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Daniel Kondziella
Gunhild Waldemar
Neurology
at the Bedside
Second Edition
123
Neurology at the Bedside
Daniel Kondziella • Gunhild Waldemar
Neurology at the
Bedside
Second Edition
Daniel Kondziella Gunhild Waldemar
Department of Neurology Department of Neurology
Rigshospitalet, University of Rigshospitalet, University of
Copenhagen Copenhagen
Copenhagen Copenhagen
Denmark Denmark
ISBN 978-3-319-55990-2 ISBN 978-3-319-55991-9 (eBook)

DOI 10.1007/978-3-319-55991-9
Library of Congress Control Number: 2017943485
© Springer International Publishing AG 2017

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software,
or by similar or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a specific statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in
this book are believed to be true and accurate at the date of publication. Neither the publisher nor
the authors or the editors give a warranty, express or implied, with respect to the material
contained herein or for any errors or omissions that may have been made. The publisher remains
neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Printed on acid-free paper
This Springer imprint is published by Springer Nature

The registered company is Springer International Publishing AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Foreword to the Second Edition
Progress in neurology is being made at an ever-increasing speed. Since the

first edition of this book only 4 years ago, extraordinary achievements have
been seen in almost all neurological subspecialties. To name just a few, there
is now firm evidence for endovascular recanalization therapy in acute stroke;
convenient oral drugs have become available for the treatment of multiple
sclerosis; and clinical exome sequencing is revolutionizing diagnostics of
hereditary diseases. Accordingly, the text has been carefully updated, and
references to major revisions such as the new classification of status epilepti-
cus have been included. In addition, the authors have added more than 40 new
cases illustrating important concepts for the young neurologist’s daily clini-
cal practice, and much of the graphic material has been revised.
The authors are grateful for the invaluable suggestions and comments to
the manuscript made by Kirsten Svenstrup, Poul Brodersen, Sára B. W. Bech,
Siska Frahm-Falkenberg, Vagn Eskesen (Copenhagen), Fredrik Asztely
(Gothenburg), and Lawrence A. Zeidman (Chicago). Further, the authors
would like to thank Vibeke Andrée Larsen and Annika Reynberg Langkilde
(Department of Neuroradiology, Rigshospitalet, Copenhagen) for their excel-
lent help in providing many of the neuroimaging figures.
Finally, the authors wish to express their gratitude to their patients and
colleagues—you have taught us neurology; it is a pleasure working with you!
Copenhagen Daniel Kondziella

2017 Gunhild Waldemar
v
Foreword to the First Edition
The days of textbooks are numbered but not for this one. In the modern com-
puter age, most of our knowledge is gained online and the need for weighty
textbooks is over. Much of our learning comes from clinical experience and
seeing our colleagues, senior and junior, at work.
During the early stages of a neurologists’ career, there is a tremendous
need for a reasonably short book which enables the beginner to grasp the
whole span of the subject, a book which will provide a framework on which
to graft their growing bedside experience. This is that book. It takes its stance
at the bedside, be that the outpatient clinic couch or the hospital bed. The
winning combination of junior neurologist Daniel Kondziella and senior pro-
fessor Gunhild Waldemar covers the spectrum from anatomy and physiology,
through history and examination, to differential diagnosis, investigation, and
treatment. Concise, clear, and written in impeccable English, each chapter
could be consumed in an evening, leaving the reader with a sound basis for
their clinical practice, further enquiry, and lifelong learning.
Each generation needs its own text to tackle the problems of the modern
era. This text is modern and full of wisdom. I warmly recommend it to the
neurologist in training and the more senior neurologist who wants to match
their knowledge against an authoritative template.
London Richard Hughes, M.D., F.R.C.P., F.Med.Sci.

2013
vii
Disclaimer
Medicine, including neurology, is an ever-changing field. As new research

and clinical experience extends our knowledge, revisions of diagnostic proce-
dures, treatment protocols, and drug therapy may become necessary. While
the authors have made considerable efforts to describe generally accepted
practices and to confirm the accuracy of the data, they are not responsible for
errors or omissions or for any consequences from application of the informa-
tion in this publication. To the fullest extent of the law, neither the authors nor
the publisher assumes any liability for any damage and/or injury to persons or
property arising from this book. The application of the information in this
book remains the professional responsibility of the practitioner. In particular,
the reader is urged to confirm the accuracy of the information relating to drug
therapy by checking the most current product information provided by the
drug manufacturer and by consulting other pharmaceutical literature to verify
the recommended dose, the method and duration of administration, as well as
possible contraindications and drug side effects. The treating physician is
responsible for the decision on dosages and the best available treatment for
each individual patient.
ix
Contents
1 What to Expect from This Book (and What Not to) �� 1
2 Clinical History and Neuroanatomy:
“Where Is the Lesion?” �� 5
2.1 Muscle�� 9
2.2 Neuromuscular Junction �� 10
2.3 Peripheral Nerves, the Plexus, and Spinal Nerve Roots �� 12
2.3.1 Peripheral Nerves�� 15
2.3.2 Brachial Plexus and Lumbosacral Plexus �� 21
2.3.3 Spinal Nerve Roots �� 23
2.4 Spinal Cord �� 24
2.4.1 Complete or Near-Complete Transection
of the Spinal Cord �� 26
2.4.2 Brown-Séquard Syndrome�� 28
2.4.3 Anterior Cord Syndrome�� 29
2.4.4 Dorsal Cord Syndrome �� 30
2.4.5 Syringomyelia �� 31
2.4.6 Central Cord Syndrome�� 32
2.4.7 Conus Medullaris Syndrome�� 32
2.5 Brainstem�� 33
2.6 Cranial Nerves�� 36
2.6.1 Olfactory Nerve�� 36
2.6.2 Optic Nerve�� 36
2.6.3 Oculomotor Nerve, Trochlear Nerve,
Abducens Nerve�� 38
2.6.4 Trigeminal Nerve�� 43
2.6.5 Facial Nerve�� 44
2.6.6 Vestibulocochlear Nerve �� 48
2.6.7 Glossopharyngeal Nerve�� 49
2.6.8 Vagus Nerve�� 50
2.6.9 Spinal Accessory Nerve�� 50
2.6.10 Hypoglossal Nerve�� 51
2.7 Cerebellum�� 52
2.8 Subcortical Gray Matter�� 54
2.8.1 Basal Ganglia�� 54
2.8.2 Diencephalon�� 55
2.9 Subcortical White Matter�� 56
xi
xii Contents
2.10 Cortex�� 60
2.10.1 Frontal Lobes�� 61
2.10.2 Temporal Lobes�� 62
2.10.3 Parietal Lobes�� 65
2.10.4 Occipital Lobes �� 66
2.11 Cerebrovascular System�� 69
2.11.1 Anterior Circulation�� 69
2.11.2 Posterior Circulation �� 71
2.12 The Healthy Brain�� 75
References and Suggested Reading �� 76
3 Neurological Bedside Examination:
“Can I Confirm My Anatomical Hypothesis?” �� 79
3.1 Cognitive and Mental Functions �� 81
3.1.1 The History as Part of the Cognitive
Examination�� 81
3.1.2 Bedside Examination of Mental and
Cognitive Functions�� 84
3.1.3 General Neurological Examination in the
Cognitively Impaired Patient�� 87
3.2 Cranial Nerves�� 87
3.3 Motor Function �� 94
3.4 Sensory Function�� 98
3.5 Cerebellar Function�� 99
3.6 Gait�� 101
3.7 System Overview�� 101
3.8 Examination of the Comatose Patient�� 106
3.8.1 Verification and Quantification of
Unconsciousness �� 106
3.8.2 Assessment of Neurological Deficits,
in Particular Signs of Brainstem Injury�� 107
3.8.3 Neurological Causes for Coma �� 110
3.8.4 Coma, Vegetative, and Minimal Conscious States�� 112
3.8.5 Prognostication of Neurological Outcome
Following Cardiac Arrest�� 113
3.8.6 Diagnosis of Brain Death�� 114
3.9 Examination of the Patient with an Acute Ischemic Stroke�� 118
3.10 Examination of the Patient with an Epileptic Seizure�� 119
3.10.1 Taking the History of a Patient with a Seizure�� 119
3.10.2 The Examination and Management of a Patient
with a Seizure�� 120
3.11 Examination of the Patient with Functional/
Nonorganic Deficits�� 121
4 Differential Diagnosis: “What Is the Lesion?”�� 127
4.1 Introduction to Heuristic Neurological Reasoning �� 128
4.2 The Differential Diagnosis of Coma�� 130
4.2.1 Structural Causes of Coma�� 131
4.2.2 Nonstructural Causes of Coma �� 134
Contents xiii
4.3 The Differential Diagnosis of Traumatic Brain Injury �� 134

4.3.1 Assessing the Need for Observation
and Imaging in Closed Head Injury�� 135
4.4 The Differential Diagnosis of Headache�� 136
4.4.1 Primary Headache Syndromes�� 136
4.4.2 Symptomatic Headaches�� 138
4.5 The Differential Diagnosis of Cognitive Impairment
and Dementia�� 143
4.5.1 Potentially Reversible Conditions�� 144
4.5.2 The Amnestic Syndrome�� 146
4.5.3 Chronic Dementia Disorders�� 146
4.5.4 Rapidly Progressive Dementias�� 152
4.6 The Differential Diagnosis of Encephalopathy�� 154
4.7 The Differential Diagnosis of Epilepsy�� 160
4.7.1 Practical Clinical Definition of Epilepsy�� 161
4.7.2 Epileptic Seizures�� 161
4.7.3 Epileptic Syndromes �� 164
4.7.4 Status Epilepticus�� 165
4.8 The Differential Diagnosis of Nonepileptic Seizures,
Blackouts, and Drop Attacks�� 166
4.9 The Differential Diagnosis of Vertigo and Dizziness �� 171
4.9.1 Vertigo of Central Origin�� 171
4.9.2 Vertigo of Peripheral Origin�� 171
4.9.3 Dizziness and Light-Headedness�� 174
4.10 The Differential Diagnosis of Sleep Disorders �� 174
4.11 The Differential Diagnosis of TIA and Stroke�� 176
4.11.1 Ischemic Stroke: Clinical Presentation and
Classification�� 176
4.11.2 Space-Occupying Ischemic Stroke �� 178
4.11.3 Transitory Ischemic Attacks: Definitions and
Risk Assessment �� 180
4.11.4 Rare Causes of Ischemic Stroke and
Transitory Ischemic Attacks �� 181
4.11.5 Ischemic Stroke Mimics �� 188
4.11.6 Hemorrhagic Stroke�� 190
4.11.7 Subarachnoid Hemorrhage�� 190
4.12 The Differential Diagnosis of Demyelinating Disorders�� 192
4.12.1 Multiple Sclerosis �� 192
4.12.2 Other Forms of Demyelinating Disease and
Related Conditions�� 194
4.13 The Differential Diagnosis of Infectious Diseases �� 203
4.13.1 Viral Infections�� 203
4.13.2 Bacterial Infections �� 207
4.13.3 Infections Due to Parasites, Fungi, and Prions �� 211
4.14 The Differential Diagnosis of Malignancy,
Including Paraneoplastic Conditions�� 214
4.14.1 Primary Tumors of the CNS and PNS and
Secondary Malignancies �� 214
4.14.2 Paraneoplastic Conditions�� 220
xiv Contents
4.15 The Differential Diagnosis of Movement Disorders�� 221

