Thieme Mumenthaler, Neurology - part 1 ppt

3 Diseases Mainly Affecting the Spinal Cord 389Characteristics of Diseases of the Spinal Cord.. 423 Degenerative and Heredode-generative Diseases Mainly Affecting the Spinal Cord.. 437 M

Thieme

Robert Wyss

Mark Mumenthaler, M.D Professor Emeritus of Neurology Former Head of the Department

of Neurology Berne University, Inselspital Berne, Switzerland

Heinrich Mattle, M.D Professor of Neurology Berne University Inselspital Berne, Switzerland Translated and adapted by Ethan Taub, M.D.

4th revised and enlarged edition

438 illustrations

210 tables

Georg Thieme Verlag Stuttgart · New York

Library of Congress

Cataloging-in-Publication Data

This book is an authorized and revised

translation of the 11th German edition

pub-lished and copyrighted 2002 by Georg

Thieme Verlag, Stuttgart, Germany Title of

the German edition: Neurologie

Translator: Ethan Taub, M.D.,

Klinik Im Park, Zurich, Switzerland

Thieme New York, 333 Seventh Avenue,

New York, NY 10001 USA

http://www.thieme.com

Cover design: Cyclus, Stuttgart

Typesetting by Mitterweger, Plankstadt

Printed in Germany by Grammlich,

Pliezhausen

ISBN 3-13-523904-7 (GTV)

ISBN 1-58890-045-2 (TNY)

1 2 3 4 5

Important note: Medicine is an

ever-changing science undergoing continual velopment Research and clinical experi- ence are continually expanding our knowl- edge, in particular our knowledge of proper treatment and drug therapy Insofar as this book mentions any dosage or application, readers may rest assured that the authors, editors, and publishers have made every effort to ensure that such references are in

de-accordance with the state of knowledge at the time of production of the book Nev-

ertheless, this does not involve, imply, or express any guarantee or responsibility on the part of the publishers in respect to any dosage instructions and forms of applica-

tions stated in the book Every user is quested to examine carefully the manu-

re-facturers’ leaflets accompanying each drug and to check, if necessary in consultation with a physician or specialist, whether the dosage schedules mentioned therein or the contraindications stated by the manufac- turers differ from the statements made in the present book Such examination is par- ticularly important with drugs that are either rarely used or have been newly re- leased on the market Every dosage sched- ule or every form of application used is en- tirely at the user’s own risk and responsibil- ity The authors and publishers request ev- ery user to report to the publishers any dis- crepancies or inaccuracies noticed.

Some of the product names, patents, and registered designs referred to in this book are in fact registered trademarks or proprie- tary names even though specific reference

to this fact is not always made in the text Therefore, the appearance of a name with- out designation as proprietary is not to be construed as a representation by the pub- lisher that it is in the public domain This book, including all parts thereof, is legally protected by copyright Any use, ex- ploitation, or commercialization outside the narrow limits set by copyright legisla- tion, without the publisher’s consent, is ille- gal and liable to prosecution This applies in particular to photostat reproduction, copy- ing, mimeographing, preparation of micro- films, and electronic data processing and storage.

IV

For Regula, Sarah, and Sofia-Rebecca

Preface to the Fourth English Edition

Since this book first appeared in

Ger-man in 1967, the reading and study

habits of medical students and

physi-cians have changed dramatically As

foreign travel is now commonplace,

many young physicians take part of

their training abroad and thereafter

continue to enjoy a lively exchange of

experiences with colleagues in

differ-ent countries International

commu-nication, of course, requires a

com-mon language, and English has

language for this purpose The

au-thors and publisher therefore decided

to arrange a translation of the 11th

German edition into English

The book is now 3 times its original

length; since 1967, the number of

il-lustrations has risen sixfold from 70

to 438, the number of tables from 30

to 210, and the number of references

from 640 to about 2000 Its scope

having widened over the years, this

text, originally intended for

begin-ning medical students, has come to

be more suitable for advanced

medi-cal students with a special interest in

neurology, for resident trainees, and

for physicians in practice

As a logical consequence of this

de-velopment, the authors and publisher

have decided to tailor the current

edi-tion for practicing physicians Thus,

we have omitted the sections

con-cerning the basic neurological

exami-nation and ancillary tests in

neurol-ogy, while introducing a substantial

amount of new material and ing greater detail on many of the top-ics previously covered We have en-tirely rewritten the chapters on epi-lepsy, disorders of cerebral perfusion,

polyneuropathies, and myopathies

We have also considerably enlargedthe sections on treatment in eachchapter, as requested by readers ofprevious editions

In order to keep the book frombecoming unwieldy through the ad-dition of so much material, we havenot printed the reference list in thebook, but have published it instead

(http://www.thieme.com/mm-refs),where the abstracts of most refer-ences can also be read The didacticaspects of the text and its graphicpresentation have been brought up todate with the help of Ms SusanneHuiss of Georg Thieme Verlag

