Neurologic Aspects of Systemic Disease Part III

666 Pages·2014·55.252 MB·English

by  José Biller and José M. Ferro (Eds.)

HANDBOOK OF CLINICAL
NEUROLOGY
Series Editors
MICHAEL J. AMINOFF, FRANC¸OIS BOLLER, AND DICK F. SWAAB
VOLUME 121
EDINBURGH LONDON NEW YORK OXFORD PHILADELPHIA
ST LOUIS SYDNEY TORONTO 2014
ELSEVIERB.V.
Radarweg29,1043NX,Amsterdam,TheNetherlands
©2014,ElsevierB.V.Allrightsreserved.
Nopartofthispublicationmaybereproducedortransmittedinanyformorbyanymeans,electronicor
mechanical,includingphotocopying,recording,oranyinformationstorageandretrievalsystem,without
permissioninwritingfromthepublisher.PermissionsmaybesoughtdirectlyfromElsevier’sRightsDepartment:
phone:(þ1)2152393804(US)or(þ44)1865843830(UK);fax:(þ44)1865853333;e-mail:
[e-mail]queston-lineviatheElsevierwebsiteat
http://www.elsevier.com/permissions.
ThisbookandtheindividualcontributionscontainedinitareprotectedundercopyrightbythePublisher(otherthan
asmaybenotedherein).
ISBN:9780702040887
BritishLibraryCataloguinginPublicationData
AcataloguerecordforthisbookisavailablefromtheBritishLibrary
LibraryofCongressCataloginginPublicationData
AcatalogrecordforthisbookisavailablefromtheLibraryofCongress
Notice
Knowledgeandbestpracticeinthisfieldareconstantlychanging.Asnewresearchandexperiencebroadenour
understanding,changesinresearchmethods,professionalpractices,ormedicaltreatmentmaybecomenecessary.
Practitionersandresearchersmustalwaysrelyontheirownexperienceandknowledgeinevaluatingandusing
anyinformation,methods,compounds,orexperimentsdescribedherein.Inusingsuchinformationormethodsthey
shouldbemindfuloftheirownsafetyandthesafetyofothers,includingpartiesforwhomtheyhaveaprofessional
responsibility.
Withrespecttoanydrugorpharmaceuticalproductsidentified,readersareadvisedtocheckthemostcurrent
informationprovided(i)onproceduresfeaturedor(ii)bythemanufacturerofeachproducttobeadministered,to
verifytherecommendeddoseorformula,themethodanddurationofadministration,andcontraindications.Itisthe
responsibilityofpractitioners,relyingontheirownexperienceandknowledgeoftheirpatients,tomakediagnoses,
todeterminedosagesandthebesttreatmentforeachindividualpatient,andtotakeallappropriatesafety
precautions.
Tothefullestextentofthelaw,neitherthePublishernorthecontributorsoreditors,assumeanyliabilityforany
injuryand/ordamagetopersonsorpropertyasamatterofproductsliability,negligenceorotherwise,orfromanyuse
oroperationofanymethods,products,instructions,orideascontainedinthematerialherein.