4.15.1 Hypokinetic Movement Disorders (Parkinsonism)�� 222
4.15.2 Hyperkinetic Movement Disorders�� 228
4.16 The Differential Diagnosis of Ataxia�� 234
4.17 The Differential Diagnosis of Gait Disorders �� 240
4.18 The Differential Diagnosis of Cranial Nerve Deficits�� 241
4.19 The Differential Diagnosis of Myelopathy �� 242
4.20 The Differential Diagnosis of Motor Neuron Disease�� 246
4.20.1 Amyotrophic Lateral Sclerosis �� 246
4.20.2 Other Motor Neuron Diseases�� 247
4.21 The Differential Diagnosis of Peripheral Nerve Disorders�� 251
4.21.1 Algorithm for Evaluation of Polyneuropathies�� 251
4.22 The Differential Diagnosis of Neuromuscular
Junction Disorders�� 255
4.22.1 Myasthenia Gravis�� 255
4.22.2 Other Neuromuscular Junction Disorders�� 256
4.23 The Differential Diagnosis of Myopathy�� 257
4.23.1 Acquired Myopathies�� 257
4.23.2 Genetic Myopathies�� 257
5 Ancillary Investigations �� 263
5.1 Neuroimaging �� 263
5.1.1 Computer Tomography (CT)�� 264
5.1.2 Positron Emission Tomography (PET) �� 267
5.1.3 Single Photon Emission Computed
Tomography (SPECT)�� 267
5.1.4 Magnetic Resonance Imaging (MRI)�� 267
5.2 Electroencephalography (EEG)�� 271
5.3 Electromyography (EMG) and Nerve Conduction
Studies (NCS) �� 274
5.3.1 EMG�� 274
5.3.2 NCS�� 275
5.3.3 Electrodiagnostic Findings in Selected
Neuromuscular Disorders �� 276
5.4 Other Neuroelectrophysiological Investigations�� 280
5.4.1 Evoked Potentials�� 280
5.4.2 Evaluation of the Autonomic Nervous System�� 281
5.4.3 Polysomnography and Other Studies of Sleep�� 281
5.5 Ultrasound of the Cerebral Vessels and the Heart�� 281
5.6 Lumbar Puncture�� 282
5.7 Neurogenetics �� 284
5.7.1 Basic Neurogenetics�� 284
5.7.2 Genetic Testing �� 286
5.8 Other Tests�� 287
5.8.1 Blood and Urine Chemistry�� 287
5.8.2 Tissue Biopsies �� 287
5.8.3 Formal Neuropsychological Examination�� 289
Contents xv
6 Treatment of Neurological Disorders �� 291

6.1 Coma and Acute Encephalopathies�� 292
6.2 Headache�� 294
6.3 Cognitive Impairment and Dementia�� 296
6.4 Epilepsy�� 297
6.4.1 General Aspects�� 297
6.4.2 Status Epilepticus�� 299
6.4.3 Antiepileptic Drugs (AED) �� 299
6.4.4 Epilepsy and Mood Disorders�� 301
6.4.5 Options for Patients with Medically
Refractory Seizures�� 301
6.5 Sleep Disorders �� 302
6.6 Cerebrovascular Disorders�� 303
6.6.1 Intravenous Thrombolysis (IVT)�� 303
6.6.2 Endovascular Therapy (EVT) for Acute
Ischemic Stroke�� 305
6.6.3 Surgical Decompression for Space-Occupying
Ischemic Stroke�� 307
6.6.4 Arterial Dissection�� 310
6.6.5 Symptomatic Treatment of Ischemic Stroke�� 310
6.6.6 Secondary Prevention of Ischemic Stroke�� 312
6.6.7 Cerebral Venous Sinus Thrombosis�� 313
6.6.8 Spontaneous Intracranial Hemorrhage
and Bleedings Associated with Vascular
Malformations�� 313
6.6.9 Unruptured Intracranial Aneurysms �� 315
6.7 Multiple Sclerosis and Other Neuroinflammatory
Disorders �� 315
6.8 Infectious Diseases�� 318
6.9 Parkinson’s Disease�� 319
6.10 Non-parkinsonian Movement Disorders�� 320
6.11 Cranial Neuropathies and Related Conditions�� 322
6.12 Autonomic Dysreflexia with Spinal Cord Injury�� 323
6.13 Neuromuscular Disorders �� 323
6.13.1 Motor Neuron Diseases and Peripheral
Neuropathies �� 323
6.13.2 Neuromuscular Junction Disorders and
Myopathies�� 324
Index�� 329
Abbreviations
ACA Anterior cerebral artery

ACE Addenbrooke’s cognitive examination
AChR Acetylcholine receptor
AComA Anterior communicating artery
ACTH Adrenocorticotropic hormone
AD Alzheimer’s disease
ADC Apparent diffusion coefficient
ADEM Acute demyelinating encephalomyelitis
ADHD Attention deficit hyperactivity disorder
AED Antiepileptic drug
AICA Anterior inferior cerebellar artery
AIDP Acute inflammatory demyelinating polyneuropathy
AIE Autoimmune encephalitis
AION Anterior ischemic optic neuropathy
ALS Amyotrophic lateral sclerosis
AMAN Acute motor axonal neuropathy
AMPA α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid
AMSAN Acute motor sensory axonal neuropathy
ANA Antinuclear antibody
ANCA Antineutrophil cytoplasmic antibodies
ARAS Ascending reticular activating system
ARUBA A randomized trial of unruptured brain arteriovenous
malformations
AV Arteriovenous
AVM Arteriovenous malformations
BAEP Brainstem auditory evoked potentials
BECTS Benign partial epilepsy of childhood with centrotemporal
spikes
BPPV Benign paroxysmal positional vertigo
BRBNS Blue rubber bleb nevus syndrome
bvFTD Behavioral variant frontotemporal dementia
C9ORF72 Chromosome 9 open reading frame 72
CAA Cerebral amyloid angiopathy
CADASIL Cerebral autosomal dominant arteriopathy with subcortical
infarcts and leukoencephalopathy
CARASIL Cerebral autosomal recessive arteriopathy with subcortical
infarcts and leukoencephalopathy
xvii
xviii Abbreviations
CAE Childhood absence epilepsy

CAG Cytosine, adenine, guanine
CASPR2 Contactin-associated protein-like 2
CBD Corticobasal degeneration
CBF Cerebral blood flow
CDT Carbohydrate-deficient transferrin
CEA Carotid endarterectomy
CES Clinical exome sequencing
CGH Comparative genomic hybridization
CIDP Chronic inflammatory demyelinating polyneuropathy
CIPM Critical illness polyneuropathy and myopathy
CIS Clinically isolated syndrome
CJD Creutzfeldt-Jakob disease
CK Creatine kinase
CLIPPERS Chronic lymphocytic inflammation with pontine perivascular
enhancement responsive to steroids
CMAP Compound muscle action potentials
CMT Charcot-Marie-Tooth disease
CN Cranial nerve
CNS Central nervous system
CO Carbon monoxide
CO2 Carbon dioxide
COHb Carboxyhemoglobin
COMT Catechol-O-methyltransferase
CPAP Continuous positive airway pressure
CPEO Chronic progressive external ophthalmoplegia
CPP Cerebral perfusion pressure
CRP C-reactive protein
CSF Cerebrospinal fluid
CT Computed tomography
CTA Computed tomography angiography
DADS Distal acquired demyelinating symmetric polyneuropathy
DAI Diffuse axonal injury
DAP 3,4-Diaminopyridine
DAT scan Dopamine transporter scanning
DBS Deep brain stimulation
DECIMAL Decompressive craniectomy in malignant middle cerebral
artery infarction
DESTINY Decompressive surgery for the treatment of malignant
infarction of the middle cerebral artery
DLB Dementia with Lewy bodies
DMD Duchenne muscular dystrophy
DMPK Dystrophia myotonica-protein kinase
DoC Disorders of consciousness
DRPLA Dentatorubral-pallidoluysian atrophy
DTI Diffusion tensor imaging
DWI Diffusion-weighted MRI
DYT Dystonia
Abbreviations xix
EEG Electroencephalography
EFNS European federation of neurological societies
EMG Electromyography
eMCS Emerged from minimal conscious state
EVT Endovascular therapy
FCMS Foix-Chavany-Marie syndrome
FDG Fludeoxyglucose
FLAIR Fluid-attenuated inversion recovery
FMD Fibromuscular dysplasia
FMR1 Fragile X mental retardation 1
fMRI Functional magnetic resonance imaging
FOUR Full outline of unresponsiveness
FSH Facioscapulohumeral muscular dystrophy
FTD Frontotemporal dementia
FXTAS Fragile X-associated tremor/ataxia syndrome
g Gram
GABA Gamma-aminobutyric acid
GAD Glutamic acid decarboxylase
GBS Guillain-Barré syndrome
GCS Glasgow Coma Scale
GHB Gamma-hydroxybutyrate
GI Gastrointestinal
GluT1 Glucose transporter type 1
h Hour
HAART Highly active antiviral therapy
HAMLET Hemicraniectomy after middle cerebral artery infarction
with life-threatening edema trial
HaNDL Headache with neurological deficits and CSF lymphocytosis
HAS-BLEED Hypertension, abnormal renal/liver function, stroke, bleeding
history or predisposition, labile INR, elderly, drugs/alcohol
HD Huntington’s disease
Hg Hectogram
HNPP Hereditary neuropathy with liability to pressure palsies
HSP Hereditary spastic paraparesis
HSV Herpes simplex virus
HTLV-1 Human T-lymphotropic virus type 1
i.a. Intra-arterial
IBM Inclusion body myositis
ICA Internal carotid artery
IC-EC Intracranial-extracranial
ICH Intracerebral hemorrhage
ICP Intracranial pressure
ICU Intensive care unit
Ig Immunoglobulin
IIH Idiopathic intracranial hypertension
ILAE International League Against Epilepsy
IM Intramuscularly
INO Internuclear ophthalmoplegia
xx Abbreviations
INR International normalized ratio

