Bilateral MLF Bilateral INO: No adduction in contralateral On attempt ed gaze in either direction, abducting eye abd ucts, but c
gaze in either eye tralateral eye does not ad duct. In some cases eyes may appear ab
bilate rally at b ase line (WEBINO).
CN: cranial nerve; MLF: me dial long itud inal asciculus; PPRF: pa rame dian po ntin e reticular orma tion
hypotropia is due to right superior rectus weakness, double A skew deviation can also cause vertical misalig
vision would worsen in upgaze. the eyes (see “Skew Deviation” above). Unlike unilate
T e superior and in erior recti and the in erior oblique palsy, skew deviation is usually comitant (although
muscles are all cont rolled by CN 3, so i vertical diplopia is incomitant), and when the ocular tilt reaction accom
due to a third nerve palsy, it is common to see multiple eye the mor e elevated eye is into rted . his is in contr ast
movement abnormalities in the a ected eye. I weakness can palsy, in which in torsion is impaired in the hypertr
be isolated to the superior and/ or in erior rectus in isolation, (because CN 4 palsy leads to impaired intorsion and i
extraocular muscle pathology (e.g., thyroid eye disease) or depression).
myasthenia gravis should be considered.
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C H A
T e Auditory and
Vestibular Pathways and
Approach to Hearing
Loss and Dizziness/
1
Vertigo
Cranial nerve 8
THE AUDITORY SYSTEM Cold Calo ric Te stin g

APPROACH TO HEARING LOSS Nystagmus
Distinguis hing Cond uctive Hearing Los s APPROACH TO DIZZINESS AND VERTIGO
From Senso rineural Hearing Los s Evaluatio n o Acute-Onset Cont inuou s
THE VESTIBULAR SYSTEM Dizziness/Vertigo
The Vest ibulo -Ocular Re ex (VOR) Evaluatio n o Chronic Dizziness
Oculoce phalic Re ex
Cranial nerve 8 (CN 8) contains two components: auditory rarely the entry zone o CN 8 at the pontom
(cochlear) and vestibular. Both begin in the inner ear and be ore arr iving at the cochlear nu clei). Centr
travel to the brainstem: the auditory component projects to brainstem or tempor al lobe) on ly rarely cause
the cochlear nuclei (at the pontomedullary junction) and the must be extensive and bilateral to do so. T
vestibular component projects to the vestibular nuclei (in the etiologies o dea ness are usually associated w
medulla). due to involvement o neighboring structu
lobe lesions can lead to de cits in word process
dea ness) and right temporal lobe lesions can c
THE AUDITORY SYSTEM (FIG. 1 2–1) music processing (amusia).
Hearing loss due to a peripheral lesion is
Auditory in ormation travels rom the inner ear through the
tive hearing loss i it caused by problems in the
auditor y (cochlear) portion o CN 8 to arrive at the cochlear
dle ear, and called sensorineural hearing loss
nuclei at the pontom edullary junction. T e cochlear nu clei
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• Bilateral
Cerebral • Aging (presbyacusis)
cortex
• Ototoxic medications (e.g., aminoglycosides)
• Sequela o meningitis (especially in children )
• Neur o bromatosis type II with bilateral
schwannomas (see “Neurocutaneous Syndromes” i
Primary
auditory • Susac’s syndrome (a syndrome causing branc
cortex artery occlusion, sensorin eural hearing loss, and
lopathy; see “Susac Syndrome” in Ch. 19)
• Super cial siderosis (which causes hearing loss
Medial
Midbrain
geniculate
panied by cerebellar dys unction and/ or uppe
diencephalon
junctu re nucleus neuron signs; see “Super cial Siderosis” in Ch. 1
Brachium of • Mitochondrial disorders (see “Mitochondrial Dis
inferior Ch. 31)
colliculus
Sudden-onset unilateral hearing loss can be ca
Inferior
in arction o the inner ear structur es due to ischem
Midbrain colliculus
territor y o the intern al auditor y artery (also called
rinthine artery), which is usually a branch o the AI
diagnosis should be strongly considered in patient
acute-onset unilateral hearing loss is accompanied b
Nuc leu s of or other brainstem or cerebellar symptoms/signs.
lateral
lemniscus
ing loss is episodic with a sense o ear ullness and
Pons
Ménière’s disease should be considered.
Disting uishing Conductive Hearing L

Lateral From Sensorineural Hearing Loss
lemniscus
T e unction o the eardrum and middle ear
Pons ampli y sound waves or tran smission into n eural i
Trapezoid In conductive hearing loss, CN 8 works properly, b
bod y
is not able to be transmitted to it by the outer/middl
sensorineural hearing loss, the pathway or auditor
Superior tion is not unctioning properly. T e Weber and
Cochlear nuclei: olivary can help to distinguish between conductive and senso
Dorsal complex hearing loss ( able 12–1).
Posteroventral
Anteroventral
Webe r’s Test
Medulla
In Weber’s test, a vibrating tuning ork is placed on t
the patient’s head. Normally, the pitch should b e hear
in both ears. I the pitch is louder on one side, this m
Cochlea r division either the opposite (so er) side is a ected by senso
of cranial nerve VIII
FIGURE 12 –1 The auditory pathway See text or expl ion.

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TABLE 17 –5 Patterns o We aknes s in Foo t Drop Due to Les ions o the Perone al Nerve, Sciatic Nerve, and
L5 Roo t. a
Pe ro ne al Ne rve -Su pp lie d Ac tio ns Tib ia l Ne rve -Su pp lie d Ac t
Dorsif exion Eversion Plantarf exion Inve
Common peroneal ne uropathy Weak Weak Spared Spar
Sciatic n europathy Weak Weak Weak Wea
L5 radiculop athy Weak Weak Spared Wea

a
Note t hat a sciatic neu ropa th y can pre eren tially a ect t he p ero nea l division, mimicking a com mo n pe rone al neu rop ath y (see t ext). Not e also th at t he d ivisions o
neal nerve may be a ected in isolation: Isolated supe rf cial peron eal neuropat hy causes eversion weakness without dorsi esion weakness; isolated deep pe roneal n
causes dorsi exion weakness without eversion weakness.
normal walking. Foot dorsi exion (tibialis anterior) weakness As described above, sciatic neuropathy may be c
can be caused by peroneal neuropathy, sciatic neuropathy, indistinguishable rom peroneal neuropathy, since
lumbosacral plexopathy, or L5 radiculopathy. Lumbosacral neal division o the sciatic nerve can be more susc
plexopathy will o en cause mor e widespread lower extrem- injury than the tibial division. In such cases, EMG
ity de cits, but oot drop may be the predomin ant mani es- tinguish between sciatic and peroneal etiologies by
tation o lesions at the other thr ee levels. Isolated common or denervation o the short head o the biceps
peroneal neuropathy causes oot drop and eversion weakness only muscle innervated by the peroneal nerve above
(loss o “up and out”), but does not a ect plantar exion or lar head (and the only hamstring muscle innervate
inversion (preserved “down and in”) since these are tibial peroneal division o the sciatic nerve). I there are
nerve–innervated unctions. Inversion is a tibial nerve–inn er- changes in the short head o the biceps emoris on E
vated unction supplied by L5, but plantar exion is a tibial patient who appears to have a peroneal neuropathy, t
nerve–innervated unction supplied by S1-S2. T ere ore, a izes to the peron eal division o the sciatic nerve pro
oot drop with loss o both eversion and inversion but with the bular head (the bular head is the more comm
spared plantar exion suggests L5 radiculopathy (a ecting peroneal nerve compression).
both tibial nerve–inn ervated and peroneal nerve–innervated During the period o recovery rom oot drop
muscles), whereas loss o dorsi exion, plantar exion, inver- oot drop not expected to recover), an ankle- oot or
sion, and eversion suggests sciatic neuropathy ( able 17–5). be used that maintain s the oot in a more neutr al po
In addition to weakness in various movements o the oot, L5 restore the natural position o the oot during wal
radiculopathy can also cause weakness o hip abduction.
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PART II DISEASES OF THE NERVOUS SYSTEM
Seizures and Epilepsy C H A P
1
DEFINITIONS AND CAUSES OF SEIZURES AND EPILEPSY Antiepilep tics and Drug Drug Inte ractions
EVALUATION OF PATIENTS WITH SEIZURES Side E ects and Toxicitie s o Antiepilep tics
CLINICAL FEATURES OF SEIZURES Antiepilep tic Drug Titration and Com binati
Gene ralized Se izures Re ractory (Drug-Resistant) Epilepsy
Focal (Partial) Seizures Tape ring O Antilep tic Drugs in Seizure-Fr
Distinguis hing Seizure From Synco pe SPECIAL SCENARIOS IN THE MANAGEMENT O
Distinguis hing Seizure From Transient Ische mic Attack AND EPILEPSY
Distinguis hing Seizure From Migraine With Aura Childhood-Onset Seizures
Distinguishing Seizures From Psychogenic Pediatric Febrile Seizures

Nonepileptic Spells (Pseudose izures) Seizu res in Patie nts With HIV
ELECTROENCEPHALOGRAPHY (EEG) IN THE EVALUATION Epilep sy and Preg nancy
OF SEIZURES Antiepilep tic Drug Prophylaxis or At-Risk P
EVALUATION AND MANAGEMENT OF PATIENTS AFTER A Who Have Not Had Seizures
FIRST SEIZURE STATUS EPILEPTICUS
Couns eling A t er a First Seizure Nonconvulsive Status Epilepticus
OUTPATIENT MANAGEMENT OF EPILEPSY
Dete rmining an Antiep ileptic Drug Reg imen or a
Patient With Epilep sy
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168 PART II Diseases o the Nervou s System
TABLE 18 –1 Causes o Provoked Seizures and Some causes o acute symptomatic (provoked)
Epilepsy. such as acute stroke or hemorrhage, head trauma, o
gitis can increase the risk or development o epilep
Causes o Acute Provoked Seizures Causes o Epilepsy
utur e since they can lead to irreversible brain damag
Acute brain patholog y: Structural brain lesions: ing an epileptogenic ocus.
•##
Acute stroke or intracranial •##
Intracranialtumor or vas-
hemorrhage cular malformation
• Acute head trauma •##

