Peripheral Nerve Disorders

953 Pages·2013·214.391 MB·2-987\953

by  Gérard Said and Christian Krarup (Eds.)

HANDBOOK OF CLINICAL
NEUROLOGY
Series Editors
MICHAEL J. AMINOFF, FRANC¸OIS BOLLER, AND DICK F. SWAAB
VOLUME 115
EDINBURGH LONDON NEW YORK OXFORD PHILADELPHIA
ST LOUIS SYDNEY TORONTO 2013
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Availabletitles
Vol.79, The humanhypothalamus: basic and clinicalaspects, Part I, D.F. Swaab ISBN 9780444513571
Vol.80, The human hypothalamus: basic and clinical aspects,Part II, D.F. Swaab ISBN 9780444514905
Vol.81, Pain, F.Cerveroand T.S. Jensen, eds. ISBN 9780444519016
Vol.82,Motor neurone disorders and relateddiseases, A.A. Eisenand P.J.Shaw, eds.ISBN9780444518941
Vol.83, Parkinson’s diseaseand relateddisorders, Part I, W.C.Koller and E. Melamed,eds. ISBN 9780444519009
Vol.84,Parkinson’sdiseaseandrelateddisorders,PartII,W.C.KollerandE.Melamed,eds.ISBN9780444528933
Vol.85, HIV/AIDS and thenervoussystem, P. Portegiesand J. Berger, eds. ISBN 9780444520104
Vol.86, Myopathies, F.L. Mastaglia and D. Hilton Jones, eds.ISBN9780444518996
Vol.87, Malformations ofthe nervous system, H.B. Sarnat and P. Curatolo, eds.ISBN 9780444518965
Vol.88, Neuropsychology and behaviouralneurology,G.Goldenberg and B.C.Miller, eds. ISBN 9780444518972
Vol.89, Dementias, C.Duyckaerts and I.Litvan, eds. ISBN 9780444518989
Vol.90, Disorders ofconsciousness, G.B. Young and E.F.M. Wijdicks, eds. ISBN 9780444518958
Vol.91, Neuromuscular junction disorders, A.G. Engel, ed. ISBN 9780444520081
Vol.92, Stroke – Part I:Basicand epidemiologicalaspects, M.Fisher, ed.ISBN9780444520036
Vol.93, Stroke– Part II: Clinical manifestations and pathogenesis,M. Fisher, ed. ISBN 9780444520043
Vol.94,Stroke– Part III:Investigationsand management, M.Fisher, ed. ISBN 9780444520050
Vol.95, Historyofneurology, S.Finger, F.Boller and K.L. Tyler, eds. ISBN 9780444520081
Vol.96, Bacterialinfectionsofthecentralnervoussystem,K.L. RoosandA.R. Tunkel, eds.ISBN9780444520159
Vol.97, Headache, G. Nappi and M.A. Moskowitz, eds.ISBN9780444521392
Vol.98, Sleep disorders Part I, P. Montagna and S. Chokroverty,eds. ISBN 9780444520067
Vol.99, Sleep disordersPart II, P. Montagna and S. Chokroverty, eds.ISBN9780444520074
Vol.100,Hyperkineticmovement disorders,W.J. Weiner and E. Tolosa, eds.ISBN 9780444520142
Vol.101,Muscular dystrophies,A.Amato and R.C.Griggs, eds.ISBN 978 0 080450315
Vol.102, Neuro-ophthalmology, C. Kennard and R.J. Leigh, eds.ISBN9780444529039
Vol.103,Ataxicdisorders, S.H. Subramony and A.Durr,eds.ISBN 9780444518927
Vol.104,Neuro-oncology Part I, W.Grisold and R. Sofietti,eds. ISBN 9780444521385
Vol.105,Neuro-oncology Part II, W. Grisold and R. Sofietti,eds. ISBN 9780444535023
Vol.106,Neurobiology ofpsychiatricdisorders, T.Schlaepfer and C.B. Nemeroff, eds. ISBN 9780444520029
Vol.107,Epilepsy, H. Stefan and W.H. Theodore, eds.ISBN 9780444528988
Vol.108,Epilepsy Part II,H.Stefanand W.H. Theodore, eds. ISBN 9780444528995
Vol.109, Spinal cord injury, J.Verhaagen and J.W. McDonald III, eds.ISBN9780444521378
Vol.110, Neurologicalrehabilitation,M. Barnes and D.C. Good, eds.ISBN9780444529015
Vol.111, Pediatric neurology Part I, O. Dulac, M. Lassonde and H.B. Sarnat, eds.ISBN 9780444528919
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Vol.113, Pediatric neurology Part III, O. Dulac, M. Lassonde and H.B.Sarnat, eds.ISBN9780444595652
Vol.114,Neuroparasitologyand tropicalneurology, H.H. Garcia,H.B. Tanowitz and O.H. Del Brutto, eds.