IRIS Immune reconstitution inflammatory syndrome
IV Intravenously
IVIG Intravenous immunoglobulin
IVT Intravenous thrombolysis
JAE Juvenile absence epilepsy
JC John Cunningham (virus)
JME Juvenile myoclonic epilepsy
kg Kilogram
KSS Kearns-Sayre syndrome
L Liter
LACS Lacunar syndromes
LEMS Lambert-Eaton myasthenic syndrome
LG1 Leucine-rich glioma-inactivated 1
LHON Leber’s hereditary optic neuropathy
LMN Lower motor neuron
MADSAM Multifocal acquired demyelinating sensory and motor
neuropathy
MAG Myelin-associated glycoprotein
MAO-B Monoamine B
MAP Median arterial pressure
MAPs Compound muscle action potentials
max Maximally
MCA Middle cerebral artery
MCI Mild cognitive impairment
MCS Minimal conscious state
MCV Motor conduction velocity
MELAS Mitochondrial encephalomyopathy with lactic acidosis and
stroke-like episodes
MEP Motor evoked potential
mEq Milliequivalent
MERFF Myoclonic epilepsy with ragged red fibers
MERS Mild encephalitis/encephalopathy with a reversible splenial
lesion
mg Milligram
MG Myasthenia gravis
mGluR5 Metabotropic glutamate receptor 5
MGUS Monoclonal gammopathy of unknown significance
MLF Medial longitudinal fasciculus
MLPA Multiplex ligation-dependent probe amplification
MLST Multiple sleep latency test
mm Millimeter
MMN Multifocal motor neuropathy
MMNCB Multifocal motor neuropathy with conduction block
mmol Millimole
MMSE Mini-Mental State Examination
MND Motor neuron disease
MNGIE Mitochondrial neurogastrointestinal encephalopathy
Abbreviations xxi
MOG Myelin oligodendrocyte glycoprotein

MR Magnetic resonance
MRC Medical Research Council
MRI Magnetic resonance imaging
MRS Magnetic resonance spectroscopy
mRS Modified Rankin Scale
MS Multiple sclerosis
MSA Multisystem atrophy
ms Millisecond
MSUD Maple syrup urine disease
MUPs Motor unit potentials
MuSK Muscle-specific kinase
NARP Neuropathy, ataxia, and retinitis pigmentosa
NBIA Neurodegeneration with brain iron accumulation
NCS Nerve conduction studies
NFL Neurofilament
NFLE Nocturnal frontal lobe epilepsy
NIHSS National Institute of Health Stroke Scale
NINDS National Institute of Neurological Disorders and Stroke
NMDA N-Methyl-d-aspartate
NMO Neuromyelitis optica
NOAC Novel (or non-vitamin K antagonist) oral anticoagulants
NPH Normal pressure hydrocephalus
NREM Non-rapid eye movement
NSAID Nonsteroidal anti-inflammatory drugs
OCD Obsessive compulsive disorder
OMIM Online Mendelian Inheritance in Man
p.o. Per oral
PACS Partial anterior circulation syndromes
PCA Posterior cerebral artery
PCommA Posterior communicating artery
PCR Polymerase chain reaction
PD Parkinson’s disease
PDE-5 Phosphodiesterase type 5
PET Positron-emission tomography
PFO Patent foramen ovale
PICA Posterior inferior cerebellar artery
PLED Periodic lateralized epileptiform discharges
PLMT Painful legs and moving toes
PML Progressive multifocal leukoencephalopathy
PNES Psychogenic nonepileptic seizures
PNET Primitive neuroectodermal tumors
PNFA Progressive nonfluent aphasia
PNS Peripheral nervous system
POCS Posterior circulation syndromes
POEMS Polyneuropathy, organomegaly, endocrinopathy, M protein,
and skin changes
PRES Posterior reversible encephalopathy syndrome
xxii Abbreviations
PROMM Proximal myotonic myopathy

PSP Progressive supranuclear palsy
RCVS Reversible cerebral vasoconstriction syndrome
REM Rapid eye movement
RIS Radiologically isolated syndrome
RLS85 Reaction Level Scale 85
RVCL Retinal vasculopathy with cerebral leukodystrophy
SAH Subarachnoidal hemorrhage
SC Subcutaneously
SCA Spinocerebellar ataxias
SCA1 Spinocerebellar ataxia type 1
SCA2 Spinocerebellar ataxia type 2
SCLC Small cell lung cancer
SD Semantic dementia
SIADH Syndrome of inadequate antidiuretic hormone secretion
SLE Systemic lupus erythematosus
SMA Spinal muscular atrophy
SNAPs Sensory (nerve) action potentials
SOD1 Copper-zinc superoxide dismutase
SPECT Single photon emission computed tomography
SSA Sjögren syndrome type A antibody
SSB Sjögren syndrome type B antibody
SSEP Somatosensory evoked potentials
SSPE Subacute sclerosing panencephalitis
SSRI Selective serotonin reuptake inhibitor
STICH Surgical Trial in Intracerebral Hemorrhage
STIR Short tau inversion recovery
SUNCT Short-lasting unilateral neuralgiform headache attacks with
conjunctival injection and tearing
SUNA Short-lasting unilateral neuralgiform headache with cranial
autonomic symptoms
SVP Spontaneous venous pulsations
SWI Susceptibility-weighted imaging
TAC Trigeminal autonomic cephalalgias
TACS Total anterior circulation syndromes
TBE Tick-borne encephalitis
TCA Tricyclic antidepressant
TEE Transesophageal echocardiography
TENS Transepidermal nerve stimulation
TGA Transient global amnesia
TIA Transitory ischemic attack
TMS Transcranial magnetic stimulation
TPO Thyroid peroxidase
TTE Transthoracic echocardiography
TTR-FAPs Transthyretin familial amyloid polyneuropathies
UMN Upper motor neuron
UWS Unresponsive wakefulness syndrome
vCJD Variant form of Creutzfeldt-Jakob disease
Abbreviations xxiii
VDRL Venereal Disease Research Laboratory

VEP Visual evoked potentials
VGCC Voltage-gated calcium channel
VGKC Voltage-gated potassium channel
VN Vestibulocochlear nerve
VOR Vestibulo-ocular reflex
VOR Volume of interest
VS Vegetative state
VZV Varicella zoster virus
w Week
WMSN Wartenberg’s migrant sensory neuritis
× Times
μg Microgram
μmol Micromol
μV Microvolt
List of Cases
Case 2.1 Botulism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Case 2.2 Split-hand sign in amyotrophic lateral sclerosis. . . . . . . . . . 17
Case 2.3 Pancoast tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
Case 2.4 Compressive cervical medullopathy in Down syndrome. . . 27
Case 2.5 Conus medullaris metastasis. . . . . . . . . . . . . . . . . . . . . . . . . 32
Case 2.6 Brainstem glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
Case 2.7 Facial synkinesis after Bell’s palsy. . . . . . . . . . . . . . . . . . . . 46
Case 2.8 Tuberculosis of the CNS. . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Case 2.9 Hypoglossal nerve palsy due to carotid artery dissection . . . . 51
Case 2.10 Crossed cerebellar diaschisis. . . . . . . . . . . . . . . . . . . . . . . . . 52
Case 2.11 Parinaud’s syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
Case 2.12 Wernicke’s encephalopathy. . . . . . . . . . . . . . . . . . . . . . . . . . 57
Case 2.13 Normal pressure hydrocephalus. . . . . . . . . . . . . . . . . . . . . . 59
Case 2.14 Alexia without agraphia . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
Case 2.15 Behavioral variant frontotemporal dementia
(bvFTD) with disinhibition�� 62
Case 2.16 Pure word deafness due to bitemporal infarctions . . . . . . . . 63
Case 2.17 Semantic dementia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64
Case 2.18 Transient global amnesia. . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Case 2.19 Balint’s syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Case 2.20 Anton’s syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
Case 2.21 Palinopsia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69
Case 2.22 Pediatric basilar artery thrombosis. . . . . . . . . . . . . . . . . . . . 72
Case 2.23 Top-of-the-basilar stroke. . . . . . . . . . . . . . . . . . . . . . . . . . . . 74
Case 2.24 Time-space synesthesia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75
Case 3.1 Alzheimer’s disease with atypical onset
(behavioral symptoms) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
Case 3.2 Idiopathic intracranial hypertension
and spontaneous CSF fistula. . . . . . . . . . . . . . . . . . . . . . . . . 89
Case 3.3 Carotid-cavernous fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Case 3.4 Vertebral artery dissection. . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Case 3.5 Focal epilepsy due to Sturge-Weber syndrome. . . . . . . . . . . 103
Case 3.6 Blue rubber bleb nevus syndrome. . . . . . . . . . . . . . . . . . . . . 104
Case 3.7 Raccoon eyes and Battle’s sign. . . . . . . . . . . . . . . . . . . . . . . 106
xxv
xxvi List of Cases
Case 3.8 Cerebral fat embolism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108

Case 3.9 Type A aortic dissection. . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
Case 3.10 Subdural hemorrhage due to ruptured
intracranial aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111
Case 3.11 Dialysis disequilibrium syndrome. . . . . . . . . . . . . . . . . . . . . 115
Case 3.12 Fatal cerebral edema due to inborn error of metabolism. . . . 116
Case 3.13 Wartenberg’s migrant sensory neuritis . . . . . . . . . . . . . . . . . 124
Case 4.1 Herpes simplex encephalitis misdiagnosed as stroke. . . . . . 129
Case 4.2 Cerebral venous sinus thrombosis and hyperthyroidism. . . 133
Case 4.3 Reversible cerebral vasoconstriction syndrome (RCVS). . . . 140
Case 4.4 Reversible cerebral vasoconstriction syndrome
(RCVS) with fatal outcome. . . . . . . . . . . . . . . . . . . . . . . . . . 141
Case 4.5 Spontaneous intracranial hypotension
and spinal CSF leak. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142
Case 4.6 Behavioral variant frontotemporal dementia (bvFTD). . . . . 148
Case 4.7 Progressive nonfluent aphasia (PNFA). . . . . . . . . . . . . . . . . 149
Case 4.8 Huntington’s disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150
Case 4.9 Progressive multifocal leukoencephalopathy (PML) . . . . . . 151
Case 4.10 Creutzfeldt-Jakob disease. . . . . . . . . . . . . . . . . . . . . . . . . . . 152
Case 4.11 Limbic encephalitis with anti-LGI1 antibodies. . . . . . . . . . . 157
Case 4.12 N-Methyl-d-Aspartate receptor (NMDAr) encephalitis. . . . 158
Case 4.13 Post-herpetic NMDAR encephalitis. . . . . . . . . . . . . . . . . . . 159
Case 4.14 Posterior reversible encephalopathy syndrome (PRES). . . . 160
Case 4.15 Moyamoya disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167
Case 4.16 Pheochromocytoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169
Case 4.17 Amyloid spells . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170
Case 4.18 Ruptured intracranial dermoid cyst. . . . . . . . . . . . . . . . . . . . 173
Case 4.19 Narcolepsy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175
Case 4.20 Cerebral autosomal dominant arteriopathy with
subcortical infarcts and leukoencephalopathy (CADASIL). . . 179
Case 4.21 Susac’s syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180
Case 4.22 Mycotic aneurysms due to endocarditis and myxoma . . . . . 182
Case 4.23 Moyamoya phenomenon due to Fanconi anemia. . . . . . . . . 184
Case 4.24 Repetitive ischemic and hemorrhagic strokes due
to COL4A1 mutation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186
Case 4.25 Paraneoplastic stroke. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
Case 4.26 Mitochondrial Encephalomyopathy,
Lactic Acidosis, and Stroke-like episodes (MELAS). . . . . . 188
Case 4.27 Intracranial dissection following traumatic brain injury. . . . 189
Case 4.28 Cerebral amyloid angiopathy . . . . . . . . . . . . . . . . . . . . . . . . 191
Case 4.29 Multiple sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 195
Case 4.30 Neuromyelitis optica. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199
Case 4.31 Neuro-Behçet . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 200
Case 4.32 Rhombenencephalitis due to Listeria monocytogenes . . . . . 201
Case 4.33 Toxic leukoencephalopathy due to cocaine. . . . . . . . . . . . . . 202
Case 4.34 HIV encephalitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205
Case 4.35 Diffusion-weighted MRI in the assessment
of brain abscesses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
List of Cases xxvii
Case 4.36 Cranial neuropathy due to malignant external otitis. . . . . . . 210