We have tried to make the book prehensive, though not complete(which would be impossible in anycase), aiming at a clear and under-standable presentation of what wethink are the more important aspects

com-of neurological disease We age readers to consult the amply citedreferences for more detail on particu-lar matters of interest

encour-Many of the illustrations show ing studies of the kinds that are nowindispensable in clinical neurology.Physicians should order such studies,

imag-VI

in our opinion, mainly to confirm a

diagnosis that has already been

for-mulated on the basis of the case

his-tory and physical examination It

should be possible to arrive at a

sin-gle diagnosis or a narrow differential

diagnosis through a critical

assess-ment and logical combination of

these elements, in the light of an

un-derstanding of topical neurologic

di-agnosis and of the patterns of

neuro-logic disease, which is what this book

seeks to impart

We have also written another, less

comprehensive text, Grundkurs

Neu-rologie (soon to be available in

En-glish as Fundamentals of Neurology),

which provides an introduction to

clinical neurology for medical

stu-dents and allied health professionals

That text, unlike this one, includes

in-troductory chapters on the

neurologi-cal examination and on ancillary

test-ing

We are glad to have secured the

col-laboration of Dr Ethan Taub, an

American-trained and certified

neu-rosurgeon practicing in Switzerland,

for this English edition He has not

only produced an accurate translation

of fine literary quality, but has also,

through his familiarity with

Ameri-can medicine, succeeded in adapting

this edition optimally to the standard

practice and terminology of

English-speaking physicians

We would like to thank Dr Thomas

Scherb, Dr Thorsten Pilgrim, Susanne

Huiss, M.A., and Rolf Dieter Zeller ofGeorg Thieme Verlag for designingand producing the German editionwith such great care, and Dr CliffordBergman and Gert Krüger of ThiemeInternational for managing produc-tion the English edition We alsothank all of the readers and friendswhose critical advice has helped useliminate errors and maximize theaccuracy of the text We gratefullylook forward to more such advice inthe future

Many people have helped us obtainreferences and illustrations, reviewedparts of the manuscript critically, andparticipated in the technical aspects

of book production We would like toexpress our particular thanks in thisregard to Professor G Schroth, Dr L.Remonda, Dr K Lövblad, Dr F Donati,

Dr K Gutbrod, Professor Ch W Hess,

Dr G Jenzer, PD Dr K Rösler, PD Dr J.Mathis, Prof C Bassetti, Dr A Nirkko,and Dr P Imesch, as well as Dr sc.nat.Karin Hänni and Dr phil AnneliesBlum

We hope that this translation of thebook into the lingua franca of modernmedicine will make it accessible to awider audience, in the English-speaking countries and throughoutthe world

Mark MumenthalerHeinrich MattleZurich and Berne, SwitzerlandAutumn 2003

Translator’s Note

This textbook of neurology has been

translated into many languages since

its original publication in German in

1967 and has earned the appreciation

of generations of readers all over the

world It has covered the field of

neu-rology in greater depth with each

new edition, while incorporating the

latest clinical and scientific advances,

and has thus doubled in size since it

last appeared in English (the 3rd

edi-tion of 1990, translated by E H

Bur-rows from the 9th German edition)

Yet, thanks to the skill of the authors,

the book has not become unwieldy

and remains eminently readable for

both study and reference

In translating and adapting the 11th

German edition to produce this

En-glish version, I have worked directly

from the current German text, but

have occasionally borrowed words

and phrases from the previous

En-glish edition when I found they could

not be improved on The text mostlycorresponds to the German, para-graph by paragraph, and even page bypage for long stretches toward the be-ginning of the book I have sparinglyadded words of explanation wher-ever this might help the reader, espe-cially in passages touching on myown specialty (neurosurgery), buthave otherwise done my best to con-vey the sense of the original unal-tered Needless to say, the opinionsand expert judgments expressed inthe book are those of the authors

I have learnt much in the process oftranslation and am grateful to Profes-sors Mumenthaler and Mattle, and toGeorg Thieme Verlag, for the privilege

of helping them put this book before

an English-speaking audience

Ethan Taub, M.D

Zurich, Switzerland, Autumn 2003

VIII

Differentiation of Central and

Peripheral Paresis 2

Bodily Distribution of Paresis 3

Monoparesis 3

Hemiparesis 4

Para- and Quadriparesis 4

Lesions Affecting the Anterior Horn Ganglion Cells 5

Lesions Affecting a Spinal Nerve Root 5

Polyradiculopathy 10

Polyneuropathy 10

Plexus Lesions 10

Lesions of a Single Peripheral Nerve 11

Dysfunction of the Neuromuscular Junction 11

Myopathy 11

Clinical Neurology 13 2 Diseases Mainly Affecting the Brain and its Coverings 14 Characteristics of Diseases of the Brain 14