Cortical malformation
EVALUATION OF PATIENTS WITH
•##
Acute meningitis/encephalitis •##
Prior stroke or
intracranial hemorrhage SEIZURES
•##
Posterior reversible
encephalopathy syndrome (PRES) •##
Prior head trauma
A patient with seizure(s) will generally present or ev
(see Ch. 19)
•##
Prior meningitis/ in one o three scenarios:
•##
Cerebral venous sinus encephalitis
thrombosis/cortical vein 1. A er a rst seizure (or other type o spell)
thrombosis (see Ch. 19) •##
Neurod eg en erat ive
disease 2. With a history o seizures (or other type o spell
Metabolic derangements, 3. Actively seizing
including: Gene tic (idiopathic)
epilepsy syndromes When a patient presents or evaluation a er a
•##
Hypoglycemia or hyperglycemia (see Table 18–3)
any scenario, the goals o the clinical encounter are
•##
Hyponatremia mine the ollowing:
• Hypocalcemia • Was the event truly a seizure? T e di erential
• Hypomagnesemia or a tran sient alteration in neurologic unction
migraine, syncope, transient ischemic attack (
Medications, including:
arrhythmia, and psychogenic nonepileptic spell.
• Bupropion description o the event by witnesses should be o
• Tramadol T e clinical eatures that can be used to aid in dis
ing between th ese are discussed below.
• Fluoroquinolones
• Were there any clear provoking actors? A
• Cephalosporins
medication and drug/alcohol history should be o
• Carbapenems laborator y tests should evaluate or potent ial meta
• Isoniazid in ectious etiologies, and neuroimaging should be
ered. I the patient has been on an antiepileptic
Drugs /drug withdrawal
prior seizures, it should be determ ined whether t
• Alcohol is taking the medication(s) properly and cons
• Cocaine Even patients with known epilepsy should be e
or potential provoking actors that may have cau
Systemic illnes s
to have a seizure at that particular moment, suc
•##
Systemic infection with fever
in ection or a new medication that could lower the
• Renal failure threshold or alter the metabolism o their anti
medication(s).
recurren t seizures due to any o these causes are considered • Was this the f rst event or have there been others
unprovoked. A patient with a brain tumor (or an y o the have been other prior events, was the patient ever e
prior CNS insults listed above) who has a rst seizure has or these? I so, was the patient ever on an ant iepile
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CHAPTER 18 Seizures and Ep
or seizure. T e sensitivity o a single 20-minute routine EEG be hard to justi y since the likelihood o capt
recording to detect interictal epilepti orm discharges is only may be low. Ultimately, in patients whose ev
around 50%, although sensitivity can be increased by per- to classi y, in requent, and in whom the EEG
ormin g the EEG in the sleep-deprived state, per or ming EEG ambiguous), a decision must be made in coll
within 24 hours o a seizure event, or repeating EEG on mu l- the patient as to whether to attempt treatmen
tiple occasions. Notably, a small proportion o the population epileptic medication as a poten tial diagnostic a
may have EEG abnormalities o no clinical signi cance, and maneuver.
a number o medications can also cause EEG abnormalities.
T ere ore, the absence o epilepti orm discharges does not
“exclude” epilepsy and their pr esence does not “con rm” epi- EVALUATION AND MANAGEME
lepsy. Seizures and epilepsy are clinical diagnoses and EEG
PATIENTS AFTER A FIRST SEIZU
ndin gs must be interpreted in light o the clinical history. I a
patient has had paroxysmal events and t here is a strong clini- In a patient who presents a er a rst seizure,
cal suspicion that the patient has had seizures, a normal EEG be made to determin e whether the seizure
should not necessarily dissuade the clinician rom tr eating T is requires a care ul medication and dr ug h
these as seizures. Similarly, i the clinician has a strong suspi- tory evaluation (including electrolytes and toxi
cion th at th e events ar e not seizures (e.g., syncope, migraine), and neuroimaging (MRI with contrast pre err
an epilepti orm ndin g on routine EEG should not necessar- or venous imaging may be considered depend
ily dissuade the clinician rom that impression. context). Lumbar puncture should be conside
What remains uncertain is how much the clinician concern or CNS in ection or inf ammator y
should weight the routine EEG in a patient or whom the his- protocol MRI generally includes coronal view
tory o the events is di cult to interpret/classi y. For exam- campi to look or asymmetries in size or signal
ple, i an otherwise healthy p atient presents with recurr ent, on 2/FLAIR (f uid-attenuated inversion recov
discrete, clinically ambiguou s episod es (e.g., “ eeling oggy” that may suggest an underlying ocus or tem
or a ew minu tes every ew mon ths), a nor mal EEG does not lepsy (mesial temporal sclerosis ) (Fig. 18–1
exclude the possibility that these are seizures, and an abnor- noted that seizures can produce transient M
mal interictal EEG does not con rm that they are seizures. I ties, most commonly di usion restriction (o
the events are requent enough, the gold standard is to cap- sequences) and/ or 2/FLAIR hyperintensity
ture events dur ing inpatient video EEG monitor ing. An inter- splenium, and/or thalamus (Fig. 18–2). Di
mediate step is at-home ambulator y EEG or 24-48 hours. on MRI can also be caused by acute stroke (
However, even when extended EEG monitoring captures a Weighted Imaging and Apparent Di u sion
spell, deep seizure oci can be missed with sur ace recordings. sequences” in Chapter 2). T e pattern o di
I the events are rare, a ew days o inpat ient monitor ing may due to seizure can be distinguished rom stro
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TABLE 18 –2 Characte ristics o Commo nly Use d Antie pile ptic Drug s (AEDs).
Most Common Unique Adverse Drug Drug IV Formulation
Indication, I Spe cif c E ects a Interactions Available Othe r
Carbamaze pine (CBZ) Focal seizures Hyponatremia Inducer (decreases Used for trigem
levels of other neuralgia
medications)
Ethosuximide (ESX) Absence seizures
Gabapentin (GBP) Used for neuro

pa in
Lacosamide (LCM) Cardiac Yes
Lamotrig ine (LTG) LTG levels Used as mood

decreased by oral stabilizer
contraceptives
Safest in pre
Levet iracetam (LEV) Psychiatric Yes
Oxcarbaze pine (OXC) Focal seizures Hyponatremia
Phenobarbital (PB) Hepatic Inducer (decreases Yes

levels of other
medications)
Phenyt oin (PHT) Inducer (decreases Yes

levels of other
medications)
Topiram ate (TPM) Neph rolithiasis Used for migra
Weight loss
Valproate (VPA) Idiopathic genetic Weight gain Inhibitor (increases Yes Used for migra
epilepsy syndromes levels of other mood stabiliz
Tremor medications)
Most teratogenic
Zon isamide (ZNS) Nephrolithiasis

a
Serious adverse a ects such as hepatotoxicity, bo ne marrow toxicity, teratogenicity, and Stevens-Johnson syndrome can occur with nearly all AEDS, as can less
a ects such as dizziness, nystagmus, mental cloudiness.
medical evaluation shou ld a rash develop. Explaining to par- In addition to potential toxicities, some AEDs ha
ents o children initiating an AED to report the development tional properties that may inf uence their use. Val
o any rash is particularly important since children requently topiramate are e ective or migraine prophylaxis, and
develop rashes o various sorts. T e rash o Stevens-Johnson considered in patients with both migraine and epilep
can begin quite innocuously and might not raise concern proate can cause weight gain and topiramate can ca
unless parents are instructed to watch or it. loss, so the latter might be pre erred in an obese patie
Carbamazepine and oxcarbazepine can cause hypona- proate has th e highest r ate o etal mal ormations an
tremia, so serum sodium should be ollowed in patients on be avoided in women o child-bearing age. Val
these AEDs. Lacosamide can cause PR interval prolongation, lamotrigine both have mood-stabilizing properties,
and so a baseline ECG should be obtained be ore initiating the levetiracetam can cause irritability and depression, so
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TABLE 18 –3 Pediatric Epileps y Syndrome s.

Ag e o On se t Se iz ure Ty pe Ot he r Clin ica l Fe at ure s EEG Fe at ure s Tre at me nt
Ohtahara 0–3 months Tonic spasms Often fatal be fore age 1 Burst suppression Often refractory
syndrome
Various
Dravet syndrome 0–1 ye ars Va rio us, often Developmental May be normal Keto gen ic diet
(Severe prolon ge d regression initially;later
myoclonic epilepsy slowing & Often refractory
o in ancy) SCN1Amutation epileptiform
(sodium channel) discharges
West syndrome 0–1 ye ars In fa nt ile spasms Developmental Hyp sa rrh yt hm ia ACTH
delay/mental
retardation Vigabatrin
Often secondary to
underlying cause
Doose s yndrome 1–5 years Myoclonic-astatic Mental development May be normal AEDs
(Myoclonic astatic (drop attacks) may be normal or initially;later
epilepsy) impaired spike/wave & Keto gen ic diet
Various slowing
Len nox-Gastau t 3–10 years GTC Mental re tardation Slow spike/wave Valproic acid
syndrome (Peak: (2.0–2.5 Hz)
3–5 years) Atonic (drop attacks
Atypical absence
Landau-Kle ner 2–10 years Any type (some Pure word deafness Tem po ral/ AEDs
syndrome (Peak: pa tien ts do not have with pre serve d hearing; temporoparietal
5–7 years) seizures) other prog ressive spikes Steroids
language de cits Surgery
Spike/wave
during sleep
Rasmussen 1–15 years Focal motor/epilep- Progressive unilateral Focal epileptiform Immunomodulato
encephalitis sia pa rtialis continua hemisphere atrophy discharges therapy
with contralateral
hemiparesis Hemispherectomy
Associated with
antibody to GluR3 (glu-
tamate receptor)
Panayiotopoulos 1–15 ye ars Eye deviation, Normal development; Occipitalspikes AEDs