Case 4.37 Neurocysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212
Case 4.38 Cerebral malaria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213
Case 4.39 Gliomatosis cerebri. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216
Case 4.40 Craniopharyngeoma and obstructive hydrocephalus . . . . . . 218
Case 4.41 Primary CNS lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . 219
Case 4.42 Anti-Ma2-associated paraneoplastic
brainstem encephalitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
Case 4.43 Multisystem atrophy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224
Case 4.44 Progressive supranuclear palsy. . . . . . . . . . . . . . . . . . . . . . . 224
Case 4.45 Carbon monoxide poisoning (interval form). . . . . . . . . . . . . 227
Case 4.46 Superficial CNS siderosis. . . . . . . . . . . . . . . . . . . . . . . . . . . 236
Case 4.47 Chronic Lymphocytic Inflammation with
Pontine Perivascular Enhancement Responsive
to Steroids (CLIPPERS). . . . . . . . . . . . . . . . . . . . . . . . . . . . 237
Case 4.48 Adult-onset adrenoleukodystrophy. . . . . . . . . . . . . . . . . . . . 240
Case 4.49 Spinal epidural abscess. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244
Case 4.50 Spinal arteriovenous dural fistula. . . . . . . . . . . . . . . . . . . . . 245
Case 4.51 Cervical flexion myelopathy. . . . . . . . . . . . . . . . . . . . . . . . . 248
Case 5.1 Mild encephalopathy with a reversible splenial
lesion (MERS). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264
Case 5.2 Clinically silent middle cerebral artery occlusion. . . . . . . . . 265
Case 5.3 Idiopathic hypertrophic pachymeningitis. . . . . . . . . . . . . . . 265
Case 5.4 Radiation toxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266
Case 5.5 Alzheimer’s disease with atypical onset
(posterior cortical atrophy). . . . . . . . . . . . . . . . . . . . . . . . . . 268
Case 5.6 Dopamine transporter (DAT) imaging
in Parkinson’s disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269
Case 5.7 Neurosarcoidosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288
Case 6.1 Thrombectomy for middle cerebral artery occlusion. . . . . . 306
Case 6.2 Hemicraniectomy for malignant middle cerebral
artery infarction�� 309
Case 6.3 Surgical decompression for space-occupying
cerebellar stroke �� 311
Case 6.4 Hemicraniectomy for cerebral sinus venous
thrombosis�� 314
What to Expect from This Book
(and What Not to) 1
Abstract
Neurologists take pride in their clinical bedside skills, perhaps even more
so than other physicians. Good clinical mentorship is mandatory in order
to develop such skills; however, due to an ever-increasing need for more
efficient working structures, the time and dedication that mentorship
requires may not always be available. The authors have therefore written
this book for neurology residents who are looking for a personal clinical
mentor, guiding them through the entire patient encounter: from a compre-
hensive history and an efficient clinical examination to a thorough differ-
ential diagnosis, ancillary investigations, and finally treatment options.
Keywords
Clinical skills • Clinical neurology • Examination • Differential diagnosis
• Examination • Mentorship • Neuroanatomy • Patient history • Treatment
Few things in medicine are as fascinating as Neither a traditional textbook nor a pocket
watching an experienced neurologist perform a manual, the aim of this book is to act as a clinical
history and bedside examination to generate a dif- mentor and provide information otherwise diffi-
ferential diagnosis prior to any laboratory investi- cult to look up in the usual reference sources. It
gations. Good clinical skills are mandatory when tries to answer the sort of questions neurology
it comes to placing the patient on the right diag- trainees typically would ask their consultants.
nostic track and to interpreting laboratory results
correctly. These skills are acquired through regu- • How do I distinguish clinically between myas-
lar training, in-depth theoretical knowledge, and thenia gravis and Lambert-Eaton myasthenic
good mentorship. The neurology trainee is syndrome?
responsible for the first two aspects, while good • What is the differential diagnosis of a rapidly
clinical mentorship requires a dedicated consul- progressive dementia if the initial workup is
tant who may not always be available. negative?
© Springer International Publishing AG 2017 1

D. Kondziella, G. Waldemar, Neurology at the Bedside, DOI 10.1007/978-3-319-55991-9_1
2 1 What to Expect from This Book (and What Not to)
• Which anatomical lesions can lead to Horner’s mary and secondary headache syndromes, the lat-
syndrome? ter of which can be further divided into intracranial
• Which neurophysiological features distinguish and extracranial disorders and so on.
axonal from demyelinating polyneuropathy? Second, this chapter provides a comprehen-
• How do I treat autonomic dysreflexia in a sive differential diagnosis of most of the disor-
patient with chronic spinal injury? ders encountered in neurological practice. Due to
space limitations, the background information is
To this end, the approach of the present book limited, but the chances are minimal that a
reflects the course of the neurological consulta- patient, even at tertiary care level, has a neuro-
tion. The history, bedside examination, and gen- logical diagnosis not covered by this chapter.
eration of a working diagnosis are discussed first, Chapter 5 provides a short overview of the
followed by a review of laboratory investigations main laboratory investigations performed in neu-
and medical treatment (Fig. 1.1). rology with emphasis on the clinical aspects.
Like any clinical mentor, this book demands Chapter 6 offers a reference of medical, surgi-
an active effort and commitment from you. It is cal, and other treatment options for most neuro-
assumed that you have at least a superficial logical conditions. Again, this chapter is written
knowledge of neurology and that you will consult from a clinical mentor’s point of view. It sug-
a good reference source if needed. gests the medication and dosage that may be
Chapters 2 and 3 review the relevant neuro- chosen for a given condition but assumes that the
anatomy from a clinical point of view and pro- reader is familiar with the pharmacodynamic
vide the tools to obtain an efficient clinical history and pharmacokinetic data, contraindications,
and bedside examination. These chapters can be and side effects. The main focus of this chapter
read straightforwardly from the first to the last is to guide the residents on call. Thus, the empha-
page. sis is on treatment of cerebrovascular disorders
Chapter 4 is somewhat more complicated and and epilepsy. Neurorehabilitation, counseling,
demands greater flexibility. The chapter’s aim is and regular follow-up, which should be an inte-
twofold. gral part of neurological management, will not
First, using a specific clinical syndrome as its be covered.
case, it intends to teach the reader how to approach The authors are grateful for any comments
differential diagnosis in a structured manner. This and suggestions for improving future editions.
is the bird’s-eye view. Although there are hun- Also, they hope that upon completion of your
dreds of etiologies to a given polyneuropathy, neurology training, you will know most of the
most can be diagnosed using a simple three-step content of this publication like the back of your
approach. Headache can be classified into pri- hand.
3
To master the clinically relevant neuroanatomy

To recognize features in the clinical history pointing towards a specific anatomical site
Chapter 2
Clinical history and neuroanatomy
To learn how to ask for these features during the history
To learn how to generate an “anatomical hypothesis”
Chapter 3 To be able to perform an efficient bedside examination
“Neurology at the bedside”: learning objectives
Neurological bedside examination

To be able to modify the bedside examination according to the history and the “anatomical hypothesis”
To learn how to approach the differential diagnosis of neurological disorders systematically
Chapter 4
Differential diagnosis
What to Expect from This Book (and What Not to)
To be able to generate a working diagnosis for (almost) every neurological complaint
Fig. 1.1 Summary of chapter learning objectives

To understand the principles of the common ancillary investigations used in clinical neurology
Chapter 5
Ancillary investigations
To be able to interpret the results of ancillary investigations correctly
Chapter 6
To know the most common treatment options for neurological disorders
Treatment of neurological disorders
1
Clinical History and
Neuroanatomy: “Where Is 2
the Lesion?”
Abstract
The first step in the management of the neurological patient is to localize
the lesion. While taking the history, the neurologist generates an anatomi-
cal hypothesis, which subsequently can be confirmed or rejected during
the bedside examination. Following this, a working diagnosis is estab-
lished and ancillary tests are chosen accordingly. Although the anatomy of
the nervous system is highly complex, distinct anatomical entities have
characteristic features. For instance, the hallmark of a myopathy is sym-
metric proximal weakness without sensory disturbances. Fatigability
together with proximal weakness, including bulbar and oculomotor fea-
tures, is typical for a disorder of the neuromuscular junction. In contrast,
diseases of peripheral nerves, the brachial and lumbosacral plexus, as well
as nerve roots usually lead to both motor and sensory deficits. Further,
injury to the spinal cord is associated with a triad of paraparesis, a sensory
level of the trunk, and sphincter disturbances. Brainstem processes often
produce ipsilateral cranial nerve deficits and contralateral sensorimotor
signs. While damage of the cerebellar hemispheres causes ataxia and
intention tremor of the ipsilateral extremity, lesions of the midline region
mainly lead to gait ataxia and truncal instability. Movement disorders due
to disease involving the basal ganglia can be divided into hypo- and hyper-
kinetic disorders. Lesions involving the subcortical white matter fre-
quently induce visual field deficits, complete hemiplegia, and dense
numbness. Impairment of higher cognitive function, incomplete hemipa-
resis (sparing the leg), and epileptic seizures are common signs of cortical
disease. This chapter reviews the relevant neuroanatomy from a clinical
viewpoint and provides the reader with the tools to perform a competent
clinical history.
Keywords
Basal ganglia • Brachial plexus • Lumbosacral plexus • Brainstem
• Cerebellum • Cortex • Cranial nerves • Muscle • Nerve roots
• Neuromuscular synapse • Peripheral nerves • Spinal cord • Subcortical
white matter • Thalamus
© Springer International Publishing AG 2017 5