Congenital and Perinatally Acquired Diseases of the Brain 14

Traumatic Brain Injury 45

Intracranial Hypertension and Brain Tumors 56

Infectious Diseases of the Brain and Meninges 75

Disturbances of Cerebral Perfusion and Nontraumatic Intracranial Hemorrhage 131

The Comatose Patient 221

Extrapyramidal Syndromes 235

Cerebellar Syndromes 274

Metabolic Disorders with Cerebral Or Other Neurologic Involvement 285

Systemic Diseases Affecting the Nervous System 299

Dementing Disorders and Other Neuropsychological Syndromes 346

IX

3 Diseases Mainly Affecting the Spinal Cord 389

Characteristics of Diseases of

the Spinal Cord 389

Ancillary Tests in Diseases of the Spinal Cord 389

Classification of Spinal Cord Syndromes 391

Congenital and Perinatally Acquired Lesions of the Spinal Cord 393

Spinal Cord Trauma 395

Fundamentals of (Complete) Spinal Cord Transection Syndrome 395

Practical Approach to Acute Traumatic Spinal Cord Transection 399

Acceleration Injury of the Spine (Whiplash Injury) 402

Tumors and Other Masses Com-pressing the Spinal Cord 405

General Aspects 405

Types of Mass Compressing the Spinal Cord 406

Infectious, Allergic, and Toxic Diseases of the Spinal Cord and Its Coverings 414

Infectious Diseases of the Spinal Cord 414

Myelitis 416

Toxic Myelopathies 417

Circulatory Disorders of the Spinal Cord 418

Blood Supply of the Spinal Cord 418

Spinal Cord Ischemia Due to Deficient Arterial Blood Flow 418

Spinal Cord Ischemia Due to Venous Disturbances 423

Degenerative and Heredode-generative Diseases Mainly Affecting the Spinal Cord 425

Diseases Affecting the Anterior Horn Cells 425

Spastic Spinal Paralysis 432

Amyotrophic Lateral Sclerosis (ALS) 434

Spinocerebellar Ataxias 437

Metabolic Disorders Mainly Affecting the Spinal Cord 441

Vitamin B12Deficiency and Funicular Myelosis 441

Syringomyelia and Syringobulbia 443

Other Diseases of the Spinal Cord 447

Radiation-Induced Myelopathy 447

Decompression Myelopathy 447

4 Autonomic and Trophic Disorders 449 Acute Pandysautonomia 454

Familial Dysautonomia (Riley-Day Syndrome) 455

Botulism 455

Insensitivity to Pain 456

Congenital Insensitivity to Pain 456

Congenital Sensory Neuropathy with Anhidrosis 456

Sensory Radicular Neuro-pathy 456

Pain Asymbolia 456

Sympathetic Syndromes 457

Typical Manifestations 457

Trophic Disorders 461

5 Demyelinating Diseases 465

Multiple Sclerosis 465

Typical Clinical Features 466

Epidemiology 467

Clinical Features 467

Ancillary Tests 471

Prognosis 474

Pathologic Anatomy 476

Etiology and Pathogenesis 477

Treatment 480

Differential Diagnosis 483

Other Demyelinating Diseases 484

Concentric Sclerosis (Bal ´o’s Disease) 484

Diffuse Sclerosis (Schilder’s Disease) 484

Acute Disseminated Encephalo-myelitis (ADEM) 484

Neuromyelitis Optica (Devic’s Disease) 484

Subacute Myelo-optic Neuropathy (SMON) 485

Hereditary Demyelinating Diseases 485

6 Injury to the Nervous System by Specific Physical Agents 487 Electrical Injury 487

Gas Embolism 488

Venous Gas Embolism 488

Arterial Gas Embolism 489

Injury Due to Ionizing Radiation 490

Radiation Injury to the Brain 490 Radiation Injury to the Spinal Cord 490

Radiation Injury to the Peripheral Nervous System 491

Hypothermic Injury 491

7 Epilepsy, Other Episodic Disorders of Neurologic Function, and Sleep Disorders 493 Epilepsy 493

History 493

Etiology and Pathogenesis 494

Epidemiology 495

Ancillary Diagnostic Tests in Epileptology 495

Classification of Epilepsy 501

Individual Seizure Types 504

Clinical Patterns of Epilepsy and Epileptic Syndromes 506

Partial (Focal) Seizures 517

Special Seizure Types and Seizure Etiologies 530

Procedure after a First Seizure or Multiple Seizures 532

Treatment of Epilepsy 532

Prognosis 546

Episodic Disturbances of Con-sciousness, Syncope, and Other Nonepileptic Episodes 547

Syncope and Drop Attacks 550

Disturbances of Consciousness of Metabolic Origin 554

Drop Attacks 555

Episodic Nonepileptic Motor Phenomena 556 Episodic Disorders that are Partly or Wholly Psychogenic 558