syndrome vomiting, autonomic usually remits
features (sweating, Usually remits
pa llor, salivation) teenage years
Benign Rolandic 2–13 ye ars No ct urn al focal Usually spontaneously Centrotemporal Spontaneously
epilepsy (Benign motor seizures of remits spikes (although can
epilepsy with mouth with with AEDs until
centrotemporal drooling, aphasia
spikes [BECTS])
Childhood absence 3–10 ye ars Brie f a bsence May be triggered by 3 Hz spike/wave Ethosuximide
epilepsy seizures hyperventilation during seizures
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C H A
Vascular Diseases of the
Brain and Spinal Cord
1
OVERVIEW OF ISCHEMIC STROKE AND INTRACEREBRAL CENTRAL NERVOUS SYSTEM VASCULAR MALFO
HEMORRHAGE SUBARACHNOID HEMORRHAGE
ISCHEMIC STROKE Aneu rysmal Suba rachnoid Hemorrhag e
Transie nt Ische mic Attack Perimesencephalic Subarachnoid Hemorrh
Etiolo gy o Ische mic Stroke Unruptured Intracranial Aneurysms
Initial Evaluatio n o a Patient With Acute Ischem ic Stroke INTRAVENTRICULAR HEMORRHAGE
Initial Treat ment o Acute Ischemic Stroke SUBDURAL HEMATOMA
Evaluatio n or Etiolo gy o Ische mic Stroke EPIDURAL HEMATOMA
Second ary Preve ntio n o Ischem ic Stroke CEREBRAL VENOUS SINUS THROMBOSIS AND
Rarer Cause s o Ischem ic Stroke : Vasculo pathie s, VEIN THROMBOSIS
Vasculitis, and Gene tic Disorders OTHER CEREBROVASCULAR DISORDERS
Long -term Seq uelae o Ische mic Stroke: Recrudes- Poste rior Reve rsible Encephalo pathy Syndr
cence , Seizures , and Cog nitive Impairmen t
Reversible Cerebral Vasoconstriction Syndro
INTRACEREBRAL HEMORRHAGE
Super cial Siderosis
Acute Manag eme nt o Intracereb ral Hemo rrhag e
VASCULAR DISEASE OF THE SPINAL CORD
Etiolo gie s o Intracerebral Hemo rrhag e
Ischem ic Stroke o the Spinal Cord
Resuming Anticoagulation A ter Anticoagulation-
Spinal Hemorrhag e
Asso ciated Intracerebral Hemo rrhage
Spinal Dural Arteriove no us Fistula
T e brain and spinal cord can be a ected by a variety o condi- OVERVIEW OF ISCHEMIC STRO
tions related to the vascular system:
AND INTRACEREBRAL
• Ischemic stroke: lack o blood ow to a portion o the brain
HEMORRHAGE
(or more rarely the spinal cord)
• Intr acranial or spinal hemorrhage at ve possible sites: T e t er m stroke re ers to the clinical scenar
• Epidural hematoma: between the skull or spine and dura patient is “struck” by a sudden-on set neu
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182 PAR II Diseases o the Nervou s System
A B
FIGURE 19 –1 Inte rnal carotid artery diss ection . A: CTangiogram of the neck in sagittal view demonstrating “ ame-shaped”
of internal carotid arte ry dissection ( arrow). B: T1-weighted axial MRI with fat saturation demonstrating “crescent”appearance of intram
hem atoma in left internal carotid artery dissection ( arrow ).
artery dissection, lower cranial nerve palsies (cranial nerves 9–12) • Reversible cerebral vasoconstriction syndrome
and/or Horn er’s syndrome (in the case o internal carotid dissec- which can cause stroke or hemorrhage (most co
tion only ptosis and miosis will be seen, but no anhidrosis because subarachnoid hemorrhage when hemorrhage occu
sweating bers travel with the external carotid; see “Impaired • Moyamoya (which can be primary or secondary)
Pupillary Dilatation” in Chapter 10). T e risk o stroke is high- • Cerebral autosomal dominant arteriopathy with su
est in the rst week a er dissection, and some patients may have in arcts and leukoencephalopathy (CADASIL) and
multiple IAs or strokes during this period. A dissected vessel autosomal recessive arteriopathy with subcortical
has a ame-shaped appearance on C A (Fig. 19–1A), and a cres- and leukoencephalopathy (CARASIL)
centic intramural hematoma can be visualized on 1-weighted
• Vasculitis: blood vessel in ammation that m ay be
at saturation MRI (Fig. 19–1B). Secondary stroke prevention in
or secondary (e.g., secondary to in ection or to a s
patients with IA or stroke due to cervical artery dissection is
vasculitic syndrome)
discussed below (see “Secondary Stroke Prevention in Patients
With Cervical Artery Dissection”). T ese and oth er vasculopathies are discussed be
“Rarer Causes o Ischemic Stroke: Vasculopathies, V
Va sospa sm a s a cause of ischem ic stroke—Vasospasm can and Genetic Disorders”)
be caused by:
Cardiac Causes o Ische mic Stroke
• Local irritation o the blood vessels by subarachn oid hem- Cardiac causes o stroke include:
orrhage or meningitis
• Atrial brillation: clot ormation due to stasis
• Failure o cerebral autoregulation, which can be seen in
atrium (especially the le atrial appendage) leads
posterior reversible encephalopathy syndrome (PRES; see
embolism
“Posterior Reversible Encephalopathy Syndrome” below)
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CHAP ER 19 Vascular Diseases o the Brain and Spinal
A B
FIGURE 19 –2 Neuroimaging in hypo xic-ischemic injury cause d by cardiac arrest . A: Axial CTdemonstrating di use
and symmetric hypod ensity of the basal gan glia. B: Axial DWI MRI demonstrating di use cortical and bilatera l ba sal ganglia di u
• Cardiac tumors on which thrombus may orm (e.g., bro- the rst time may mimic stroke, especially be
elastoma, atrial m yxoma, metastasis) headache emerges. Although acute metabolic
• Patent oramen ovale, which can serve as a conduit or o en present with global neurologic de cits
thrombus ormed in the venous circulation to nd its way ones, ocal ndin gs can occur in the setting
to the arterial system and cause stroke. rauma and intoxications are generally appa
• Cardiac arrest with hypoxic-ischemic injury. T e gray mat- history and examination, but may require colla
ter is most sensitive to hypoxia, so hypoxic-ischemic injury tion an d toxicology screening (especially i
can cause di use in arction o the cortex and/ or basal gan- ply “ oun d down”). I a patient presents with
glia (Fig. 19–2). and ace tingling, evaluation or myocardial
be und ertaken, since chest pain may not be a p
Hema tolo g ic Cause s o Acute Ische mic Stroke ture o cardiac ischemia in elderly patients or
Problems with the blood itsel can also lead to stroke: impaired pain perception due to diabetic neuro
For any acute-on set neurologic de cit,
• Hypercoagulable states, which may be inherited (e.g., actor vital signs is essential, and ECG, blood sugar,
V Leiden mutation, prothrombin gene mutation, protein C tries, complete blood count, and coagulation pr
de ciency, protein S de ciency, antithrombin III de ciency) obtained while clinical evaluation is undertake
or acquired (e.g., antiphospholipid antibodies, hypercoagula- In pr actice, when acut e stroke is suspecte
bility o malignancy, disseminated intravascular coagulation) examination are o ten per ormed en route
• Sickle cell anem ia since the use o throm bolytic treatment or a
• Hyperviscosity, which can be caused by polycythemia vera stroke (IV tPA) requires rapid con rmation o
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Neuroimag ing Findings in Acute Ischemic

T e C scan may show no abnormalities in the acut
o acute ischemic stroke since the C hypoden sity
ischemic stroke can t ake up to 12 hou rs to emerge.
cases, however, subtle nd ings related to vessel
or early ischemia may be seen on non contr ast
acute setting: a hyperdense vessel (a sign o clot and
ow in the vessel) (Fig. 19–3), blurring o the gra
jun ction /su lcal e acement (Fig. 19–3), and , in mid
bral artey (MCA) str oke, hypo den sity o the insu
(Fig. 19–4A). Early parench ymal hypodensity m
easily visible when changing the window setting t
(Fig. 19–4B). I the clinical impression is that the patien
ing an ischemic stroke, the C scan does not reveal
tive explanation or the patient’s symptoms, and the
onset o symptom s is well established with the patien
presented within the 3-hou r (or in some cases 4.5-ho
dow, the patient can be considered or thrombolytic
i there are not contraindications (see “T rombolys
reatment o Acute Ischemic Stroke” below).
MRI with di usion weighted imaging (DWI) an
ent di usion coef cient (ADC) sequences can dem
ischemic stroke within an h our a er onset, and so MR
more sensitive than C in the acute setting. Acute
strokes appear bright on DWI and dark on ADC (Fig
C A can be per ormed to look or arterial occlusion
be apparent be ore signs o tissue ischemia are visi
(Fig. 19–6). However, these studies take longer and
FIGURE 19 –3 Early signs o ischemia on no ncontrast CT be accessible acutely, and neither is required or th
imaging I: hyperdense vesse l and sulcal e acement. Axial non- tration o IV tPA in the appropriate clinical setting.
contrast CTdemonstrating hyperdense left MCA (arrow ) and di use MRI may be per ormed acutely to determine whether
sulcal e acement with loss of gray–white di erentiation in the left is a candidate or intra-arterial intervention (see “T
MCAterritory. sis in the reatment o Acute Ischemic Stroke”).
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A B
FIGURE 19 –5 Di usion we ighte d imaging (DWI) and apparent di usion coe cient (ADC) MRI in acute ischemic stro
(A) and ADC(B) demonstrate di usion restriction in th e territory of the left MCA.
Ischemic stro kes do not become visible on luid- than 6 hours rom onset. ADC darkness is
attenuated inversion inversion recovery (FLAIR) imaging or several days be ore norm alizing, althou gh D
unt il about 6 hours rom onset, so a stroke visible on di u- may persist or approximately 7–10 days.
sion sequ ences that is not yet visible on FLAIR is generally less Although DWI/ADC sequences are belie
gold standard in stroke diagnosis, it should be
negatives do occur in th e rst 24 hours, especia
that are very small and/ or in the posterior ossa
Subacute strokes (about 1 week to 1 mon
onstrate enhancement on postcontrast C or M
T is radiographic appearance may be mistake
there is no clear clinical history o stroke, but s
can be radiologically distinguished rom tu
ways: subacute strokes typically con orm to a
tory, and usually demonstrate no or minima
edema or mass e ect on surroun ding structur
seen with tumors. In ambiguous cases, serial i
be per ormed to see i the lesion expands as wou
with tumor, or develops volume loss (enceph
would be expected with in arction.
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Susac Syndrome Cerebral Auto so mal Domina nt Arte r

Susac syndrome is an autoimmune vasculopathy character- Subcortical In arcts and Leuko ence pha
ized by the triad o encephalopath y, bran ch retinal artery (CADASIL) and Cerebral Auto so mal R
occlusion, and sensorineural hearing loss. Lesions in the Arte riop athy With Subco rtical In arcts
central corpus callosum are characteristic, but additional Leuko ence phalo pathy (CARASIL)
non speci c white matter lesions are also comm on and may Cerebral autosomal dominant arteriopathy w
lead to misdiagnosis o multiple sclerosis. CSF is typically in arcts and leukoencephalopathy (CADAS
in ammat ory. Patients may not pr esent with all three ele- CNS vasculopathy that causes migraines, stro
ment s o the triad, but will most o en develop them within gressive neuropsychiatric dys unction leading
mon ths o onset. In patients in whom th e diagnosis is sus- MRI in CADASIL demonstrates a subcortical
pected but not all aspects o the triad ar e clinically presen t, alopathy (con uent 2/FLAIR hyperinten
laboratory testing (i.e., MRI, uorescein angiogram, audi- matter) with characteristic extension into the w
ometry) may reveal subclinical evidence o other elements the anterior temporal lobes (Fig. 19–8) (this r
o the triad. be similarly a ected in myotonic dystrophy;
Anti-endothelial cell antibodies have been described in Dystrophy” in Chapter 30). Diagnosis o CAD
association with the disorder. reatment is with immunomod- genetic testing (mutation o the NOTCH3
ulatory therapy. T e disease may be monophasic or relapsing. some 19) or skin biopsy to evaluate blood ves
T ere is no de nitively proven stroke-preve
Intravascula r Lymphoma although any coexisting vascular risk actors
Intr avascular large B-cell lymphoma is a rar e lymphoma th at be well controlled, and many patients are gi
develops within the lumens o small and medium blood ves- A recessive and much less common
sels. It is a multisystem disorder, but th e presenting eatures der (CARASIL) is caused by mutation in the H
may be neurologic due to strokes caused by lymphomatous chromosome 10. It is similar in clinical and r
occlusion o cerebral blood vessels. T ese are most commonly sentation to CADASIL, but a ected patients ad
small vessel subcortical strokes. Strokes may be clinically prematu re alopecia and lumbar spondylosis.
apparent as acute-onset de cits, or the accumulation o small
subclinical subcortical strokes may lead to a presentation with Mito chondrial Encephalop athy With L
subacute cognitive decline. Any level o the n ervous system Acidosis and Strokelike Episodes (MEL
may be involved, including myelopathy and neuropathy con- Patients with mitochon drial encephalopathy wi
current with or independ ent o brain involvement. Diagno- sis and stroke-like episodes (MELAS) may pre
sis o en requires biopsy o a ected nervous system tissue, sient neurologic stroke-like episodes, generally
although biopsy o skin lesions (i there is skin involvement) On neuroimaging, the stroke-like episodes in
or random skin biopsy may be diagnostic in some cases. signal changes that are typically isolated to
reatment is with combination chemotherapy including anti– do not con orm to individual vascular territori
B-cell therapy with r ituximab. tion o cerebral damage due to stroke-like e
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encephalopathy. Migraines and seizures are common. Patients thrombosis or cortical vein thrombosis), but are mo
typically have other eatures o mitochondrial disease including mon in the setting o acute intracerebral hemorrhage
short stature, dea ness, myopat hy, and lactic acidosis.
MELAS is associated with mitochondrial mutation A3243G. Post-stroke Cog nitive Impairment
Acute strokelike episodes may resolve with administration o Cerebrovascular disease can a ect cognition due to
L-arginine, and prophylactic administration may reduce the re- in regions such as the hippocampus, thalamus, o
quency o stroke-like episodes (Koga et al., 2005). lobe(s), and/or can cause progressive cognitive imp
due to accumulation o chronic subcortical ischemi
Long -term Sequelae o Ische mic Stroke: (see “Vascular Demen tia” in Chapter 23).
Recrudesce nce, Seizures , and Cognitive
Impairment INTRACEREBRAL HEMORRHAGE
Recrudescence
Intracerebral hemorrhage (ICH) presents with sudd
Patients who have had a prior stroke may present with reemer-
ocal neurologic de cits, as does acute ischemic strok
gence o resolved de cits or worsening o baseline de cits in
pared to ischemic stroke, however ICH is more o
the setting o in ection or oth er systemic illness, a phenome-
panied by one or more o the ollowing clinica
non kn own as recrudescence.A ny patient with a prior stroke
(Runchey and McGee, 2010):
is at risk or anoth er, and so recurrent stroke is the primary
di erential diagnosis in this setting. However, it would be • Headache
somewhat unlikely to have a stroke in the exact same place • Nausea/vomiting
with the exact same de cits as a prior stroke, so when patient s • Depressed level o consciousness at onset (due to
present with worsening o prior de cits, they should be evalu- ment o brain tissue by the hematoma and/or intrav
ated or an in ection or metabolic abnormality that could be a lar extension o hemorrhage)
cause o recrudescence.
• Extreme hypertension (d iastolic pressure >110 mm
Post-stroke Seizures • Seizures at pr esentation
Prior stroke is a common cause o epilepsy in older adu lts. Sei- Any o these ndin gs can also occur in the s
zures generally emerge about 6 months to 1 year a er in arct ischemic stroke, and so de nitive diagnosis requires
and require treatment with antiepileptic medications to prevent C , acute blood is hyperdense and visible at pres
recurrence. An acute precipitant o seizures should be sought (Fig. 19–9); this is in contrast to acute ischemic s
(e.g., in ection, electrolyte abnormality, new medication; see which C scan may be normal at presentation (se
able 18–1), though may not be present. Seizures at the time imaging in Acute Ischemic Stro ke” above). Vascular
o presentation o an acute ischemic stroke are uncomm on (e.g., C A or MRA) should be per o rmed to assess
(though more common i the stroke is due to venous sinus cular mal ormation that may require surgical inter
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CHAPTER 21 Demyelinatin g Diseases o the Central Nervous S
A B
FIGURE 21 –4 MRI in acute disse minate d e nce phalo mye litis (ADEM). A:A xial FLAIR image showing multiple large hype
the periventricular white m atte r. Note that the lesions in ADEM ten d t o b e larger t han those seen in MS (see Fig. 21–1). B: Axi
T1-weight ed image d emon strating that t he lesions in A exhibit incomplete (open) rings o enh ancem ent .
OPTIC NEURITIS Optic neuritis may be the rst “attack”

