The Neurologic Examination-Scientific

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2022)_(0197556302)_(Oxford University
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 V.
The
Neurologic
Examination
Scientific Basis for Clinical Diagnosis

Hiroshi Shibasaki
& Mark Hallett
SECOND EDITION

OXFORD
T H E N E U R O L O G I C E X A M I N AT I O N
THE NEUROLOGIC EXAMINATION
Scientific Basis for Clinical Diagnosis

SECOND EDITION

Hiroshi Shibasaki and Mark Hallett

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Library of Congress Cataloging-in-Publication Data

Names: Shibasaki, Hiroshi, 1939– author. | Hallett, Mark, 1943– author.
Title: The neurologic examination : scientific basis for clinical diagnosis /
Hiroshi Shibasaki and Mark Hallett.
Description: 2. | New York, NY : Oxford University Press, [2022] |
Includes bibliographical references and index.
Identifiers: LCCN 2021060585 (print) | LCCN 2021060586 (ebook) |
ISBN 9780197556306 (hardback) | ISBN 9780197556320 (epub) |
ISBN 9780197556337 (online)
Subjects: MESH: Neurologic Examination—methods | Nervous System
Diseases—diagnosis | Medical History Taking—methods |
Neurologic Manifestations
Classification: LCC RC386.6.N48 (print) | LCC RC386.6.N48 (ebook) |
NLM WL 141 | DDC 616.8/0475—dc23/eng/20220307
LC record available at https://lccn.loc.gov/2021060585
LC ebook record available at https://lccn.loc.gov/2021060586

DOI: 10.1093/​med/​9780197556306.001.0001

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Printed by Integrated Books International, United States of America

 IN MEMORIAM

Hiroshi made many contributions to the physiology of

movement and the pathophysiology of movement disor-
ders. He authored pioneering papers with his deep knowl-
edge of the field and his ability to innovate. Our interests
continued to run in tandem, and our mutual interest in
myoclonus was a constant theme over the years.
Hiroshi was an excellent teacher who devoted consider-
able time to teaching even after officially retiring well into his
later years. A good example of that is this book. Originally
written in Japanese, it was so popular he thought it would be
good to have it in English too. He asked me to help, and we
had many interesting discussions about the fine points of the
Hiroshi Shibasaki, MD, PhD neurologic examination. He also had a large number of vid-
1939–2022 eotapes of patients with different movement disorders that
formed the core of another book on hyperkinetic movement
disorders that we wrote together with some other experts in
Hiroshi Shibasaki died as we were finalizing this second
2020 (also with Oxford University Press).
edition of The Neurologic Examination. He was enthusias-
Hiroshi was a very pleasant person and always a gentleman.
tic about the book, pleased with the updates from the first
Outside of work, he had a large family which he enjoyed. He
edition, and liked the new cover, a variation on the first edi-
also had a strong partnership with his devoted wife, Shinobu,
tion cover.
also a physician. His family and many neurologists around the
I first met Hiroshi at a myoclonus meeting organized
world will miss him. I have lost a good friend.
by Dr Stanley Fahn in the mid-1980s. Unbeknownst to
Mark Hallett
the attendees, the retreat location, owned by Columbia
June 2022
University, only comprised double rooms, all of which
were assigned by Dr Fahn. Hiroshi was my roommate. I
had never met him, but we knew each other’s work on the
physiology of myoclonus. We were fast friends ever since.
Over the years, we met at many meetings, and Hiroshi sent
some excellent neurologists for post-doctoral fellowships
during my tenure at the National Institute of Neurological
Disorders and Stroke. When he retired as Chair of
Neurology at Kyoto University—mandatory retirement at
the early age of 63—he spent two years in my Section at the
National Institutes of Health.
Dedication

To Shinobu, Judy, and our families for their constant support, and to the many patients
we have examined who have taught us about the neurologic examination
 CONTENTS

List of Boxes xv G. General physical findings and neurological

Preface to the First Edition xix findings 13

Preface to the Second Edition xxi 2. Neurological manifestation of systemic

medical diseases 14
Explanatory Notes xxiii
A. Collagen vascular diseases 14
Acknowledgments xxv
B. Endocrine diseases 18
C. Metabolic diseases and syndromes 19
1. DIAGNOSIS OF NEUROLOGICAL DISEASES 1 D. Hematologic diseases 21
1. Axis 1: anatomic diagnosis 1 E. Behçet disease 22
A. Distribution of the lesion at the tissue level 1 F. Sarcoidosis 22
B. Selective vulnerability 2 G. Neoplasm 22
C. Negative versus positive neurological 3. Steps of the neurologic examination 23
symptoms and signs 2
Bibliography 23
2. Axis 2: etiologic diagnosis 2
3. Axis 3: clinical diagnosis 4 4. EVALUATION OF CONSCIOUSNESS 25
Bibliography 4 1. Anatomic basis of consciousness 25
2. Mechanisms of disturbances of consciousness 26
2. HISTORY TAKING 5
3. Observation of the state of consciousness 26
1. The present age and the age at onset 5
4. Modes of consciousness disturbance and the
2. Sex 6 coma scale 27
3. Handedness 6 5. Conditions that should be distinguished from
4. Chief complaints 6 clouding of consciousness 28
5. History of the present illness 7 A. Deep sleep 28
6. Past medical history 7 B. Advanced stage of dementia 29
7. Social history 7 C. Akinetic mutism 29
8. Family history 8 D. Locked-​in syndrome 29
Bibliography 8 Bibliography 30

3. PHYSICAL EXAMINATION 9 5. BRAINSTEM AND CRANIAL NERVE TERRITORIES 31

1. General physical examination and neurologic 1. Brainstem 31
examination 9 2. Common structures of cranial nerves 32
A. General physical examination at the initial 3. Cranial nerves related to the motor function 33
clinical evaluation 9
4. Cranial nerves related to somatosensory
B. Skin abnormalities seen in congenital or function 33
hereditary neurological diseases 10
5. Cranial nerves related to autonomic nervous
C. Skin abnormalities seen in inflammatory function 33
neurologic diseases 11
6. Location of the cranial nerve nuclei in
D. Skin abnormalities seen in toxic neurologic brainstem 34
diseases 12
7. Neurologic examination of the cranial nerve
E. Sunlight photosensitivity of skin 13 territory 34
F. Abnormalities of oral cavity seen in Bibliography 35
neurologic diseases 13
 6. OLFACTORY SENSATION 36 9. EXTRAOCULAR MUSCLES, GAZE, AND EYE MOVEMENTS 56
1. Structures and neurotransmitters of the 1. Nerve innervation of extraocular muscles 56
olfactory system 36 A. Third cranial nerve (oculomotor nerve) 57
2. Examination of olfactory sensation 36 i. Intramedullary lesion of the oculomotor
Bibliography 37 nerve and crossed hemiplegia 57
ii. Extramedullary lesion of the oculomotor
7. VISUAL FUNCTIONS 38 nerve 58
1. Anatomy and function of the visual system 38 iii. Cavernous sinus syndrome 59
A. Retina 38 B. Fourth cranial nerve (trochlear nerve) 59
B. Fovea centralis and optic disc 38 C. Sixth cranial nerve (abducens nerve) 59
C. Blood supply to the retina 39 2. Neural control mechanism of gaze 60
D. Optic nerve and optic chiasm 41 A. Central control mechanism of gaze 60
E. Lateral geniculate body and optic radiation 43 B. Lateral gaze and lateral gaze palsy 61
F. Visual cortex 43 C. Vertical gaze and vertical gaze palsy 61
G. Blood supply to the visual pathway 43 D. Convergence and convergence palsy 62
H. Cytoarchitecture of the visual system and 3. MLF and internuclear ophthalmoplegia 62
higher cortical functions 43
4. Central control mechanism of eye movements 63
I. Special visual functions 44
A. Central control mechanism of saccades 63
2. Examination of visual functions 44
B. Central control mechanism of smooth pursuit 64
A. Visual acuity 44
C. Roles of cerebellum and basal ganglia in
B. Visual fields 45 eye movements 65
i. Central scotoma 46 5. Examination of extraocular muscles and gaze 65
ii. Other visual field defects 46 A. Ptosis 65
iii. Constriction of peripheral visual field 46 i. Bilateral ptosis 65
C. Ophthalmoscopic examination of ocular ii. Unilateral ptosis 66
fundus 47
iii. Blepharospasm 66
i. Optic disc 47
B. Examination of extraocular muscles 66
ii. Physiologic cup of optic disc 48
i. Vertical paralysis of one eye 66
iii. Fovea centralis 48
ii. Congenital disorders of extraocular
iv. Retinal pigment degeneration 48 muscles 67
v. Observation of retinal blood vessels 48 iii. Causes of diplopia or extraocular
Bibliography 48 muscle paralysis 67
iv. Ocular myopathy 68
8. PUPILS AND ACCOMMODATION 49 C. Examination of fixation and gaze 68
1. Nerve innervation of intraocular muscles 49 i. Lateral gaze palsy and conjugate
A. Light reflex and convergence reflex 49 deviation of eyes 68
B. Sympathetic nervous system and Horner ii. Oculogyric crisis 69
syndrome 51 iii. Gaze paralysis and ocular motor apraxia 69
C. Accommodation and its abnormality 52 iv. Eye-​tracking test 69
2. Examination of intraocular muscles 53 v. Optokinetic nystagmus 70
A. Oculomotor nerve palsy and Horner 6. Involuntary movements of eyes 70
syndrome 54
A. Spontaneous nystagmus 70
B. Examination of the light reflex and its
abnormality 54 B. Gaze nystagmus 70
C. Special abnormal findings of pupils 55 C. Ocular myoclonus 71
D. Examination of the convergence reflex 55 D. Opsoclonus 71
E. Examination of accommodation 55 E. Ocular bobbing and ocular dipping 72
Bibliography 55 7. Comprehensive examination of eye movements 73
Bibliography 73

viii
10. TRIGEMINAL NERVE 75 13. SENSE OF EQUILIBRIUM 93
1. Structures and functions 75 1. Structures and functions of the pathways
A. Somatosensory pathway 75 related to equilibrium 93

B. Cortical reception of somatosensory input 2. Examination of the sense of equilibrium 94

from face 76 A. Vestibular nystagmus 95
C. Nerve innervation of masticatory muscles 76 B. The vestibulo-​ocular reflex 95
D. Reflex pathway of jaw jerk 77 C. Caloric test 95
E. Neural pathway of corneal reflex 78 Bibliography 96
2. Examination of functions related to the
trigeminal nerve 78 14. SWALLOWING, PHONATION, AND ARTICULATION 97
A. Motor nerve 78 1. Innervation of swallowing, phonation, and
articulation 97
i. Trismus (lockjaw) 78
A. Glossopharyngeal nerve and vagus nerve 97
ii. Jaw-​winking phenomenon 79
B. Hypoglossal nerve 98
B. Sensory nerve 79
C. Cortical innervation of bulbar muscles 99
C. Jaw jerk (masseter reflex) 80
D. Involvement of the cranial nerves
D. Corneal reflex 80 innervating bulbar muscles 99
E. Primitive reflexes 81 2. Somatosensory innervation of pharynx
Bibliography 82 and larynx 100
3. Taste sense 101
11. FACIAL NERVE 83
4. Salivation 101
1. Structures and functions 83
5. Autonomic innervation of visceral organs 101
A. Motor nerve 83
6. Afferent pathway from the visceral organs 102
B. Somatosensory nerve 84
7. Examination of swallowing, phonation, and
C. Taste sense 84 articulation 103
D. Lacrimation and salivation 85 A. Examination of the tongue 103
E. Parasympathetic innervation of facial skin B. Examination of the soft palate 103
and mucosa 86
C. Spasm of the deglutition muscles 104
2. Examination of functions related to the
facial nerve 86 D. Dysphonia 104

A. Motor function 86 E. Dysarthria 104

i. Central facial paralysis and peripheral F. Bulbar palsy and pseudobulbar palsy 104
facial paralysis 87 Bibliography 105
ii. Crossed hemiplegia of facial muscle 87
15. NECK AND TRUNK 106
iii. Mimetic muscles 87
1. Examination of the neck 106
B. Somatosensory function 87
A. Postural abnormality of the neck 106
C. Gustatory sense 87
B. Examination of muscles innervated by the
D. Lacrimation and salivation 87 accessory nerve 106
E. Facial skin and mucosa 88 C. Cervical spondylosis 107
Bibliography 88 D. Lhermitte sign 107

12. AUDITORY FUNCTION 89 E. Meningeal irritation 107

1. Structures of the auditory pathway 89 2. Examination of the trunk 107

A. Brainstem auditory evoked potentials 90 Bibliography 108

B. Cognitive functions related to the auditory 16. MOTOR FUNCTIONS AND MOVEMENT DISORDERS 109
sense 90
1. Final common pathway of the motor system 109
2. Examination of auditory function 91
A. Motor cortex and corticospinal tracts 109
A. Tinnitus 91
i. Pyramidal tract and upper motor neuron 109
B. Sensorineural deafness 92
ii. Corticospinal tract 109
Bibliography 92
iii. Lesion of the corticospinal tract and
distribution of motor paralysis 111

 Contents
 B. Structure and function of spinal cord 112 I. Toxic polyneuropathy 145
i. Laminar structure of the lateral J. Metabolic polyneuropathy 145
corticospinal tract 112 K. Hereditary polyneuropathy 145
ii. Blood perfusion of the spinal cord 113 L. Spastic paralysis 146
C. Anterior horn cells and peripheral motor M. Gowers sign 146
nerves 114
N. Disuse atrophy 146
i. α motor fibers 114
4. Muscle tone 147
ii. Motor unit and reinnervation 114
A. Spasticity 147
iii. γ motor fibers 115
B. Clonus 147
D. Neuromuscular junction and muscle 115
C. Rigidity 148
2. Central control of voluntary movement 115
D. Hypotonia 149
A. Higher functions of motor cortices 116
5. Muscle spasm and cramp 149
B. Basal ganglia 116
A. Hemifacial spasm 150
i. Cytoarchitecture of striatum and neural
circuits of basal ganglia 116 B. Tetanus 150
ii. Nigrostriatal system 119 C. Stiff-​person syndrome and progressive
encephalomyelitis with rigidity and
iii. Deep brain stimulation in Parkinson myoclonus 151
disease 119
D. Painful muscle spasm (cramp) 151
iv. Significance of raphe nucleus and locus
coeruleus 119 6. Myotonia 152
v. Parkinsonism 121 7. Coordination 152
vi. Causes of parkinsonism 123 Bibliography 153
vii. Disability rating score of Parkinson
disease 124 18. TENDON REFLEXES AND PATHOLOGIC REFLEXES 157

C. Cerebellum 125 1. Physiologic mechanism of the tendon reflex 157

i. Cytoarchitecture of cerebellum 125 2. Examination of tendon reflexes 157

ii. Cerebellar circuit and clinical A. Tendon reflex of the distal extremity muscles 158
symptomatology 127 B. How to confirm loss of ankle jerk 160
iii. Spinocerebellar degeneration 129 C. Significance of hyperactive tendon reflexes 160
iv. Acquired cerebellar ataxia 130 3. Pathologic reflexes and their generating
v. Tumors of the cerebellum 131 mechanisms 160