Neurological Findings in the

Unconscious Patient and in

Psychogenic Pseudo-Neurological

Conditions 560

Neurological Findings in the Unconscious Patient 560

The Neurological Examination in Psychogenic Pseudo-Neurological Conditions 560

Sleep and Disturbances of Sleep 563

Sleep 563

Sleep Disorders 564

Hypersomnia 565

Sleep Apnea Syndrome 569

8 Polyradiculitis and Polyneuropathy 575 Polyradiculitis 575

Classic Acute Polyradiculitis (Guillain-Barr ´e Syndrome, Landry-Guillain-Barr ´e Syndrome) 576

Atypical Polyradiculitis 580

Polyneuropathy 582

General Features 582

Hereditary Polyneuropathy 588

Polyneuropathy Due to Metabolic Disorders 598

Polyneuropathy Due to Improper or Inadequate Nutrition 604

Polyneuropathy Due to Vitamin B12Malabsorption 604

Autoimmune Poly-neuropathy 605

Polyneuropathy Due to Infectious Disease 606

Polyneuropathy Due to Arterial Disease 606

Polyneuropathy Due to Sprue and Other Malabsorptive Dis-orders 608

Polyneuropathy Due to Exogenous Toxic Substances 609 Polyneuropathy of Other Causes 613

9 Diseases Affecting the Cranial Nerves 617 Disturbances of Olfaction 623

Anosmia 623

True Combined Anosmia and Ageusia 624

Cacosmia 624

Visual Disturbances of Neurologic Origin 624

Loss of Vision 624

Visual Field Defects and Perceptual Disturbances 625

Abnormal Findings in the Optic Disks 630

Oculomotor Disturbances 630

The Neuroanatomical Basis of Ocular Motility 634

Preliminary Remarks on the Examination of Ocular Motility 636

Ancillary Tests in Oculomotor Disturbances 639

General Principles of Ocular

Motility 641

Some Diseases in which Oculomotor Disturbances are Prominent 652

Diseases that Mimic Supra-nuclear Disturbances of Eye Movement 653

Lesions of the Cranial Nerves Subserving Eye Movement and their Brainstem Nuclei 654

Ptosis 662

Treatment of Oculomotor Disorders 663

Pupillary Disorders 664

Anatomy and Clinical Examin-ation of the Pupils 664

Abnormal Size and Shape of the Pupils 664

Abnormalities of Pupillary Reactivity 664

Trigeminal Disturbances 670

Facial Nerve Disturbances 673

Cryptogenic Peripheral Facial Nerve Palsy 673

Bilateral Facial Nerve Palsy 676

Melkersson-Rosenthal Syndrome 677

Other Causes of Peripheral Facial Nerve Palsy 677

Disturbances of Taste 678

Hemifacial Spasm 678

Facial Myokymia 679

Facial Tic 679

Progressive Facial Hemiatrophy 679

Disturbances of the Vestibulo-cochlear Nerve (Statoacoustic Nerve, Auditory Nerve) 680