15 years a er an episode o optic neuritis,
Optic neuritis is in ammat ion o the optic nerve. It pres- hal o patients will develop MS. T e risk
ents with pain ul visual loss over days. It is most common ly ulated by whether or not there are MRI lesio
unilateral, although it can occur bilaterally. T e pain is typi- o MS at the time o optic neuritis. Patients w
cally worse when the pat ient mo ves the a ected eye. On lesions have a 15-year risk o 25% o developin
examination, decreased acuity, decreased color vision, and patien ts with one or more MRI lesions have a
an a erent pu pillary de ect may be observed (see “Impaired 72% o developing MS (Opt ic Neuritis Stud
Pupillary Constr iction Due to a Lesion o Cranial Nerve 2” A patient with isolated optic n euritis who does
in Chap ter 10). Although an in amed optic nerve head on evidence o dissemination in space and t ime is
und oscopy con rms the diagnosis in the appropriate clini- a clinically isolated synd rom e, the man ageme
cal context, in ammation o the optic nerve may be retrobul- discussed above.
bar with no visible abnormality o the optic nerve head itsel . I optic neuritis occurs sequentially in o
Optic nerve enhancement can o en be seen on contrast- and then th e other within a short period o time
enhanced MRI o the orbit. be considered.
T e time course o optic nerve dys unction is key to
the di erential diagnosis: A more insidious progression o
monocular visual loss may suggest a mass lesion (e.g., optic
nerve glioma, optic nerve sheath meningioma), whereas a
TRANSVERSE MYELITIS
more acute pr esentation may suggest a vascular etiology (e.g., ransverse myelitis ( M) re ers to in amm
ischemic optic neuropathy) (see “Monocular Visual Loss” in nal cord. Like optic neuritis, M can occur
Chapter 6).
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TABLE 22–1 Clinical Features o Dement ia Syndromes.

Fronto temp oral Demen tia (FTD)
Alzheimer’s Disease Demen tia With Lewy Behavioral Variant FTD Primary Progressive
(AD) Bodies (DLB) (bvFTD) Aphasia (PPA) Vascul
Most common initial cognitive de cits Memory Visuospatial dys unction Behavior/personality change Language de cits Executi
Executive dys un ction Cognitive s
Additional eatures Visuospatial Parkinsonism Executive dys unction May hav

dys unction
Hallucinations
Executive dys un ction
Fluctuations
Word- nding
dif culties
Age o onset > 60 > 60 50s–60s > 60
Locations o atrophy Medial temporal Va ria ble , m ay b e m in im al Fro nt ot em po ra l La ng ua ge a re as Sit es o
Parietal
Locations o hypometabo lism/ Tempo roparietal Occipital Frontotemporal Language areas N/A
hypope r usion on nuclear imaging
Posterior cingulate Tempo roparietal
Precuneus
Cerebrospinal f uid ndings A!42' Tau ( N/A N/A N/A N/A
Pathology/Proteinopathy A!42 plaques and tau Alpha-synuclein Tau

neuro brillary tang les
TDP-43
FUS
Alzheimer path ology in logope nic variant PPA
Respo nse to cho linesterase inhibitors Yes Yes No Yes

and memantine
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254 PART II Diseases o th

thee Nervou s Sy
System
stem
Faciob rachial Dysto nic Seizures ataxia, and/ or chorea. T e class

classic
ic tremor o Wilson’
a wing-beating tremor elicited by having the patien
An importan t di erential diagnosis
diagnosis to consider
consider in patients
thee arm
th armss elevated with elbows
elbows exed (like wings).
with hemi acial spasm is aciobrachial dystonic seizures.
and psychiatric symptoms are common. Seizures ma
T es
esee are vevery
ry brie epis
episodes
odes in
in which
which there is momentary
momentary
A pathogn
pathognomon
omon ic ocular ndnding,
ing, the Kayser-Fleische
dystonic postur
posturing
ing o one side side o the ace and the ipsila
ipsilateral
teral
dark rin g encircling
encircling the periphery o the iris), is almo
arm, which may occuroccur many
m any times per day (or even
even per hour)
ho ur)
present in patients with neur ologic mani estations o
a er onset. T is entity
entity is important to recognize
recognize as it is ass
asso-
o-
ease.. T e diagnosis
ease diagnosis can usually
usually be made by decreas
ciated with anti-LGI1 (leucine-rich, glioma inactivated 1)
ceruloplasmin and increased urinary copper on 24
antibodies.
antibod ies. Faci
Faciobrachial
obrachial dystonic seizures
seizures o en precede the
collection, but liver biopsy may be necessary i th
developmen
devel opmentt o limbic encephalitis
encephalitis associated
associated with
with an ti-LG
ti-LGI1I1
inconclusive. reatment with with copper
copper chelation
chelation (D-p
antibodies,
antibod ies, and development o limbic encephalitis may be
mine or trientin
t rientine)
e) can be highly e ectiv
ective.
e. In cases
cases r
prevented by early institution
in stitution o steroids upon recognition
to such treatments, liver transplantation may be cons
o aciobrachial dystondyston ic seizures (Iran i et al.,
al., 2011; Iran i
et al.,
al., 201
2013).
3). T is is one o the autoimmune
autoimmun e encephalitide
encephalitidess that
is most commonly not associated with an underlying malig-
nancy,
nan cy, although it m ay rarely be associ
associated
ated with thymoma
th ymoma or REFERENCES
small cell
cell lun
lungg cancer (see “Paraneoplastic
“Paraneoplastic Syndrom
Syndrom es o the Conn olly BS, La Lang
ng AE. Pharm acologi
acological
cal treatmen t o Park
Nervous Syste
System”
m” in Ch. 24). disease: a review. JAMA 2014;311:1670-1683.
Irani SR, Michell AW, Lang B, Pettingill P, Waters P, Johns
Wilson
ilson’’s Di
Dise
se ase et al. Faciobrachial dystonic seizures pr ecede Lgi1
Lgi1 antib
bic encephalitis
encephalitis Ann
Ann Neurol 2011;
2011;69:
69:892-900.
892-900.
Wilson ’s disease is an autosomal
Wilson’ auto somal recessive disease that a ects Iran i SR,
SR, Stagg CJ,
CJ, Schott JM, Rosenthal
Rosenth al CR, Schn
Schneider
eider SA, Pe
the liver and brain, presenting in childhood or early adult- et al.
al. Faciobrachial
Faciobrachial dystonic seizures:
seizures: the in uence o imm
hood. Many o the neurologic mani estations are movement therapy
therap y on seizure
seizure contr
contr ol and prevention o cognitive
disorders, which can include parkinsonism, tremor, dystonia, in a broadening
bro adening pheno
phenotype.
type. Brain
Brain 2013:
2013:136;
136;3151-316
3151-316
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C H A
Neoplast
eop lastic
ic and
an d
Paraneoplastic
Disordeer s of
Disord of t h e Ne
System and Neurologic
Ner vous 2
Complic
Com plication
ationss of
Chem
Che m oth
otheer apy and
Radi
diaati
tioon T era rapy
py
INTRACRAN
INTRACRANIAL
IAL TUMORS NEUROCUTANEOUS SYNDROMES AND ASSOC
Brain
Brain Metastas es NERVOUS SYSTEM NEOPLASMS
Lepto meninge al Metastase s (C

(Car
arcinomatous
cinomatous NEUROTOXICITY OF CHEMOTHERAPY AND RA
Meningitis) THERAPY
Primary Intracranial Tumors Neuroto