Bibliography 132 4. Loss of superficial reflex as a pyramidal sign 162

A. Abdominal reflex (abdominal wall reflex) 162
17. EXAMINATION OF MOTOR FUNCTIONS 136 B. Cremasteric reflex 162
1. Posture 136 Bibliography 163
2. Muscle atrophy and fasciculation 136
A. Muscle atrophy 136 19. INVOLUNTARY MOVEMENTS 164

B. Fasciculation 138 1. Examination of involuntary movements 164

C. Muscle hypertrophy 138 2. Tremor 164

3. Muscle strength 139 A. Resting tremor 165

A. Manual muscle testing 139 B. Postural tremor 167

B. Muscle pain 139 i. Essential tremor 168

C. Sites of lesion for monoplegia 139 ii. Other conditions presenting with
postural tremor 169
D. Root lesions 141
iii. Titubation 170
E. Plexus lesions 142
iv. Physiologic tremor 170
F. Acute polyneuritis 142
C. Action tremor 170
G. Atypical acute polyneuritis 144
3. Chorea 171
H. Chronic inflammatory demyelinating
polyneuropathy 145 4. Ballism 172

x
 5. Athetosis 172 iii. Sensory impairment in a transverse
6. Dystonia 172 spinal cord lesion 200

A. Functional abnormality of the sensori-​ iv. Brown–​Séquard syndrome 200

motor cortex in focal dystonia 173 Bibliography 203
B. Fixed dystonia 173
21. AUTONOMIC NERVOUS SYSTEM 204
C. Sensory trick 174
1. Structure and function of the autonomic
D. Causes of generalized dystonia 175 efferent system 204
E. Hereditary dystonia 176 A. Sympathetic nervous system 204
7. Dyskinesia 177 B. Parasympathetic nervous system 206
8. Myoclonus 178 C. Disorders of the peripheral autonomic
A. Myoclonus of cortical origin 178 nerves 206
i. Cortical reflex myoclonus 179 D. Cortical center of the autonomic nervous
ii. Causes of cortical myoclonus 179 system 206

iii. Negative myoclonus 179 E. Neural control of urination 207

B. Myoclonus of brainstem origin 181 2. Structure and function of the autonomic

afferent system 207
C. Myoclonus of spinal cord origin 182
3. Examination of the autonomic nervous
D. Myoclonus of undetermined origin 183 function 207
i. Creutzfeldt–​Jakob disease 183 A. Autonomic symptoms of skin 208
ii. Subacute sclerosing panencephalitis B. Micturition and sexual functions 208
(SSPE) 184
C. Gastrointestinal symptoms 208
9. Motor stereotypies 184
D. Orthostatic hypotension 208
10. Involuntary movements that can be
suppressed momentarily 185 Bibliography 209

A. Tics 185 22. POSTURE AND GAIT 211

B. Functional (psychogenic) involuntary 1. Central control mechanism of gait 211
movements 186
2. Examination of gait 211
C. Restless legs syndrome 186
A. Freezing of gait 212
11. Involuntary movements of peripheral
nerve origin 186 B. Ataxic gait 213
Bibliography 187 C. Waddling gait and steppage gait 214
Bibliography 214
20. SOMATOSENSORY FUNCTION 191
1. Structure and functions of the somatosensory 23. MENTAL AND COGNITIVE FUNCTIONS 216
system 191 1. Examination of mental and cognitive
A. Somatosensory receptors 191 functions 216
B. Peripheral somatosensory nerves 192 A. Orientation 217
C. Somatosensory pathway in the spinal cord 193 B. Memory 217
D. Nociceptive pathway 193 i. Examination of memory 218
E. Proprioceptive pathway 194 ii. Korsakov (Korsakoff) syndrome 218
F. The third sensory neuron and cortical C. Calculation 218
receptive areas 194 D. Common knowledge and judgment 219
2. Examination of somatosensory function and E. Emotion and character 219
abnormal findings 195
F. Illusion, hallucination, and delusion 220
A. Examination of somatosensory function 195
G. State of daily living 220
B. Somatosensory symptoms and signs 196
2. Dementia 222
C. Impairment of the somatosensory nervous
system and distribution of the sensory A. Alzheimer disease and related disorders 223
symptoms 197 i. Alzheimer disease 223
i. Polyneuropathy primarily presenting ii. Lewy body dementia and Parkinson
with sensory symptoms 198 disease 223
ii. Segmental sensory impairment 199 iii. Basal ganglia and cognitive functions 223

 Contents
 B. Frontotemporal lobar degeneration 224 27. SLEEP DISORDERS 255
C. Leukoencephalopathy 225 1. Obstructive sleep apnea–​hypopnea syndrome 255
Bibliography 227 2. Central sleep apnea–​hypopnea syndrome 255
3. REM sleep behavior disorders (RBD) 255
24. APHASIA, APRAXIA, AND AGNOSIA 230
4. Narcolepsy and related disorders 256
1. Neural circuits related to language, praxis,
and recognition 230 5. Periodic limb movement in sleep 257

A. Focal neurological deficit and disconnection 6. Kleine–​Levin syndrome 258

syndrome 230 7. Fatal familial insomnia 258
B. Language 230 Bibliography 258
C. Classification of aphasia 231
28. EPISODIC NEUROLOGICAL DISORDERS RELATED TO
D. Spoken language and written language 232 ION CHANNELS 259
E. Praxis 233 1. Hereditary channelopathies 259
F. Classification of apraxia 234 A. Hereditary channelopathies of the central
G. Task-​specific apraxia 236 nervous system 259
H. Mirror neuron 236 i. Familial hemiplegic migraine 259
I. Recognition 236 ii. Episodic ataxia 259
J. Disturbance of auditory recognition 237 iii. Hereditary startle disease, hyperekplexia 260
K. Higher visual functions and their disturbance 237 iv. Epilepsies 261
L. Visuospatial agnosia 238 B. Hereditary channelopathies of the
M. Positive symptoms of higher visual functions 238 peripheral nervous system 262

2. Examination of language, praxis, and recognition 239 C. Hereditary channelopathies of muscles 262

A. Examination of language 239 i. Hypokalemic periodic paralysis 262

i. Motor aphasia 239 ii. Hyperkalemic periodic paralysis 262

ii. Sensory aphasia 239 iii. Myotonia congenita 262

iii. Conduction aphasia 240 iv. Paramyotonia congenita 262

B. Examination of praxis 240 v. Andersen–​Tawil syndrome 263

C. Examination of recognition 240 vi. Malignant hyperthermia susceptibility 263

i. Visual recognition 240 vii. Central core disease 263

ii. Hemispatial neglect 241 viii. Congenital myasthenic syndrome 264

D. Use of a simple test battery 241 2. Autoimmune channelopathies 264

Bibliography 242 A. Myasthenia gravis 264

B. Lambert–​Eaton syndrome 264
25. EPILEPSY AND CONVULSION 244 C. Isaacs syndrome (neuromyotonia) 265
1. Classification of epileptic seizures 244 Bibliography 266
A. Focal epileptic seizures 244
29. FUNCTIONAL (PSYCHOGENIC) NEUROLOGICAL
B. Generalized epileptic seizures 247
DISEASES 268
2. Diagnosis of epilepsy 248
1. Functional motor paralysis 268
A. Evaluation of medically intractable epileptic
seizures 248 2. Functional sensory loss 269

B. Psychogenic non-​epileptic seizures 249 3. Constriction of visual field 269

Bibliography 249 4. Common features of functional neurological

diseases 269
26. HEADACHE AND MIGRAINE 251 Bibliography 270
1. Classification of headache 251 30. RELAY CENTER OF ALL SENSORY AND MOTOR
2. Migraine and related disorders 252 FUNCTIONS (THALAMUS) 271
A. Cluster headache 253 1. General structure of the thalamus 271
B. Reversible cerebral vasoconstriction 2. Ventral posterior nucleus as the sensory relay
syndrome 253 center 272
3. Tension-​type headache 253 3. Ventral anterior nucleus and ventrolateral
Bibliography 253 nucleus as the motor thalamus 273

xii
 4. Medial dorsal nucleus as a relay center to the B. Tetanus 289
prefrontal cortex 273 5. Conditions expected to have poor functional
5. Anterior nucleus and memory 274 recovery unless appropriately treated at early
6. Pulvinar as a relay center to the parietal lobe 274 clinical stage 289

7. Intralaminar nucleus as a relay center of the A. Acute compression of spinal cord 290
ascending reticular activating system 274 B. Progressive ischemic cerebrovascular
8. Relay nucleus for the visual and auditory diseases 290
system 275 6. Examination of coma 291
Bibliography 275 A. Degree of coma (coma scaling) 291
B. Presence or absence of asymmetry in
31. ADJUSTMENT TO THE EXTERNAL ENVIRONMENT neurological signs 291
AND CONTROL OF THE INTERNAL MILIEU
(HYPOTHALAMUS AND NEUROENDOCRINOLOGY) 276 C. Examination of brainstem functions 292

1. Structure and function of the hypothalamus 276 i. Observation of respiration 292

2. Neuroendocrinology 276 ii. Brainstem reflexes 292

A. Anterior lobe of the pituitary gland D. Cerebral herniation 294

(adenohypophysis) 276 i. Anisocoria 294
B. Posterior lobe of the pituitary gland ii. Decerebrate posture 294
(neurohypophysis) 277 iii. Tonic neck reflex 294
3. Adjustment to the external environment and iv. False localizing signs 294
control of the internal milieu 278
E. Meningeal irritation 295
A. Control of circadian rhythm 278
7. Judgment of brain death 295
B. Control of feeding 278
Bibliography 295
C. Control of body temperature 278
D. Homeostasis 278 34. DISABILITY, FUNCTIONAL RECOVERY, AND PROGNOSIS 297
Bibliography 279 1. Assessment of disability scale 297
2. Mode of tissue damage, functional disability,
32. INFECTIOUS DISEASES OF THE NERVOUS SYSTEM 280 and recovery 297
1. Bacterial infection of the nervous system 280 A. Gray matter lesion versus white matter lesion 297
2. Spirochete infection of the nervous system 280 B. Axonal damage versus myelin damage 298
3. Viral infection of the nervous system 280 3. Mechanisms of functional recovery 298
4. Parasitic infection of the nervous system 281 A. Plastic reorganization 299
5. Fungal infection of the nervous system 282 B. Mobilization of reserve functions 299
6. Transmissible form of prion disease 282 Bibliography 299
Bibliography 283
35. HOW TO PLAN LABORATORY TESTS 300
33. NEUROLOGICAL EMERGENCY 285 1. Functional neuroimaging studies and
1. Disorders with respiratory paralysis 285 electrophysiologic studies 300
A. Crisis of myasthenia gravis 285 2. Lumbar puncture 300
B. Edrophonium (Tensilon) test 285 3. Genetic tests 301
C. Guillain–​Barré syndrome 286 4. Use of laboratory tests to prevent disease 302
D. Acute anterior poliomyelitis 286 Bibliography 303
E. Acute brainstem lesion 287
F. Amyotrophic lateral sclerosis 287 Afterword: For those who wish to study neurology 305
2. Disorders with disturbance of consciousness 287 Index 307
3. Stroke 288
4. Status epilepticus 289
A. Treatment of convulsive status epilepticus 289

 Contents
 LIST OF BOXES

1-​1 Onset of neurological symptoms in 11-​1 Orbicularis oris muscle and orbicularis
cerebrovascular diseases 3 oculi muscle 86

2-​1 Risk factors for vascular diseases in the young 11-​2 Crocodile tears syndrome 88
population 6
11-​3 Bilateral facial paralysis 88
2-​2 Lacunar infarction 7
14-​1 Wallenberg syndrome and related conditions 97
3-​1 Clinical neurology from a wide viewpoint 9
14-​2 Why does the tongue deviate on protrusion
3-​2 Inflammatory diseases of the nervous system 17 even in a unilateral hemispheric lesion? 99

4-​1 Subclavian steal syndrome 26 14-​3 Named syndromes related to brainstem and
cranial nerves 100
4-​2 Etiologic classification of syncope 26
14-​4 Frey syndrome 102
4-​3 Causes of syncope 27
16-​1 Mirror movement 111
4-​4 The Glasgow Coma Scale 28
16-​2 Non-​motor symptoms and signs in Parkinson
4-​5 The Japan Coma Scale 28 disease 122

4-​6 Etiologic classification of acute encephalopathy 28 16-​3 Pedunculopontine nucleus 124

4-​7 Locked-​in syndrome 30 16-​4 Neurodegenerative diseases presenting with

parkinsonism 125
7-​1 Clinical features of demyelinating diseases:
Is the Uhthoff phenomenon due to physical 16-​5 Neurodegeneration with brain iron
exercise or increase in the body temperature? 40 accumulation (NBIA) 125

9-​1 Weber syndrome 58 16-​6 Lysosomal storage disease 126

9-​2 Top of the basilar artery syndrome 59 16-​7 Conditions other than Parkinson disease in
which l-​dopa may improve the parkinsonian
9-​3 Saccadic eye movement and smooth pursuit symptoms 127
eye movement 63
16-​8 The Hoehn and Yahr scale for the level of
9-​4 Causes of extraocular muscle paralysis 67 disability in Parkinson disease 127

9-​5 Miller Fisher syndrome 68 16-​9 Bassen–​Kornzweig disease 130

9-​6 Comprehensive examination of eye movements 72 16-​10 Hereditary disorders characterized by cerebellar
ataxia, spastic paralysis, and cataract 131
10-​1 Marcus Gunn phenomenon 79
17-​1 Inclusion body myositis 137
10-​2 Raeder (paratrigeminal) syndrome 80
17-​2 Spinal and bulbar muscular atrophy 137
10-​3 Forced grasping 81
17-​3 Juvenile muscular atrophy of unilateral upper
10-​4 Head retraction reflex and opisthotonos 81 extremity (Hirayama disease) 141
17-​4 Neuromyelitis optica spectrum disorders 19-​15 Transient myoclonic state with asterixis in
(NMOSD) 148 elderly patients 181

17-​5 Acute disseminated encephalomyelitis (ADEM) 19-​16 Palatal tremor and Guillain–​Mollaret triangle 182
versus multiple sclerosis (MS) 148
19-​17 Diaphragmatic flutter 183
17-​6 Degenerative disorders of upper motor neurons 149
19-​18 Startle reflex in progressive supranuclear palsy 184
17-​7 Adrenomyeloneuropathy 149
20-​1 Modalities of somatosensory system 191
17-​8 HTLV-​I-​associated myelopathy/​tropical spastic
paralysis (HAM/​TSP) and subacute 20-​2 Pain associated with herpes zoster 197
myelo-​optico-​neuropathy (SMON) 150
20-​3 Carpal tunnel syndrome 197
17-​9 Malignant syndrome and malignant hyperthermia 150
20-​4 Thalamic pain and Dejerine–​Roussy syndrome 198
17-​10 What can occur in Parkinson patients during
l-​dopa treatment? 151 20-​5 Fabry disease 198