D. Kondziella, G. Waldemar, Neurology at the Bedside, DOI 10.1007/978-3-319-55991-9_2
6 2 Clinical History and Neuroanatomy: “Where Is the Lesion?”
The neurological history differs from the his- The subcortical white matter and the cortex
tory in other medical specialties insofar as it is can be further differentiated into:
primarily anatomy based. When examining a
new patient, the first question a neurologist • Frontal lobes
attempts to answer is, “Where is the lesion?” • Temporal lobes
The main principle is to use the history to gen- • Parietal lobes
erate an anatomical hypothesis and to use the • Occipital lobes
bedside examination to confirm this hypothesis.
Following this, other features of the history, Many diseases can be classified according to
such as epidemiological data and the speed of which of these entities they affect. For instance,
symptom development, are taken into account myasthenia gravis (MG) is a disease of the neuro-
in order to answer the next question, “What is muscular synapse, while Alzheimer’s diesease
the lesion?” Thereafter, the neurologist forms a (AD) and epilepsy are mainly disorders of corti-
differential diagnosis and a working diagnosis cal function. Importantly, each anatomical unit
and then accordingly orders the most relevant has a specific symptomatology that can be elic-
laboratory tests. Strictly adhering to this schema ited during the history. The neurologist can there-
allows for a safe and rapid diagnostic proce- fore “examine” the patient from the muscle to the
dure. Obviously, in many cases, the experi- cortex solely by performing a good neurological
enced neurologist uses a shortcut called instant history. The essential anatomical and clinical fea-
pattern recognition to reach a diagnosis. Yet, tures are summarized in the following pages and
when confronted with a difficult diagnostic in Table 2.1.
problem, nothing is more useful than to return Although it is of preeminent importance not to
to the bedside and take a more detailed history. push the patient into reporting the symptoms that
Also, the history is more likely to lead to the one is trying to elicit, the history must be meticu-
correct diagnosis than the physical examina- lous and detailed. A useful clinical rule is that
tion. Therefore, as a rule, more time should be after finishing the history, one should have a clear
devoted to the history compared to the bedside and detailed idea of what has happened and be
examination. able to fully visualize the sequence of events. The
In order to obtain a neurological history, it is focus should be on the principal symptoms and
helpful to divide the complexity of the nervous signs; the neurologist should not let minor find-
system’s anatomy into small manageable entities ings and uncertain clinical data distract from the
(Fig. 2.1). From peripheral to central, these greater picture. (Admittedly, this is not easy with-
include: out experience.) If the patient has many different
and seemingly unrelated complaints, it is useful
• Muscle to ask what bothers him most and then concen-
• Neuromuscular synapse trate on this complaint.
• Peripheral nerves Taking a history is difficult in patients with a
• Brachial and lumbosacral plexus disorder that affects the level of consciousness,
• Nerve roots communication skills, and/or cognitive function,
• Spinal cord e.g., due to amnesia, aphasia, impaired judgment,
• Cranial nerves (CNs) lack of insight, and confabulation. A patient with
• Brainstem AD, for instance, may not be able to explain his
• Cerebellum symptoms or the course of the disease; if he can,
• Subcortical gray matter such as the thalamus it might well be that not all of the information can
and basal ganglia be taken at face value. Likewise, the aphasic
• Subcortical white matter patient following a left middle cerebral artery
• Cortex (MCA) occlusion may have difficulties under-
2 Clinical History and Neuroanatomy: “Where Is the Lesion?” 7
Nervous system
PNS CNS
Nerve roots
Spinal cord
Brainstem
Cerebellum
Subcortical gray matter
Subcortical white matter
Cortex
functions
autonomic
Sensory/
Brachial/lumbosacral plexus
Peripheral nerves
Neuromuscular junction
Muscle
Medulla oblongata
Pons
Mesencephalon
Occipital lobes
Parietal lobes
Temporal lobes
Frontal lobes
Cranial nerves
functions
autonomic
Sensory/
Fig. 2.1 The nervous system’s anatomy can be divided into small manageable entities. Each anatomical unit has a
specific symptomatology that can be elicited during the history
standing the examiner’s questions, or he may despite an obvious inability to perform basic
well understand but be unable to formulate an activities of daily living. In contrast, a patient
appropriate answer. Further, a patient with a fron- with locked-in syndrome due to a large pontine
tal brain tumor may have become so apathetic infarct following a basilar artery thrombosis is
and indifferent that he might not complain at all awake and may fully understand the examiner but
8 2 Clinical History and Neuroanatomy: “Where Is the Lesion?”
Table 2.1 Summary of key anatomic features for history taking and bedside examination
Muscle: Proximal, symmetric weakness; no sensory symptoms
Neuromuscular junction: Fatigability; proximal, symmetric (or asymmetric) weakness; no sensory symptoms
Peripheral nerve: Sensory symptoms; distal, asymmetric weakness (except for symmetric polyneuropathy); normal
or decreased muscle tone; hyporeflexia; eventually atrophy and fasciculations; motor and sensory deficits correlate
to peripheral nerve distribution; sympathetic function (sweating) may be disturbed
Brachial plexus, lumbosacral plexus: Sensory symptoms; often distal greater than proximal weakness; normal or
decreased muscle tone; hyporeflexia; eventually fasciculations and atrophy; motor and sensory deficits are neither
consistent with a single peripheral nerve distribution nor with a specific dermatome/myotome; sympathetic function
(sweating) may be disturbed; radiculopathic pain is common
Nerve root: Sensory symptoms; distal greater than proximal weakness; normal or decreased muscle tone;
hyporeflexia; possibly atrophy and occasionally fasciculations; motor and sensory deficits associated with specific
dermatome/myotome; radiculopathic pain; sympathetic function normal
Spinal cord:
Complete or near-complete transection or myelitis: Triad of paraparesis (spastic below site of lesion), sensory level,
and sphincter disturbances
Anterior cord syndrome: Dissociated sensory loss with preserved vibration and proprioception but impaired
perception of pain and temperature; paraparesis
Dorsal cord syndrome: Impairment of vibration and proprioception
Brown-Séquard syndrome (=hemitransection): Ipsilateral paresis; ipsilateral loss of vibration and proprioception;
contralateral loss of pain and temperature
Syringomyelia (most often enlargement of cervicothoracic central cord): Dissociated sensory loss with bilateral
impaired perception of pain and temperature; later bilateral paresis due to injury to nucleus of second motor neuron
Brainstem and cranial nerves: The brainstem includes (a) mesencephalon, (b) pons, and (c) medulla oblongata and
consists of (x) all long afferent and efferent tracts, (y) vegetative centers (e.g., respiration), and the ARAS (arousal)
and (z) nuclei of CN III–XII (mesencephalon III–V, pons V–VIII, medulla oblongata V and IX–XII). Thus,
brainstem reflexes can be used to locate the lesion on a vertical axis (e.g., pupillary reflex, in, CN II, out, III;
corneal and eyelash reflexes, in, V, out, VII; vestibulo-cephalic reflex and VOR, in, VIII, out, III and VI; and gag
reflex, in, IX, out, X). Lesions often lead to ipsilateral CN and contralateral long tract signs, e.g., ipsilateral,
peripheral (!) facial palsy, and contralateral hemiparesis. (However, if lesions affect the site above the nucleus of
the facial nerve, there may be a contralateral, central facial palsy.) Other typical brainstem symptoms are, e.g.,
dysphagia, diplopia, and dysarthria (but not dysphasia, which is a cortical phenomenon)
Cerebellum: Cerebellar hemispheric lesions lead to ipsilateral (!) cerebellar ataxia and intention tremor. Cerebellar
midline lesions lead to gait and truncal ataxia (without severe ataxia of the extremities)
Subcortical gray matter: Consists of basal ganglia, thalamus, and hypothalamus; deficit of dopamine functions leads
to parkinsonism (tremor, bradykinesia, rigidity, postural instability) and dopamine hyperfunction to, e.g., chorea
Subcortical white matter: Visual field deficits, dense numbness, complete hemiparesis, executive dysfunction, and
decreased psychomotor speed
Cortex: Impairment of higher cognitive function, including amnesia, aphasia, apraxia, visuospatial deficits, and
neglect; epileptic seizures; incomplete hemiparesis sparing the leg; frontal lobe (executive dysfunction, personality
impairment, hemiparesis due to lesions of the supplemental motor area and primary motor cortex, nonfluent or motor
dysphasia); temporal lobe (auditory hallucinations due to lesions of the primary auditory cortex, memory disturbance
with damage to the hippocampal formation, rising epigastric sensation and automatisms with temporal lobe seizures,
fluent or sensory dysphasia); parietal lobe (visuospatial disorientation, sensory hemisymptoms due to lesions of the
sensory cortex); and occipital lobe (visual hallucinations and cortical blindness with damage to primary visual cortex)
has lost all efferent motor control except for a ate the cognitive capabilities of the patient and to
few eye movements. Similarly, patients with ter- find appropriate means of communication, e.g.,
minal amyotrophic lateral sclerosis (ALS) or by establishing a reliable code for locked-in
fulminant Guillain-Barré syndrome (GBS) may patients to indicate yes and no using blinking or
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Very true—swear him, added another.
You’ll excuse me, said the Attorney-general. I say, you—what’s your
name?
Jerry Smith.
And you appear on the side of the prisoner at the bar, I take it?
Well, what if I do?
Why in that case, you see, you are not to be sworn, that’s all.
Not sworn! cried Burroughs. And why not Sir?
Why we never allow the witnesses that appear against the crown, to
take the oath.
Against the crown Sir! what on earth has the crown to do here?—
what have we to do with such absurdity?
Have a care, brother Burroughs!
Do you know Sir—do you know that, if this man be not allowed to
say what he has to say on oath, less credit will be given to what he
says?
Can’t help that Sir.—Such is the law.
Judges—judges—do ye hear that?—can this be the law? Will you
give the sanction of oaths to whatever may be said here against life?
—and refuse their sanction to whatever may be said for life? Can
such be the law?
The judges consulted together and agreed that such was the law, the
law of the mother-country and therefore the law of colonies.
Of a truth, said Burroughs, in reply; of a truth, I can perceive now
why it is, if a man appear to testify in favor of human life that he is
regarded as a witness against the crown.—God help such crowns, I
say!
Brother!—dear brother!
God help such crowns, I say! What an idea of kingship it gives! What
a fearful commentary on the guardianship of monarchs! How much it
says in a word or two of their fatherly care! He who is for the subject,
even though a life be at stake, is therefore against the king!
Beware of that Sir.—You are on the very threshold of treason.
Be it so.—If there is no other way, I will step over that threshold—.
If you do Sir, it will be into your grave.—
Sir!—
Dear brother, I beseech you!
Enough—enough—I have nothing more to do—nothing more to say,
Sir—not another word, Sir—forgive me Sir—I—I—I—the tears of the
aged I cannot bear; the sorrow of such as are about to go before
God, I am not able, I never was able to bear. I beseech you,
however, to look with pity upon the poor soul there—poor Martha!—
let her gray hairs plead with you, as your gray hairs plead with me—I
—I—proceed, Mr. Attorney-General.
I have nothing more to say?
Nothing more to say!
With submission to the court—nothing.
Do you throw up the case then? said a judge.
Throw up the case! no indeed—no!—But if Mr. Counsellor Burroughs
here, who has contrived in my humble opinion, to make the
procedure of this court appear—that is to say—with all due
submission—appear to be not much better than a laughing-stock to
the—to the—to my brethren of the bar—if Mr. Burroughs, I say, if he
has nothing more to say—I beg leave to say—that is to say—that I
have nothing more to say—.
Say—say—say—whispered one of his brethren of the bar—what say
you to that Mr. Burroughs?
CHAPTER VIII.
What should I say? replied Burroughs. What would you have me
say? standing up and growing very pale. What would you have me
say, you that are of counsel for the prisoner, you! the judges of the
court? You that appear to rejoice when you see the last hope of the
prisoner about to be made of no value to her, by the trick and
subterfuge of the law. Why do you not speak to her?—Why do you
not advise me? You know that I depend upon the reply—You know
that I have no other hope, and that she has no other hope, and yet
you leave us both to be destroyed by the stratagem of an adversary.
How shall I proceed? Speak to me, I entreat you! Speak to me
judges! Do not leave me to grope out a path blind-folded over a
precipice—a path which it would require great skill to tread—O, I
beseech you! do not leave me thus under the awful, the tremendous
accountability, which, in my ignorance of the law, I have been
desperate enough to undertake!—Here by my side are two men of
the law—yet have you assigned her, in a matter of life and death, no
counsel. They are afraid I see—afraid not only to rise up and speak
for the wretched woman, but they are afraid even to whisper to me.
And you, ye judges! are you also on the side of the prosecutor and
the witnesses—are you all for the king?—all!—all!—not so much as
one to say a word for the poor creature, who being pursued for the
king, is treated as if she were pursued by the king—pursued by him
for sacrifice! What! no answer—not a word! What am I to believe? ...
that you take pride in the exercise of your terrible power? that you
look upon it as a privilege? ... that you regard me now with
displeasure ... that if you could have your own way, you would permit
no interference with your frightful prerogative?... O that I knew in
what way to approach the hearts of men! O that I knew how to
proceed in this affair! Will nobody advise me!
Sir—Sir!—allow me, said a man of the law who sat near, allow me
Sir; I can bear it no longer—it is a reproach to the very name of law
—but—but (lowering his voice) if you will suffer me to suggest a step
or two for your consideration—you have the courage and the power
—I have not—my brethren here have not—you have—and you may
perhaps be able to—hush, hush—to bring her off.
Speak out, Sir—speak out, I beseech you. What am I to do?
Lower if you please—lower—low——er—er—er—we must not be
overheard—Brother Trap’s got a quick ear. Now my notion is—allow
me—(whispering) the jury are on the watch; they have heard you
with great anxiety—and great pleasure—if you can manage to keep
the hold you have got for half an hour—hush—hush—no matter how
—the poor soul may escape yet—
I’ll address the jury—
By no manner of means! That will not be suffered—you cannot
address the jury—
Good God! what shall I do!
Thirteen-pence more—carry five—paid to watchman.
I’ll put you in the way (with a waggish leer.) Though you are not
allowed to address the jury, you are allowed to address the court—
hey?—(chucking him with his elbow)—the court you see—hey—sh!
—sh!—you understand it—hey?
No—how cool you are!
Cool—you’d be cool too, if you understood the law.
Never—never—in a case of life and death.
Life and death? poh—everything is a case of life and death, Sir—to a