Anatomy 680

Testing of Hearing 680

Cochlear, Retrocochlear, or Central Hearing Loss? 682

Diseases Causing Hearing Loss 683

Ancillary Tests in the Diagnostic Evaluation of Hearing Loss 690

Tinnitus and Other Abnormal Sounds 690

Vertigo 691

Diseases Causing Prominent Vertigo 702

Glossopharyngeal and Vagus Nerve Dysfunction 712

Accessory Nerve Palsy 713

Hypoglossal Nerve Palsy 715

Multiple Cranial Nerve Palsies 716

Cranial Polyradiculitis 716

(Recurrent) Multiple Cranial Nerve Palsies 716

Progressive Palsies of Multiple Cranial Nerves 716

Garcin Syndrome 716

Rarer Causes 716

10 Spinal Radicular Syndromes 717 General Symptoms and Signs 718

Intervertebral Disk Disease as a Cause of Radicular Syndromes 728

Cervical Disk Herniation and Spondylosis 729

Thoracic Radicular Syndromes 730

Lumbar Disk Herniation 730

Mass Lesions in and Adjacent to the Spinal Nerve Roots 738

Other Radicular Syndromes 739

Herpes Zoster 739

11 Lesions of Individual Peripheral Nerves 741

General Clinical Features 741

Ancillary Tests 742

Classification and Quantification of Peripheral Nerve Lesions 746

Peripheral Nerve Regener-ation 748

Pain Syndromes due to Peripheral Nerve Lesions 748

Brachial Plexus Palsies 749

Traumatic Brachial Plexus Palsies 751

Other Causes of Brachial Plexus Palsy 762

Differential Diagnosis of Brachial Plexus Palsies 766

Long Thoracic Nerve 767

Axillary Nerve 767

Suprascapular Nerve 768

Musculocutaneous Nerve 768

Radial Nerve 768

Median Nerve 771

Ulnar Nerve 775

Lumbosacral Plexus 780

Genitofemoral and Ilioinguinal Nerves 780

Femoral Nerve 787

Lateral Femoral Cutaneous Nerve (Meralgia Paresthetica) 788

Obturator Nerve 789

Gluteal Nerves 790

Sciatic Nerve 791

Common Peroneal Nerve 792

Tibial Nerve 795

12 Headache and Facial Pain 797 General Aspects 798

History-Taking from Patients with Headache 798

Classification of Headache and Facial Pain 798

Examination of Patients with Headache 802

Pathogenesis of (Primary) Headache 802

The Major Primary Headache Syndromes 803

Tension-Type Headache 803

Post-Traumatic Headache 804

Migraine 805

Headache in Organic Vascular Disease 815

Cranial Arterial Occlusion 815

Aneurysmal Subarachnoid Hemorrhage 815

Arterial Hypertension 816

Pheochromocytoma 816

Temporal Arteritis 816

Spondylogenic Headache and Cervical Migraine 817

Neck-Tongue Syndrome 818

Other Symptomatic Forms of Headache 818

Headache Due to an Intracranial Mass 818

Headache Due to Intermittent Obstruction of CSF Flow 819

Syndrome of Low Cerebro-spinal Fluid Volume (Hypo-liquorrhea) 819

Pseudotumor Cerebri 820

Headache Due to Ocular Disorders 820

Headache Due to Disorders of the Ear, Nose, and Throat 820

Headache Due to Systemic Disease 820

Psychogenic Headache 821

Drug-Induced Headache 821

Facial Pain 821

Neuralgias 821

Other Types of Facial Pain 825

13 Pain Syndromes of the Limbs and Trunk 833

Pain in the Shoulder and Arm

Lower Limb 843Other Regional and GeneralizedPain Syndromes 848

Clinical Presentation and

Diagnostic Evaluation of the

Diseases 907Muscular Manifestations of

Electrolyte Disturbances 908Muscular Manifestations Due

to Medications, Intoxications,and Nutritional Deficiencies 909Disorders of Neuromuscular

Transmission 911Myasthenia Gravis (MyastheniaGravis Pseudoparalytica, Erb-Goldflam Disease) 911Lambert-Eaton MyasthenicSyndrome 922Congenital Myasthenic

Syndromes 923Other Myasthenic

Syndromes 924Common Muscle Cramps 924

See also http://www.thieme.com/mm-refs

Appendix 927 Scales for the Assessment of Neurologic Disease 928

Detailed Instructions for the

Unified Parkinson’s Disease

Rating Scale (UPDRS) 930

Simplified Scale for Evaluating

the Severity of Individual Signs

of Parkinson’s Disease 938

Epworth Sleepiness

Questionnaire 939

Barthel Index (of Disability) 940

Modified Rankin Scale(for Stroke) 941Modified NIH Stroke Scale

(adapted from Brett et al andLyden et al.) 942Expanded Disability Status Scale(DSS) for Multiple Sclerosis 946Major Neurogenetic Diseases 947Glossary of Common Abbrevi-ations in Neurology 957

Clinical Syndromes

1 Clinical Syndromes in Neurology

Because of the anatomical

construc-tion of the nervous system and the

manner in which functions are

as-signed to its components, lesions in

specific areas of the central or

periph-eral nervous system are regularly

associated with characteristic

symp-toms and signs An acquaintance withthese recurring patterns allows one

to trace individual findings or stellations of findings back to the re-sponsible dysfunctional component

con-of the nervous system (Table 1.1) Table 1.1 Components of the nervous system

Spinal cord

Brachial and lumbar plexusesPeripheral nerves

Motor end platesMusclesThe following discussion will concern

the most important typical

constella-tions of findings (syndromes):

junction (motor end plate),

Differentiation of Central and Peripheral Paresis

Central and peripheral forms of paresis may be differentiated from each other

by the criteria listed in Table 1.2.

2

Table 1.2 Characteristics of central and peripheral paresis

Proprioceptive muscle

reflexes

Exteroceptive muscle

Bodily Distribution of Paresis

The distribution of paresis in the

body enables a number of inferences

to be made about the nature and

ana-tomical localization of the

respon-sible lesion

Monoparesis

Monoparesis is defined as isolatedweakness of an entire limb or of amajor part of it Possible causes are

Anterior horn of spinal

cord (chronic lesion)

Paresis of individual muscles with accompanying atrophyand decreased tone

No sensory deficitPossibly accompanied by fasciculationsDecreased proprioceptive muscle reflexes (but may be in-creased in amyotrophic lateral sclerosis)

Brachial or

lumbar plexus Mixed sensory and motor deficitDecreased muscle tone

Muscle atrophy, decreased proprioceptive muscle reflexesSensory deficit for all modalities

Multiple peripheral

nerves

Same as in plexus lesions in a single limb

Muscle Hardly ever a pure monoparesis; if so, then flaccid

Purely motor deficit, sometimes with muscle atrophy

Hemiparesis may be due to any of the

causes listed in Table 1.4.