ISBN 9780444534903
Foreword
TheHandbookofClinicalNeurology,originallyconceivedandeditedbyPierreVinkenandGeorgeBruyn,isapres-
tigious, multivolume reference work that covers all the disorders encountered by clinical neurologists, providing
both a detailed account of their relevant clinical features and a critical analysis of the experimental and scientific
advances that relate to these disorders. The first volume was published in 1968. The first series of the Handbook
endedin1982andwasfollowedbyasecondseriestoupdatetheearliermaterial,whichconcludedin2002.Wethen
assumed the responsibility of developing a new (third) series. The first volume in the new series was published in
2003, and 36 volumes have since been published, making a total of 114 volumes so far in the combined series of
theHandbook.
Peripheral neuropathies have received extensive—but very appropriate—coverage in the Handbook over the
years. Volumes 7 and 8 in the first series covered diseases of nerves, followed 18 years later by Volume 51 in the
secondseries,alsodealingwiththeneuropathies.Inaddition,manyofthechaptersinothervolumeshavefocused
ondiseasesoftheperipheralnervoussystem.Nevertheless,rapidadvancesinmolecularbiology,genetics,immunol-
ogy,pathology,basicandclinicalneurophysiology,andimagingproceduresnecessitateanewvolumededicatedto
thistopic.Asserieseditors,wefeelprivilegedthatProfessorGerardSaidfromParisandProfessorChristianKrarup
fromCopenhagen,bothofwhomareeminentcliniciansandinvestigatorswhohavemadeimportantcontributionsto
thefield,agreedtotakeonthetaskofdevelopingsuchavolumeintheHandbookseries.Together,theyhavepro-
ducedacomprehensiveandwide-rangingaccountoftheperipheralneuropathiesthatwillserveasastandardrefer-
enceforcliniciansandneuroscientistsinyearstocome.ItsavailabilityelectronicallyonElsevier’sScienceDirectsite
aswell asin a print format should ensure its ready accessibilityand facilitate searches forspecificinformation.
Thevolumebringsintofocustheadvancesthatoccurredintheclinicalandbasicneurosciencesoverthelastfew
years, and the individual chapters consider not just the clinical aspects of disorders of the peripheral nervous
system—the epidemiology, natural history, semiology, prognosis, and treatment of these disorders—but also the
mechanismsofdisease,therelevantexperimentalevidence,andthescientificprinciplesgoverningtheirmanagement.
WearegratefultoProfessorSaidandProfessorKrarupfortheirworkasvolumeeditorsandtothecontributors,
who gave freely of their time and expertise to produce scholarly reviews of the literature and subject matter.
As series editors, we read and commented on each of the chapters and can attest to their uniformly high quality.
WealsothankMr.MichaelParkinson,Ms.KristiAnderson,andMr.ThomasStoneofElsevierfortheirunfailing
and expertassistance in thedevelopmentand production ofthis volume.
MichaelJ. Aminoff
Franc¸oisBoller
Dick F.Swaab
Preface
The peripheral nerves are an essential part of the nervous system, and disorders of the peripheral nervous system
(PNS) are the cause of prominent neurological symptoms, including weakness, sensory loss, pain, and autonomic
dysfunction, which are associated with significant morbidity and mortality. These disorders may be hereditary or
sporadicneurologicaldisordersconfinedtothePNSorpartofamorewidespreaddiseaseaffectingboththecentral
andperipheralnervoussystems.However,mostPNSdisordersaresecondarytodysfunctionofotherorgansystems
and may respond to treatment of the primary disease. Greater understanding of these groups of PNS diseases in
recent years has led to important advances in treatment both to prevent and to repair damage of the peripheral
nerves.