ThePublisher
PrintedinChina
The
Publisher's
CommissioningEditor:ThomasE.Stone policy is to use
DevelopmentEditor:MichaelParkinson paper manufactured
from sustainable forests
ProjectManager:AnithaKittusamyRamasamy
Designer/DesignDirection:AlanStudholme
HandbookofClinicalNeurology3rdSeries
Availabletitles
Vol.79,Thehumanhypothalamus:basicandclinicalaspects,PartI,D.F.SwaabISBN9780444513571
Vol.80,Thehumanhypothalamus:basicandclinicalaspects,PartII,D.F.SwaabISBN9780444514905
Vol.81,Pain,F.CerveroandT.S.Jensen,eds.ISBN9780444519016
Vol.82,Motorneuronedisordersandrelateddiseases,A.A.EisenandP.J.Shaw,eds.ISBN9780444518941
Vol.83,Parkinson’sdiseaseandrelateddisorders,PartI,W.C.KollerandE.Melamed,eds.ISBN9780444519009
Vol.84,Parkinson’sdiseaseandrelateddisorders,PartII,W.C.KollerandE.Melamed,eds.ISBN9780444528933
Vol.85,HIV/AIDSandthenervoussystem,P.PortegiesandJ.Berger,eds.ISBN9780444520104
Vol.86,Myopathies,F.L.MastagliaandD.HiltonJones,eds.ISBN9780444518996
Vol.87,Malformationsofthenervoussystem,H.B.SarnatandP.Curatolo,eds.ISBN9780444518965
Vol.88,Neuropsychologyandbehaviouralneurology,G.GoldenbergandB.C.Miller,eds.ISBN9780444518972
Vol.89,Dementias,C.DuyckaertsandI.Litvan,eds.ISBN9780444518989
Vol.90,Disordersofconsciousness,G.B.YoungandE.F.M.Wijdicks,eds.ISBN9780444518958
Vol.91,Neuromuscularjunctiondisorders,A.G.Engel,ed.ISBN9780444520081
Vol.92,Stroke–PartI:Basicandepidemiologicalaspects,M.Fisher,ed.ISBN9780444520036
Vol.93,Stroke–PartII:clinicalmanifestationsandpathogenesis,M.Fisher,ed.ISBN9780444520043
Vol.94,Stroke–PartIII:Investigationsandmanagement,M.Fisher,ed.ISBN9780444520050
Vol.95,Historyofneurology,S.Finger,F.BollerandK.L.Tyler,eds.ISBN9780444520081
Vol.96,Bacterialinfectionsofthecentralnervoussystem,K.L.RoosandA.R.Tunkel,eds.ISBN9780444520159
Vol.97,Headache,G.NappiandM.A.Moskowitz,eds.ISBN9780444521392
Vol.98,SleepdisordersPartI,P.MontagnaandS.Chokroverty,eds.ISBN9780444520067
Vol.99,SleepdisordersPartII,P.MontagnaandS.Chokroverty,eds.ISBN9780444520074
Vol.100,Hyperkineticmovementdisorders,W.J.WeinerandE.Tolosa,eds.ISBN9780444520142
Vol.101,Musculardystrophies,A.AmatoandR.C.Griggs,eds.ISBN9780080450315
Vol.102,Neuro-ophthalmology,C.KennardandR.J.Leigh,eds.ISBN9780444529039
Vol.103,Ataxicdisorders,S.H.SubramonyandA.Durr,eds.ISBN9780444518927
Vol.104,Neuro-oncologyPartI,W.GrisoldandR.Sofietti,eds.ISBN9780444521385
Vol.105,Neuro-oncologyPartII,W.GrisoldandR.Sofietti,eds.ISBN9780444535023
Vol.106,Neurobiologyofpsychiatricdisorders,T.SchlaepferandC.B.Nemeroff,eds.ISBN9780444520029
Vol.107,EpilepsyPartI,H.StefanandW.H.Theodore,eds.ISBN9780444528988
Vol.108,EpilepsyPartII,H.StefanandW.H.Theodore,eds.ISBN9780444528995
Vol.109,Spinalcordinjury,J.VerhaagenandJ.W.McDonaldIII,eds.ISBN9780444521378
Vol.110,Neurologicalrehabilitation,M.BarnesandD.C.Good,eds.ISBN9780444529015
Vol.111,PediatricneurologyPartI,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444528919
Vol.112,PediatricneurologyPartII,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444529107
Vol.113,PediatricneurologyPartIII,O.Dulac,M.LassondeandH.B.Sarnat,eds.ISBN9780444595652
Vol.114,Neuroparasitologyandtropicalneurology,H.H.Garcia,H.B.TanowitzandO.H.DelBrutto,eds.