18-​1 Inverted tendon reflex 158 20-​6 Impairment of dorsal root ganglion cells 200

18-​2 Significance of finger flexor reflex 159 21-​1 Familial amyloid polyneuropathy 206

18-​3 Achilles tendon reflex and peripheral nerve 21-​2 Diagnostic criteria of multiple system atrophy 209
lesions 160
22-​1 How does paradoxical kinesia occur? 212
18-​4 Complete motor paralysis of toes and
Babinski sign 161 22-​2 Progressive supranuclear palsy and
corticobasal degeneration 213
18-​5 Alternatives to the Babinski sign; the Bing sign 161
22-​3 Idiopathic normal-​pressure hydrocephalus 213
18-​6 Abdominal reflex and abdominal muscle reflex 162
22-​4 Tandem gait and Romberg test 214
18-​7 How is the resting tremor in Parkinson disease
suppressed by voluntary movement? 162 22-​5 Intermittent claudication 214

19-​1 Asymmetry of tremor 168 23-​1 Montreal Cognitive Assessment (MoCA) 217

19-​2 Involuntary movements suppressed by ethanol 23-​2 Transient global amnesia 217
intake 168
23-​3 Wernicke encephalopathy 220
19-​3 Tremor caused by midbrain lesion 170
23-​4 Subacute and fluctuating behavioral
19-​4 Fragile X-​associated tremor/​ataxia syndrome 170 abnormality 220

19-​5 Genotype and phenotype of Huntington disease 171 23-​5 Change of emotion and character by a lesion of
the right temporal lobe 222
19-​6 Generating mechanism of dystonia 174
23-​6 Dementia pugilistica (chronic traumatic
19-​7 Hereditary myoclonus–​dystonia syndrome 176 encephalopathy) 224

19-​8 Causes of cortical myoclonus 179 23-​7 HIV-​related neurological disorders 224

19-​9 Causes of progressive myoclonus epilepsy 179 23-​8 Prion disease 225

19-​10 Hereditary paroxysmal dyskinesia 180 23-​9 Vanishing white matter disease 226

19-​11 Unverricht–​Lundborg disease 180 23-​10 Nasu–​Hakola disease 226

19-​12 Benign adult familial myoclonus epilepsy 180 23-​11 Progressive multifocal leukoencephalopathy (PML) 226

19-​13 Mitochondrial encephalomyopathy and 24-​1 Alexia with and without agraphia 233
abnormality of mitochondrial functions 181
24-​2 Kanji (morphogram) and kana (syllabogram) of
19-​14 Unilateral asterixis due to thalamic lesion 181 Japanese language 233

xvi
24-​3 Inability to open eyes 235 27-​1 Could sleep apnea syndrome cause cognitive
impairment? 257
24-​4 Gerstmann syndrome 236
27-​2 REM sleep behavior disorder (RBD) as a
24-​5 Syndrome of Alice in Wonderland 236 synucleinopathy 257

24-​6 Amusia 237 28-​1 Limbic encephalitis 265

24-​7 Savant syndrome 237 29-​1 Hoover sign 268

24-​8 Ventriloquist effect and cocktail party effect 237 29-​2 Functional gait disorders 269

24-​9 Williams syndrome 238 32-​1 Acute meningoencephalitis 281

32-​2 General paresis and tabes dorsalis 282

24-​10 Landau–​Kleffner syndrome 239
32-​3 Neurological manifestations of COVID-​19 282
24-​11 Balint syndrome 241
33-​1 Causes of neurological emergencies 286
25-​1 Classification of epileptic seizures by ILAE
Commission 245
33-​2 Acute poisoning causing respiratory paralysis 286
25-​2 Gelastic epilepsy (ictal laughter) 248 33-​3 Reye syndrome 289
25-​3 Cerebrovascular diseases and epilepsy 249 33-​4 Causes of coma 292
26-​1 International classification of headache 251 34-1 Factors Influencing the Degree of Functional
Disability and its Recovery in Neurological
26-​2 Post–​lumbar puncture headache 252 Disorders 298

 List of B oxes
 PREFACE TO THE FIRST EDITION
A number of books related to neurology and neurological
diagnosis have been published and are currently available in
updated editions, but most of them are disease-​oriented or
anatomy-​oriented, and only few are function-​oriented. As a
consequence of recent advances of laboratory testing including
electrophysiology and neuroimaging, the importance of his-
tory taking and physical examination in neurological diagnosis
tends to be neglected. However, the correct interpretation of
symptoms and signs based on modern scientific knowledge is
of utmost importance in the diagnosis of neurological diseases.
The problems of any patient have to be based on a detailed and
thoughtful analysis of the patient; the laboratory testing is just
an extension of the examination. In this regard, this book is
organized in terms of functional anatomy of the nervous sys-
tem and aims at providing a bridge from the basic sciences such
as anatomy, physiology, pharmacology and molecular biology
to neurological symptoms and signs. As one of the unique fea-
tures of this book, 113 boxes are attached in order to discuss
some specific topics of current interest and some clinical issues
that the authors have been particularly interested in.
This book is primarily aimed at neurology residents and
registrars, but it is hoped that it will be also useful for neu-
rologists in general practice, pediatric neurologists, neurosur-
geons, psychiatrists, physical therapists, technicians of clinical
neurophysiology and neuroimaging, and medical students.
Hiroshi Shibasaki learned clinical neurology from a
number of senior neurologists, including Dr. Yoshigoro
Kuwoiwa and Dr. Shukuro Araki of Japan, Dr. A.B. Baker
and Dr. John Logothetis of USA, and Dr. A.M. Halliday
and Dr. Ian McDonald of UK. He also received a profound
impact from Dr. Jun Kimura while they worked together in
Kyoto University. He has also collaborated with Dr. Mark
Hallett who is a co-​author of this book and Dr. Hans
O. Lüders of USA for many years. By integrating all the
information collected from those distinguished neurolo-
gists and based on his own vast clinical experience, he has
created his own concept of neurological diagnosis. He pub-
lished a book entitled For Those Who Learn Neurological
Diagnosis in Japanese from Igaku-​Shoin in Tokyo, the first
edition in 2009 (Shibasaki, 2009) and the second edition
in 2013 (Shibasaki, 2013). Since those books have been
widely accepted among Japanese neurologists, he decided
to publish an updated English version for international
readers and asked Mark Hallett to help.
Mark Hallett learned his clinical neurology in Boston
from Drs. Raymond Adams, C. Miller Fisher, H. Richard
Tyler, and Norman Geschwind. The examination in
Boston in those days was heavily influenced by Dr. Derek
Denny-​Brown, who himself was influenced by Dr. Gordon
Holmes. Denny-​Brown (1946) authored a very brief book-
let on the neurologic examination that was the manual.
Like Dr. Shibasaki, Dr. Hallett spent time in London and
was influenced there by Dr. C. David Marsden. Dr. Hallett
notes that while he and Dr. Shibasaki had the good fortune
to be originally trained by giants in the field, the examina-
tion does continue to evolve and we appreciate the contri-
butions of all our neurologic colleagues over our years of
practice.
Since the two authors have had their main interests in
the field of human motor control, movement disorders,
involuntary movements and clinical neurophysiology, the
number of references cited for each chapter have been influ-
enced, leading to a relatively larger number of literature
citations for the chapters related to those specialty fields.
We are grateful to Prof. Per Brodal of Oslo for allowing
us to use many of the neuroanatomy figures from his book
The Central Nervous System (Brodal, 2010).
HS expresses his thanks to Dr. Masao Matsuhashi
of Kyoto University for his valuable help in computer
processing.
B IB LIOGR APH Y
Brodal P. The Central Nervous System. Structure and Function. 4th ed.
Oxford University Press, New York, 2010.
Denny-​Brown D. Handbook of Neurological Examination and Case
Recording. Cambridge: Harvard University Press; 1946.
Shibasaki H. Diagnosis of Neurological Diseases. Igaku-​Shoin, Tokyo,
2009, pp. 1–​332 (in Japanese).
Shibasaki H. Diagnosis of Neurological Diseases. 2nd ed. Igaku-​Shoin,
Tokyo, 2013, pp. 1–​381 (in Japanese).
 PREFACE TO THE SECOND EDITION
Since the first edition of this book appeared in 2016, it has
been widely accepted and has developed a favorable reputa-
tion. As the scientific information in this specialty field has
been rapidly advancing and expanding, we thought it useful
to update in this second edition many issues in relation to
the phenomenology, pathophysiology, pathology, diagnos-
tic skills, and treatment of disorders of the nervous system.
In view of the recent interest in infectious diseases such as
Zika virus and the new coronavirus, Chapter 32, “Infectious
Diseases of the Nervous System,” stands as an independent
chapter in this second edition. We also thought it appro-
priate to make a new section in the physical examination
on Neurologic Manifestations of the Systemic Medical
Diseases, which encompasses collagen vascular diseases, sar-
coidosis, endocrine diseases, metabolic diseases, and cancer.
We acknowledge helpful review of this revised edition
by Dr. Stephen G. Reich of the University of Maryland
School of Medicine and Dr. Yiwen Shi of Johns Hopkins
Hospital. H.S. expresses his thanks to Prof. Takashi
Nagamine of Sapporo Medical University for his valuable
help in computer processing and to Dr. Asako Makino for
excellent assistance. Finally, we acknowledge the efficient
help of Mr. Craig Allen Panner of Oxford University Press
in preparing this book.
 EXPLANATORY NOTES
Regarding the use of medical terminology, the following
principles are adopted in this book.
A symptom is a subjective condition determined from
the complaints of the patient during the history taking.
A sign is an objective finding identified by the physician
during the physical examination.
Lesion is the site of the nervous system that is affected
by pathology or functionally impaired. Disturbance is an
abnormality of nervous functions.
Rostral (applied to the brainstem and spinal cord)
means superior, or directed toward the cranium. Caudal
(applied to the brainstem and spinal cord) means infe-
rior, or directed toward the feet. In the brain, “anterior”
and “posterior” are used to describe front and back,
respectively. Ventral means inferior in the brain, and
anterior in the brainstem and spinal cord. Dorsal means
superior in the brain, and posterior in the brainstem and
spinal cord.
Neurologic refers to neurology (e.g., the neurologic
examination) whereas neurological refers to a disease
process (e.g., a neurological symptom).
 ACKNOWLEDGMENTS
The following figures and table were reproduced from
Diagnosis of Neurological Diseases, 2nd edition, Igaku-​
Shoin Ltd. Tokyo, 2013, with kind permission:
Chapter 1: Figures 1-​1, 1-​2, 2-​1, 4-​1, 5-​1, 5-​2, 5-​3, 5-​4,
5-​5, 7-​2, 7-​3, 7-​5, 7-​9, 8-​2, 8-​3, 8-​4, 8-​5, 9-​1, 9-​2, 9-​3, 9-​4,
9-​5, 9-​6, 9-​8, 10-​1, 10-​2, 10-​3, 11-​1, 11-​2, 11-​3, 12-​1, 12-​3,
13-​1, 14-​2, 14-​4, 14-​6, 14-​7, 16-​1, 16-​2, 16-​5, 16-​6, 16-​7,
16-​8, 16-​13, 16-​14, 16-​15, 16-​16, 16-​19, 16-​21, 16-​24, 16-​
25, 16-​26, 16-​28, 16-​29, 16-​30, 16-​31, 16-​32a, 17-​2, 17-​3,
18-​1, 18-​2, 18-​5, 18-​7, 18-​10, 18-​12, 18-​13, 19-​1, 19-​2, 19-​
3, 19-​4, 19-​5, 19-​6, 19-​7, 19-​8, 19-​9, 19-​10, 19-​11, 20-​1,
20-​2, 20-​3, 21-​1, 22-​1, 22-​2, 22-​4, 22-​5, 23-​1, 23-​3, 23-​5,
23-​7, 23-​8, 23-​9, 24-​1, 24-​3, 29-​1, 30-​1.
Chapter 4: Table 4-​1, 4-​3, 8-​1, 9-​1, 14-​1, 16-​1, 16-​2,
16-​3, 18-​1, 18-​2, 18-​3, 19-​1, 19-​2, 22-​1, 24-​2, 25-​1, 25-​2,
25-​3, 29-​1, 29-​2, 30-​1, 30-​2, 30-​3.
Copyright to the following figures do not belong to the
Work. The third parties’ permissions are required.
Chapter 2: Figures 6-​1, 7-​1, 7-​4, 7-​6, 7-​7, 7-​8, 8-​1, 9-​7,
12-​2, 14-​3, 14-​5, 16-​3, 16-​4, 16-​9, 16-​10, 16-​11, 16-​12,
16-​17, 16-​18, 16-​20, 17-​1, 18-​3, 18-​4, 18-​6, 18-​11, 22-​3,
23-​2, 23-​4, 23-​6, 27-​1, 28-​1, and Table 24-​1.
 1.
DIAGNOSIS OF NEUROLOGICAL DISEASES
T
o diagnose neurological diseases in a systematic way,
it is practical and useful to take into account three
axes: axis 1, anatomic; axis 2, etiologic; and axis 3,
clinical. It is effective to consider these three axes through
all steps of neurologic diagnosis, from the history taking
to the neurologic examination, before choosing the neces-
sary laboratory tests.
1 . A XIS 1: AN ATO MI C D I AG N O SI S
While taking the history and carrying out the neurologic
examination, it is essential to consider whether the lesion
involves the central nervous system, the peripheral ner-
vous system, the muscular system, or more than one of
these systems. Furthermore, within each of these systems,
the sites of lesion should be estimated as precisely as pos-
sible. This is a special feature of neurology because any site
of the nervous system, if affected, gives rise to the symp-
toms and signs that are specific for that particular site. In
the cerebrum, for example, whether the lesion involves
the superficial gray matter (cortex), the deep gray matter
such as the thalamus and basal ganglia, or the intermediate
white matter should be considered. Furthermore, it is pos-
sible to estimate the precise location of the lesion within
each site of the cerebral hemisphere. As for the peripheral
nervous system, whether the lesion involves a particular
nerve, more than one nerve, or all the nerves diffusely
can be estimated. In addition, the precise location of the
lesion (the proximal or distal part of the nerve fibers) can
be also estimated. Furthermore, careful clinical examina-
tion allows us to estimate the kind of nerve fibers affected
(motor, sensory, or autonomic) and whether the axon or
the myelin sheath is affected.
For example, when the patient complains of muscle
weakness in one hand, it is useful to systematically take
into account the motor cortex, the corticospinal tract
through the cerebral white matter, internal capsule and
brainstem to the cervical cord, the anterior horn cells,
and then the α motor fibers, neuromuscular junction, and
finally muscles, in this order. Of course, this process can
be done in the opposite order from the periphery to the
motor cortex. For another example, if the patient com-
plains of numbness in one hand, it is practical to consider
the responsible site of lesion first in the peripheral nerve
innervating that region of the skin, then the brachial
plexus, dorsal root ganglion, dorsal root, gray matter of
the cervical cord, the somatosensory fibers in the cervi-
cal cord and brainstem, the relay nucleus in the thalamus,
the thalamocortical fibers, and finally the hand area of
the contralateral somatosensory cortex. This process is
often possible even during the step of history taking, if
carefully done.
A. D
 ISTRIBUTION OF THE LESION AT THE
TISSUE LEVEL
For neurologists, it is not enough to just estimate the
location of lesion at the level of gross neuroanatomy;
they are expected to be able to consider the more precise
distribution of pathology at the tissue level. The mode
of distribution of the tissue damage is important not
only to make the correct diagnosis but also to predict the
severity and prognosis of the symptoms and signs caused
by the lesion.
Five forms of representative distribution of tissue dam-
age are illustrated in Figure 1-​1, taking the localized lesion
in the cervical cord as an example. In a diffuse lesion, the
tissue is homogeneously damaged throughout. A typical
example of this case is transverse myelitis of the cervi-
cal cord, in which the patient is expected to show severe
quadriplegia at least in the acute stage. In a disseminated
lesion, the lesion is scattered as if the seeds are spread on
the ground. Pathologically it is characterized by perivas-
cular cell infiltration. An example is disseminated myelitis
as seen in neuromyelitis optica spectrum disorders (Sato
et al, 2014; Takahashi et al, 2007; Wingerchuk et al,
2015), acute disseminated encephalomyelitis (ADEM),
 Diffuse
Focal
Figure 1-​1 Five forms of representative distribution of tissue damage.
neuro-​Behçet, and HTLV-​I associated myelopathy (HAM/​
TSP) (Osame, 2002). In this condition, the lesion usually
extends also longitudinally over a relatively long distance
in the spinal cord. In contrast with acute transverse myeli-
tis, which usually manifests with quadriplegia (paraplegia
in case of the thoracic cord lesion) of severe degree below
the affected segment, the disseminated cord lesion typi-
cally presents with severe spasticity of the lower extremities
while muscle strength is relatively spared, and it is especially
so in the chronic stage.
A focal lesion is localized to an area in the central ner-
vous system and is typically seen in vascular ischemic disease
or tumor. Multiple sclerosis and metastatic tumors typically
show multifocal lesions. System involvement is character-
istically seen in many neurological diseases, and it affects a
group of neurons or a neuronal network that has specific
function(s). Typical examples are neurodegenerative dis-
eases such as Parkinson disease, motor neuron disease, and
Alzheimer disease. Intoxication due to heavy metals and
drugs also belongs to this group.
B. SELECTIVE VULNERABILITY
This is the basic concept for explaining system involvement
of the nervous system, for example due to heavy metal
or drug intoxication. A typical example is organic mer-
cury poisoning (Minamata disease), which is clinically
characterized by dysarthria and ataxia due to cerebellar
damage, constriction of the peripheral visual field due to
involvement of the occipital cortex, and hearing difficulty
(Hunter-​Russell syndrome) (see Chapter 7-​2B, p. 46).
2
Disseminated Multifocal
System
C. N
 EGATIVE VERSUS POSITIVE NEUROLOGICAL
SYMPTOMS AND SIGNS
Neurological symptoms and signs can be divided into
two types: negative and positive. The negative neurologi-
cal symptoms and signs are neurological deficits caused by
impairment or loss of functions of the nervous system, and
the positive ones are caused by over-​excitation of the nervous
system or loss of its inhibition. Examples of negative symp-
toms and signs are unconsciousness, memory loss, aphasia,
visual field defect, motor paralysis, bradykinesia, freezing of
gait, sensory deficit, and orthostatic hypotension. Examples
of positive ones are convulsion, involuntary movements,
spasm/​cramp, pain, and dysesthesia. Psychiatric symptoms
like hallucinations and delusions may also be interpreted
as positive symptoms. As positive phenomena are not
always present during the physical examination, it often
depends on the patient’s description or situations reported
by observers. Most of the negative signs are observable by
physical examination, but intermittent or paroxysmal nega-
tive phenomena like transient ischemic attacks and periodic
paralysis have to be judged by careful history taking.
2 . AX IS 2 : E T IOLOGIC DIAGNOS IS
Information about how the neurological symptoms started
(mode of onset) and how they have changed after the onset
(clinical course) provides the most useful clue to the cause
or pathogenesis of the disease. The mode of onset is classi-
fied into four types: sudden, acute, subacute, and insidious
(Figure 1-​2 and Box 1-​1).
 Sudden/acute onset Acute/subacute onset Remissions
& improvement & improvement & exacerbations