Importantadvanceshaveoccurredinseveralareasincludingmoleculargenetics,imaging,biochemistry, immu-
nology,morphology,andphysiology,andthesehaveledtoagreaterunderstandingofthecausesandconsequences
of damage to peripheral nerves, and thereby improved the diagnosis and management of patients with peripheral
nerve disorders. The 54 chapters of the present volume have been subdivided into areas that include Structure
and Function (describing anatomy, embryology, physiology, biochemistry, molecular biology, and imaging of
PNScomponents),ClinicalAspects(examinationandmanagementofPNSdisorders),andspecificnervedisorders.
Eachchapter aims to provide boththe scientific underpinningof thetopic and clinically relevantinformation.
Itisourhopethatthisvolumewillprovideastate-of-the-artoverviewoftheexamination, diagnosis,andtreat-
mentofpatientswiththesediversedisorders.Wehavebeenfortunateenoughtorecruitexpertsondifferentaspects
ofthefieldtocontributetothebookwiththisinmind.Webelievethatthevolumewillservenotonlyasareference
sourceforacademicandpracticingneurologistsbutalsobecomeaneducationalresourcefortraineesinneurology
and neuroscience.
Ge´rardSaid
ChristianKrarup
Contributors
M.J.Aminoff Centro deInvestigacio´nBiome´dicaen Red de
Departmentof Neurology,Schoolof Medicine, Enfermedades Neurodegenerativas, Santander,Spain
University ofCalifornia, San Francisco,CA,USA
P. Bouche
R. Amouri DepartmentofClinical Neurophysiology,
DepartmentofNeurology,InstitutNationalMongiBen Salpeˆtrie`re Hospital,Paris,France
HamidadeNeurologie, Medical Faculty ofTunis,
Universite´ ElManar,Tunis,Tunisia C. Briani
DepartmentofNeurosciences,UniversityofPadova,Italy
X. Arcila-Londono
Departmentof Neurology,WayneState University B.Calabek
School ofMedicine, Detroit, Michigan,USA Kaiser Franz Josef Hospital,Vienna, Austria
J.C. Arezzo A.Castelo
Departments of Neuroscience and Neurology, Albert DepartmentofInfectious Diseases, UNIFESP —
Einstein College of Medicine, New York, USA Escola Paulista deMedicina, Sa˜oPaulo,Brazil
A.K. Asbury M.Catala
Departmentof Neurology,Hospital ofthe University DepartmentofNeurology, Hoˆpital deLa Pitie´-
ofPennsylvania, Philadelphia,PA, USA Salpeˆtrie`re and UMR 7622 CNRS,Universite´ Pierre et
Marie Curie, Paris, France
M. Auer-Grumbach
Departmentof Orthopaedics, Medical University M.W.Chapleau
Vienna,Vienna, Austria Departments ofInternal Medicine and Molecular
Physiologyand Biophysics, UniversityofIowa Carver
J. Baets CollegeofMedicine,VeteransAffairsMedicalCenter,
Neurogenetics Group, VIB Departmentof Molecular Iowa City, IA,USA
Geneticsand Laboratory ofNeurogenetics,Institute
Born-Bunge, UniversityofAntwerpand Division of C. Crone
Neurology, UniversityHospital Antwerp, Antwerp, DepartmentofClinical Neurophysiology,
Belgium Rigshospitalet and University ofCopenhagen,
Copenhagen,Denmark
E. Battalog˘lu
Departmentof Molecular Biology and Genetics, M.R.G. de Freitas
Bog˘azic¸i University, Istanbul, Turkey HospitalUniversita´rio Antonio Pedro, Universidade
Federal Fluminense, Nitero´i,Brazil
M. Bendszus
Departmentof Neuroradiology,University of S. Delanian
Heidelberg, Germany Oncologie-Radiothe´rapie-Radiopathologie, Hoˆpital
Saint-Louis, Paris, France
J. Berciano