ISBN9780444534903
Vol.115,Peripheralnervedisorders,G.SaidandC.Krarup,eds.ISBN9780444529022
Vol.116,Brainstimulation,A.M.LozanoandM.Hallett,eds.ISBN9780444534972
Vol.117,Autonomicnervoussystem,R.M.BuijsandD.F.Swaab,eds.ISBN9780444534910
Vol.118,Ethicalandlegalissuesinneurology,J.L.BernatandH.R.Beresford,eds.ISBN9780444535016
Vol.119,NeurologicAspectsofSystemicDisease,J.BillerandJ.M.Ferro,eds.ISBN9780702040863
Vol.120,NeurologicAspectsofSystemicDisease,J.BillerandJ.M.Ferro,eds.ISBN9780702040870
Foreword
Although neurology and psychiatry are closely linked specialties, many neurologists see their specialty as part of
internalmedicine.Indeed,neurologydepartmentsintheUnitedStatesoftenbeganasdivisionswithindepartments
ofinternalmedicine,attestingtotheirspecialrelationship.Withtheevolutionofneurologyasanindependentdisci-
pline,ithasbecomeparticularlyimportantforitspractitionerstoremainfamiliarwiththeneurologicaspectsofsys-
temic diseases as well as with the systemic aspects of neurologic disorders. This has been recognized since the
Handbook of Clinical Neurology was founded by Pierre Vinken and George Bruyn, with volume 1 appearing in
December 1968. That first series concluded in 1982 and was followed by a second series, edited by them, that
concluded—in turn—in 2002. We then took over as editors of the current third series, with volume 79 appearing
inlate2003andseveralvolumesappearingannuallysincethen.
Twovolumes(38and39)werepublishedinthefirstseriesoftheHandbook,focusingontheneurologicmanifes-
tationsofsystemicdiseases.Thesecondseriesincludedafurtherthreevolumes(63,70,and71)onthesametopic,
publishedin1993and1998,withoneofus(MJA)servingasaneditorofthosevolumes.Advancesinthefield,but
especiallyinimmunology,genetics,imaging,pharmacotherapeutics,andintensivecare,sincethattimehaveneces-
sitatedareappraisalofthefieldandthepublicationofthreenewvolumesonthetopic.Wearethereforeparticularly
delightedatthepublicationofthisscholarlycontributiontothemedicalandneurologicliteratureandwelcomeitas
partoftheHandbook.Webelievethatitwillappealnotonlytoneurologistsbuttophysiciansinallspecialties,helping
intheirinteractionswitheachotherandwiththeirpatients.
ProfessorsJose´ BillerandJose´ M.Ferrohavetogetherproducedanauthoritative,comprehensive,anduptodate
accountofthetopicandhaveassembledatrulyinternationalgroupofauthorswithacknowledgedexpertisetocon-
tributetotheseimportantmultifacetedvolumes.Wearegratefultothemandtoallthecontributorsfortheireffortsin
creatingsuchaninvaluableresource.Weareconfidentthatcliniciansinmanydifferentdisciplineswillfindmuchin
thesevolumestoappealtothem.
Itisapleasure,also,tothankElsevier,ourpublishers–andinparticularTomStone,MichaelParkinson,andKristi
Anderson–forunfailingandexpertassistanceinthedevelopmentandproductionofthesevolumes.
MichaelJ.Aminoff
Franc¸oisBoller
DickF.Swaab
Preface
Medicinehasalwaysbeeninastateofevolutionandtoday,morethanever,withtheacceleratedgrowthofscientific
knowledge,patientsareevaluatedandtreatedbyteamsofphysicians.Theextensivebodyofknowledgeandthemajor
scientificandclinicaladvancesinneurologyandinternalmedicineareagaindrawingbothspecialtiesclosertogether.
Whateverthesubspecialtyareaofinterest,thenatureofmodernclinicalmedicinecallsformultidisciplinarycollab-
orativeeffortstobettermeettheneedsofindividualpatients.Theaimofthesethreevolumesofthethirdseriesofthe
HandbookofClinicalNeurologyistointegrateandprovideathoroughframeworkofthecoreneurologicmanifes-
tations ofawidearray ofsystemicdisorders. Each chapterprovides acritical appraisaland extensivebackground
informationregardingthevarietyofpresentationsofeachdisorder,thecharacteristicclinicalcourse,thetypicalneu-
rologic manifestations of each disease, and current therapeutic strategies. Comprehensive and updated references
alsobringforthvaluableresourcesforfurthertopicalreadingandresearch.Ourintendedaudienceincludesexperi-
encedpractitionersandresidentsinneurology,neurosurgery,andinternalmedicine,aswellasotherhealthcarepro-
fessionalsindifferentsubspecialtiescaringforthesechallengingpatients.