Insidious onset Insidious onset Paroxysmal

& progressive & slowly progressive & repetitive

Fluctuating

Figure 1-​2 Mode of onset.

BOX 1-​1 ONSET OF NEUROLOGICAL SYMPTOMS IN CEREBROVASCULAR DISEASES

Cerebral infarction is classified into atherothrombosis, lacunar infarction (see Box 2-​2), and cardiogenic embolism. The type of in-
farction can often be estimated from the clinical symptoms. In cardiogenic embolism, the initial neurological symptom is completed
suddenly all at once. Moreover, if the functions of cerebral regions supplied by different arteries are simultaneously impaired, it
supports the diagnosis of cardiogenic embolism. Atherothrombosis is further classified into three types: thrombotic, embolic, and
hemodynamic. The hemodynamic form tends to occur in the border zone territory supplied by two large arteries, especially when the
blood pressure drops (watershed infarction). Therefore, this form of infarction tends to be completed over a period of a few hours
during sleep at night. The embolic form of atherothrombosis occurs when a blood clot is detached from the wall of a large artery
and flows into its smaller branch to form an embolic infarction there. A typical example is occlusion of the ophthalmic artery by a
blood clot originating from atherothrombosis of the internal carotid artery. In the thrombotic form, thrombus formation in a cerebral
artery causes a local occlusion or stenosis of that artery, clinically causing acute neurological deficit of the corresponding functions.
Intracerebral hemorrhage is sudden in onset and the symptoms rapidly progress to cause loss of consciousness and headache.
Hemorrhages are common in the putamen, thalamus, pons, and cerebellum, and subcortical hemorrhages are not uncommon. Lobar
intracerebral hemorrhage is often caused by amyloid angiopathy. In this condition, there are β-​amyloid deposits in the wall of small
arteries in the cortex and leptomeninges. Its sporadic form is common in the elderly (Biffi & Greenberg, 2011). Cerebral amyloid
angiopathy may also be associated with transient focal neurological episodes (Smith et al, 2021) and magnetic resonance imaging
(MRI) findings of lobar cerebral microbleeds, cortical superficial siderosis, lobar lacunes, and white matter hyperintensities (Cha-
ridimou et al, 2015; Gokcal et al, 2021). Subarachnoid hemorrhage is characterized by sudden onset of severe headache rapidly
followed by loss of consciousness.
In case of atypical stroke presenting with headache, disturbance of consciousness, and convulsion, venous sinus thrombosis
has to be kept in mind. MRI with magnetic resonance venography (MRV) may help with the diagnosis (Idiculla et al, 2020). Small
juxtacortical hemorrhages on computed tomography (CT) imaging are characteristically seen in cerebral venous thrombosis (Coutin-
ho et al, 2014).

1 . D iagnosis of neurological diseases

 When the symptom reaches the maximal degree within
a week, its mode of onset is defined as acute. Acute onset is
commonly encountered in vascular diseases, inflammatory
diseases, and acute poisoning. When the symptom occurs
abruptly, its mode of onset is defined as sudden, commonly
seen in embolism and subarachnoid hemorrhage. The condi-
tions that start acutely tend to be followed by some improve-
ment if properly diagnosed and treated. The prognosis of
the conditions that start suddenly depends on the severity
of the pathology. When the symptom reaches the maximum
over a period of more than 1 week but less than 4 weeks after
the clinical onset, its mode of onset is classified as subacute.
It is commonly seen in inflammatory diseases or metabolic/​
toxic diseases. This definition of categories for indicating the
mode of onset is rather arbitrary, and it is actually important
to describe the exact time in the medical history.
When the symptom begins gradually and reaches the max-
imum over a period of more than a month, its mode of onset
is called insidious. The following clinical course in this case
is either progressive, as seen in brain tumors, or slowly pro-
gressive, as seen in neurodegenerative diseases. A polyphasic
clinical course with remissions and exacerbations is typically
seen in inflammatory demyelinating diseases such as multiple
sclerosis. Repetition of short episodes with intervals of variable
length is seen in paroxysmal disorders such as epilepsy and
migraine. Ion channel disorders such as periodic paralysis and
episodic ataxia may also show this pattern of clinical course.
A fluctuating clinical course is commonly seen in metabolic
or endocrine encephalopathy. In the disorders showing a par-
oxysmal or repetitive clinical course, the onset of each attack
or each episode is usually sudden or acute.
When describing the history of the present illness, it is
sometimes practical and useful to illustrate the mode of onset
and the clinical course in a diagram showing the severity of each
symptom in the vertical axis along the horizontally drawn time
course, as shown in Figure 1-​2. Since more than one symptom
may appear in many diseases, illustration of the time course for
each individual symptom will help you to understand the tem-
poral relationship among different symptoms.
As described earlier, neurological symptoms are either
negative or positive. When indicating the severity of each
symptom in the vertical axis of the illustration (see Figure
1-​2), a negative symptom or a neurological deficit is usually
correlated with the severity of the lesion or the tissue dam-
age, but that is not necessarily the case for positive symp-
toms. That is because the positive symptoms may result
from excessive excitation of the nervous system or loss of
inhibition. Furthermore, some positive symptoms, such
as pain and involuntary movements, may also result from
4
plastic changes of the nervous system during the process
of functional restoration following an acute insult, such as
what is typical following a stroke (see Chapter 33-​3, p. 288).
3 . AX IS 3 : C LINIC AL DIAGNOS IS
By considering the anatomic diagnosis and the etiologic diag-
nosis throughout the process of history taking and neurologic
examination as we have described, the most likely sites of
lesion and the most likely cause of the lesion can be combined
together, which will help you reach the most reasonable clini-
cal diagnosis. A few additional conditions might come out as
candidates for the differential diagnosis. Of course, a num-
ber of conditions may appear in mind as the candidates for
differential diagnosis during the history taking, but if an ade-
quate history is obtained, the differential diagnosis is typically
expected to be limited to two or three at most at the end of
the history taking. An excessive list of differential diagnoses
may be difficult to manage and may even make the neurologic
examination more difficult since the logic of the examination
needs to take the differential diagnosis into account.
B IB LIOGR APH Y
Biffi A, Greenberg SM. Cerebral amyloid angiopathy: A systematic
review. J Clin Neurol 7: 1–​9, 2011.
Charidimou A, Linn J, Vernooij MW, Opherk C, Akoudad S, Baron
JC, et al. Cortical superficial siderosis: Detection and clinical signif-
icance in cerebral amyloid angiopathy and related conditions. Brain
138: 2126–​2139, 2015.
Coutinho JM, van den Berg R, Zuurbier SM, VanBavel E, Troost D,
Majoie CB, et al. Small juxtacortical hemorrhages in cerebral venous
thrombosis. Ann Neurol 75: 908–​916, 2014.
Gokcal E, Horn MJ, van Veluw SJ, Frau-​Pascual A, Das AS, Pasi M, et al.
Lacunes, microinfarcts, and vascular dysfunction in cerebral amyloid
angiopathy. Neurology 96: e1646–​e1654, 2021.
Idiculla PS, Gurala D, Palanisamy M, Vijayakumaar R, Dhandapani S,
Nagarajan E. Cerebral venous thrombosis: A comprehensive review.
Eur Neurol 83: 369–​379, 2020.
Osame M. Pathological mechanisms of human T-​cell lymphotropic virus
type I-​associated myelopathy (HAM/​TSP). J Neurovirol 8: 359–​
364, 2002.
Sato DK, Callegaro D, Lana-​Peixoto MS, Waters PJ, de Haidar Jorge
FM, Takahashi T, et al. Distinction between MOG antibody-​positive
and AQP4 antibody-​positive NMO spectrum disorders. Neurology
82: 474–​481, 2014.
Smith EE, Charidimou A, Ayata C, Werring DJ, Greenberg SM. Cere-
bral amyloid angiopathy-​related transient focal neurologic episodes.
Neurology 97: 231–​238, 2021.
Takahashi T, Fujihara K, Nakashima I, Misu T, Miyazawa I, Nakamura
M, et al. Anti-​aquaporin-​4 antibody is involved in the pathogenesis
of NMO: A study on antibody titre. Brain 130: 1235–​1243, 2007.
Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis
T, et al. International consensus diagnostic criteria for neuromyelitis
optica spectrum disorders. Neurology 85: 177–​189, 2015.
 2.

HISTORY TAKING

H
istory taking, if appropriately done, is expected to
provide the most important information for reach-
ing the correct neurological diagnosis. Usually the
history is obtained from the patient, but if the patient is
unconscious, demented, aphasic, or uncooperative, the his-
tory must depend on the information obtained from the
patient’s family or accompanying persons. The information
to be obtained when taking the medical history includes the
present age, sex, chief complaints, the age of onset of symp-
toms, history of the present illness, past medical history,
social history, and family history. When describing the med-
ical history in the record or when presenting it at a medical
conference, it is customary and convenient to describe the
information in the above order, but when actually taking the
history, it does not always have to follow this sequence.
1 . T H E PR E S E NT AGE AND T H E AGE
AT ONS E T
Each neurological disease occurs in the population of
a certain age range (Figure 2-​1). However, as a con-
sequence of recent advances in the understanding of
pathogenesis and with changes in the environment, it
is increasingly recognized that there are exceptions to
the age predilection. For example, ischemic cerebro-
vascular diseases are common in the aged population
but can also occur in the young population (Box 2-​1).
Likewise, the paroxysmal disorders or the hereditary
degenerative diseases, which were thought to be com-
mon in children and young adults, may occur for the
first time in adults.