Neuroto xici
xicity
ty o Chemo therapy
TUMORS OF THE SPINE Neuroto xicity o Radiatio n Therapy
TUMORS OF THE CRANIAL NERVES PARANEOPLASTIC SYNDROMES OF THE NERVO
Numb Chin
Chin Sig n Autoimmune Limbic Enceph
Enceph alitis
Primary
Primary Cranial
Cranial Nerve Tumo rs: Schwan no mas Opsoclonus Myoclonus/Opsoclonus Myoclo
TUMORS OF THE PERIPHERAL NERVOUS SYSTEM CEREBROVASCULAR COMPLICATIONS OF CAN
T e nervous sys
system
tem can be a ected in
in several
several ways
ways in patients e ects o sys
systemic
temic malignan
malignancy
cy on the
th e nervou
with neoplastic disease: also be the presenting eature o a systemic
systemic can
• Directly due to pr imary nervous system
system tum ors or metasta-
ses to nervous system structures
INTRACRANIAL
INTRACRANIAL TUMORS
TUMO RS
• Indirectly due to:
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thee Nervou s Sy
System
stem
Particular tumors to consider in particular clinical sce- carcinoma, and choriocarcinoma have the highest p
narios include: or intratumoral hemorrhage. However, given th
cancer is the most common cause o cerebral meta
• Hearing loss, tinnitus, imbalance: cerebellopontine angle
is the most common cause o hemor hemorrhagic
rhagic brain met
tumor (most commonly vestibular schwannoma or
Gastrointestinal cancer metastases
metastases have a predile
meningioma)
the posterior ossa. Prostate cancer only very very rarely
• Bitemporal hemianopia: pituitary tumors, craniopharyngioma
sizess to the brain, but can m etastasi
size etastasize
ze to the skull
• Unilateral visual disturbance: optic glioma, optic nerve mater, causing neurologic symptoms by impinging
sheath meningioma, o r ol actory groove m eningioma brain and /or cran ial nerevs.
(ol actory groove meningioma may also cause unilateral T e evevalua
aluation
tion o a singl
singlee brain lesi
lesion
on suspi
loss o smell) neoplasm requires a search
search or systemic
systemic malignan
malignan
• Multiple cranial neuropathies: skull base lesion, brainstem ing imaging o the chest,
chest, abdomen
abdomen,, and pelvis
pelvis with
lesion,
lesion, or leptomenin
leptomeningeal
geal metastases
metastases or PE . Howeve
However,r, even
even i a systemic
systemic malignancy
In general,
general, steroids are o en used or the treatment o isolated CNS lesion could still be an independent
peritum oral vasogenic
vasogenic edema
edema in patients whose brain tum ors brain tu mor r ather th an a metastasis, so brain tissue
are symptom
symptom atic (e.g.,
(e.g., headache and/ or ocal de cits) as as a ultimately be required or diagnosis.
diagnosis.
result o the location
location and sizesize o the mass and its surro
surround
und - reatmentt o brain metastases involve
reatmen involvess surgical
ing edema. It should be noted that th at steroids are
are part o the ( or large, sympt
symptomatic
omatic metastases), stereotactic
stereotactic radio
treatmen t or primary nervous sy system
stem lymphom
lymphomaa and can and/or whol
wholee brain radiation.
alterr biopsy resul
alte results
ts i administe
administered
red prior to biopsy.
biopsy. T ere-
ore, when there is concern or primary
prim ary centr
centr al nervous sys-
sys-
tem lymphoma, steroids should ideally ideally be avoided until a er
Lepto meninge al Metastase
Metastase s
biopsy. Antiepileptics should be initiated i seiz seizures
ures occur
occur due (Car
(Carcinom
cinomato
ato us Me
Me ning
ningitis)
itis)
to intracranial tumors, although
although there is no bene t (and there Leptomeningeal metastases (carcinomatous mening
may be harm) to administering prophylactic antiepileptics to cause headache, nausea/vomitin g, cranial nerve invo
patients with brain tumor s who who have not had seizures.
seizures. and/or con usion. Spinal leptomeningeal involvem
cause back and radicular pain due to involvement
involvement
roots. Common causes o leptomenin
leptomeningeal
geal metastase
Brain Me
Me tas
tastas
tasee s breast cancer, lung cancer, hematologic malignanc
Metastases to the brain rom systemic
Metastases systemic cancer
cancer are ar more melanoma. Leptomen
Leptomeningeal
ingeal metastases o en have
common than primary brain tumors. Brain metastases panying brain
br ain metastase
m etastases.
s. T ey may rarely be the pr
rom lung cancer, breast cancer, melanoma, and colon eature o a systemic
systemic malignancy. Contr ast-enhan
cancers are most common. Metastases are most commonly imaging reveals
reveals enhan
enhancement
cement o the leptomeninge
ound at the gray–white
gray–white junction, appearing as one or more can be noted in the cerebellar
cerebellar olia, surro
surround
und ing th
ring-enhancing lesions on contrast-enhanced neuroimaging stem, and invaginating the cerebral sulci (Fig. 24–2
(Fig. 24–1). Melanoma, renal cell carcinoma, thyroid cephalus may also
also be present.
pr esent. A normal
norm al MRI
MRI does no
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CHAPTER 24 Neoplastic and Paran eoplastic Disorders o the Nervous S
A B
FIGURE
FIGURE 24 –2 Lepto meningeal metastase s.A xi
xial
al T1-wei
T1-weighte
ghte d p ostcont rast MR
MRI image
image s dem onstrat ing enh ancem ent
sulci (Aa nd B) as well as in
in the ce rebe ll
llar
ar oli
oliaa and surround ing the brainstem ( B) inin a pat ient with breast cance r.
the possibility
possibility o leptomenin
leptomeningealgeal metastases, and de nitive • Pituitary: pituitary adenoma
diagnosis is made by detecting malignant cel cells
ls in the cerebro- • Hematologic system:
system: primary
prim ary CNS lymphom
lymphom
spinal f uid (CSF)
(CSF) by cytolog
cytology/f
y/f ow cytometry. reatmen
reatmentt o lar lymphoma
leptomeningeal
leptomenin geal metastases is directed at th e primary
primar y underly-
Meningiomas and glial tumors are the
ing cancer, but progno
prognosis
sis is generally
generally poor.
primar y intracranial tum ors, although both
than metastases.
Prima
Primarry Int
ntrracran
acranial
ial Tum
umoo rs
Primary intr
intracranial
acranial tum
tumors
ors can arise rom any o the stru
struc-
c- Meningiomas
tures
tur es and constitu ent cell types o the central nervous system,
system, Meningiomas are dural based lesions that enha
including: on contrast-enhanced neuroimaging
neuroimaging and o
• Glial cells: astrocytoma, oligodendroglioma, glioblastoma tail o enhan cement at the margins o the tum
(see able 24–1) Compress
Compr ession
ion o local nervous system
system structu
• Neuron s: neur
neurocytomas,
ocytomas, gangliogl
gangliogliomas,
iomas, gangliocy
gangliocytomas
tomas ocal de cits and/ or seiz
seizures.
ures. reatmen
reatmentt o
• Neural progenitor cel
cells:
ls: medulloblastoma, neuroblastoma surgical.. Postoperative radiation th erapy is
surgical
(anaplastic) meningiomas and incompletely re
• T e meninges
meninges:: meningi
meningioma
oma
(atypical) meningiomas. Radiation therapy
• Choroid plexus: choroid plexus papilloma and choroid gery
ge ry may be considered or patient s whose
plexus carcinom
carcinomaa are inoperable, or in patients who are not
• Ependyma: ependymoma candidates. Meningiomas may be discovere
• Pineal gland:
gland: pinoplastoma,
pino plastoma, pineocytoma when brain imaging is is per ormed or an
TABL
ABLE
E 24 –1 Tumo rs o Glial Line ag e.
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thee Nervou s Sy
System
stem
FIGURE
FIGURE 24–3 Meningioma . Axial
Axial T1-weigh te
tedd p ost
ostcon
contra
trast
st MRI FIGURE
FIGURE 24 –4 Low grade glioma. A xial FLAIR MRI im
image demonstrating a le t rontal meningioma that enhances homog- on strat ing T2/FL
T2/FLAAIR hype rinten sity with
with g yral expansion in t
enously and h as a dural tail extend
extend ing peripherally
peripherally on each side. rontal lobe. There was no enhancement on postcontrast im
shown). Patho
Patho logy revealed
revealed oli
oligod
god end rogli
roglioma
oma (grade 2)
Incidentally discovered
discovered meningiomas can generally
generally be ol- with HIV/AIDS). Neuroimaging demonstrates one
lowed
lowe d with serial imaging
imaging i they are small and asymptom atic. contrast-enhancing lesions in the supratentorial or
torial white matter th at may showshow centr
centr al di usion
Gliomas tion du e to high cellula
cellularity
rity (Fig.
(Fig. 24–7). T is appearan
Gliomas range rom low grade (grades 1–2) to high grade speci c, and there ore biopsy
biopsy is o en necessary
necessary or d
(grades 3–4). Grade 4 (glioblastoma) is associated with the diagnosis. Lumbar pun cture may reveal the diagnos
worst prognosis (usually just over 1 year survival even with is evaluated
evaluated with cytology
cytology and f ow cytometr
cytometry,y, al
treatment), although patients with lower grade tumors can can take up to three large-volume lumbar punctures
survive or over a decade with
with treatment.
treatmen t. the diagnosis. I lymphoma is un under
der consideration,
On neuroimaging, low-grade gliomas are typically should be avoided prior to biopsy as they may alte
2/FLAIR hyperintense lesions with little or no contrast results. reatment is with chemoth
chemotherapy,
erapy, and rad
enhancement
enhan cement (Fig. 24–4).
24–4). Glioblastoma
Glioblastoma appears as a con trast- used in some cases; surgery is not indicated. Intra
enhancing
enhan cing mass with a necrotic appearing center,
center, and o en B-cell ly
lymph
mph oma limited to thet he lumen o blood vess
progresses along white
white matter tracts such as across the corpus cussed in Chapter
Ch apter 19 (see “Intravas
“Intr avascular
cular Lymph
Lymphoma”
oma”
callosum
callosum (“butterf y glioma”)
glioma”) (Fig. 24–5).
24–5).
reatmentt regimens
reatmen regimens or gliomas
gliomas involve maximal surgical
TUMORS OF THE SPINE
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A B
FIGURE 24 –5 Glioblastoma.A xial ( A) and Coronal (B) T1-weight ed postcon trast MRI image s dem onstrating an e nhan cing
the corpus callosum (“but ter y glioma”).
A B
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A B
C D
FIGURE 24 –7 Primary CNS Lymph om a. A xial FLAIR ( A), T1-postco ntra st (B), DWI (C), and ADC (D) MRI image s dem onst rating
occipital homog eno usly enh ancing (B), di usion restricting (C–D) lesion.
• Intramedullary (in the spin al cord itsel ): intramedul- TUMORS OF THE CRANIAL NERVE
lary metastases, ependymoma, astrocytoma, glioblastoma,
lymphoma T e cranial nerves can be a ected by:
• Extramedullary (outside the spinal cord), urther divided • Primary tumor s o cranial nerves, such as schw
into two subcategories: optic nerve glioma, cranial nerve neuro broma
• Extramedullary intradural: dural metastases, menin- • Skull base tumors, includin g ol actory groove men
gioma, schwannoma, neuro broma Metastases to the orbit, cavernous sinus, skull
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Cranial nerves can be a ected individually or together

in any o the above scenarios, and a new cranial neurop a-
thy in a patient with known malignancy requires MRI with
contr ast to look or metastases in any o the sites above. I
no lesion is revealed, C o the skull base may demon strate
bon y disease causing cran ial neu rop ath y. As men tion ed
above, imaging may be normal in patients with leptomen-
ingeal disease, and in such cases, diagnosis requires CSF
cytology.
Numb Chin Sig n

Numbn ess o the chin should raise concern or potential met-
astatic malignancy involving the mental nerve (a branch o the
trigeminal nerve). T e numb chin sign can be the rst sign o
cancer in a patient without a known history o cancer, or it can
be the rst sign o metastatic disease in a patient with kno wn
cancer (see “Numb Chin Sign” in Chapter 13).
Primary Cranial Ne rve Tumors:

Schwannomas
Schwannomas can arise on any peripheral nerve myelinated
FIGURE 24 –8 Ves tibular schwannoma . Axial
by Schwann cells. T is includes all o the cranial nerves except
po stcon trast MRI image d em onst rating a h omo ge
cranial nerve 2, which is part o the CNS, and so it is not mass at the le t cerebellopontine angle.
myelinated by Schwann cells.
Ves tibular Schwannoma TUMORS OF THE PERIPHERAL

Schwannom a o the eighth cran ial nerve (vestibular NERVOUS SYSTEM
schwannoma) is the most common cranial nerve schwan-
noma and on e o the most common cerebellopontine angle Nerve root s, the br achial and lumbar plexus,
tumors (meningioma is another common tumor at the nerves can be compressed or in ltrated by tum
cerebellopontine angle). Vestibular schwannoma is also tumor or metastasis can cause root compressio
re erred to as acoustic neuroma, which is a less accurate tum ors and axillary lymph node disease
term since the tum or arises rom Schwann cells (not n eu- cer are examples o malignancies that can a
rons) and rom the vestibular portion o the nerve (not the plexus; colon, urologic, or gynecologic canc
auditory portion). Vestibular schwannoma typically pres- the lumbosacral plexus. Di erentiating plexus
ents with subacute to chronic unilateral sensorineural hear- in ltration rom radiation-induced plexopathy
ing loss and/or tinnitus. Dizziness/vertigo is less common Chapters 16 and 17.
because th e tumor develops slowly, allowing or centr al Neurolymphomatosis re ers to in ltrati
compensation or peripheral vestibular dys unction. T e more nerves or nerve roots with lymphoma. T
tumor can impinge on the seventh cranial nerve (both cra- in patients with known lymphoma or it may be
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TABLE 24-4 Paraneo plastic/Antibody Mediated Syndrome s that Af ect the Nervous System.
Leve l o Most Commonly
Nervous System Syndrome Antibodya Associated Cancer(s)
Brain Limbic Encephalitis Anti-NMDA* Ovarian teratoma