Wehavepurposelydividedthesethreevolumesintochaptersuniformlyorganizedbyorgansystem,whicharefur-
therdividedbyspecificconditionsanddiseasecategories.VolumeIisdedicatedtotheneurologicaspectsofcardio-
pulmonary diseases, renal disorders, and selective rheumatologic and musculoskeletal disorders. Volume II
encompasses core neurologic aspects of gastrointestinal and hepatobiliary disorders, endocrinologic diseases, and
agamutofmetabolic,nutritionalandenvironmentalconditions.VolumeIIIconcentratesontheneurologicaspects
of hematologic and oncologic disorders, organ transplantation, infectious diseases, and tropical neurology. It also
includesamiscellaneousgroupofdisordersincludingneurodermatology,neurologicalcomplicationsofpregnancy,
iatrogenicneurology,neuromusculardisordersintheintensivecaresetting,posteriorreversibleleukoencephalopathy
and reversible cerebral vasoconstriction syndromes, neuro-Behcet’s, complications of neuroimaging, neuro-
traumatology,andobservationspertainingtoneurologyinthedevelopingworld.Thesevolumesgobeyondthescope
of classic neurology and examine the neurologic manifestations of a wide range of medical conditions, spanning
mostareasofmedicine,thatneurologists,neurosurgeons,internists, andotherspecialistsmustdiagnoseandtreat
ineverydaypractice.
Wearehopefulthatthesethreevolumeswillcontributetothebestpossiblecareofpatientswiththesedisorders,and
thatthereadershipwillfindthematerialinformative,authoritative,reliable,andstimulating
Weareextremelygratefultoallthecontributorsfromacrosstheglobe,whobysharingtheirknowledgeandexper-
tisemadethesevolumespossible.Tobringtofruitionaworkofthismagnituderequiresahighlyprofessionaleditorial
effort,andforthiswethankLindaTurnerforherwonderfulorganizationalskillsandadministrativeexpertise,and
MikeParkinsonandtheeditorialstaffatElsevierfortheirunfailingdedication,professionalism,andexpertassistance
inthedevelopmentandproductionofthesethreevolumes.
Jose´ Biller,MD
Jose´ M.Ferro,MD
Contributors
H.M.Ahmed D.Dean
DepartmentofNeurologicalSurgery,LoyolaUniversity DepartmentofInternalMedicine,IndianaUniversity,
MedicalCenter,Maywood,IL,USA Indianapolis,IN,USA
G.Akman-Demir O.H.DelBrutto
DepartmentofNeurology,SchoolofMedicine,Istanbul SchoolofMedicine,UniversidaddeEspecialidades
BilimUniversity,Istanbul,Turkey EspirituSantoandDepartmentofNeurological
Sciences,HospitalClinicaKennedy,Guayaquil,
Z.Argov Ecuador
DepartmentofNeurology,Hadassah–Hebrew
UniversityMedicalCenter,Jerusalem,Israel A.Didelot
CentredeRe´fe´rence,deDiagnosticetdeTraitementdes
J.F.Bale,Jr. SyndromesNeurologiquesParane´oplasiquesand
DepartmentsofPediatricsandNeurology,Universityof INSERMU842,UMR-S842,Lyon,France
UtahSchoolofMedicine,SaltLakeCity,UT,USA
A.Ducros
J.R.Berger DepartmentofNeurology,HoˆpitalGuideChauliac,
DepartmentofNeurology,UniversityofKentucky Montpellier,France
CollegeofMedicine,Lexington,KY,USA
S.Edelstein
M.Bilgrami DepartmentofAnesthesiology,LoyolaUniversity
DepartmentofMedicine,LoyolaUniversityMedical MedicalCenter,Maywood,IL,USA
Center,Maywood,IL,USA
O.Fustinoni