Vascular

Heredodegenerative Degenerative

Infectious

Autoimmune, demyelinating

Paroxysmal Trauma

Congenital Tumor

0 20 40 60 80
Age (years)

Figure 2-​1 Each neurologic disease occurs in the population of a certain age range.
 3 . H ANDE DNE S S
RISK FACTORS FOR VASCULAR DISEASES IN THE
BOX 2-​1
YOUNG POPULATION Since handedness is an important factor for higher corti-
cal functions, it can be confirmed when taking the history,
Strokes in young persons have become increasingly common
although it can also be confirmed while carrying out the
(Aarnio et al, 2014; Larrue et al, 2011). In addition to the com-
physical examination.
mon risk factors for vascular diseases such as diabetes mel-
litus, hypertension, hyperlipidemia, increased blood viscosity,
obesity, and heavy smoking, special conditions have to be con-
4 . C H IE F C OMPLAINT S
sidered for the young population. Those are congenital heart
disease, among others the patent foramen ovale associated
Some physicians might believe that the chief complaints
with aneurysm of the atrial septum, atrial myxoma, positive
are symptoms the patient mainly complains of, but that is
antiphospholipid antibody, migraine with aura, use of contra-
not always correct. Some patients might complain of what
ceptives, eclampsia, systemic lupus erythematosus, Behçet
they are mainly suffering from at the time of the history
disease, malignant atrophic papulosis, Degos disease, fibro-
taking, but that symptom might not be directly related to
muscular dysplasia, homocystinuria, Fabry disease (see Box
the neurological disease that the patient has. It is reason-
20-​5, p. 198), pseudoxanthoma elasticum, moyamoya disease,
able to choose the most important complaint(s) of that
and mitochondrial encephalomyopathy (see Box 19-​13, p. 181;
particular patient during the process of history taking.
see Chapter 3-​1, p. 9, for some of the skin abnormalities). Ho-
In this sense, to identify the chief complaint for a patient
mocystinuria is due to lack of cystathionine synthetase and is
is to find what the main problem is for that patient, and
inherited via autosomal recessive transmission. Clinically it is
thus it is a process of problem-​finding. When writing in
characterized by dislocation of lens and arachnodactylia sim-
the medical record or when presenting in conferences, the
ilar to Marfan syndrome. Moyamoya disease is characterized
chief complaint is usually described or reported before the
by an extensive network of abnormal collaterals associated
history of the present illness, but in clinical practice, the
with occlusion of the proximal cerebral arteries (Albrecht et al,
most appropriate chief complaint(s) can be chosen after
2015; Al-​Yassin et al, 2015). It has been commonly reported
completing the history taking.
in East Asian children, but it is also seen in whites (Tho-​Calvi
It is of utmost importance to find out what the real
et al, 2018). It causes ischemic attacks or convulsion in chil-
problem is for the patient and what he or she really means.
dren and subarachnoid hemorrhage in adults. Physiologically,
For this purpose, it is not always advisable to describe
hyperventilation causes marked, prolonged slowing in the elec-
exactly what the patient complains of; it is more reasonable
troencephalogram (EEG). Mitochondrial encephalomyopathy,
to interpret the patient’s words in reference to the medical
lactic acidosis, and stroke-​like episodes (MELAS) is known to
knowledge. For example, a patient who complains of “numb-
cause strokes at a young age (see Box 19-​13). This condition
ness” in the hand usually means a sensory abnormality such
also shows convulsions, migraine attacks, and mental retarda-
as a tingling or pinprick sensation, but some patients may
tion. Lactic acid is increased in the serum and cerebrospinal
express weakness by using the word “numb.” In this case, it
fluid, and ragged red fibers are seen on muscle biopsy. Recent
is not appropriate to describe just “numb” in the medical
use of drugs such as cocaine has drawn particular attention to
record; rather, it should be described as “weak.” Dizziness
this behavior as a risk factor for strokes in the young popula-
is a symptom commonly reported, but some patients use
tion (Sordo et al, 2014).
“dizzy” when they feel like fainting, others when they feel
unsteady, with real vertigo. In this case, these two should
be distinguished as much as possible by taking the history
2 . S EX carefully (see Chapter 13-​2, p. 14).
When describing symptoms when you are taking pre-
Many diseases are more common in one sex than in the liminary notes as well as when you are making the final
other. The sex of the patient is usually obvious from his notes in the medical record, it is advised not to use med-
or her appearance and from the medical record, but if ical terminology. For example, when a patient complains
any uncertainty exists, it should be confirmed during the of difficulty in speaking, it is not advisable to describe
history taking. Sex is the biological designation of male/​ it as either “dysarthria” or “aphasia.” Instead, it is much
female, while gender refers to identification. better to call it just “speech disturbance.” It is because

6
the first impression might have to be changed later as a
result of neurologic examination. It is reasonable to use
the medical terminology at the final stage of neurologic
evaluation. The chief complaint may not be single, and
more than one symptom can be listed as chief complaints
as necessary.
5 . HIS TORY O F THE PRESEN T I LLN ES S
When taking the history of the present illness, it is import-
ant to know how each symptom started and how it has
changed since its onset. Patients may certainly have more
than one symptom. Regardless of whether those symptoms
are caused by a single disease or multiple diseases, mode of
onset and clinical course should be carefully described for
each individual symptom. In this case, it is convenient to
illustrate the time course of each symptom in a diagram as
shown in Figure 1-​2 (p. 3).
For each symptom, it is also important to obtain the
information as to what time of the day, what season of the
year, or in what kind of conditions it tends to occur or to
worsen (Box 2-​2). Furthermore, it is useful to identify any
precipitating factor that might bring up or increase the
symptom or any factor that may help ameliorate it.
BOX 2-​2 LACUNAR INFARCTION
A small infarction (less than 15 mm) in the territory supplied
by a penetrating arterial branch in the deep cerebral hemi-
sphere is called a lacunar infarction. It mainly occurs in the
corona radiata, internal capsule, thalamus, basal ganglia,
and brainstem. Typical clinical manifestations are pure motor
hemiplegia or hemianesthesia without motor paralysis, but it
can be asymptomatic. An infrequent manifestation caused
by lacunar infarction in the internal capsule or pons is known
as dysarthria-​clumsy hand syndrome (Arboix et al, 2004). An
underlying pathology of lacunar infarction has been believed
to be lipohyalinosis of the penetrating arteries, which is com-
monly seen in hypertensive patients, but atherothrombosis at
the bifurcation of the penetrating arterial branch from a main
cerebral artery (branch atheromatous disease) has drawn
more recent attention (Caplan, 1989). The latter condition in
the internal capsule causes repetitive transient hemiparesis
(capsular warning syndrome), and a similar condition of the
lenticulostriatal artery and anterior pontine artery also caus-
es progressive motor disturbance (Yamamoto et al, 2011).
2 . History taking
If a patient reports experiencing a neurological symp-
tom in the past, you should describe in the history of the
present illness if you judge it to be related to or a phase of
the present illness or in the past medical history if you judge
it to be unrelated to the present illness. Likewise, if you
judge the present neurological condition to be caused by or
part of a systemic condition such as hypertension and dia-
betes mellitus, you can describe those systemic conditions
in the history of the present illness as well. Other systemic
conditions can be listed in the past medical history.
6 . PAS T ME DIC AL H IS T OR Y
Regarding the past medical history, the amount of detail
to be described in the medical record depends on the clin-
ical situation, but all medical histories must be recorded
if they are judged to be related to the present illness. As a
patient does not necessarily tell the physician all the past
medical history, it may be necessary to ask specific ques-
tions to make sure certain topics are covered. For example,
when the history of the present illness suggests a possi-
bility of strokes, information about the risk factors for
vascular diseases, including diabetes mellitus, hyperten-
sion, hyperlipidemia, history of cardiovascular diseases,
and history of smoking, should be obtained as much as
possible (Otite et al, 2017). Among cardiac arrhythmias,
atrial fibrillation is known to be a leading cause of recur-
rent stroke. In particular, paroxysmal atrial fibrillation,
which is also an important risk factor, may be detected
only by long-​term electrocardiographic (ECG) monitor-
ing (Gladstone et al, 2014). Therefore, it is important to
know whether such testing was done.
It is noteworthy that some infections might cause neu-
rological disorders a long time afterwards. Subacute scleros-
ing panencephalitis can occur in adults following childhood
measles virus infection, and myelitis can occur years after
varicella zoster virus infection (postherpetic myelitis).
In these cases, confirmation of the past medical history is
important for the correct diagnosis.
7 . S OC IAL H IS T OR Y
This includes the information about the patient’s occupa-
tion, allergies, smoking, drinking, hobbies, sports, and envi-
ronmental situations at home and at work. Just like the past
medical history, the information needed depends on the
history of the present illness.
 8 . FA M ILY H I STO RY
As many neurological diseases are inherited and as the
background or precipitating factors might be genetically
determined, information about the family is quite import-
ant in the history taking. Occurrence of similar diseases
among the family members is important, and a history of
consanguineous marriage in the patient’s parents or grand-
parents is also important. When there is a possibility of
familial conditions, it is useful to draw a family tree (with
the patient’s permission). It is as important to know the
family members who are normal as well as those who are ill.
BIBL IOGRA PHY
Aarnio K, Haapaniemi E, Melkas S, Kaste M, Tatlisumak T, Putaala J.
Long-​term mortality after first-​ever and recurrent stroke in young
adults. Stroke 45: 2670–​2676, 2014.
Albrecht P, Blasberg C, Lukas S, Ringelstein M, Müller A-​K , Harmel J,
et al. Retinal pathology in idiopathic moyamoya angiopathy detected
by optical coherence tomography. Neurology 85: 521–​527, 2015.
Al-​Yassin A, Saunders DE, Mackay MT, Ganesan V. Early-​onset bilat-
eral cerebral arteriopathies: Cohort study of phenotype and disease
course. Neurology 85: 1146–​1153, 2015.
8
Arboix A, Bell Y, Garcia-​Eroles L, Massons J, Comes E, Balcells M, et al.
Clinical study of 35 patients with dysarthria-​clumsy hand syndrome.
J Neurol Neurosurg Psychiatry 75: 231–​234, 2004.
Biffi A, Greenberg SM. Cerebral amyloid angiopathy: A systematic
review. J Clin Neurol 7: 1–​9, 2011.
Caplan LR. Intracranial branch atheromatous disease: A neglected,
understudied, and underused concept. Neurology 39: 1246–​
1250, 1989.
Charidimou A, Martinez-​Ramirez S, Shoamanesh A, Oliveira-​Filho J,
Frosch M, Vashkevich A, et al. Cerebral amyloid angiopathy with
and without hemorrhage. Evidence for different disease phenotypes.
Neurology 84: 1206–​1212, 2015.
Gladstone DJ, Spring M, Dorian P, Panzov V, Thorpe KE, Hall J, et al.
Atrial fibrillation in patients with cryptogenic stroke. N Engl J Med
370: 2467–​2477, 2014.
Larrue V, Berhoune N, Massabuau P, Calviere L, Raposo N, Viguier A,
et al. Etiologic investigation of ischemic stroke in young adults. Neu-
rology 76: 1983–​1988, 2011.
Otite FO, Liaw N, Khandelwal P, Malik AM, Romano JG, Rundek T,
et al. Increasing prevalence of vascular risk factor in patient with
stroke: A call to action. Neurology 89: 1985–​1994, 2017.
Sordo L, Indave BI, Barrio G, Degenhardt L, de la Fuente L, Bravo MJ.
Cocaine use and risk of stroke: A systematic review. Drug Alcohol
Depend 142: 1–​13, 2014.
Tho-​Calvi SC, Thompson D, Saunders D, Agrawal S, Basu A, Chitre M,
et al. Clinical features, course, and outcomes of cohort of pediatric
moyamoya. Neurology 90: e763–​e770, 2018.
Yamamoto Y, Ohara T, Hamanaka M, Hosomi A, Tamura A, Akiguchi
I. Characteristics of intracranial branch atheromatous disease and its
association with progressive motor deficits. J Neurol Sci 304: 78–​
82, 2011.
 3.
PHYSICAL EXAMINATION
1. G
 ENE RAL PHYSI CAL EX AMI N ATI ON AND
NEURO LO G I C EX AMI N ATI O N
Even in the specialty clinic of neurology, general physi-
cal examination is as important as neurologic examina-
tion, mainly because all health aspects are important for
the patient and also because the neurological symptoms
are often related to systemic diseases in terms of clinical
manifestation and pathogenesis (Box 3-​1).
The general physical examination and neuro-
logic examination can be carried out separately in
this order, but it is often more practical to carry out
the two together. Otherwise, a series of examinations
BOX 3-​1 CLINICAL NEUROLOGY FROM A WIDE VIEWPOINT
It is important to take a wide view throughout the diagnostic
procedure of neurological diseases. That is because (1) the
neurological symptoms in question might be a manifestation
of a systemic disease, like Crow–​Fukase syndrome (polyneu-
ropathy, organomegaly, endocrinopathy, M-​protein, and skin
changes; POEMS) (p. 12); (2) the neurological symptom
might be a complication of a systemic disease, like diabet-
ic polyneuropathy; (3) the neurological symptom might have
resulted from a systemic disease, like cerebrovascular dis-
eases as a result of hypertension, hyperlipidosis, and cardi-
ac diseases; and (4) the neurological disease might have a
common pathogenesis with a systemic disease, like para-
neoplastic syndrome (see Box 3-​2). In bulbospinal muscular
atrophy, as another example of this last category, the genetic
abnormality of the androgen receptor might be related to the
involvement of anterior horn cells (see Box 17-​2, p. 137). Ap-
proaching neurological conditions with a wide view has con-
tributed to the discovery of some new neurological diseases
or to elucidation of their pathogenesis.
from the top of the head to the toes will need to be
repeated, which is not practical particularly when the
time spent for each patient is limited in an outpatient
clinic. Thus, when examining the eyes for example, we
can check the cornea, palpebral conjunctiva, bulbar
conjunctiva, and ocular bulb first, and then we can
examine the visual acuity, visual fields, pupils, extra-
ocular muscles, ocular movements, and ocular fundi if
necessary. When examining the oral cavity, for another
example, we check the mucosa of the oral cavity and
pharynx, teeth, tongue, and tonsils first, and then
check for the presence or absence of muscle atrophy,
fasciculation and movement of the tongue, position
and movement of the soft palate, gag reflex if neces-
sary, and voice, speech, and swallowing. This way, we
do not have to come back to the eyes or mouth after
having completed the general physical examination.
This approach is more efficient not just for neurolo-
gists but also for the patient’s convenience.
A. G
 ENERAL PHYSICAL EXAMINATION AT THE
INITIAL CLINICAL EVALUATION
When examining each patient for the first time, the gen-
eral physical examination usually includes observation of
(1) head, hair, and facial skin, (2) eyes (eyelids, cornea,
palpebral and bulbar conjunctivae, and iris), (3) ears and
nose, (4) oral mucosa, pharynx, and tongue, (5) neck
(lymph nodes, thyroid, and vascular bruit if necessary),
(6) arterial pulse and blood pressure, (7) chest (skin, res-
piration, percussion and auscultation of heart, and breast
as necessary), (8) abdomen (skin, percussion, palpation
and percussion of liver, and bowel sounds as necessary),
and (9) extremities (skin, joints, and edema), in this order.
Pulsation of arteries should be checked at the radial arter-
ies bilaterally, and the dorsalis pedis arteries should also be
palpated as necessary.
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 CHAPTER XXIII
A FRIEND IN ADVERSITY