man o’ the law—everything—all cases are alike, Sir—hey—provided
—a—a—
Provided what, Sir?
Where the quid is the same.
The quid?
The quid pro quo—
How can you, Sir?—your levity is a—I begin to be afraid of your
principles—what am I to do?
Do—just keep the court in play; keep the judges at work, while I run
over to the shop for an authority or two I have there which may be of
use.—You have the jury with you now—lay it on thick—you
understand the play as well as I do now—
Stop—stop—am I to say to the judges what I would say to the jury, if
I had leave?
Pre—cisely! but—but—a word in your ear—so as to be heard by the
jury.—Tut—tut—
The head-prosecutor jumped up at these words, and with a great
show of zeal prayed the judges to put a stop to the consultation, a
part of which was of a character—of a character—that is to say, of a
character—
Burroughs would have interrupted him, but he was hindered by his
crafty law-adviser, who told him to let the worthy gentleman cut his
own throat in his own way, now he was in the humor for it.
Burroughs obeyed, and after his adversary had run himself out of
breath, arose in reply, and with a gravity and a moderation that
weighed prodigiously with the court, called upon the chief-judge to
put a stop to such gladiatorial controversy—
What would you have us do? said the judge.
I would have you do nothing more than your duty—
Here the coadjutor of Burroughs, after making a sign to him to face
the jury, slid away on tip-toe.
—I would have you rebuke this temper. Ye are the judges of a great
people. I would have you act, and I would have you teach others to
act, as if you and they were playing together, in every such case—
not for your own lives—that were too much to ask of mortal man; but
for another’s life. I would have you and your officers behave here as
if the game that you play were what you all know it to be, a game of
life and death—a trial, not of attorney with attorney, nor of judge with
judge, in the warfare of skill, or wit, or trick, or stratagem, for fee or
character—but a trial whereby the life here, and the life hereafter it
may be, of a fellow-creature is in issue. Yea—more—I would have
you teach the king’s Attorney-General, the prosecutor himself, that
representative though he be of majesty, it would be more dignified
and more worthy of majesty, if he could contrive to keep his temper,
when he is defeated or thwarted in his attack on human life. We may
deserve death all of us, but we deserve not mockery; and whether
we deserve death or not, I hope we deserve, under our gracious
Lord and Master, to be put to death according to law—
That’ll do!—that’ll do!—whispered the lawyer, who had returned with
his huge folios—that’ll do my boy! looking up over his spectacles and
turning a leaf—that’ll do! give it to ’em as hot as they can sup it—I
shall be ready for you in a crack—push on, push on—what a capital
figure you’d make at the bar—don’t stop—don’t stop.
Why, what on earth can I say!
Talk—talk—talk—no matter what you say—don’t give them time to
breathe—pop a speech into ’em!
A speech!
Ay, or a sermon, or a whar-whoop, or a prayer—any thing—anything
—if you do but keep the ball up—no matter-what, if the jury can hear
you—they are all agog now—they are pricking up their ears at you—
now’s your time!
Very well——Judges!
Proceed.
Judges. I am a traveller from my youth up. I have journied over
Europe; I have journied over America—I am acquainted with every
people of both hemispheres, and yet, whithersoever I go, I am a
stranger. I have studied much—thought much—and am already a
show among those who watched over my youth. I am still young,
though I appear old, much younger than you would suppose me to
be, did you not know me—
Here he turned to the lawyer—I never shall be able to get through
this; I don’t know what I am saying.—
Nor I—So much the better—don’t give up—
——A youth—a lad in comparison with you, ye judges, you that I
now undertake to reprove——a spectacle and a show among men.
They follow me every where, (I hope you’ll soon be ready) they
pursue me day after day—and week after week—and month after
month—
—And year after year—by jings, that’ll do!—
—And year after year; they and their wives, and their little ones—
And their flocks and their herds, and their man-servants and their
maid-servants, whispered the lawyer.
Do be quiet, will you.—They pursue me however, not because of
their veneration or their love, but only that they may study the
perpetual changes of my countenance and hear the language of one
to whom all changes and all languages are alike, and all beneath
regard. They follow me too, not because they are able to interpret
the look of my eyes, or to understand the meaning of my voice, but
chiefly because they hear that I have been abroad in the furthermost
countries of all the earth, because they are told by grave men, who
catch their breath when they speak of me, though it be in the House
of the Lord, as you have seen this very day, that I have been familiar
with mysterious trial and savage adventure, up from the hour of my
birth, when I was dropped in the wilderness like the young of the
wild-beast, by my own mother—
I say—Brother B.—I say though—whispered the lawyer, in much
perplexity—I say though—what are you at now? You are not on trial
—are you?
Yes—yes—let me alone, I beseech you....
Fire away ... fire away ... you’ve got possession of the jury, and that’s
half the battle ... fire away.
Peace ... peace, I pray you ... Judges! whenever I go abroad ...
wherever I go ... the first place into which I set my foot, is the tribunal
of death. Go where I may, I go first in search of the courts ... the
courts of justice, I should say, to distinguish them from all other
courts—
Good!—
—And I go thither because I have an idea that nations are to be
compared with nations, not in every thing—not altogether, but only in
a few things; and because after much thought, I have persuaded
myself that matters of religion, politics and morals, are inadequate
for the chief purposes of such comparison—the comparison of
people with people, though not for the comparison of individual with
individual perhaps; and that a variety of matters which regard the
administration of law, in cases affecting either life or liberty, are in
their very nature adequate, and may be conclusive. We may
compare court with court and law with law; but how shall we
compare opinion with opinion, where there is no unchangeable
record of either? goodness with goodness—where goodness itself
may be but a thing of opinion or hearsay, incapable of proof, and
therefore incapable of comparison?
Very fair—very fair—but what on earth has it to do with our case?
Wait and you shall see; I begin to see my way clear now—wherefore
judges, I hold that the liberty of a people and therefore the greatness
of a people may be safely estimated by the degree of seriousness
with which a criminal is arraigned, or tried, or judged, or punished—.
—Very true—and very well spun out, brother B.... but a non sequitur
nevertheless. That wherefore, with which you began the period was
a bit of a——
Pray—pray—don’t interrupt me; you will be overheard—you will put
me out.—In a word, ye Judges of Israel! I have had a notion that
arbitrary power would betray itself in every case, and every-where on
earth, by its mode of dealing with liberty and life—being, I persuade
myself, more and more summary and careless, in proportion as it is
more and more absolute of a truth, not as it is more and more
absolute by character. You had for a time, while the northern
savages were at your door, a downright military government.—You
know therefore that my words are true. Your government was called
free—to have called it arbitrary, would have offended you; yet for a
season you dealt with human life as the Turk would. You know, for
you have seen the proof, that in proportion to the growth of power in
those who bear sway among you, the forms and ceremonies which
fortify and hedge in, as it were, the life and liberty of the subject, are
either disregarded or trampled on.—
Oh ho!—I see what you are driving at now!
—For my own part, I love to see the foreheads of them who are
appointed to sit in the high-places and give judgment forever upon
the property or character, life or liberty of their fellow man.—
Property or character—life or liberty—of a fellow man! Very fair—
very fair indeed.
—Expressive at least of decent sorrow, if not of profound awe. I
would have them look as if they were afraid—as if they trembled
under the weight of their tremendous authority; as if they were
deeply and clearly and reverentially sensible of what they have
undertaken to do—which is, to deal with the creatures of God, as
God himself professes to deal with them—according to their
transgressions—to do a part of his duty with his own Image—to
shelter the oppressed and to stay the oppressor, not only now and
for a time, but hereafter and forever—
Don’t stop to breathe now; I shall be at your back in a jiffy—
I would that every man who has to do with the administration of law,
wherever that law is to touch the life or liberty of another; and
whoever he may be, from the highest judge in the highest court of all
the earth, down to the humblest ministerial officer—I would that he
should feel, or at least appear to feel, that for a time he is the
delegate of Jehovah—I do not stop to say how, nor to ask why. That
is for others to say.—I would have the judges remarkable for their
gravity, not for their austerity; for their seriousness and for their
severe simplicity, not for a theatrical carriage. I would have the bar,
as you call it, above the trick and subterfuge of the law—incapable of
doing what I see them do every day of my life; and I would have the
bench as you call it, incapable of suffering what I see them not only
suffer, but take pleasure in, every day of my life——are you ready?
Persevere—persevere—you may say what you please now, said the
lawyer, shuffling his papers about with both hands, chuckling in his
sleeve, and whispering without appearing to whisper.—Have your
own way now ... they like to hear the lawyers and the judges, and the
law cut up; it’s a new thing to hear in such a place ... fire away, fire
away ... you see how they enjoy it ... you’ve got us on the hip now ...
fire away.
If a criminal be arraigned on a charge that may affect his life or
character, limb or property, or if a witness be to be sworn, or the oath
administered, ... I care not how ... I care not why ... if you will have
oaths ... ye should order silence to be proclaimed by the sound of
trumpet.
—Pho! pho!
I would have a great bell, one so large that it might be heard far and
wide over the whole town—I would have this tolled on the day of
condemnation, if that condemnation were to death. And if it must be
—if you will have it so—if you will that a man be put to death by the
rope or the axe, on the scaffold or over an open grave—as the poor
soldier dies—I would have him perish at night—in the dead of
midnight—and all the town should wake up at the tolling of that
heavy bell, or at the roar of cannon, with a knowledge that a fellow-
creature had that instant passed away from the earth forever—just
gone—that very instant—before the Everlasting Judge of the quick
and the dead—that while they were holding their breath and before
they could breathe again—he would receive the sentence from
which there would be no appeal throughout all the countless ages of
eternity.
Very fair—very fair—I see the foreman of the jury shudder—keep
him to it—
I love theory, but I love practice better—
Zounds! what a plunge!
—Bear with me, I beseech you. I had come to a conclusion years
and years ago, before I went away into the far parts of the earth,
Judges and Elders, that where human life is thought much of, there
liberty is; and that just in proportion to the value of human life are the
number and variety, the greatness and the strength of the safe-
guards forms and ceremonies, which go to make it secure, if not
altogether inaccessible.
Very fair—stick to the foreman—keep your eye on his face—don’t
take it off, and you’ll be sure of the jury.
I can hardly see his face now—
So much the better—we’ll have candles for them yet; and if we do,
my boy, the game is our own ... fire away; my authorities are almost
ready now—fire away.
—I journeyed the world over, but I found little to prove that human life
was of much value anywhere—anywhere I should say, except
among the barbarians and the savages. My heart was troubled with
fear. I knew not whither to go, nor where to look. Should I pursue my
way further into the cities of Europe, or go back into the wilderness
of America?—At last I heard of a nation—bear with me judges—
where all men were supposed by the law to be innocent, until they
were proved to be guilty, where the very judges were said to be of
counsel for the accused, where the verdict of at least twelve, and in
some cases of twenty-four men—their unanimous verdict too, was
required for the condemnation of such accused; where if a man were
charged with a crime, he was not even permitted to accuse himself
or to acknowledge the truth, till he had been put upon his guard by
the judges—who would even allow him, nay press him to withdraw
an avowal, though it were made by him with serious deliberation;
where the laws were so tender of human life to say all in a word, and
so remarkable for humanity as to be a perpetual theme for
declamation. I heard all this.... I had much reason to believe it ... for
everybody that I knew believed it.... I grew instantly weary of home....
Lights there! lights....
—I could not sleep for the desire I had to see that country.
You’d better stop awhile, Mr. Burroughs, whispered the lawyer.
—And I lost no time in going to it.
Pull up where you are ... but keep your face toward the jury.
CHAPTER IX.
Well, continued Burroughs, I departed for the shores of that other
world, where human life was guarded with such care and jealousy. I
inquired for the courts of justice and for the halls of legislation ... I
hurried thither; ... I elbowed my way up to the sources of their law,
and I had the mortification to discover that in almost every case, their
courts were contrived, not as I had hoped from the character of the
people, so as to give the public an opportunity of seeing the
operation of power at work in the high-places of our earth, for the
detection of guilt and for the security of virtue, but so as to hinder
that operation, whether evil or good, from being viewed by the public.
Everywhere the courts of justice were paltry ... everywhere
inconvenient. Seeing this I grew afraid for the people. I found but one
large enough to accommodate its own officers, and but one which it
was possible for a stranger to enter, even by the aid of money,
without much delay, difficulty and hazard. Ye do not believe me—ye
cannot believe that such things are, such courts or such men, or that
ever a price hath been fixed in a proud free country, for which a few
and but a few of a mighty and wise people may see, now and then,
wherefore it is that some one of their number is to be swept away
from the earth forever. What I say is true. To the Halls of Legislation I
proceeded—to the place where that law is made of which I have had
occasion to speak this day. I went without my dinner; I paid my last
half-crown to see the makers of the law—and I came away, after
seeing—not the makers of the law, but the door-keepers of their
cage—it is true that while I was there, I was happy enough to see a
man, who was looking at another man, which other man declared
that the wig of the Speaker was distinctly visible—
Are you mad?
Be quiet Sir—
You have broken the spell—the jury are beginning to laugh—
Leave the jury to me—what I have to say Sir, may provoke a smile,
but if I do not much mistake, a smile for the advantage of poor