Anti-AMPA* Breast, lung, thymoma
Anti-GABA-B* SCLC
Anti-LGI1* (see Ch. 23) SCLC, thymoma
Anti-CV2/ CRMP5 SCLC, t hym oma
Ant i-Hu (ANNA1) Sm all ce ll lun g can ce r
Anti-Ma2 Testicular cancer
Anti-GAD* SCLC, thymoma
Cerebellum Cerebellar degeneration Anti-Yo Ovarian, breast
Anti-Hu (ANNA1) SCLC
Anti-Tr Hodgkin’s lymphoma
Anti-Ma2 Testicular
Anti-GAD* SCLC, thymoma
Optic nerve Optic neuropathy Anti-CRMP5 SCLC
Retina Retinopathy Anti-recoverin SCLC
Anti–bipolar cell Melanoma
Spinal cord Myelitis (o ten part o an Anti-Hu (ANNA1) SCLC
encephalomyelitis b ut can
be iso late d) Anti-CRMP5
Necrotizing myelopathy Anti-Hu (ANNA1) SCLC, hematologic

malignancies
Anti-CRMP5
Dorsal root g anglia Ganglionopathy/sensory Anti-Hu SCLC

neu ronopa thy (see Ch. 15)
Peripheral nerves Sensorimotor neuropathy Anti-Hu (ANNA1) SCLC
Anti-CRMP5 Thymoma
Paraprotein-associated (see Ch. 26) Plasma cell disorders (see
Autonomic neuropathy (see Ch. 27) Anti-ganglionic AChR SCLC, thymoma
Anti-Hu (ANNA1) SCLC
Motor neurons ALS-like syndrome ? Rare case reports o ass
hematologic malignancies
Neuromuscular Lamb ert-Eaton myasthe nic Voltage gate d calcium Small cell lung can cer
junctio n syndrome (see Ch. 29) channels
Myasthenia gravis (see Ch. 29) AChR* Thymoma
Muscle Dermatomyositis (see Ch. 30) Anti-NXP2 Gastrointe stinal, breast
Anti-TIF1-gamma
Other Opsoclonus-myoclonus ? Neuroblastoma
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I ischemic stroke, intracerebral hemorrhage, or venous REFERENCES

sinus thrombosis occurs in a patient with cancer, the patient
Hershman DL, Lacchetti C, Dworkin RH, Lavoie S
should be evaluated or other potential causes o cerebrovas-
Bleeker J, Cavaletti G, et al. Prevention and mana
cular disease prior to attributing the cerebrovascular compli- chemotherapy-induced peripheral neuropathy in
cation directly to the u nderlying malignancy. adult cancers: American Society o Clinical On
When malignancy is thou ght to be the cause o ischemic Practice Guideline. J Clin Oncol 2014;32:1941-19
stroke, therapeutic anticoagulation with low molecular weight
heparin may be considered i there are no contrain dications,
although this is based on data or venous thromboembolism
in the setting o malignancy rather than speci c data or stroke
in this setting.
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C H A P
Disorders of
Intracranial Pressure
2
INCREASED INTRACRANIAL PRESSURE (INTRACRANIAL DECREASED INTRACRANIAL PRESSURE (INTRA
HYPERTENSION) HYPOTENSION)
Gen eral Principle s o Intracranial Press ure Cause s o Intracranial Hypo ten sion
Sympto ms and Signs o Increas ed Intracranial Pres sure Sympto ms and Signs o Intracranial Hypo
Treat ment o Acute ly Increas ed Intracranial Press ure Diagno sis o Intracranial Hypo ten sion
Treatm en t o Chronica lly Increas ed Intracranial Treat ment o Intracranial Hypo ten sion
Pres sure: Vent riculope riton eal Shunt a nd
Endo scop ic Third Vent riculosto my
Pseudo tumo r Cerebri
INCREASED INTRACRANIAL PRESSURE or due to increased arterial blood ow (e.g

vasodilation).
(INTRACRANIAL HYPERTENSION)
Gene ral Principle s o Intracranial Pressure Increased intracranial pressure due t
cerebrospinal fluid volume—Increased
Intracranial Conte nts Contributing to Intracranial
( hydrocephalus) can occur due to obstruction
Volume and Pressure
lation or rarely due to increased CSF productio
T e skull contains three components that contribute to intra- choroid plexus papilloma). Obstruction o CS
cranial volume: the brain, its blood supply, and the cerebro- be caused by a blockage anywhere within the v
spinal uid (CSF). T e brain accounts or approximately 80% (e.g., tumor, intraventricular hemorrhage, conge
o the intracran ial volume, the arterial and venous blood tal stenosis) or a blockage o the arachnoid gran
approximately 10%, and t he CSF approximately 10%. T e skull CSF is absorbed into the venous circulation (e.g
is xed and can only accommodate small changes in intracra- ingitis, subarachnoid hemorrhage). Ventricul
nial volume be ore intracran ial pressure rises. Increased intra- causes noncommunicating hydrocephalus: T
cranial pressure can arise due to increased volume o any o not communicate with one another to allow
the thr ee intracranial contents (brain, blood volume, or CSF). late. In noncommunicating hydrocephalus, only
proximal to the obstruction will dilate (e.g., obs
Increased intracranial pressure due to increased brain third ventricle will lead to dilation o the lateral v
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TABLE 30–5 Clinical Features o the Metabo lic Myopath ies.
Disorders o Glycogen Storage Disorders o Lipid Metabolism
Carnitine Carnitine Fatty acid

Type 2 Type 3 Type 4 Type 5 Type 7 transport metabolism dehydrogenase
Pompe Cori Andersen McArdle Tarui def ciencies disorders def ciencies
Dynamic/static Static Static Static Dynamic Dynamic Static and Dynamic Dynamic
dynamic
Enzyme Acid Glycogen Glycogen Muscle Phospho ructokinase Carnitine Carnitine Very long, long,
maltase debrancher bran ching ph osp hor ylase transporter pa lmitoyl medium, and
enzyme trans erase 2 short-chain
acyl-coenzyme A
dehydrogenase
Exercise onse t N/A Early in exertion With sust aine d exercise
Second wind N/A Yes No
Forearm exercise Normal Decrea sed lact ate /pyru vate rise Normal
test
Treatment Alpha Liver Ca rn it in e Lo w- a t d ie t / Avoid asts

glucosi- transplant avoidance o
dase pro longe d
exercise
Other Can be unmasked by valproate

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318 Index
lymphoma level o consciousness, 4 Multicentr e rial or Early Epilepsy

intr avascular, 191 memory, 4–5 Seizures (MESS), 168–173, 172
neurolymphomatosis, 261 meralgia paresthet ica, 164 multi ocal acquired demyelinating sen
primar y CNS, 258, 260f MERRF. See myoclonic epilepsy with ragged motor neuropathy (MADSAM
red fbers multiple sclerosis (MS)
M mesencephalic nucleus o 5, 119 clinically isolated syndrome (CIS), 2
Machado-Joseph disease, 73 mesial temporal sclerosis, 171, 171f clinical eatures o , 223–224
macular sparing, 49 MESS. See Multicent re rial or Early Epilepsy ulminant demyelinating disease, 22
Maddox rod test, 102–104, 103f and Single Seizures neuroim aging in, 224, 224–225f
MADSAM. See multi ocal acquired metabolic myopathies, 304, 305t, 306 radiologically isolated syndrom e (R
demyelinating sensory and motor methotrexate, 227 225–226
neuropathy methylprednisolone, 226 treatment o
magnesium, 202, 277 metoclopram ide, 250, 277 acute ares, 226
Magnetic gait, 8 metronidazole, 219 long-term o relapsing-remitt
magnetic resonan ce angiography (MRA), Meyer’s loop, 48 226t
12, 19 middle cerebral artery (MCA), 57 progressive, 227
magnetic resonan ce imaging (MRI), 11–12 arterial supply o , 58, 61f symptomatic management, 227
o brain , 13–17, 15–17f, 16t territory in arction o , 59–60, 64f multiple systems atrophy (MSA), 250–
magnetic resonance per usion studies, 12 migraine, 276–278 muscle disease, 299–308
magnetic r esonance spectr oscopy (MRS), 12, acephalgic, 277 muscle tone, 6
17, 18f chronic, 277 muscular dystrophies, 301, 302t
magnetic r esonance venography (MRV), 12, 21 episodic, 277 congenital, 304, 304t
mannitol, 271 hemiplegic, 277 distal, 303–304, 303t
MAO-B inhibitors. See monoamine oxidase B ocular, 277 musculocutaneous neuropathy, 150
inhibitors retinal, 277 myasthenia gravis, 293–296, 294f, 296t
Marburg variant, 223 symptomatic, 275 myasthenic crisis, 294–295
marche à petit pas, 248 vestibular, 115 myasthenic syndrom es, congenital, 29
Martin-Gruber anastomosis, 148 mild cognitive impairment (MCI), 234 mycophenolate, 228, 295
May-T urner syndrome, 188 Mini-Mental State Examinat ion (MMSE), 5 mydriasis, 83
MCA. See middle cerebral ar tery miosis, 83, 85 myeloneuropathy, 44
MCI. See mild cognitive impairm ent mitochon drial d iseases, 309, 311t myelopathy, 45, 221
McLeod syndrome, 245 mitochondrial encephalopathy with lactic Myerson’s sign, 248
Meckel’s cave, 117 acidosis and str okelike episodes myocardial in arction, 182
medial lemniscus, 37 (MELAS), 191–192 myoclonic epilepsy with ragged red f
medial longitudin al asciculus (MLF), 91, 98 mitochondrial myopathies, 306 (MERRF), 244
medial rectus, 91 mitoxantrone, 227 myoclonus, 169, 243–244
median neuropathy, 148–149 mixed transcortical aphasia, 55 myositis, in ectiou s, 220
median raphe nuclei, 75 MLF. See medial longitudinal asciculus myotonic dystroph y, 303
medication overuse headache, 279 MMSE. See Mini-Ment al State Examination myxopapillary ependymoma, 163
MELAS. See mitochond rial en cephalopathy with MoCA. See Montreal Cognitive Assessment
lactic acidosis and str okelike episodes modaf nil, 227 N
memant ine, 236, 237 Mollaret’s m eningitis, 211 natalizumab, 227
Ménière’s disease, 106, 115 monoamine oxidase B (MAO-B) inhibitors, NCC. See neurocysticercosis
meninges, 26, 28, 29f 248 NCS. See nerve conduction studies
meningiomas, 257–258, 258f monocular, 101 NCSE. See nonconvulsive status epilep
meningitis, 2, 2f, 175, 208t monocular visual loss, 49–50 neglect, 53
aseptic, 209 monon europath ies, 39, 130, 135 neonatal myasthenia gravis, 296
bacterial o lower extremit y, 163–165, 164f neoplastic and radiation-induced plex
clinical eatures o , 209 o upper extremity, 148–150 147
complications o , 210 monon europath y multiplex, 39, 130, 135 nerve condu ction studies (N CS), 11, 1
isolation and prophylaxis o contacts, 210 monoplegia, 6 136f, 138–139f, 138t
lumbar puncture in, 210, 210t Monro-Kellie doctrine, 269 nerve roots
treatment o , 209–210 Montreal Cognit ive Assessment (MoCA), 5 anatomy o , 130, 132–133f
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o upper extremit y, 141–145, 142–143f, radial, 149–150 otolithic crises o umarkin