Blue skies and sunshine and a rippling silver sea, all nature
jocund and gay, and for the first time in her young life Meriel was
unresponsive to the appeal. The romance had gone out of life. The
man she had learned to love was a thief—a thief. She told herself
fiercely that she no longer loved him, that she had never loved him,
but the ache at her heart gave the lie to the declaration.
The perpetual ache at her heart! If she could only have shared
the burden with Mrs. Marven, taken advantage of her Aunt's
sympathetic counsel, sobbed out her trouble and her despair on that
motherly breast, the pain would have been easier to bear. But she
could not do that. Thief though he was, Meriel could not betray Guy's
confidence.
Mrs. Marven wondered at the girl's reticence. Yet she waited in
patience for the confidence which was not yet given. Sooner or later
she felt it would be given to her. Her husband had told her of Guy's
conversation with him. She gathered that no ordinary blow had
shattered her darling's romance, and, though she longed to counsel
and to comfort, to mingle her tears with Meriel's, yet she did not
press her for the confidence which was withheld.
Yet, though the girl suffered, she strove to put her misery from
her, to busy herself with trivial duties. She went to the garden, but
she could not remain amongst the flowers. Her glance fell on a rose
Guy had tied. Now it was battered by the storm, and shapeless—like
her romance. She passed through the garden, across the meadow,
and on to the sea-wall. She desired to be alone, that was all. She
strolled idly along, unseeing anything but the tide slowly rising over
the mud, quite unconscious of the approach of an acquaintance.
 Mr. Hildebrand Flurscheim had recognised Meriel while yet
some distance away, and had hastened his footsteps. He had
wondered that she was alone, but congratulated himself on the fact,
for then Guy would probably be alone, too. He wanted a private
conference with Guy. He, too, had been troubled greatly in his
thoughts during the previous day and night. Ever since Cornelius
Jessel had revealed to him the fact that one item of his missing
property was in Guy's possession, he had been rejoicing in the
thought that vengeance on his spoliators was soon to be within his
reach. Not that he wholly trusted the valet's word. The shadow-man
was not possessed of a confidence-inspiring personality, but the man
had not asked for any cash on account for the information he had
furnished. Flurscheim judged that the informer must have been quite
convinced as to the truth of his information, or he would not have
been so confiding. Flurscheim had arranged with him to secure the
miniature so that he might identify it. After that his course would be
simple. The police would do the rest. He blessed the lucky chance
which had brought him to recognise the strange likeness which
existed between Meriel and the missing miniature. But for that
curious coincidence he might never have had the opportunity of
getting on the track of his missing treasures.
Meriel had been correct in her intuition when she read another
meaning in his warning to Guy to beware of the approaching storm.
He could not resist the jest. But then the events which had
immediately followed played havoc with his plans and projects.
Guy had saved his life. Would the man who had robbed him
have done that? Hildebrand Flurscheim set great store by his life.
When he felt himself falling, when the water closed over his head, a
great horror possessed him. He struggled madly, blindly, against the
fearful thing. Then a strong hand had gripped him. He had still
struggled until dimly he had comprehended that a cool voice was
commanding him to be still, telling him that he was safe. The words
repeated again and again had impressed themselves upon his
consciousness. He had ceased to struggle. He had trusted entirely to
the strong hand which supported him. Then, he knew not how, other
hands had lifted him up and taken him into a boat. He had opened
his eyes and seen Guy clamber up over the stern, had heard him
say, "Not much the matter, eh, Mr. Flurscheim?"
The experience seemed to have lasted hours. He said little, but
he had insisted, when Guy was put aboard the Witch, on shaking the
young man's hand. And when, afterwards, the horror of those
moments returned to him there was renewed in his mind the feeling
of gratitude to his preserver. He was glad that it was in his power to
repay in some measure the debt of gratitude he owed. Guy had
given his life. Well, he could give Guy liberty. Yet he was not
altogether satisfied in his mind. He had no reason for thinking that
Guy had worked single-handed, and he saw no reason why other
parties should be participants in his gratitude. Yet, rather than Guy
should suffer, all the guilty parties should escape. Certainly the
experience of being at hand-grips with death had wrought a
wonderful change in Mr. Hildebrand Flurscheim's views, for it was
with this determination in his mind that he left his yacht, intending to
call at the Hall and tell Guy of Jessel's communication, and of his
determination not to act upon the information.
Not until he was a yard away and he spoke, did Meriel
recognise the connoisseur. A shadow of annoyance crossed her
face. This man seemed to her to be the cause, though a perfectly
innocent cause, of Guy's undoing. She bowed slightly, and would
have passed by, but Flurscheim stood in her path.
"Pardon me, Miss Challys," he said, "can you tell me where I
can find Mr. Guy Hora? I have something of importance to say to
him."
There was a shade of agitation in his voice. Meriel was startled.
Again the thought came to her, "Did he suspect Guy? Was that the
explanation of the presence of himself and his yacht at Whitsea?"
She strove to answer steadily.
"Mr. Hora left for London by the first train this morning."
An exclamation of annoyance escaped Flurscheim. "It is most
important that I should see him at once. Most important. Will you
give me his address? I must send him a wire immediately."
 The colour left her face. She trembled. She was sure now that
Flurscheim knew who had robbed him of his treasures, that his
anxiety was due to the fear lest the robber should escape him. How
could she warn Guy? How could she refuse to supply Flurscheim
with the address he asked? Even if she were to refuse, her aunt or
uncle would give it. They knew of no reason why it should be kept
secret.
Flurscheim saw her hesitation, and a strange suspicion
germinated in his mind. "Surely she is not one of the gang," he
thought.
His intent gaze increased her discomposure. His suspicions
increased. He ventured a bold stroke.
"I wish to make some return for the service Mr. Hora has
rendered me," he said quietly. "Facts have come to my knowledge
which go to show that he is in considerable danger. If you do not
trust me, will you convey to him the warning?"
Again she looked at him doubtingly. "You would be his friend?"
she asked amazedly.
"If Mr. Hora will allow me," he answered, and seeing that she
still hesitated, he continued earnestly. "Will you not believe me, Miss
Challys, when I say that I would do anything in my power to save Mr.
Hora from a fate which would mean absolute ruin to him. May I go so
far as to say that if you are in the slightest degree interested in his
welfare you will not waste any time in communicating to him the
message I will give you."
There was a deep feeling expressed more in his tone than the
words he used. Instinctively Meriel felt that he was to be trusted.
"I will give you Guy—Mr. Hora's address," she remarked. "I
cannot communicate with him myself."
"Why—I thought——" he remarked and paused.
Meriel did not pretend to misunderstand the swiftly checked
exclamation. She shook her head sadly.
 "I am a very unhappy girl, Mr. Flurscheim," she said, and despite
her efforts tears mounted to her eyes.
"There! There! There! Say no more," interrupted the
connoisseur hastily. "Misunderstandings will occur between young
people."
Meriel again shook her head. "Yesterday Mr. Hora told me
something regarding himself which I could not have suspected; part
of what he told me concerned you, Mr. Flurscheim, and—so he has
gone."
"Then the miniature did not come into his possession by
accident!" ejaculated Flurscheim. "And all the time I was hoping that
it had."
"No," said Meriel. "It was no accident." The words slipped from
her. Flurscheim realised that she knew all about the robbery. He
began to question her eagerly, but she would answer him nothing.
Already she had been betrayed into a confidence which she
regretted, and when he realised her difficulty, he ceased to ask for
details.
"It makes no difference in my intentions," he said. "Whether Mr.
Hora was himself the burglar who stole my picture and the
miniatures, or whether he was only one of the parties who handled
them afterwards, would make no difference to my course of action. If
he is warned immediately there will be time for him to clear out of the
way. If not——" He shrugged his shoulders expressively.
"But why?" asked Meriel.
Flurscheim repeated the story Jessel had told him.
"I can keep the man's mouth closed for a certain time," he said,
"but sooner or later he will blab it out, and once the police get on the
track——" Again he shrugged his shoulders.
Meriel was more than ever amazed at the Jew's attitude. She let
some of her surprise escape in speech.
"You, knowing where some of your valuables are, are willing to
forego all chance of their recovery, to let the—the thief—go
unpunished? You who everybody says had determined to spend the
whole of the rest of your life on the recovery."
The Jew spread out the palms of his hands in a
characteristically racial gesture.
"The rest of my life," he said. "My life would have been ended
yesterday but for Mr. Guy Hora. Strange as it may seem, life is very
good in my eyes. I might never have known how good but for my
accident. It was slipping away and he gave it back to me. That was a
debt which I must repay. Miss Challys, never yet have I failed to
meet every obligation that I have incurred. Ask Christian or Jew who
has ever had dealings with me if Hildebrand Flurscheim has ever
failed to take up his bond when it became due."
She murmured something about the nobility of his attitude, but
he would not accept any such complimentary description.
"It is just my business point of view," he remarked drily.
"Suppose I put the value of the pictures at fifty thousand pounds.
Personally I would give double that amount for my life, though I
should very much doubt whether anybody else would give as many
pence."
Laughter and tears strove for mastery in her face.
"You have greatly relieved my mind, Mr. Flurscheim," she said
softly. "Though I shall never see Mr. Hora again, yet I—I could not
bear to think of him in prison."
"You had better see him again, and quickly, too, if you want to
keep him out of it," snapped the Jew promptly in response. "It will
want a woman's hand to do that, and from what I have observed you
are about the only person in the world who has half a chance of
succeeding."
The direct attack, voicing the thought she had striven to
suppress, brought the colour surging to her cheeks. "Don't you
understand," she cried. "He is a thief—a thief."
"He seems to be a rare plucky one anyway," answered
Flurscheim. Meriel laughed hysterically. "There! There! There!" said
the connoisseur in his most soothing manner, "I didn't mean to hurt
you, and I can see you are very much upset. Perhaps we will talk
over this bad business later and see what we can manage between
us."
There was so much kindness in his manner, so real a delicacy in
his whole attitude, that Meriel felt more than ever inclined to confide
the whole story to him. He was a man of the world. He owed Guy a
debt of gratitude. She had not promised Guy to keep silence. He had
never asked her, for he had realised that the request would have
been an insult. There was little time to argue the matter with herself.
Flurscheim was impatient to depart. She obeyed the impulse.
"Mr. Flurscheim," she said, "will you treat what I tell you as
strictly confidential, and take no action without my permission?"
"I give you my word," he said gravely.
Meriel glanced round her. No living soul was in sight. They stood
alone upon the sea-wall. Flurscheim noted her glance.
"There's no chance of being overheard," he remarked. At a little
distance was a stile in a rail fence which separated one meadow
from another. Flurscheim pointed to it.
"Come along," he said brusquely, and there the girl made known
to her companion the story of Guy's life as she had heard it from his
lips.
"He is not so greatly to blame, is he, Mr. Flurscheim?" she
asked eagerly, when she had finished the narration. "And yet nothing
can alter the fact that he is a thief."
The Jew had listened with growing amazement. To him the story
seemed the wildest, maddest romance he had ever heard. He could
scarcely believe his ears. To Meriel's appeal he could only reply at
first with an Hebraic invocation, uttered beneath his breath. Then he
asked, "And you really think he was telling the truth?"
"If you had seen his face, you would have had no need to ask
the question," she answered sorrowfully.
 "It's the tallest story I've ever heard," he remarked. "But whether
true or not, if we want to do anything for him the first step is to get
him out of the hands of that old scoundrel of a father, and," he
added, "I am more than ever convinced that only a woman can do
that. Think it over, Miss Challys, think it over." He glanced at his
watch. "Now if you will give me his address, I will be off. I have a
number of things to do before returning to town."
She gave him the address. She responded heartily to his
farewell, and as she stood watching him as he hastened along the
path towards the quay it seemed to her that already the burden of
her trouble was lightened. She trusted Flurscheim implicitly, and yet
twenty-four hours previously she would have been prepared to
assert that he would have been the last person in the world of whom
she would have taken counsel in her distress.
Not that her mind was at rest. She merely was relieved from
anxiety as regards Guy's immediate safety. As to the future there
was no trace of a silver lining to the clouds. Guy and she had parted.
Yet Flurscheim's words stuck in her brain. "Only a woman could help
him." Only a woman!
 CHAPTER XXIV
INSPECTOR KENLY CONTEMPLATES ACTION