Departmentof Neurology and Clinical J.D. England
Neurophysiology,UniversityHospital “Marque´s DepartmentofNeurology, Louisiana State University
de Valdecilla (IFIMAV)”, UniversityofCantabria and Health Science Center, New Orleans,LA, USA
xii CONTRIBUTORS
D.G.R. Evans W. Grisold
Department ofGenetic Medicine, Manchester DepartmentofNeurology,Kaiser-Franz-Josefhospital,
Academic Health Science Centre,CentralManchester Austrian Cluster forTissueRegenerationand Ludwig
NHSFoundation Trust, Manchester Royal Infirmary, Boltzmann Institute for Experimentaland Clinical
Manchester, UK Traumatology, Vienna, Austria
E.L. Feldman A.Gutierrez
Department ofNeurology,University ofMichigan, Department ofNeurology, Louisiana State University
Ann Arbor, MI,USA Health Science Center, New Orleans,LA, USA
R.E. Ferner D.H.Gutmann
Department ofNeurology,Guy’s and St. Thomas’ Department ofNeurology and Neurofibromatosis
NHSFoundation Trust, Guy’sHospital, Center, Washington University, Schoolof Medicine,
London, UK St.Louis, MO,USA
N.B. Finnerup
M.Haltia
Danish Pain ResearchCenter,Aarhus Universityand
Department ofPathology, HaartmanInstitute,
DepartmentofNeurology,AarhusUniversityHospital,
UniversityofHelsinki, Helsinki, Finland
Aarhus,Denmark
K. Hansen
R. Freeman
Department ofNeurology, Rigshospitalet,
Department ofNeurology,Beth Israel Deaconess
UniversityClinic Copenhagen, Denmark
MedicalCenter,Harvard MedicalSchool, Boston,
MA, USA
H.-P. Hartung
Department ofNeurology, Heinrich–Heine University,
K. Fugleholm
Du¨sseldorf,Germany
Department ofNeurosurgery, Rigshospitalet,
Copenhagen, Denmark
F.Hentati
DepartmentofNeurology,InstitutNationalMongiBen
H. Fujimura
Hamida de Neurologie,MedicalFaculty ofTunis,
Department ofNeurology,ToneyamaNational
Universite´ ElManar, Tunis, Tunisia
Hospital,Toneyama, Toyonaka,Japan
E.Hentati
A.A. Gabbai
DepartmentofNeurology,InstitutNationalMongiBen
DepartmentofNeurology,UNIFESP—EscolaPaulista
Hamida de Neurologie,MedicalFaculty ofTunis,
de Medicina,Sa˜o Paulo, Brazil Universite´ ElManar, Tunis, Tunisia
A.Garc´ıa R.A.C. Hughes
Department ofNeurology and Clinical MRC Centre for Neuromuscular Disease,
Neurophysiology, UniversityHospital “Marque´s de Institute ofNeurology, UniversityCollege London,
Valdecilla (IFIMAV)”, University ofCantabria and London, UK
Centrode Investigacio´n Biome´dicaen Redde
EnfermedadesNeurodegenerativas, Santander, Spain
J.Infante
Department ofNeurology and Clinical
L. Ginsberg Neurophysiology, University Hospital“Marque´sde
Department ofClinicalNeurosciences, Institute of Valdecilla (IFIMAV)”, UniversityofCantabria and
Neurology, UniversityCollege London, Royal Free Centro de Investigacio´nBiome´dicaenRed de
Campus,London, UK Enfermedades Neurodegenerativas, Santander, Spain
F.Graus A.Jani-Acsadi
Neurology Department, Hospital Clinic, August Pi i Department ofNeurology, Universityof
SunyerBiomedical Research Institute (IDIBAPS), ConnecticutSchool ofMedicine, Farmington,
Barcelona, Spain Connecticut, USA
CONTRIBUTORS xiii
T.S. Jensen N. Kubis
Danish Pain Research Center,AarhusUniversityand PhysiologyDepartment,HoˆpitalLariboisie`reand
DepartmentofNeurology,AarhusUniversityHospital, Universite´ParisDiderot,SorbonneParisCite´,Paris,France