145f, 151–153t sciatic, 164–165 OXC. See oxcarbazepine
ventricular system and cerebrospinal uid sensory, 129, 134 oxcarbazepine (OXC), 174t
ow, 29, 31–32f, 32 small f ber, 282 oxybutynin, 227
nervus intermedius, 120 tibial, 165
neuralgic amyotroph y, 147 ulnar, 149 P
neuroacant hocytosis, 244, 245 vasculitic, 135–136 p-ANCA. See perinuclear antin
neurocut aneous syndrom es, 262, 262t neurot oxicity, o chemother apy and radiation cytoplasmic antibody
neurocysticercosis (NCC), 216, 217f therapy, 262–264, 263t, 264f pachymeninges, 28
neurocysticercotic en cephalitis, 216, 217f nimodipine, 202 palmoment al re ex, 234
neurodiagnostic testing NMO. See neuromyelitis optica Pancoast tumor, 147
CSF analysis, 11, 21–24, 23t nodulus, 67 pantot henate kinase-associated
EEG, 11, 210 noncon vulsive status epilepticus (NCSE), 178 neurodegenerat ion
EMG and NCS, 11, 136–139, 136f, 138–139f, nondominant hemisphere, 53 245f
138t nonst eroidal anti-in ammat ory drugs (NSAIDs), papilledema, 270
neurogenic claudication, 161 207, 277 paradoxical embolism, 181, 18
neuroimaging normal pressure hydrocephalus (NPH), paramedian pontin e reticular
in clinical p ractice, 11–12 237–238, 238f 98
contrast- enhanced, 17–19, 19–20f NPH. See normal pressure hydroceph alus paraneoplastic syndromes, 244
interpretation o , 12 NSAIDs. See nonsteroidal anti-in ammat ory o nervous system, 264–266,
interpr etation o brain C , 12–13, 13–15f drugs paraparesis, 6
interpr etation o brain MRI, 13–17, 15–17f, nuclear imaging, 12 paraplegia, 6
16t nuclear medicine studies, 21 parasitic brain lesions, ocal in
nuclear medicine studies, 21 nucleus am biguus, 82t, 126 neurocysticercosis, 216, 217f
o the spine, 21, 22f nucleus solitar ius, 82t, 120, 126 toxoplasmosis, 215–216, 216
vascular, 19–21, 20f num b chin sign, 119–120, 261 parasympathet ic pathway
neurologic diagnosis nystagmus, 109–110 constriction , 83, 84
associated symptoms and signs in, 3 end-gaze, 110 paresis, 6
diagnostic reasoning in gaze-evoked, direct ion-changing, 109 parietal eye f elds, 96
localization, 1–2, 9t jerk, 109 parietal lobes, 53–54
time cour se, 2–3, 2f pendular , 109 Parinaud ’s syndrome, 100
examination o pure tor sional, 109 Parkinsonian gait, 8
coordination, 3, 8 pure vertical, 109 Parkinsonian tremor, 243
cranial n erves, 3, 5–6 Parkinson-plus syndromes, 236
gait, 3, 8 O 251t, 253
general, 8 obtunded, 4 Parkinson’s disease (PD), 247–
mental status, 3–5 obturator nerve, 159 diagnosis and di erential di
motor, 3, 6 obturator neuropathy, 164 248
neuroanatomic structures and pathways, 9t occipital n euralgia, 279–280 gait in, 248
re exes, 3, 7–8 ocular apraxia, 50 treatm ent o , 248–250, 24
sensory, 3, 6–7 ocular migraine, 277 Parsonage- urn er syndrome,
neurolymphomatosis, 261 ocular myasthenia, 293 partial th romboplastin time (P
neurom yelitis optica (N MO), 223, 227–228, ocular tilt reaction, 101 patent oramen ovale (PFO), 1
227f, 228t oculocephalic re ex, 108 PB. See phenobarbital
neurons, 26 oculogyric crisis, 246 PCA. See posterior cerebral art
neuropat hy, 217, 221 oculomotor nerve, 5, 84, 93–95, 94f PD. See Parkinson’s disease
acute po lyneuropathy, 283–284 oculopharyngeal muscular dystrophy (OPMD), pediatric ebrile seizures, 175
adrenomyeloneuropathy, 309 303 pedunculopon tine nu clei, 7
AIDP, 131t, 136–137, 145, 281–283 OKN. See optokinet ic nystagmus pendular n ystagmus, 109
anterior interosseous (AIN), 149 ol actory nerve, 5, 125 perception, 25
autonom ic, 282–283 oligoclonal bands, 225 pergolide, 248
chronic po lyneuropathy, 284–288 ondansetron, 277 perilymphatic f stula, 115–116
CIDP, 2, 2f, 40, 131t, 136, 145, 281, 285–287, one-and -a-hal syndrome, 99–100, 100f perimesencephalic subarachno
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320 Index
PE . See positron emission tomography pronat or dri , 35 hypore exia, 7, 35

PFO. See patent oramen ovale propran olol, 242, 243 jaw jerk, 119
Phalen’s sign, 148 propriocept ion, 6 optokinetic, 96–97, 96f
pharm acologic localization o Horn er’s prosopagnosia, 50, 54, 63 palmoment al, 234
syndrome, 87–88t pseudoaneurysm, 190 rootin g, 234
phenobarbit al (PB), 173, 174t pseudoathetosis, 38, 72 snout, 234
phenytoin (PH ), 173, 174t pseudoephedrin e, 202 suck, 234
phoria, 102 pseudoseizures, 170 VOR, 101, 107–108, 108f
PH . See phenytoin pseudotum or cerebri, 27, 272–273 re r actory epilepsy, 175
pia mater, 26 PSP. See progressive supranuclear palsy relapsing-remitting multiple sclerosis,
PICA. See posterior in erior cerebellar art ery psychogenic nonepileptic spells, 170 relative a erent pupillary de ect, 83
pilocarpine eye drops, 90 P . See plasma thromboplastin REM. See rapid eye moment
PIN. See posterior interosseous ner ve pterygopalatine ganglion, 120 REM sleep behavior disorder (RBD), 2
PION. See posterior ischemic optic neuropathy ptosis, 85, 89t reserpine, 250
PKAN. See pant othenat e kinase-associated P . See partial throm boplastin time rest tremor, 241
neurodegeneration pull test, 247 restless leg syndrome (RLS), 253
plasma exchange, 228, 230 pupil-sparin g, 94, 94f retinal migraine, 277
plasma throm boplastin (P ), 183 pupillary constriction , 83–84, 84–85f retrobulbar, 49
plegia, 6 pupillary dilation retrocollis, 246
plexopathy, 129–130, 134, 135t abnormalities o , 90 retropulsion, 247
PML. See progressive multi ocal impaired, 85–87, 87t, 88–89f reverse straight leg raise test, 159
leukoencephalopathy sympathetic pat hway o , 84–85, 86f reversible cerebral vasoconstriction sy
PNS. See peripheral nervous system pure mot or stroke, 63 (RCVS), 181, 196, 202, 203f
poliomyelitis, 219 pure sensory stroke, 63 rheumatoid arthritis, 135, 207
polycythemia vera, 244 pure tor sional nystagmus, 109 rhinocerebral mucormycosis, 215
polymyositis, 301 pure vertical nystagmus, 109 rhombencephalitis, 209
polyneuropath y, 39, 135–136 pure word dea n ess, 105 ribo avin, 277
acute, 283–284 pyramidal system, 33 ri ampicin, 219
chronic, 284–285, 288 pyrazinamide, 212 ri ampin, 212
peripheral, 281–285, 286t pyridostigmine, 295 rigidity, 247
polyradiculopathy, 130, 132–134 riluzole, 291
positron emission tomography (PE ), 12, 21, 175 Q Rinne’s test, 107
post-stroke cognitive impairm ent, 192 quadrantanopia, 48 RIS. See radiologically isolated syndro
post-stroke seizures, 192 rituximab, 227, 228
posterior cerebral art ery (PCA), 51, 57 R rivaroxaban, 188
arterial supply o , 58, 61f rabies, 213 RLS. See restless leg syndrome
e tal, 58, 60f, 63 racemose cysts in n eurocysticercosis, 216 Romberg’s sign, 7, 37, 72
territ ory in arction o , 60, 60f, 63, 65f radial neu ropath y, 149–150 rootin g re ex, 234
posterior cort ical atrophy, 234 radiculopath y, 129–130 ropinirole, 248
posterior emoral cutan eous nerve, 159 lumbosacral, 159–161, 162f, 163f RPR. See rapid plasma reagin
posterior in erior cerebellar artery (PICA), 126 radiologically isolated syndrome (RIS), 225–226 rubral tremor, 243
posterior in terosseous nerve (PIN), 150 Ramsay-Hun t syndrom e, 122, 217 ruck-sack paralysis, 147
posterior ischemic opt ic neuropath y (PION), 49 rapid eye movement (REM) sleep disorder,
posterior r eversible encephalopathy syndrome 236, 248 S
(PRES), 201–202, 202f rapid p lasma reagin (RPR), 239 saccades, 5, 96
posterolateral disc herniat ion, 130, 159 rapidly progressive dementia, 231 SAH. See subarachnoid h emorrh age
postictal state, 169 rasagiline, 248 sarcoid, 135
postpart um an giopathy, 202 RBC count. See red blood cell count sarcoidosis, 207
postural tr emor, 241 RBD. See REM sleep behavior disorder schistosomiasis, 219
Pott’s disease, 212, 218, 218f RCVS. See reversible cerebral vasoconstriction sciatic nerve, 159
PPRF. See paramedian pontine reticular syndrome sciatic neur opathy, 164–165
ormation rebleeding, 197t scintillating scotoma, 277
pramipexole, 248 receptive aphasia, 55 scissoring, 8
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post-stroke, 192 spinal meningitis, 218 throm bolysis, 186