J.Biller INEBAInstituteofNeurosciencesandFacultyof
DepartmentofNeurology,LoyolaUniversityChicago, Medicine,UniversityofBuenosAires,BuenosAires,
StritchSchoolofMedicine,Maywood,IL,USA Argentina
H.S.Block J.C.Garcia-Monco
DepartmentofNeurology,LoyolaUniversityChicago, ServiceofNeurology,HospitaldeGaldacano,
StritchSchoolofMedicine,Maywood,IL,USA Galdacano,Vizcaya,Spain
M.C.Brouwer G.Godeneche
DepartmentofNeurology,AcademicMedicalCenter, DepartmentofNeurology,CHULaMile´trie,Poitiers,
Amsterdam,TheNetherlands France
F.D.Dastur W.Grisold
DepartmentofMedicine,P.D.HindujaNational DepartmentofNeurology,KaiserFranzJosepHospital,
Hospital,Mumbai,India Vienna,Austria
xii CONTRIBUTORS
M.Guarino W.S.Jellish
NeurologyUnit,S.Orsola-MalpighiUniversityHospital, DepartmentofAnesthesiology,LoyolaUniversity
Bologna,Italy MedicalCenter,Maywood,IL,USA
G.Guillet S.V.Khadilkar
DepartmentofDermatology,CHULaMile´trie,Poitiers, DepartmentofNeurology,GrantMedicalCollegeand
France SirJ.J.GroupofHospitalsandBombayHospital
InstituteofMedicalSciences,Mumbai,India
J.J.Halperin
DepartmentofNeurosciences,OverlookMedical D.K.Kochar
Center,Summit,NJ,USA MedicalResearch,RajasthanUniversityofHealth
Sciences,Jaipur,India
S.G.B.Heckenberg
DepartmentofNeurology,KennemerGasthuis, C.Lamy
Haarlem,TheNetherlands DepartmentofNeurology,Universite´ ParisDescartes,
HoˆpitalSainte-Anne,Paris,France
R.Helbok
DepartmentofNeurology,MedicalUniversityHospital N.Latronico
Innsbruck,Innsbruck,Austria DepartmentofAnesthesiaIntensiveCareand
PostoperativeCare,DivisionofNeuroanaesthesiaand
A.Heroux NeurocriticalCare,UniversityofBrescia,SpedaliCivili,
HeartFailureandHeartTransplantProgram, Brescia,Italy
LoyolaUniversityMedicalCenter,Maywood,
IL,USA R.B.Love
DepartmentofCardiothoracicSurgery,MedicalCollege
{
J.Hildebrand ofWisconsin,Milwaulkee,WI,USA
Fe´de´rationdeNeurologieMazarin,GroupeHospitalier
Pitie´-Salpeˆtrie`re,Paris,France C.R.Marino
DepartmentofMedicine,UniversityofTennessee
S.E.Hocker HealthScienceCenter,Memphis,TN,USA
DivisionofCriticalCareNeurology,Collegeof
Medicine,MayoClinic,Rochester,MN,USA J.L.Mas
DepartmentofNeurology,Universite´ ParisDescartes,
D.Holt HoˆpitalSainte-Anne,Paris,France
DepartmentofSurgery,LoyolaUniversityMedical
Center,Maywood,IL,USA S.Mathis
DepartmentofNeurology,CHULaMile´trie,Poitiers,
J.Honnorat France
CentredeRe´fe´rence,deDiagnosticetde
TraitementdesSyndromesNeurologiques E.Melian
Parane´oplasiquesandINSERMU842,UMR-S842, DepartmentofRadiationOncology,LoyolaUniversity
Lyon,France Chicago,StritchSchoolofMedicine,Maywood,IL,
USA
S.Hou
DepartmentofMedicine,DivisionofNephrologyand A.A.Miravalle
Hypertension,LoyolaUniversityMedicalCenter, DepartmentofNeurology,UniversityofColorado
Maywood,IL,USA SchoolofMedicine,Aurora,CO,USA
M.Jacewicz M.T.Mitchell
DepartmentofNeurology,UniversityofTennessee DepartmentofRadiology,AdvocateChristHospital,
HealthScienceCenter,Memphis,TN,USA OakLawn,IL,USA
{(deceased)
CONTRIBUTORS xiii
J.M.K.Murthy G.C.Roma´n
ContinentalInstituteofNeurosciences&Rehabilitation, DepartmentofNeurology,WeillCornellMedical