Aarhus,Denmark
P. Landrieu
R. Kaji DepartmentofPediatricNeurology, CHU Paris sud,
Departmentof Neurology,Tokushima University Hoˆpital Biceˆtre, Paris, France
Graduate School ofMedicine,Tokushima, Japan
M.Laura´
P. Kerschen MRC Centre for Neuromuscular Diseases,UCL
Departmentof Neurology,CHU Henri Mondor and Institute ofNeurology, Queen Square,London, UK
FacultyofMedicine,Universite´ Paris-EstCre´teilValde
Marne,Cre´teil,France H.C. Lehmann
DepartmentofNeurology, Heinrich–Heine University,
G.J. Kidd Du¨sseldorf,Germany
Departmentof Neuroscience, LernerResearch
Institute,Cleveland Clinic, Cleveland, Ohio, USA R.A. Lewis
DepartmentofNeurology, Cedars–Sinai Medical
M.C. Kiernan Center, LosAngeles,California,USA
Princeof Wales Clinical School, UniversityofNew
SouthWales, and Neuroscience Research Australia, C.S.-Y. Lin
Sydney,Australia School ofMedicalSciences, Faculty ofMedicine,
UniversityofNewSouthWales,Sydney, Australia
R. King
Departmentof ClinicalNeurosciences,Institute of S.K.W. Lloyd
Neurology, UniversityCollege London, Royal Free Salford Royal NHSFoundation Trust, Salford, and
Campus,London, UK DepartmentofOtolaryngology, Manchester Academic
Health Science Centre, Central Manchester NHS
S. Kiuru-Enari FoundationTrust, Manchester Royal Infirmary,
Departmentof Neurology,Unitfor Neuromuscular Manchester,UK
Diseases, Helsinki UniversityCentralHospital,
Helsinki, Finland M.J.A. Malessy
DepartmentofNeurosurgery, Leiden University
H. Koike Medical Centre, The Netherlands
DepartmentofNeurology,NagoyaUniversityGraduate
School ofMedicine, Nagoya, Japan H. Manji
MRC Centre for Neuromuscular Diseases,
K. Koshy National Hospitalfor Neurology and Neurosurgery,
Departmentof ClinicalNeurosciences and the London, UK
Hotchkiss BrainInstitute, UniversityofCalgary,
Alberta,Canada C. Marchesi
Clinics of Central and Peripheral Degenerative
C. Krarup Neuropathies Unit, Departmentof Clinical
DepartmentofClinicalNeurophysiology,Rigshospitalet Neurosciences, IRCCS Foundation, C. Besta
andUniversityofCopenhagen,Copenhagen,Denmark Neurological Institute, Milan, Italy
A.V. Krishnan W. Marques, Jr.
School ofMedical Sciences,Faculty ofMedicine, Medical Faculty ofRibeira˜o Preto, UnivesityofSa˜o
University ofNew South Wales, Sydney,Australia Paulo, Ribeira˜o Preto,Brazil
W. Kristoferitsch S.I.Mellgren
Karl-Landsteiner-Institute for Neuroimmunological DepartmentofClinical Medicine-Neurology,
and NeurodegenerativeDisorders, SMZ-Ost – UniversityofTromsøandUniversityHospitalofNorth
Donauspital Vienna, Austria Norway, Tromsø, Norway
xiv CONTRIBUTORS
S.M. Murphy M.M. Reilly
MRC Centre forNeuromuscularDiseases, UCL MRC Centre for Neuromuscular Diseases,
InstituteofNeurology,QueenSquare,London,UKand UCL Institute ofNeurology, Queen Square,
Department ofNeurology,Adelaide & Meath London, UK
HospitalsIncorporating theNational Children’s
Hospital,Tallaght,Dublin,and Trinity College Dublin, L.Roberts
Ireland Department ofNeurology, Universityof Michigan,
Ann Arbor,MI, USA
O.J.M.Nascimento
Antonio Pedro UniversityHospital, Universidade G.Said
FederalFluminense, Nitero´i, Rio deJaneiro,Brazil Department ofNeurology, Hoˆpital dela Salpeˆtrie`re,
Paris, France
E. Nobile-Orazio
E.Salsano