Para- and Quadriparesis

Paraparesis is weakness affectingboth lower limbs, and quadriparesis

is weakness affecting all four limbs(but sparing the head) These may bedue to any of the causes listed in

Table 1.5.

Table 1.4 Sites of lesions causing hemiparesis, and corresponding clinical features

Cerebrum Spastic hemiparesis, possibly also involving facial

muscles, characterized by:

Increased muscle toneIncreased reflexesPyramidal tract signs

No atrophyUsually associated with a sensory deficit

Face involved or not, depending on level of lesionCranial nerve deficits contralateral to hemiparesis

Upper cervical spinal cord Spastic hemiparesis, as above

Face sparedPossible ipsilateral loss of position and vibration senseand contralateral loss of pain and temperature sensebelow the level of the lesion (Brown-S´equard syn-drome)

Table 1.5 Sites of lesions causing para- or quadriparesis, and corresponding clinical

features

Cerebrum (bilateral lesion) Clinical picture of “bilateral hemiparesis,” or

parapare-sis due to a bilateral parasagittal cortical lesion

Corticobulbar pathways in

the brain stem (bilateral

lesion) (e.g., lacunar state,

Corticospinal pathways in

the spinal cord (bilateral

lesion)

Para- or quadriparesisFace sparedHyperreflexia, pyramidal tract signs

No sensory deficitOnly mild weakness

Lesions Affecting the Anterior Horn Ganglion Cells

Lesions selectively affecting the

effer-ent neurons (ganglion cells) of the

anterior horn of the spinal cord (p

425 ff.) produce the characteristic

clinical findings listed in Table 1.6 Table 1.6 Clinical features of an isolated lesion of the anterior horn ganglion cells Muscle atrophy

Weakness

Fasciculations (in chronic phase)

Intact sensation

Decreased or absent reflexes

But: hyperreflexia and pyramidal tract signs in amyotrophic lateral sclerosis, which

in-volves not only the anterior horns but also the corticospinal pathways (thus the term

“lateral” sclerosis, as these pathways are lateral in the spinal cord)

Lesions Affecting a Spinal Nerve Root

Lesions affecting a spinal nerve root

(p 717 ff.) always produce both motor

and sensory deficits Individual spinal

nerve roots always supply more than

one muscle, and no muscle is

sup-plied exclusively by a single root

Thus, the motor deficit produced by a

monoradicular lesion has the

Nevertheless, certain muscles are

predominantly supplied by a single

root and are, therefore, weakened to a

particularly severe degree by a

corre-sponding monoradicular lesion

Pro-prioceptive muscle reflexes partiallysubserved by the affected root may

be decreased or even absent (cf

Table 1.4) The sensory deficit lies

within the corresponding sensory

dermatome (cf Table 1.1) and thus

usually has a band-like cutaneousdistribution Pain, if present, is re-ferred into the dermatome of the af-fected root Although the deficit ismixed (both motor and sensory), theclinical picture may be dominated byeither the motor deficit or the sen-sory deficit in individual cases Atro-phy of the affected muscles is evidentabout 3 weeks after the onset ofweakness

Fig 1.1a–g Cutaneous sensation.

Fields of sensory innervation of peripheral nerves

and spinal nerve roots, depicted on the left and right

sides of the body, respectively

6 Superior lateral cutaneous n

of the arm (a branch of the illary n.)

ax-7 Medial cutaneous n of thearm

8 Lateral cutaneous branches ofthe intercostal nn

9 Posterior cutaneous n of thearm (a branch of the radial n.)

10 Posterior cutaneous n of theforearm

11 Medial cutaneous n of theforearm

12 Lateral cutaneous n of theforearm

13 Superficial branch of radial n

14 Palmar branch of median n

15 Median n

16 Common palmar digital nn

17 Palmar branch of ulnar n

18 Iliohypogastric n (lateral neous branch)

cuta-19 Ilioinguinal n (anterior scrotalnn.)

20 Iliohypogastric n (anterior taneous branch)

cu-21 Genitofemoral n (femoralbranch)

22 Lateral femoral cutaneous n

23 Femoral n (anterior cutaneousbranches)

24 Obturator n (cutaneousbranch)

25 Lateral sural cutaneous n

C 3

3 2

5

4

4

5 7 9

9

10 12

24

25 27

29

14 13 11

7 Superior lateral cutaneous n

of the arm (a branch of the illary n.)

ax-8 Dorsal branches of the cal, thoracic, and lumbar spinalnn

cervi-9 Lateral cutaneous branches ofthe intercostal nn

10 Posterior cutaneous n of thearm

11 Medial cutaneous n of thearm

12 Posterior cutaneous n of theforearm

13 Medial cutaneous n of theforearm

14 Lateral cutaneous n of theforearm

15 Superficial branch of radial n

16 Dorsal branch of ulnar n

22 Lateral femoral cutaneous n

23 Posterior femoral cutaneous n

24 Obturator n (cutaneousbranch)

25 Lateral sural cutaneous n

7 12 13 15

16 14

18

17 19 20

22 23

26 27 4

28

Fig 1.1c Radicular innervation: lateral view.