Martha. We have been too serious ... we may do better by showing
that we have no fear—if the lawgivers of that country are what I say
they are—if the judges are what I say they are, and what I shall
prove them to be—and if the people of that country are what I am
afraid they are, under such law—why should we bow to its authority?
Pho—pho—pho.... You are all at sea now.
Well Judges ... I enquired when there would be a trial to prove the
truth of what I had been told, and whither I should go in search of a
Temple of Justice, where I might see for myself how human life was
regarded by the brave and the free. I found such a temple, and for
the price of another dinner, was carried up into a gallery and put
behind a huge pile of masonry, which as it stood for a pillar and
happened to be neither perforated nor transparent, gave me but a
dreary prospect for my money.... Do not smile—do not, I beseech
you—I never was more serious in my life.... At last I heard a man
called up, heard I say, for I could not see him, called up and charged
with I know not what fearful crime—I caught my breath—are you
ready Sir?...
Almost ... almost—fire away—writing as fast as he could make the
pen fly over the paper ... fire away for a few minutes more....
I caught my breath ... I trembled with anxiety.... Now said I to myself,
(To the lawyer; I am afraid I shall drop.)
No no, don’t drop yet ... fire away!
Now, said I to myself, I shall see one of the most awful and affecting
sights in the world. Now shall I see the great humanity of the law ...
the law of this proud nation illustrated ... the very judges becoming of
counsel for the prisoner ... and the whole affair carried through with
unspeakable solemnity. I addressed myself to a man who stood near
me with a badge of authority in his hand ... the very key wherewith
he admitted people at so much a head, to see the performance.
Pray, Sir, said I, what is that poor fellow charged with? He didn’t
know, not he, some case of murder though, he thought, (offering me
a pinch of snuff as he spoke) or of highway-robbery, or something of
the sort ... he would enquire with great pleasure and let me know.
The case opened. A speech was made by a prosecutor for the
crown, a ready and a powerful speaker. The charge a capital one.
The accused ... a poor emaciated miserable creature, was on trial for
having had in possession, property which had been stolen out of a
dwelling-house in the dead of night. Well, prisoner at the bar, what
have you to say for yourself? said the judge with a stern look, after
the case had been gone through with by the prosecutor. Now is your
time ... speak, said the judge. I have nothing to say for myself, said
the poor prisoner; nothing more than what I have said four or five
times already. Have you no witnesses? No my lord——
Soh soh, Mr. Burroughs! We understand your parable now, cried one
of the judges with a look of dismay. We all know what country that is
where a judge is a lord ... have a care Sir; have a care.... Be wary ...
you may rue this if you are not.
I shall endure the risk whatever it be ... shall I proceed?
We have no power to stop you....
No my lord, was the reply of the prisoner. I could not oblige them to
appear; and they would not appear. How came you by the property?
said his lordship. It was left with me by a man who stopped at my
house; he wanted a little money to carry him to see a sick wife ... and
as I did not know him, he left this property in pledge. Who was that
man? I do not know my lord, I never saw him before ... but one of my
neighbors in the same trade with me knew him, and if you had him
here, he would say so.
Judges, you have now heard my story. You know what I was
prepared to see; you know what I expected. Here was a man who,
for aught we know, told the truth. But he had no witnesses—he had
no power to make them testify—he had no refuge—no hope—the
law was a snare to him—the law of our mother-country.
How so pray?
Property being found in his possession—property which had been
stolen, he was to suffer, because—mark what I say, I beseech you—
because he could not prove his innocence!
Tut—tut—tut—rigmarole! said the prosecutor.
Rigmarole Sir—what I say is the simple truth. Hear me through. The
moment that poor fellow was found with the property in his
possession, he was concluded by the law and by the judges of the
law to be guilty; and they called upon him to prove that he was not
guilty—
Nature of things, my good brother—
Well—and if it is the nature of things, why deny the existence of the
fact? Why do you, as all men of the law have done for ages and still
do—why say over and over again every day of your lives, that it is
the characteristic of the law, that law of which you are the
expounders, to regard every man as innocent, until he be proved to
be guilty? Why not say the truth? Why quibble with rhetoric? Why not
say that where a man is charged with a crime, you are, in the very
nature of things, under the necessity of taking that for proof which is
not proof? Look you Sir—how came you by the coat you wear?
Suppose I were to challenge that cloth and put you to the proof, how
could you prove that you purchased it fairly of a fair trader?
I would appeal to the trader—
Appeal to the trader! If he had not come honestly by it Sir, would he
ever acknowledge that you had it of him? or that he had ever seen
your face before?
Well then—I would prove it by somebody else.
By somebody else, would you! Are you so very cautious—do you
never go abroad without having a witness at your heels? do you
never pick up anything in the street Sir, without first assuring yourself
that you are observed by somebody of good character, who will
appear of his own accord in your behalf, should you be arraigned for
having stolen property in your possession? What would you have to
say for yourself?—your oath would not be received—and if it was,
there would only be oath against oath—your oath against that of the
trader of whom you purchased, or the individual of whom you
received the property—and his oath against yours.—How would you
behave with no witnesses to help you out?—or with witnesses who
would not appear and could not be made to appear on your side,
though your life were at stake?—nay, for that very reason, for if your
property only were at stake, they might be made to appear—
Very well!
—Or with witnesses, who having appeared on your side, are not
allowed to make oath to what they say—lest they may be believed—
to the prejudice of our good king?
Really, cried one of the judges, really, gentlemen, you appear to be
going very wide of the mark. What have we to do with your snip-snap
and gossip? Are we to have nothing but speech after speech—about
nobody knows what—now smacking of outrage—now of treason?
Are we to stay here all night Sirs of the bar, while you are whispering
together?
With submission to the court, said the Attorney-general—we have a
case put here, which would seem to require a word of reply. We are
asked what we should do if we were without witnesses—and the
court will perceive that the sympathy of the jury is relied on—is relied
on, I say!—on the authority of a case—of a case which!—of a case
which I never heard of before! The court will please to observe—to
observe I say!—that the prisoner at the bar—at the bar—has no
witnesses—in which case, I would ask, where is the hardship—
where we cannot prove our innocence—our innocence I say!—of a
particular charge—we have only to prove our character.
Here the Attorney-general sat down with a smile and a bow, and a
magnificent shake of the head.
Only to prove our character, hey?
To be sure—
But how—if we have no witnesses—
Very fair—very fair, brother B.
What if you were a stranger?—what if you had no character?—or a
bad one?
It would go hard with me, I dare say—and—and (raising his voice
and appealing to the bar with a triumphant look) and it should go
hard with me.
Why then Sir—it would go hard with every stranger in a strange
country, for he has no character; and it would go hard with every
man who might be unable to produce proof, though he had a good
character; and with every man who might be regarded as a profligate
or a suspicious character—as a cheat, or a jew, or a misbeliever.
And what have such men to complain of?
Judges—Fathers—I appeal to you. I have not much more to say, and
what I have to say shall be said with a view to the case before you. I
have always understood that if a man be charged with a crime here,
he is to be tried for that particular crime with which he is charged,
and for no other till that be disposed of. I have always understood
moreover, not only in your courts of law and by your books of law,
but by the courts and by the books of which you are but a copy, that
character is not to be put in issue as a crime before you; and that
nobody is to be put to death or punished merely because he may
happen to have no character at all—nor because he may have a bad
one—
You have understood no more than is true, said a judge.
If so ... allow me to ask why you and other judges are in the habit of
punishing people of a bad character ... nay of putting such people to
death ... for doing that which, if it were done by people of good
character, you would overlook or forgive?
How Sir.... Do you pretend that we ever do such things?
I do.... Will you say that you do not?...
Yes ... and waive the authority of a judge, and the irregularity of your
procedure that you may reply.
Then ... if what I hear is true ... if it is law I mean ... the judges before
me will not regard character?
Why as to regarding character ... that’s another affair Mr.
Burroughs....
I implore you ... take one side or the other! Say whether you do or do
not regard character.... I care not for the degree, nor do I care which
side you take. For if you say that you do, then I say that you act in
the teeth of all your professions; for you declare in every shape,
every man of you, every day of your lives, that nobody shall be
punished by law but for that which he has been charged with in due
course of law ... technically charged with and apprised of ... and you
never charge a man with having a bad character....
Well, then ... suppose we say that we do not regard character?
When character is not in issue, brother, added the chief-judge; for it
may be put in issue by the traverser—in which case we are bound to
weigh the proof on both sides along with the jury.
If you say that, in your character of judge ... and if you are all agreed
in saying that.... Lo, I am prepared for you.
We are agreed—we perceive the truth now.
Lo, my answer!—You have heard the whole of our case. You have
heard all the witnesses for the crown; you have gathered all the
proof. Now ... bear with me, judges ... bear with me ... what I say is a
matter of life and death ... we have no witnesses ... we have not put
the character of Martha in issue ... all that you know of her, you know
from your witnesses, and they have not said a syllable touching her
character. Now ... fathers! and judges!... I ask you if that proof, take it
altogether, would be enough in your estimation, to prove ... I beg you
to hear me ... would it be enough to convict any one of your number,
if he had no witness to speak for him?... Ye are astounded! Ye know
not how to reply, nor how to escape; for ye know in your own souls
that such proof ... such proof and no more, would not be enough to
convict any one of you in the opinion of the other six.
Well Sir——what then?
Why then Sir ... then ye Judges—if that poor old woman before you
—if she be not on trial for character—on trial for that which has not
been charged to her ... by what you have now said, she is free.
Stand up, on your feet Martha! stand up and rejoice! By what ye
have now said, ye judges, that poor old woman hath leave to go free!
The judges were mute with surprise, and the lawyer started upon his
feet and clapped Burroughs on the back, and stood rubbing his
hands at the Attorney-general and making mouths at the jury—
Capital! ... Capital! ... never saw the like, faith—never, never ... never
thought of such a view myself ... but I say though (in a whisper) you
did begin to put her character in issue—tut—tut—yes you did, you
rogue you ... say nothin’—tut—tut—
Say nothing Sir!—excuse me. If I have said that which is not true, I
shall unsay it—
Pooh, pooh ... your argument’s all the same, and besides, you did
not go far enough to make Jerry Smith your witness ... pooh, pooh—
what a fool you are—
But the judges recovered their self-possession, and laid their heads
together and asked Burroughs if he had anything more to say.
More to say—yes—much more—enough to keep you employed for
the rest of your lives, ye hard-hearted inaccessible men! What—are
ye so bent upon mischief! Will ye not suffer that aged woman to
escape the snare! Ye carry me back all at once to the spot of which I
spoke. Ye drive me to the parable again. I saw the judges behave to
their prisoner as I now see you behave to yours; and I would have
cried out there as I do here, with a loud voice.... Are ye indeed the
counsel for the prisoner!—Why do ye not behave as other counsel
do? But when I looked up and beheld their faces, and about me, and
beheld the faces of the multitude, my courage was gone—I had no
hope—my heart died away within me. They were as mute as you are
—and their look was your look—a look of death. But where, said I, is
the advocate for the prisoner? why does he not appear? He has
none, was the reply. What, no advocate, no help—there is a
provision of your law which enables the very pauper to sue.... I have
heard so, and surely he is not so very poor, the man I see at the bar;
why do not the counsel that I see there unoccupied—why do they
not offer to help him? They are not paid Sir. Do they require pay
before they will put forth a hand to save a fellow-creature from
death? Of course. But why do not the court assign counsel to him?—
The reply there was the reply that you have heard here this day. The
accused have no counsel in a matter of life and death ... it is only by
courtesy that counsel are permitted even to address the court on a
point of law, when they are employed by a prisoner.
But why do I urge all this? Are not we, and were not they, living in a
land of mercy, a land remarkable for the humanity of her laws? Do
not mistake me, fathers! I would not that the guilty should escape.... I
have no such desire. But I would have the innocent safe, and I would
have the guilty, yea the guiltiest in every case and everywhere,
punished according to law. To know that a man has committed
murder is not enough to justify you in taking his life—to see him do
the deed with your own eyes, would not be enough to justify you in
putting him to death—wherefore it is that however certain the guilt of
the accused, and however great his crime, he should have
counsel....
Absurd!—
Yea—counsel, judge—counsel!
You would allow the guilty every possible chance of escape.
Even so, judge! every possible chance of escape. For the guilty have
some rights to guard—rights the more precious for being so few, and
for being in perpetual risk of outrage; the more to be guarded Sir,
because they are the rights and the privileges of the wicked, who
have nothing to hope from the public sympathy, no hope of pity, no
hope of charity. Even so, Judge! for the innocent are liable to appear
otherwise. Even so—for till the trial be over, how do we know who is
guilty and who not? How do we know—how is it possible for us to
know, till the accused have undergone their trial, whether they are, or
are not unjustly charged? For the innocent as well as for the guilty
therefore, would I have counsel for the accused—yea, counsel,
whatever were the charge, and however probable it might appear—
nay the more, in proportion both to the probability and to the
magnitude of the charge.
A fine theory that Sir. You have been abroad to much purpose, it
would appear.