simple ebrile, 175 spinal m uscular at rophy (SMA), 291, 291t IA, 180–181
simple part ial, 169 spinal nucleus o 5, 119 lacunar, 63, 65f
seizures and epilepsy. See also epilepsy spinal tract o 5, 119 pure mot or, 63
def nition s and causes o , 167–168, 168t spine pure sensory, 63
special scenarios in management o in ections o , 218–219, 218f seizures a er, 192
AED prophylaxis, 177–178 tumor s o , 258, 260 stuporous, 4
childhood-on set seizures, 175, 176t spinocerebellar ataxia, 73, 244 subacute combin ed degenerati
in patients with HIV, 177 spinothalam ic tracts, 33, 36, 75 subacute to chronic neurologic
pediatric ebrile seizures, 175, 177 spondylolisthesis, 162 2–3, 2f
in pr egnancy, 177 spondylosis, 162 subarachnoid hemorrhage (SA
selective serotonin reuptake inhibitor s (SSRIs), spontan eous activity on EMG, 138, 138t aneurysmal, 196–197
182, 202, 227, 237, 244 Spurling’s maneuver, 145 prevention an d managem
selegiline, 248 SREA . See steroid-responsive enceph alopat hy complications o , 197–1
semiology, 169 with autoimmune thyroiditis as hyperacute neurologic sym
sensorineural h earing loss, 105 SSRIs. See selective serotonin reuptake inhibitor s perimesencephalic, 198, 198
sensory ataxia, 8, 70, 72, 72t statin-induced myopathy, 307 unruptured intracranial ane
sensory nerve action p otentials (SNAPs), 136 status epilepticus, 178 subarachnoid space, 29
sensory neuropat hy, 129, 134 steppage gait, 8 subcortical stru ctures, 55–56, 5
sensory pathways, 55 stereotypies, 247 subdural empyema, 214
sensory trick, 246 steroid-responsive encephalopathy with subdural h ematoma (SDH), 19
shoulder d ystocia, 147 autoimm une thyroiditis (SREA ), 240 subdural space, 28
SIADH. See syndrome o inappr opriate secretion steroids, 227–230 sub alcine herniation, 270
o antidiuretic hormone Stevens-Johnson syndr ome, 173 substantia nigra, 75
sigmoid sinuses, 29 sti person syndrome, 253 suck re ex, 234
simple ebrile seizures, 175 stinger syndrome, 147 sulci, 25–26
simple part ial seizures, 169 strabismus, 101 superf cial peroneal nerve, 16
simultanagnosia, 50 straight leg raise test, 159 superf cial siderosis, 203
single photon emission computed tomography straight sinus, 29 superior oblique, 92t
(SPEC ), 12, 21, 175, 248 striatum, 56 superior rectus, 91
sinus stroke, 179 superior sagittal sinus, 29
cerebral venous, 29, 30f anticoagulants or secondary prevention o , superior salivatory n ucleus, 82
VS , 200–201, 201f 188–189 superior semicircular canal deh
con uence o , 29 anticoagulants in acute, 186–187 supranu clear lesions, 101
sigmoid, 29 antiplatelets or, 186–187, 188 survival motor neuron (SMN)
straight, 29 cognitive impairm ent a er, 192 Susac syndrome, 191
superior sagittal, 29 cryptogenic, 187 swinging ashlight test, 83,
transverse, 29 hemorrhage and, 179–180 Sydenham’s chorea, 244–245
Sjögren’s syndrome, 135, 224 induced hypertension in treatment o , 186 sympathomimetics, 202
skew deviation, 101 ischemic, 180t symptomatic migraine, 275
SMA. See spinal muscular atroph y anticoagulation or secondary prevention, synangiosis, 190
small f ber neuropathy, 282 188–189 syndrome o in appropr iate
SMN gene. See survival motor neuron gene antiplatelet agents or secondary antidiuretic hormone (S
smooth pursuit, 5, 96 prevention, 188 synkinesis, 124
SNAPs. See sensory ner ve action potent ials antiplatelets and anticoagulants, 186–187 syphilitic meningitis, 212
snout re ex, 234 CADASIL, 191 syrinx, 42
somatosensory pathways, 36–38, 36–38f CARASIL, 191
somnolent, 4 cerebral vasculitis, 190 T
spastic gait, 8 etiology o , 181–182, 181t, 182–183, 182f, 1 black holes, 224
spasticity, 35 183, 183f tardive d yskinesia, 253
SPEC . See single photon emission computed evaluation or etiology o , 187–188 tardy uln ar palsy, 149
tomography as hyperacute neurologic symptom, 2, 2f tarsal tunnel syndrome, 165
sphenopalatine ganglion, 120 initial evaluation o , 183–184, 184–185f, 186 task-specif c dystonias, 246
RELATED TITLES
208 views 1 0

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322 Index
360 roll maneuver, 114 triptans, 277 vestibular schwannom a, 261, 261f
throm bolysis, 186 trismus, 219 vestibular system, 107–108f, 107–110
thrombosis, 181 trochlear nerve, 5, 95, 95f vestibulo-ocular re ex (VOR), 101
thunderclap headache, 276 tropical spastic par aparesis, 218 107–108, 108f
IA. See transient ischemic attack rousseau’s syndrome, 266 vestibulocochlear nerve, 6, 105
tibial ner ve, 159 tuberculom a, 212, 215 viral brain lesions, ocal in ectious
tibial neuropathy, 165 tuberculosis, o spine, 218–219, 218f PML, 214–215, 215f, 227
tics, 246 tuberculous brain lesions, ocal in ectious, 215 viral meningitis, 211
time course o neurologic symptom s, 1, 2f tuberculous m eningitis, 211–212 visual cognition , disorders o , 50–51
inel’s sign, 148 ullio’s phenomen on, 114 visual evoked p otentials (VEPs), 22
tissue plasminogen activator (tPA), 180 tume active demyelination, 223 visual f elds, 47
tizanidine, 227, 291 tumors visual loss, 48–50, 50f
M. See transverse myelitis brain m etastases, 256 visual pathway, 47–48, 48f
odd’s paralysis, 169 o cran ial nerves, 260–261, 261f vitamin B1, 233
tolcapone, 248 intracranial, 255–258, 256–260f, 257t vitamin B12, 239, 262
tonic-clonic, 169 Pancoast, 147 def ciency, 2f, 3, 8, 44, 45, 52, 131t, 2
tonsillar herniation, 270 o PNS, 261–262 234, 281, 285, 290, 309
topiram ate ( PM), 174t, 243 primar y intracran ial, 257–258, 257t, 258f voluntary activity, 138, 138f
tort icollis, 246 o spine, 258, 260 VOR. See vestibulo-ocular re ex
ourett e’s syndrom e, 246–247 VP shunt. See ventriculoperitoneal shu
toxoplasmosis, 215–216, 216f, 244 U VPA. See valproate/valproic acid
tPA. See tissue plasmin ogen activator Uhtho ’s phenom enon, 224 VPL. See ventral p osterior lateral
PM. See topiram ate ulnar neuropathy, 149 VPM. See ventral p osterior medial nu
transcortical motor aphasia, 55 uncal herniation, 270 VS . See cerebral venous sinus thr
transcortical sensory aphasia, 55 unruptured intracranial aneurysms, 198 VZV. See varicella zoster virus
tran scranial Doppler ultrasoun d ( CD), 12, 21, upper motor n euron acial weakness, 121–122
198 upper motor neuron lesions, 35–36, 35t W
tran sient global amnesia ( GA), 232, 232f upper motor neurons, 35 wall-eyed bilateral internuclear ophtha
tran sient ischemic attack ( IA), 112, 115, upward herniation, 270 (WEBINO), 100, 100f
180–181 uremia, 2 war arin, 173, 188
transtentorial herniation, 270 Warten berg’s syndrome, 150
transverse myelitis ( M), 223, 229–230 V watershed territories
tran sverse sinuses, 29 vacuolar myelopathy, 218 o cerebral hemispheres, 58–59
traumatic brachial plexopathy, 147 vagus nerve, 6, 125–126, 127t in arction in, 63, 66, 66f
tremor , 243t, 247 valproate/valproic acid (VPA), 173, 174t, 244, WBCs. See white blood cells
action, 241 250 Weber’s syndrom e, 94
cerebellar, 243 Valsalva maneuver, 189 Weber’s test, 106–107
enhanced physiologic, 241–242 vancomycin, 209 WEBINO. See wall-eyed bilater al in
essential, 242–243 varicella zoster virus (VZV), 122, 212 ophthalmoplegia
FX AS, 73, 73f encephalitis, 214 Wegener’s granulomatosis, 135, 207
Homes, 243 vascular dem entia, 237 Wernicke’s aph asia, 55
intention, 241 vascular imaging, 19–21, 20f Wernicke’s area, 54
kinetic, 241 vascular mal orm ations, o CNS, 195, 195t, 196f Wernicke’s encephalopathy, 233, 233f
orthostatic, 243 vasculitic neuropat hy, 135–136 Weston-Hurst syndrome, 228
Parkinsonian, 243 vasculopathy and vasculitis, 182 Westphal variant o Hun tington ’s Dis
postural, 241 vasospasm, 182, 197t white blood cells (WBCs) in cerebrosp
rest, 241 ventral posterior lateral (VPL), 36 1, 23, 210t, 284
rubral, 243 ventral posterior medial (VPM) nucleus, 119 white matter, 26
wing-beating, 254 ventricular system, and cerebrospinal uid ow, Wilbrand’s knee, 49
rendelenbur g gait, 8 29, 31–32f, 32 Wilson’s disease, 244, 254
trigeminal autonom ic cephalalgias, 278, 279t ventriculoperitoneal (VP) shunt, 272 wing-beating tremor, 254
trigeminal motor pathways, 119 VEPs. See visual evoked potentials
trigeminal nerve, 5–6, 117–120, 118f, 127t verapamil, 202 X

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Clinical Neurology and

Clinical Neurology and

a LANGE medical book

Aaron L. Berkowitz, MD, PhD

Clinical Neurology and

Copyright © 2017 by McGraw-Hill

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THE WORK IS PROVIDED

Clinical Neurology and

My mentors Dr. Martin A. Samuels, Dr. Allan H

Clinical Neurology and

This page intentiona lly left

Clinical Neurology and

P A R I 4 T e Motor and Somatosensory Path

Clinical Neurology and

10 Pupillary Control and Approach to 16 Radiculopathy, Plexopathy, and

12 T e Auditory and Vestibular Pathways

Clinical Neurology and

20 In ectious Diseases o the Nervous System 207 26 Headache 275

Clinical Neurology and

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Clinical Neurology and

Clinical Neurology and

Clinical Neurology and

Attention Frontal and parietal lobes

Memory Temporal lobes

Language Frontal lobe (usually le t)

Praxis Frontal and parietal lobes

Abstract reasoning Frontal lobes

Visuospatial processing Occipital and parietal lobes C

Smell CN 1 and ol actory cortex

Pupillary light re ex CNs 2 and 3; midbrain nuclei and pathways C

Eye m ove m en ts CNs 3, 4 , a nd 6, b ra in st em p at hwa ys, ro nt al a nd p arie t al e ye f e ld s, ce r-

Fa cia l se n sa tio n CN 5, b ra in st em p at hwa ys, t ha la mu s (ve nt ra l p ost e rio r m ed ia l n ucle us

Fa cia l m ove me nt s CN 7, m ot or p at hwa y ro m p re ce nt ra l g yru s t o CN 7 n ucle us in p on s

He arin g In ne r e ar, CN 8, b rain st em a ud it ory p at hways, t hala mus (m ed ia l g en iculat e

Ve st ib ula r sys te m In ne r e ar, CN 8 , b ra in st e m p at hwa ys a nd t he ir co nn ec tio ns wit h n ucle i o

St re ng th Cort icosp ina l t ract (p re ce nt ra l g yrus t hro ug h su bco rt ica l wh it e m at te r,

Initiation o movements and assessment Basal ganglia

Clinical Neurology and

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Clinical Neurology and

NEUROIMAGING IN CLINICAL PRACTICE CONTRAST-ENHANCED NEUROIMAGING

Clinical Neurology and

40 PART I Neuroan atomy and Neuroanatom ic Localization

Hemispheric Brainstem Spinal cord Root Ne rve Poly- Myopa

In sum mary (Fig. 4–5): Isolated bilateral sensory changes generally su

Clinical Neurology and

Anterior Cord Synd rome CAUSES OF MYELOPATHY

OVERVIEW OF SP INAL CORD ANATOMY T e lateral corticospinal tracts are lateral a

Clinical Neurology and

42 PART I Neuroan atomy and Neuroanatom ic Localization

Spinal Vertebral Dorsal columns

T12 o central cord syndrome( e.g., as can be caused by a

L2 SPINAL CORD SYNDROMES

Clinical Neurology and

CHAPTER 5 T e Spinal Cord and Approach to Mye

Dorsal column nuclei

To leg From leg

Dorsal columns Dorsal columns

Corticos pinal trac t Corticos pinal tract

A Anterolatera l (spinotha lamic) trac t B Anterolateral (spinothalamic) tract