Fig 1.1d Peripheral innervation: lateral view.

1 Ilioinguinal n

2 Iliohypogastric n

3 Genitofemoral n (femoral branch)

4 Lateral femoral cutaneous n

5 Dorsal n of penis (pudendal n.)

16 Superior lateral cutaneous n of the arm

(a branch of the axillary n.)

17 Intercostobrachial nn (intercostal nn.)

18 Dorsal branches of the thoracic nn

19 Posterior cutaneous n of the arm

20 Lateral cutaneous n of the arm

21 Posterior cutaneous n of the forearm(a branch of the radial n.)

22 Superior lateral cutaneous n of theforearm

23 Medial cutaneous n of the forearm

24 Lateral cutaneous branch of the pogastric n

iliohy-25 Superior cluneal nn

26 Superficial branch of radial n

27 Autonomous area of superficial branch

8 7 9

10 11

14 13 12

10 43

1 Cutaneous branch of obturator n

2 Posterior femoral cutaneous n

3 Lateral sural cutaneous n

4 Ilioinguinal n and genital branch of

(puden-2 Posterior scrotal or labial nn

3 Anterior cutaneous branches of thefemoral n

Polyradiculopathy (p 575 ff.) is

mani-fested by a rapidly progressive and

symmetric bilateral paresis, which

usually begins in the lower limbs The

motor deficits dominate the clinical

picture in most forms of lopathy The affected muscles areflaccid, and reflexes are absent Mic-turition is not impaired

polyradicu-Polyneuropathy

The clinical picture of

polyneuropa-thy (p 582 ff.) usually develops very

slowly, over the course of several

years The initial symptoms are

prac-tically always confined to the lower

limbs Only sensory abnormalities are

walk-ing on cotton wool

Shortly after these symptoms arise,the Achilles reflex disappears, fur-nishing the first objective clinicalsign Vibration sense is impaired,more so distally than proximally.Weakness first manifests itself as aninability to spread the toes, and,when the toes are dorsiflexed, the ex-aminer feels no contraction of the ex-tensor digitorum brevis muscles onthe dorsum of the foot Later, cutane-ous sensation to touch is impaired,and the proximal calf muscles be-come weak Finally, severe bilateralfoot drop appears and leads to a char-acteristic bilateral steppage gait

Plexus Lesions

Plexus lesions (p 749 and 780 ff.)

al-ways give rise to a mixed motor and

sensory deficit The involved muscles

and the distribution of the sensory

deficit do not correspond to the field

of innervation of a single peripheral

nerve, but rather to that of a

combi-nation of nerves There is a flaccid

pa-resis, and the reflexes subserved bythe affected nerves are absent Thesensory deficit is sharply delimitedand complete and involves all modal-ities Sweating is absent in the area ofthe sensory deficit Muscle atrophybecomes evident a few weeks afterthe onset of weakness

Lesions of a Single Peripheral Nerve

A lesion of a single peripheral nerve

(p 741 ff.) produces a characteristic

motor deficit in the muscles it

sup-plies, and a characteristic sensory

deficit in its field of sensory

innerva-tion Lesions of purely motor or

purely sensory nerves (or nerve

branches) will obviously produce

purely motor or purely sensory cits Sweating is absent in the area of

defi-a sensory deficit, defi-and defi-any reflex thdefi-at

is subserved by the affected nerve isabsent There is a flaccid paresis, andmuscle atrophy becomes evident2–3 weeks after the onset of weak-ness

Dysfunction of the Neuromuscular Junction

In dysfunction of the neuromuscular

junction (motor end plate) (pp

875 ff.), there is a purely motor

pare-sis of variable severity However, as

in the (other) myopathies (see low), sensation remains intact There

be-is no muscle atrophy

Myopathy

Myopathy (p 815 ff.) is characterized

by paresis in the absence of a sensory

deficit, because the lesion lies within

the striated muscle itself The clinical

picture is thus comparable to that of

an anterior horn lesion (see above)

slowly and affect both sides of the

body symmetrically The paresis is

flaccid and the muscles become phic, albeit usually less severely than

atro-is seen with anterior horn or eral nerve lesions The correspondingreflexes are decreased or absent inadvanced stages of the illness Fascic-ulations do not occur, in contrast toanterior horn lesions