Events were coiling themselves swiftly, and Lynton Hora at the

very centre of the coil was inert, motionless, unaware of the web
which was being spun about him. Indeed some of those various
parties who were the actors in the events, the puppets who had been
set in motion by Hora's initiative to spin the web for his own
entwining, were unaware even that they were spinning such a web.
But Inspector Kenly knew. He guessed that there must have
been some master mind behind Guy's exploits, and once he had
fixed upon Lynton Hora as the possessor of that master mind he was
a sleuth hound on the trail. Where Hora was, Kenly was not far
distant. When Hora left Westminster Mansions on the night of Guy's
arrival, Kenly was at his heels. He tracked him to a railway station,
back from the railway station to one of the big London hotels. He did
not leave the premises until Hora had retired for the night. Then he
went back to Westminster Mansions and learned of Guy's arrival
there. He was very curious as to the meaning of the movements of
the two men. He could not watch them both, and though he longed
to have followed the business through without assistance the risk
had become too great. He went in haste to Scotland Yard and
requisitioned the assistance of a subordinate to watch the Mansions.
He himself returned to the hotel. He was still more astounded next
morning when Hora returned quietly to his own abode, and was
totally unable to deduce anything from the fact that Guy had left the
flat at daybreak. It was so mysterious that he could not rest. He went
again to the Yard and asked for another man.
"I think I am on the track of the Flurscheim gang," he explained.
"It's only a case of suspicion at present, and I still have the Foreign
Office business in hand."
 "The Yard" was sore at its failure to solve the mystery of the
stolen picture, and a dozen men would have been at the Inspector's
command if he had so desired. He promised to explain later, saying
that the necessity for placing his men was urgent, and so he
managed to keep his information to himself. His instructions to his
subordinates were simple and explicit. Unseen themselves, one was
to follow Lynton Hora, the other was not to lose sight of Guy. If either
of the two under surveillance attempted to leave the country he was
to be detained. The warrant would be forthcoming if needed. Already
the Inspector had his information drawn out. But there were three
names in that information, and the third was that of Captain Marven.
His subordinates instructed in their duties, the detective hurried
off to the Foreign Office. Ever since the Permanent Secretary had
suggested asking Captain Marven for an explanation Kenly's mind
had been busy with the idea. Though he had been horror stricken at
first, the more he pondered over it, the more advantageous it
seemed. Though Captain Marven's name appeared in his unsworn
information, he still doubted whether the King's Messenger could be
hand in glove with the Horas. If the suggested interview took place in
his presence, he would be able to judge by the Captain's demeanour
as to whether he was in any way implicated in the despatch
business. If implicated in that, there would be every reason to
suspect that he must have had a hand in the Flurscheim burglary. So
he sought out the Permanent Secretary and proffered his request. It
met with a ready acceptance. "Captain Marven," said the Secretary,
"is next on the rota for service. If I had not employed him, the mere
fact would have caused comment as well as arousing his suspicions,
and I really could not have employed him while this cloud is hanging
over him."
"He will be expecting a call to town then?" asked Inspector
Kenly. "If he gets the usual notice he will be taken entirely by
surprise on finding why he has been summoned."
"I will wire for him, if you like," said the Permanent Secretary.
"No," said the detective, "to-morrow will do."
 "Then I'll have a letter sent to him in the ordinary way," said the
Permanent Secretary. "Of course, you understand," he continued,
"that we shall take no action in regard to the despatch business
beyond dispensing with Captain Marven's services. It would be fatal
to our diplomacy if the impression were to get abroad that any of our
people were not absolutely trustworthy."
Inspector Kenly looked aghast. "But, what if he is concerned in
the other business?" he asked. "Mustn't he pay the penalty?"
"The penalty of twenty-four hours' law in which to get out of
England, with someone in charge of him to see that he does not
communicate with the rest of the gang," said the Permanent
Secretary drily. "Exile will be punishment enough in itself."
Inspector Kenly sighed. He saw his case robbed of one of its
most sensational features, and he loved to see sensational
headlines to the newspaper reports of the cases in which he was
engaged. He left the Foreign Office less elate than he had entered it,
but, indefatigable still, he visited each of his subordinates in turn.
Neither had any movement to report. Both Lynton Hora and Guy
remained in their own abodes. Kenly thought that he had earned a
little repose. He got into a cab, drove to the railway station, and took
a train to Wimbledon. He fell into a doze before the train started, and
in his dreamy state he thought longingly of white sheets which were
awaiting him at Woodbine Cottage. For forty-eight hours he had not
closed his eyes. He felt thoroughly wearied.
He alighted at his station and stepped out briskly homewards.
Then, as he turned into Melpomene Road, he saw in front of him a
figure which he recognised. He was so surprised that he pinched
himself to assure himself that he was not dreaming.
"My friend Mr. Jessel, by Henry!" he remarked. "I wonder what
he is doing down in this quarter again."
He moderated his pace and followed Jessel until the latter, on
arriving at the gate of Woodbine Cottage, lifted the latch and
entered. By the time the Inspector had reached the gate Cornelius
had knocked at the door, and as Kenly raised the latch, Mrs.
Inspector Kenly appeared in answer to the summons of her late
lodger. But Mrs. Kenly paid no attention to her visitor. Her glance had
travelled beyond him and rested on her husband, whom she had not
seen for a fortnight. She brushed the astonished Cornelius aside and
darted along the little path. The shadow-man felt quite embarrassed
by the warmth of the connubial greeting. He called attention to his
presence by a little cough. It was successful. Kenly looked over his
wife's shoulder and gave a well feigned start of surprise.
"Blessed if it isn't our friend, Mr. Jessel," he remarked. "Who
ever would have thought of seeing you here again? Anyhow, I take it
very kindly your looking us up so soon. Come in, old chap, come in.
You must excuse the missus and me. I'm only just back from my little
holiday, you know. We don't do this sort of thing in the front garden
every night."
Mr. Jessel smiled and Mrs. Kenly opened her eyes widely. She
had never known her husband to be so effusive with a lodger, new or
old. But like a wise woman she said nothing, but, blushing a little,
seconded her husband's invitation.
"If—if I had known you were expected," said Jessel turning to
Kenly, "I would have postponed my call for an hour. Perhaps you will
allow me to come back a little later."
"Not at all," said Kenly heartily. "Come in and have a cup of tea
with us. I'm sure the wife will be proud to give you one."
Mrs. Kenly opened her eyes wider than ever. She could very
well have dispensed with her late lodger's company, but she had
suddenly realised that her husband must have some hidden reason
for his geniality. Had he not arrived at Jessel's very heels? Scenting
a mystery she played up to the Inspector's lead, and Jessel, visibly
gratified by the warmth of the greeting, no longer rejected the
proffered hospitality. In a very few minutes he was seated at the
detective's table doing justice to the delicacies which Mrs. Kenly
produced from larder and cupboard in honour of her lord's return.
He had soon revealed the motive for his call. He wanted to know
if the room he had occupied was still unlet, for, if so, he desired,
having suddenly lost his situation, to return to the place where he
had been so comfortable. Mrs. Kenly hardly knew what to reply. She
was already in negotiation with another party. But her inclination
towards so quiet and well behaved a lodger as Mr. Jessel,
strengthened by the surreptitious nod of approval from her husband,
ultimately led to her declaring that she thought the negotiations in
progress might be broken off and Mr. Jessel installed in his former
quarters.
Cornelius was delighted, and said so.
"I should like to come in this very night," he said. "I have
nowhere but an hotel to go to, and I never can sleep at an hotel."
Again in obedience to her husband's mute request Mrs. Kenly,
after a decent amount of hesitation, acceded to his wishes, and
when the shadow-man at last left them it was merely to fetch his
baggage.
Mrs. Kenly accompanied the returned lodger to the door, and
when she returned to the parlour she found that her husband had
disappeared.
"Well, I do think he is not treating me quite fairly," she
murmured, and unaccustomed tears rose to her eyes. But they dried
rapidly as she heard a thud on the floor of the room overhead and
recognised the fall of a boot. She hastened upstairs and found her
husband already half-undressed.
"Whatever in the world is the matter, Joe?" she asked. Inspector
Kenly grunted.
"Can't keep my eyes open any longer, not if you were to prop up
the lids with steel bars, Loo," he answered.
His eyes were not so closely shut, however, that they were
unable to detect the disappointment which was expressed in his
wife's face. He caught her in his arms and imprinted a couple of
kisses on her lips.
"I haven't had a wink of sleep for forty-eight hours," he said,
"and I only wish I could do without for another forty-eight. But a nap
while I get the chance will make another man of me. Now listen, Loo.
What time did Jessel say he was coming back?"
"He said he was afraid that it would not be much before
midnight," replied Mrs. Kenly.
"Give me a call at 11.30, then," said Inspector Kenly, and he
tumbled into bed and gave a huge sigh of relief. Then he raised his
head. "If he gets back earlier call me."
"But what makes you so interested in Mr. Jessel?" she asked,
unable any longer to restrain her curiosity.
"Come here, Loo," said Kenly, and as his wife drew near he
whispered, "That young man is mixed up in some way with the
Flurscheim burglary. No, don't interrupt. I haven't time to tell you all
about it, and that is much more than I ought to have told you. Now
you know why I was so anxious that you should get him back here."
"But suppose he doesn't come back," said Mrs. Kenly.
"He will come back right enough," answered the detective
drowsily as he laid his head again upon the pillow.
"But," said Mrs. Kenly, and then paused. Her husband's eyes
had closed. His mouth was slightly open. In another few seconds an
unmistakable snore made itself heard. Mrs. Kenly drew the blinds
and noiselessly withdrew downstairs. She was excited, but not too
excited to neglect her ordinary duties.
The hours passed slowly. When ten and eleven had gone
without any sign of Jessel she began to be alarmed. Still he had said
he might not be back until midnight. At half past eleven she aroused
her husband, thoughtfully taking with her a cup of tea. He was
sleeping so soundly that she was compelled to shake him before he
could be aroused. She had just succeeded when the sound of a cab
drawing up in the street outside the garden gate arrested her
attention. Kenly heard it too, and sprang out of bed.
"Keep him talking until I come down," he said.
The next minute the modest knock at the door announced
Jessel's arrival, and Mrs. Kenly hurried downstairs to let him in.
 "Half afraid I should have found you all in bed," he said as he
entered. "You don't mind leaving the door open while I fetch my
bags?"
By the time he had made two journeys to the cab Inspector
Kenly was standing by his wife's side, and he was inviting the
shadow-man to join him in a nightcap before retiring to rest.
Cornelius was agreeable. He followed his host into the tiny
parlour where Mrs. Kenly produced a bottle and glasses from the
sideboard and a jug of hot water and a lemon from the kitchen.
"It's just like coming 'ome," said Jessel.
"We'll make you feel at home here, right enough," replied the
Inspector.
When a little later Mrs. Kenly said good-night and retired Jessel
felt so much at home indeed that he was easily persuaded to take
"just one more" before following her example. He grew quite
talkative, yet even under the detective's skilful guidance he could not
be led to speak upon the one subject which his host was anxious to
get him to talk about. Kenly was afraid to put leading questions lest
he should become alarmed too early.
There was a time for all things, however, and the time came
when the Inspector thought it desirable that his companion should
receive a shock. It arrived when, after Jessel had knocked the ashes
out of his pipe and remarked that he thought it was about time to turn
in.
"There's one thing I want to ask you first," remarked the
detective quietly. "Who was the old gentleman, got up like a parson,
who came to visit you here?"
The shot told. Jessel grew suddenly pale and his jaw dropped.
"Old gentleman? What old gentleman?" he stammered.
Kenly did not reply immediately. He walked across the room and
deliberately turned the key in the lock and placed the key in his
pocket. Then returning he took a card from his waistcoat pocket and
laid it on the table before his companion.
 "You don't seem to be aware who I am, Jessel," he said
pleasantly.
Mechanically Cornelius lifted up the card, and as he read his
face grew longer than ever. He laid the card on the table. Kenly,
noticing his shaking hand, smiled.
"Detective Inspector Kenly from Scotland Yard," said Cornelius,
as if bewildered.
"That's me," declared Kenly. "And I mean to have an answer to
the question I have just asked you." Jessel's confusion was almost
pitiable to witness. The mine that had been sprung upon him took his
breath away. To think that he had been a tenant of a member of the
detective force, sleeping under the same roof, and that the Master
had come to see him there was too preposterous to be believed.
"You—you're joking," he stammered at last.
"Not the least little bit in the world, as you will find out to your
cost, Mr. Jessel, if you don't answer my questions," replied Kenly
readily. Then he added, "I know all about you, so I really think it will
be best for you to meet me in a friendly way."
Cornelius remembered a dozen little schemes of his which had
brought him in cash which would not bear the light of day, and his
heart quailed. He never for a moment imagined that Kenly was
merely bluffing. Still he made an effort in his own defence.
"You—you know nothing against me," he asserted. "I—I've
never done anything wrong." His tone and manner gave the lie to his
assertion, and Kenly began to feel on safe ground.
"You may not think so, but I'm quite sure a magistrate would not
agree with you," said Kenly with conviction. "Still I don't suggest
testing the matter if you will tell me what I want to know."
Cornelius saw no means of escape. He surrendered at
discretion, and when the conference was ended the detective was in
possession of the whole story of the shadow-man's connection with
Guy Hora from the moment when he had been employed by "the
Master" to watch over him until that day when Guy had suddenly
paid him a month's wages and bidden him depart at once.
Long after the conference was ended the detective brooded
over the information which had come into his possession. He could
not understand it. Jessel was manifestly unaware of the identity of
the man he called "The Master" with Lynton Hora. Nor could he
suggest any reason why Guy should have so suddenly dispensed
with his services. The detective did not enlighten Cornelius on the
first point, nor did he suggest that the second fact might have been
due to some discovery made by Guy that the casket containing the
stolen miniature had been tampered with. Jessel had revealed
everything, even the momentous discovery he had made, the
discovery he had communicated to Mr. Hildebrand Flurscheim.
Kenly tired of puzzling his brain after a while with theories. He
made his way once more up to his bedroom and resumed the sleep
from which he had been awakened.
 CHAPTER XXV
THE PARTING OF THE WAYS