2ndNeurology,DepartmentofTranslationalMedicine,
Clinics of Central and Peripheral Degenerative
Milan University, IRCCS Humanitas Clinical Institute,
Neuropathies Unit, Departmentof Clinical
Rozzano,Milan,Italy
Neurosciences, IRCCS Foundation,C. Besta
Neurological Institute,Milan, Italy
M. Nolano
Department ofNeurology,Salvatore Maugeri
H.H. Schaumburg
Foundation, Telese Terme (BN), Italy
Departments ofNeurology and Pathology,
Albert EinsteinCollege ofMedicine, New York, USA
N. Ohno
Department ofNeuroscience,Lerner Research
S.H. Sindrup
Institute,Cleveland Clinic, Cleveland, Ohio, USA
DepartmentofNeurology,OdenseUniversityHospital,
Odense, Denmark
A.S.B.Oliveira
DepartmentofNeurology,UNIFESP—EscolaPaulista S. Seto
de Medicina,Sa˜o Paulo, Brazil Department ofNeuroscience, Albert EinsteinCollege
ofMedicine,NewYork, USA
D.Pareyson
Clinics ofCentraland Peripheral Degenerative G.Sobue
Neuropathies Unit,Department ofClinical DepartmentofNeurology,NagoyaUniversityGraduate
Neurosciences, IRCCS Foundation, C.Besta School ofMedicine,Nagoya, Japan
NeurologicalInstitute, Milan,Italy
C. Sommer
Department ofNeurology, Universityof Wu¨rzburg,
S.B. Park
Wu¨rzburg, Germany
School of Medical Sciences,Faculty ofMedicine,
University ofNew South Wales, Sydney,
A.Steck
Australia
Department ofNeurology, UniversityHospital Basel,
Basel, Switzerland
Y.Parman
DepartmentofNeurology,IstanbulUniversity,Istanbul
I.Steiner
MedicalFaculty, Istanbul, Turkey
Department ofNeurology, Rabin Medical Center,
BeilinsonCampus, Petach Tikva, Israel
V.Plante´-Bordeneuve
DepartmentofNeurology,CHUHenriMondor,Cre´teil
G.Stoll
and Inserm 1016, Universite´ Paris-Descartes, Institut
Department ofNeurology, Universityof Wu¨rzburg,
Cochin,Paris, France Wu¨rzburg, Germany
P.-F. Pradat M.Suzuki
De´partement desMaladies du Syste`meNerveux, Department ofNeurology, Tokyo Women’s Medical
HoˆpitalPitie´-Salpeˆtrie`re, Paris, France University, Tokyo, Japan
CONTRIBUTORS xv
K. Toyooka A.M.Vincent
Departmentof Neurology,National Hospital DepartmentofNeurology, Universityof Michigan,
OrganizationToneyamaNational Hospital, Toyonaka, Ann Arbor,MI, USA
Osaka,Japan
A.F.J.E. Vrancken
B.D. Trapp
DepartmentofNeurology, Rudolf Magnus Institute
Departmentof Neuroscience, LernerResearch
ofNeuroscience,University Medical Centre, Utrecht,
Institute,Cleveland Clinic, Cleveland, Ohio, USA TheNetherlands
J. Valls-Sole´
E.Wilder-Smith
EMG Unit,NeurologyService,Hospital Clinic,
DepartmentofMedicine, National University
Departmentof Medicine,University ofBarcelona,
ofSingapore,Singapore and Department
AugustPi iSunyerBiomedical Research Institute
ofOrthopedics,Universityof Heidelberg,
(IDIBAPS), Barcelona,Spain
Germany
N. van Alfen
Departmentof Neurology and Clinical N. Yuki
Neurophysiology,Radboud UniversityNijmegen DepartmentofMedicine, Yong Loo Lin School of
MedicalCentre,The Netherlands Medicine, National UniversityofSingapore,
Singapore
A.Vass
DepartmentofNeurology,Kaiser-Franz-Josefhospital, D.W. Zochodne
AustrianClusterfor Tissue Regeneration and Ludwig DepartmentofClinical Neurosciencesand
BoltzmannInstitute forExperimental and Clinical theHotchkissBrainInstitute,University ofCalgary,
Traumatology,Vienna, Austria Alberta, Canada