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Neurology at the Bedside 2nd Edition

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Geriatric rehabilitation : from bedside to curbside 1st

ISBN 978-3-319-55990-2 ISBN 978-3-319-55991-9 (eBook)

Library of Congress Control Number: 2017943485

© Springer International Publishing AG 2017

Printed on acid-free paper

This Springer imprint is published by Springer Nature

Progress in neurology is being made at an ever-increasing speed. Since the

Copenhagen Daniel Kondziella

London Richard Hughes, M.D., F.R.C.P., F.Med.Sci.

Medicine, including neurology, is an ever-changing field. As new research

4.3 The Differential Diagnosis of Traumatic Brain Injury ������������ 134

4.15 The Differential Diagnosis of Movement Disorders���������������� 221

6 Treatment of Neurological Disorders �������������������������������������� 291

ACA Anterior cerebral artery

CAE Childhood absence epilepsy

INR International normalized ratio

MOG Myelin oligodendrocyte glycoprotein

PROMM Proximal myotonic myopathy

VDRL Venereal Disease Research Laboratory

Case 2.1 Botulism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Case 3.8 Cerebral fat embolism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108

Case 4.36 Cranial neuropathy due to malignant external otitis. . . . . . . 210

© Springer International Publishing AG 2017 1

To master the clinically relevant neuroanatomy

Neurological bedside examination

To be able to generate a working diagnosis for (almost) every neurological complaint

Fig. 1.1 Summary of chapter learning objectives

© Springer International Publishing AG 2017 5

Subcortical gray matter

Subcortical white matter

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Copenhagen Daniel Kondziella

London Richard Hughes, M.D., F.R.C.P., F.Med.Sci.

4.3 The Differential Diagnosis of Traumatic Brain Injury �� 134

4.15 The Differential Diagnosis of Movement Disorders�� 221

6 Treatment of Neurological Disorders �� 291