A minute after Guy had peeped into her bedroom Myra

awakened. Her sleep had been short and she awoke unrefreshed.
She arose mechanically and was surprised that her maid was not
there, that her bath was not ready. She looked at a clock and saw
that the hour was not yet five. She lay down again upon her bed and
watched the clouds chasing each other across the sky. She fell to
counting them as they crossed her field of vision, bounded by the
two sides of the window frame. In the first hour there were seventy-
two, between six and seven, twenty-one, between seven and eight,
only three. When the maid came at half past eight the sky had been
untarnished for a whole half hour.
She told the girl to make the bath hot. The hot water was very
comforting. She found a physical satisfaction in the caress of the
warm water. As a child she had always delighted in her bath. She
recalled her childish delight. Anything to keep thought at bay.
After the bath she dressed slowly. The maid was exasperated,
but Myra was quite heedless of the fact. The day was hardly begun,
and there were so many hours in the day to be filled somehow with
anything that would stave off thought. At eleven she ordered
breakfast and sat down alone to it. The dishes went away
untouched. She took a newspaper into the drawing-room, but she
got no further than the door. It was there Guy had rejected the love
she had offered him. She had no feeling of shame, only she could
not remain there. She went instead to Lynton Hora's study. The room
awakened another thought. What would the Commandatore say? He
had told her to keep Guy, and Guy had gone. She remembered
Hora's unuttered threat, but she had not great fear of his anger. Still
she knew he would be angry, for Guy had offered to marry her, and
she had refused the offer. It was not marriage she wanted, only to be
loved, and she was compelled to refuse. But the Commandatore
would blame her, for Guy had gone. Her lips drooped at the thought.
Her spirit was broken.
Lynton Hora returned. She heard his step in the hall, the firm
footstep followed by the shuffle of his lamed leg. But she did not
attempt to move. He came straight to his own room. She did not
even glance up as he entered.
"Where's Guy?" His voice was harsh.
"Gone," she replied without lifting her eyes.
For a minute no other word was spoken. Hora paced the room,
up and down from door to window, and every time he turned to face
Myra the scowl on his face deepened. Her manifest distress
awakened no pity in him. He even marvelled that he had ever
thought her beautiful. Her face was dull and expressionless, the
lustre had gone from her hair, her figure drooped despondently. She
recalled to his mind a dropsical old woman, clad in rags, with a
palsied hand grasping at a bottle of gin in a dilapidated outhouse in
Fancy Lane.
"When is he coming back?" he snarled again.
"Never."
Did her lips fashion the word? She had no warrant for making so
definite a reply, but she knew that it was true.
Hora's anger nearly loosened a torrent of invective. But he
refrained. What was the use? Myra had failed. Guy was lost to him.
She was of no use to him now.
"If Guy has gone, you had better take yourself off, too," he said
deliberately.
She did not appear to hear him, and he repeated the command
with growing irritation.
He was surprised to see the tears trickle down her cheeks and
the corners of her mouth turn downwards. There had been no
snivelling about Myra in the past.
"I could not help Guy going. He does not love me," she said
meekly.
Hora's scorn could no longer be restrained. "Love," he sneered.
"The world is mad on the subject, the besotted idiocy of immature
brains. Because a girl would and a boy won't a man's plans and
schemes are to be wrecked. I'll be alone in future. You can take
yourself off as soon as you like."
"Where am I to go?" she asked.
Hora shrugged his shoulders. "You have the whole world to
choose from," he sneered. "Go where you like; your native gutter is
about the only place which is really suitable, but I don't care where
you go so long as you do not cross my path again."
For the first time Myra looked up. She met his glance, and so
fierce an anger blazed in his eyes that a thrill of physical fear passed
through her. Had she been in her normal condition the anger would
have awakened an answering flame in herself. But she was broken
in spirit. She shrank from meeting his anger. She rose listlessly from
her chair and went out of the room. She supposed that she must
obey. She had always obeyed Hora. But it was very hard to be
turned out thus. Where to go? That was a difficult question to decide
at a moment's notice. Perhaps the Commandatore was right, and
that her proper place was the gutter. The Commandatore was
usually right.
She gathered together some of her jewels, and dressed herself
in one of her smartest frocks. She had a vague idea that she was
doing unwisely, but the bright colour attracted her. Her brain had
room for only one thought. She pinned on her hat carefully and went
quietly out. She did not cast one glance backwards. The bracelet
Guy had had made for her from the stones which had originally
encircled the miniature was still clasped upon her arm.
Hora saw her leave, but he made no effort to check her. He had
not intended to turn her from his door, and noting the frock and the
hat he was quite easy in his mind. "She will return," he said to
himself, and straightway began to think of Guy. If Guy was never
going to return, Hora foresaw that he must seek him out. He acted
upon the decision at once, and drove away to the Albany. He still
had belief in his own powers of persuasion. The thought of using
Guy as a tool for his revenge had passed entirely out of his mind. He
wanted nothing but that Guy, the son of his adoption, should come
back to him.
At the Albany he arrived to find the newly furnished chambers in
confusion. Guy himself opened the door to admit him. He did not
appear surprised at the visit.
Hora enquired why Guy had answered the door himself.
"I've got rid of my man," said Guy.
"Retrenchment?" asked the Commandatore.
"Yes," said Guy.
"I don't see the necessity," said Hora.
"I do," answered Guy with gravity.
Hora had followed him into the sitting-room and stood there
expectantly. "Why?" he asked pointedly.
Guy hesitated. There was so much to explain that he did not
know where to begin. Hora's next question did not make his task
easier.
"Myra tells me that you have deserted us, is it true?" he asked.
"I can hardly say definitely. The answer in all probability will not
rest with me," replied Guy.
He realised suddenly the whimsicality of the position in which he
was momentarily placed—the position of defending himself from the
charge of refusing to continue a criminal existence. The thought won
a smile from his lips.
"You cannot tell me?" said Hora. "Have you considered
thoroughly?" He stepped forward and laid his hand on the young
man's arm. "Have you considered what such a decision would mean
to me, Guy? I am getting on in years. You have always been with
me. I might go far to meet your wishes, even to the extent of
abandoning my profession, if I could keep you with me."
"It would not be enough." Guy's answer was stern and hard.
Hora was startled by the tone. "What more can you require?" he
asked.
"What more?" said Guy bitterly. "What more?" His face flushed
and he held up his hand. "Atonement," he replied, "atonement for the
past."
There was accusation in Guy's tone, and Hora shrank under it,
but he rallied his wits. "Why so melodramatic?" he sneered.
"Oh, I know it sounds ridiculous in your ears," he answered, "but
I see no other way of regaining my own self-esteem." He turned
fiercely on Hora. "Why did you bring me up differently from other
boys? Why did you, day by day, week by week, and year by year,
instil into my ears your lying philosophy? Why did you make your son
a thief—a thief?"
All the concentrated bitterness of Guy's musings was infused
into the concluding words. Hora's lips grew pale and his hands
trembled as he listened. He recognised the emotion from which Guy
suffered by the memory of his own experience when he had himself
been branded in the light of day and the sight of all men. Still he
strove to meet the point of view.
"I thought you had learned to place their true value upon
conventional terms," he remarked.
"I have," said Guy, more bitterly than before. "I have learned that
a thief is a thief whatever sophistry may be used to throw a glamour
of romance over his actions."
"I never taught you otherwise," remarked Hora, "only that all
men are thieves, only that the hypocritical many steal under the
cloak of the law, and the intellectually honest few pursue their
avocation in defiance of the law. Why reproach yourself for
intellectual honesty?"
 Guy made no reply and Hora plunged into argument. "What is
theft? Merely the acquisition of the desirable by unconventional
means. Is it a virtue to gratify your desire by the same process as the
dull souls that are presumably dignified by the name of common
humanity? If so, virtue is a mere synonym for mediocrity. I thought
you knew better, Guy. I thought that you had learned that man owes
his chief duty to himself, that his desires are meant to be gratified,
that the most courageous way of gratifying his desires is the only
way for man to attain his highest development."
He pursued the theme with animation. Guy had seated himself,
leaning his head on his hand. Hora thought that his attitude was one
of deep attention. When he paused for breath Guy spoke:
"It is of no use, Commandatore. I have gone over the same
arguments with myself a hundred times, but I can no longer
persuade myself that they are anything but sheer sophistry."
Lynton Hora shrugged his shoulders. "I don't understand why
your opinions should have undergone so sudden a change."
"And yet you have known a good woman's love?" said Guy
suddenly.
The remark stung. Hora's eyes flashed and his lips closed tightly
for a few seconds before he trusted himself to speak.
"So that's the explanation," he said at last. "I thought as much. A
woman is responsible for every man's folly, and you like the rest are
ready to abuse your intellect at the bidding of some muling miss
whose intelligence will never allow her to discern the asses' ears
which adorn the image of the great divinity convention which she
worships in common with the majority of her feeble-minded fellows.
Who is this wonder who has robbed you of the use of your brains?
Am I right in guessing that she is of the family of that prince of
hypocrites, Marven? I can see I am right. And for one of that brood
you will cut yourself adrift from me, clothe yourself in the ready-made
fustian of the dull herd! An honest woman's love! There was never
an honest woman to be found amongst the Challys or Marvens——"
 His anger had carried him out of himself, and too late Hora
perceived that his virulent tongue had said too much.
Guy had drawn himself up, pale with anger.
"Sir," he said, "I must ask you to leave me to myself. I cannot
listen to abuse of one who is more to me than anyone else in the
world."
Hora strove to undo the effect of his words. "Chivalrous as ever,
Guy," he remarked quietly, though despite his intention a sneer
curled his lips at what appeared to him a ridiculous exhibition of
sentimentalism.
Guy did not reply to the taunt. He continued steadily:
"I must ask you to leave me, yet before you go, I will give you
fair warning of my intentions. You have learned of the alteration in
my opinions. I have told you that only by atonement shall I feel that I
can regain my self-esteem. There is only one atonement I can
make."
"Yes," said Hora breathlessly.
"I intend to surrender myself to justice," said Guy, "within the
next forty-eight hours."
Lynton Hora was stunned. The utter madness of the idea left
him bereft of the power of speech for a moment, and when the
capacity returned to him he could only think of one argument.
"You are not reckoning on Meriel Challys marrying you when
you have 'atoned,' as you call it," he said.
Guy shook his head. "I have no hope," he said wearily. "Good-
bye, Commandatore."
Hora made no answer. He knew that it was useless to argue
with Guy any longer. The set of his lips, the angle of his jaw, the
quietude with which he made the announcement were eloquent of
determination. The door closed behind him and he went out into the
street as one dazed. The first, a merely momentary impulse, was to
leave Guy to his own devices. But that passed. He became
possessed by fear—an overpowering fear of imminent danger to
himself. He judged rightly that Guy's chivalry, the chivalry which was
leading him to sacrifice himself to an ideal, would equally compel him
to keep silence in regard to his confederate. But Guy's silence would
not protect him if enquiries were pushed home in regard to either of
the two adventures in which Guy had taken part. Lynton Hora knew
that he could not escape suspicion, and suspicion once awakened
he knew that his career would come to an end. There loomed before
his mind the long days of dull routine, the still longer nights behind
the locked doors, the coarse food of the prison, the horrible convict
dress. Furtively he looked over his shoulder, for it seemed to him that
a hand was almost outstretched to grasp him.
Pooh! There was nobody taking any notice of him. The
pleasant-featured, sunburnt man who passed him at the moment
could have no idea whose sleeve he had brushed. The Master
crushed down his fear. Guy must be protected against himself, and
there was no time to waste. A lunatic asylum? Certainly Guy was
mad enough for one. But there would be many difficulties to be
surmounted, and time was short. Hora's mind became active as it
always did under stress of necessity. Was there no one who could
prevail upon Guy to forego his intention, no argument which would
appeal to him? Stay. There was one which might succeed.
Supposing Guy were to learn his real parentage.
Lynton Hora hastened his steps. He saw one chance of saving
Guy from the consequences of his folly, of saving himself also, and
at the same time paying his debt of hatred. Captain Marven
assuredly would never allow his son to consign himself to a gaol.
Guy would be too chivalrous to smirch the fair fame of a family to
which Meriel belonged. With his mind dwelling on this expedient,
Hora looked behind him no more. He was not aware that the man
with the sunburnt face kept him steadily in view until he disappeared
into his own abode. He did not suspect that Detective Inspector
Kenly, for he was the man who had brushed his sleeve, waited
patiently until he reappeared again and followed him discreetly until
he knocked at the door of Captain Marven's town house. The
Inspector only saw in that fact one additional piece of evidence of
Marven's guilty connection with the Horas. He saw that Hora put a
package into the hands of the servant who opened the door, and he
made a mental note of the fact. He guessed that the King's
Messenger had arrived in town in obedience to the summons which
had been sent him, and he assumed that he had communicated the
fact of his arrival to Hora. Still at the heels of his quarry he returned
again to Westminster Mansions, and there he transferred the duty to
one of his subordinates. The hour was two in the afternoon, and at
three he was to be a fourth at the interview between Captain Marven
and the Great Man and the Permanent Secretary at the Foreign
Office.
 CHAPTER XXVI
CAPTAIN MARVEN'S SURPRISE PACKET

When in response to the official summons Captain Marven

returned to town he was more perturbed than he would have cared
to confess, at the disastrous ending to Meriel's love affair. The
intimacy of everyday life had only confirmed the favourable
impression Guy had produced upon him, and he had looked forward
with pleasure to welcoming him as a member of his family. But
altogether apart from the question of his own gratification, he was
deeply pained that a cloud should have cast its shadow on the girl's
happiness, and he be able to do nothing to dissipate it. He was in
that condition of mind when trifles are apt to irritate the best
conditioned of men, and he was consequently as nearly
discourteous as it was possible for him to be when Mr. Hildebrand
Flurscheim thrust himself into the same compartment of the railway
train. As a travelling companion Flurscheim was the last person in
the world he would have chosen, and he strove to ignore his
presence by burying himself in a newspaper.
But Flurscheim was not accustomed to be ignored. He took no
notice of Marven's coolness, but chattered away incessantly, and at
last he succeeded in capturing the Captain's attention.
"There seems to have been some trouble between the young
people," he had remarked.
"Really, I cannot conceive that if there is it can be any business
of yours, Mr. Flurscheim," replied Marven frigidly.
The Jew had taken no notice of the snub.
"I'm not so sure of that," he had answered. "I am not so sure but
that I may not be successful in putting matters straight between
them."
 "What on earth are you driving at?" asked the Captain.
Flurscheim smiled. "It's not a matter I can talk about," he
answered, "without the permission of others, but I've seen how
interested you are in Mr. Guy Hora, and I've put my own construction
on your looking a bit down in the mouth this morning. I hope you'll
excuse me speaking straight what's in my mind, and if I'm mistaken,
I apologise for my interference. That's my impression, anyway," he
continued, as Captain Marven did not reply, "so I thought that I would
tell you that I think I know what is troubling Mr. Hora, and that I also
think it is in my power to clear up the trouble. Of course, I may be
mistaken, but I hope I am not, for I owe your young friend a debt I
can never hope fully to repay."
He spoke so earnestly that Marven's reserve and irritation
melted away, and the two men parted at the London terminus on the
best of terms with each other.
But although Marven had not learned anything as to the nature
of Flurscheim's intended action he felt easier in his mind, for he
realised that the Jew was very much in earnest, and he drove off to
his town house to make his preparations for his anticipated journey
with a far lighter heart than he had possessed when leaving Whitsea.
These preparations were soon completed, and he was sitting
down to a hastily prepared luncheon when Lynton Hora had knocked
at his door. Hora had not anticipated finding Captain Marven in town,
but had merely called in order to ascertain with certainty where the
letter he had written would find him. Then finding that Marven was in
the house, he had left the packet with instructions that it was to be
immediately delivered.
The package Hora had left was a bulky one. Marven merely
glanced at it when the servant brought it to him. Not until he had
finished lunch did he cut the string. When the wrapper was unfolded
and he had shaken out the contents his face paled, and he gasped
for breath. There seemed but little reason for his agitation; the parcel
contained nothing but a child's pinafore and a letter. Yet the sight of
the pinafore was quite sufficient to blur his vision and set his hands
shaking. He recognised it. He knew it instantly, without the necessity
for turning to the corner where the letters G. M. were embroidered by
his wife's own hand. He sprang to his feet and rang the bell violently.
"Where is the man who brought this parcel?" he demanded
directly the servant who had waited on him made her appearance.
His anxiety was so great that the woman was terrified, and some
minutes elapsed before he could obtain from her a connected
account of Hora's call. She seemed to think she must have been in
some way to blame for receiving the package. Marven succeeded
ultimately in reassuring her, and sent her out hastily to see if the
messenger still lingered in the neighbourhood. He followed to the
door and was grievously disappointed when she declared that he
was nowhere to be seen. Bethinking himself of the unopened letter
he returned to the room where he had left it. The envelope was
similar to many which had reached him previously, on the
anniversary of his child's disappearance, but when he opened it he
saw that it contained much more than the three-lined typed message
telling him that his child was alive. There were many sheets of note
paper covered in a bold handwriting which seemed familiar to him.
His hand shook more than ever as he smoothed out the sheets, and
his eyes grew dim again. Was his son at last to be restored to him?
He laid down the letter deliberately, and not until he had succeeded
in mastering his emotion did he attempt to make himself acquainted
with the contents. The opening sentence made his heart leap with
joy. The epistle opened baldly, without any of the customary methods
of address.
"The time has arrived when I am compelled to restore your son
to you. I hope you will be proud of him. He is known to the world as
Guy Hora."
Then his instinct had been right. Guy was his son. He wanted to
read no more. That was quite enough. He would hasten to make
himself known to his son. He rang the bell and ordered the servant to
summon a cab immediately. He would send a wire to his wife
informing her of the good news. He picked up the pinafore, folded it
carefully and placed it in his pocket. The letter he could read on his
way to Guy's chambers. But first he would see if his correspondent
revealed himself. He turned to the last page. Yes, there was a
signature, "Hartley Ruthven, now known as Lynton Hora."
He remembered his brother officer and unsuccessful rival
perfectly. He had thought, like all the rest of the world, Ruthven had
been dead years since. The reason for his child's disappearance
ceased to be a mystery any longer. Yet why should Ruthven now
desire to return to life? A sudden dread seized upon Marven as he
remembered his old comrade's cold, revengeful nature, the nature
which had been the real reason for his unpopularity in the regiment,
instead of the possession of narrow means, to which Hora had
always ascribed it. Perhaps this letter was only a part of Ruthven's
revenge upon the successful rival. Perhaps he had better read it to
the end before starting in search of Guy. He passed through the
entire gamut of the emotions before he had come to the end of the
epistle. Hora had deliberately set himself to describe Guy's history in
plain, matter-of-fact terms. He gave details of the manner of the
kidnapping of the child and particulars concerning him which left no
doubt that he was writing the truth. Then he went on to relate how
from the first he had trained Guy to a criminal career. Captain
Marven's heart was eaten out with rage, and he swore to himself that
the sun should not set before he exacted a reckoning from his
enemy.
Hora wrote of Guy's university career, and as he read Marven's
heart expanded again with joy. His boy had apparently been
uninjured by his earlier education. He thanked God for that. Then
came two pages in which Hora related the episodes of the
Flurscheim robbery and of the despoiling of himself of the
despatches entrusted to him. "The latter was an unpremeditated link
in my chain of revenge," wrote Hora. "Fortune does not always
favour the virtuous." The paper became blank to Marven's eyes. The
servant who came to announce that the cab was waiting at the door
had to speak twice before she could make her master comprehend.
The cabman must wait until he had finished the letter. He read
on.