ContinentalHospitals,IT&FinancialDistrict, College,MethodistNeurologicalInstitute,Houston,
Gachibowli,Hyderabad,India TX,USA
J-P.Neau K.L.Roos
DepartmentofNeurology,CHULaMile´trie,Poitiers, DepartmentofNeurology,IndianaUniversitySchoolof
France Medicine,Indianapolis,IN,USA
P.O’Keefe J.D.Rosenblum
DepartmentofMedicine,LoyolaUniversityMedical DepartmentofRadiology,LoyolaUniversity
Center,Maywood,IL,USA Chicago,StritchSchoolofMedicine,Chicago,
IL,USA
C.Oppenheim
DepartmentofNeurology,Universite´ ParisDescartes, R.Ruda`
HoˆpitalSainte-Anne,Paris,France
DivisionofNeuro-Oncology,Departmentof
Neuroscience,UniversityandSanGiovanniBattista
T.C.Origitano Hospital,Turin,Italy
DepartmentofNeurologicalSurgery,LoyolaUniversity
MedicalCenter,Maywood,IL,USA
S.Saip
DepartmentofNeurology,CerrahpasaSchoolof
S.V.Pamboukian
Medicine,IstanbulUniversity,Istanbul,Turkey
SectionofAdvancedHeartFailure,CardiacTransplant,
MechanicalCirculatorySupportandPulmonary
V.Sanghi
VascularDisease,UniversityofAlabamaat
DivisionofNeurology,BombayHospitalandMedical
Birmingham,Birmingham,AL,USA
ResearchCentre,KEMHospitalandSethG.S.Medical
College,Mumbai,India
O.Pasternak
DepartmentofRadiology,LoyolaUniversityChicago,
E.Schmutzhard
StritchSchoolofMedicine,Chicago,IL,USA
DepartmentofNeurology,MedicalUniversityHospital
Innsbruck,Innsbruck,Austria
J.Ga´llegoPe´rez-Larraya
DepartmentofNeurologyandNeurosurgery,Clinicof
T.Schreiner
theUniversityofNavarra,UniversityofNavarra,
DepartmentofNeurology,Children’sHospital,Aurora,
Pamplona,SpainandFe´de´rationdeNeurologie
CO,USA
Mazarin,GroupeHospitalierPitie´-Salpeˆtrie`re,Paris,
France
B.S.Singhal
E.C.PerryIII DepartmentofNeurology,BombayHospitalInstituteof
DepartmentofNeurologicalSurgery,LoyolaUniversity MedicalSciences.Mumbai,India
MedicalCenter,Maywood,IL,USA
A.Siva
K.Potluri DepartmentofNeurology,CerrahpasaSchoolof
DepartmentofMedicine,DivisionofNephrologyand Medicine,IstanbulUniversity,Istanbul,Turkey
Hypertension,LoyolaUniversityMedicalCenter,
Maywood,IL,USA E.M.Sizoo
DepartmentofNeurology,VUUniversityMedical
T.E.Rodriguez Center,Amsterdam,TheNetherlands
Bone Marrow Transplantation Program, Loyola
University Medical Center and Department of R.Soffietti
Medicine, Loyola University Chicago, DivisionofNeuro-Oncology,Departmentof
Stritch School of Medicine, Chicago, Neuroscience,UniversityandSanGiovanniBattista
IL, USA Hospital,Turin,Italy
xiv CONTRIBUTORS
L.A.Sposato D.vandeBeek
DepartmentofClinicalNeurologicalSciences,London DepartmentofNeurology,AcademicMedicalCenter,
HealthSciencesCentre,UniversityofWesternOntario, Amsterdam,TheNetherlands
London,Ontario,Canada
E.F.M.Wijdicks
A.Stracciari DivisionofCriticalCareNeurology,Collegeof
NeurologyUnit,S.Orsola-MalpighiUniversityHospital, Medicine,MayoClinic,Rochester,MN,USA
Bologna,Italy
C.H.Wigfield
C.Sundaram Department of Surgery, Section of Cardiac &
DepartmentofPathologyNizam’sInstituteofMedical Thoracic Surgery, University of Chicago, Chicago,
Sciences,Panjagutta,Hyderabad,India IL, USA
M.J.B.Taphoorn S.A.Zˇivkovic´