Clinical Neurology

2 Diseases Mainly Affecting the Brain and its Coverings

Characteristics of Diseases of the Brain

syn-bradycardia) Localizing signs include focal neurological and

neuropsycho-logical deficits, visual disturbances, cranial nerve deficits, and focal tic seizures None of these features are obligatory for diagnosis, and theymay be present in varying combinations and degrees of severity

epilep-Congenital and Perinatally Acquired Diseases of the BrainDefinition:

Both genetic defects and disturbances occurring during pregnancy may lead

to developmental disorders of the brain (and of the remainder of the vous system, as well as other organs of the body) These may already be ev-

ner-ident in the newborn infant (e.g., microcephaly), or they may become dent only in the course of further development The same is true of brain

evi-injuries occurring during delivery, which are of two types: hemorrhages,

and more or less diffuse hypoxic injuries The more common modes of

pre-sentation in the early postnatal phase are abnormalities of muscle tone and

pathological reflexes Later manifestations include delayed psychomotordevelopment, motor deficits (para- or hemiparesis) and involuntary move-

ments (e.g., athetosis) Epileptic seizures in children and adolescents are

not uncommonly an expression of a congenital or perinatally acquired ease of the brain

dis-14

The Neurological Examination in

Infancy and Early Childhood

Techniques used in the neurological

examination of adults are generally

not applicable to infants and very

young children Information about

the child’s functional state is more

usefully derived from observation of

spontaneous behavior and of

com-plex motor reflexes (104, 311)

In the infant, spontaneous posture

should be noted, as well as any bodily

asymmetries or constantly

main-tained postures It should also be

noted whether the head is

asymmet-ric (plagiocephalic) or otherwise

ab-normal in shape (p 44) The head

cir-cumference, body weight, and body

length should be measured, entered

into a table for future reference, and

compared with normal values for age

and sex (Fig 2.1).

Primitive motor function in children

is initially governed by a number of

reflex mechanisms These are listed in

Table 2.1, and their temporal

develop-ment and clinical significance are

briefly described

The stages of normal motor

develop-ment are shown in Fig 2.2.

In the initial months of life, the

follow-ing motor abnormalities may indicate

the presence of a cerebral movement

disorder (cerebral palsy):

> hypotonia,

the child is lifted,

dur-ing diaper changes,

persisting into the 3rd month of

life,

re-flex,

trunk postural reflex and the on-trunk reflex

head-By the end of the 4th month, the infant

should be able to control its headwhile sitting, lift its head whileprone, and use both hands for play.The Moro reflex fades, and the Lan-dau and parachute reflexes maketheir first appearance The phenom-ena listed above are pathological inthis stage also

By the end of the 6th month, the

fol-lowing findings are highly suggestive

or, if pronounced, definitely tive of a cerebral movement disorder:retained tonic neck reflex and Mororeflex, and absent Landau, labyrin-thine positional, and parachute re-flexes The child should be able to liftits head while supine, turn from su-pine to prone, sit up with support,turn toward an external noise, anduse the whole hand, including thethumb

indica-By the end of the 9th month, the child

should be able to sit unaided, and thelumbar kyphosis becomes less pro-nounced The following findings aresuggestive of a cerebral movementdisorder, in addition to the pathologicreflexes already mentioned: absence

of the body righting and limping actions, and presence of a trunk pos-tural reflex

0 3 6 9 12 15 18 21 24 30 36 42 48

0 3 6 9 12 15 18 21 24 30 36 42 48 5

20

15

kg 90 100 110

%

97 90 75 50 25 10 3

%

a

Fig 2.1a–h Head circumference, body length, and weight in childhood and

adoles-cence (Adapted from: Berner Datenbuch der Pädiatrie: Praktische Richtlinien, Therapie,

Ernäh-rungsgrundlagen, Referenzwerte, 4th ed., Stuttgart: Fischer, 1992).

54 53 52 51 50 49 48 47 46 45 44 43 42 41 40 39 38 37 36 35 34 33 32

97 90 75 50 25 10 3

%

b

0 3 6 9 12 15 18 21 24 30 36 42 48

0 3 6 9 12 15 18 21 24 30 36 42 48 5

kg 90 100 110

cm cm

%

97 90 75 50 25 10 3

%

c

54 53 52 51 50 49 48 47 46 45 44 43 42 41 40 39 38 37 36 35 34 33

6 9 12 15 18 21 24 30 36 42 48

0 3 6 9 12 15 18 21 24 30 36 42 48

Mother cm Father cm

Head circumference

Boys 0 – 4 years

Age (months)

97 90 75 50 25 10 3

%

d

97

90

75 50 25 10 3

%

e

1 2

55 56 57 58 cm

Head circumference

Girls 1 – 15 years

Age (years)

90 97

75 50 25

P :

–

97 90 75 50 25 10 3

%

97

90 75 50 25 10 3

%

g

1 2

57 58 59 60 cm

Head circumference

Boys 1 – 15 years

90 97

75

50 25