DepartmentofNeurology,MedicalCenterHaaglanden, NeurologyService,DepartmentofVeteransAffairsand
TheHague,Netherlands DepartmentofNeurology,UniversityofPittsburgh
SchoolofMedicine,Pittsburgh,PA,USA
E.Trevisan
DivisionofNeuro-Oncology,Departmentof
Neuroscience,UniversityandSanGiovanniBattista
Hospital,Turin,Italy
HandbookofClinicalNeurology,Vol.121(3rdseries)
NeurologicAspectsofSystemicDiseasePartIII
JoseBillerandJoseM.Ferro,Editors
©2014ElsevierB.V.Allrightsreserved
Chapter77
{
Brain metastases
JAIMEGA´LLEGOPE´REZ-LARRAYA1,2*ANDJERZYHILDEBRAND2(deceased)
1DepartmentofNeurologyandNeurosurgery,ClinicoftheUniversityofNavarra,UniversityofNavarra,Pamplona,Spain
2F(cid:2)d(cid:2)rationdeNeurologieMazarin,GroupeHospitalierPiti(cid:2)-Salp(cid:3)tri(cid:4)re,Paris,France
INTRODUCTION and the most common type of brain tumor in adults.
Theyexceedthenumberofprimarybraintumorsatleast
Despite better prevention and treatment advances
fourfold.
achievedduringthelastdecades,cancerisstillamajor
Theincidenceofbrainmetastasesisthoughttoberis-
public health concern and remains one of the leading
inginthelastfewdecadesduetoacombinationoffac-
causesofdeathworldwide(Lopezetal.,2006).Thecen-
torsotherthanpopulationaging.First,improvementsin
tralnervoussystem(CNS)isafrequenttargetformetas-
and wider use of neuroimaging have resulted in an
tasesfromsystemiccancer.Themostcommonlocation
increasedandearlierdetectionofclinicallysilentmetas-
ofCNSmetastasesisthebrainparenchyma,followedby
tases. For example, routine brain scans are performed
theleptomeningealspace.
during the staging evaluation in neurologically asymp-
Parenchymalmetastasesdifferfromleptomeningeal
tomaticpatientswithnewlydiagnosedlungcancer(Shi
disease in clinical presentation, treatment modalities,
et al., 2006) or metastatic melanoma (Gavrilovic and
andprognosis.However,theircombinationiscommon:
Posner, 2005). Second, more effective treatments for
on one hand, superficial brain lesions may invade the
systemic disease have extended survival of cancer
subarachnoidspace,andontheotherhand,primarylep-
patients,leadingtoalargerpopulationatriskforbrain
tomeningeal carcinomatosis often invades the brain
metastases. Third, some highly effective anticancer
parenchymaviaperivascularVirchow–Robinspaces.
agentspoorlycrosstheblood–brainbarrier(BBB),and
Thischapterdealsonlywithmetastasesrestrictedto
are thereby unable to eradicate dormant micrometas-
thebrainparenchyma.
tasesinpatientswithcontrolledsystemicdisease.
Everymalignanttumorisabletometastasizetothe
INCIDENCEANDPRIMARYTUMORS
brain.However,onlyalimitednumberaccountforthe
The exact incidence of metastatic brain tumors is vast majority of brain metastases. In adults, three
unknown. Most epidemiologic studies may underesti- tumors, lung and breast carcinomas and malignant
mate their true incidence, in part because some brain melanoma,accountforupto75%ofbrainmetastases
metastases remain asymptomatic, in part because even (Nussbaum et al., 1996; DeAngelis and Posner, 2009).
symptomatic lesions are often ignored in severely ill Inchildrenandveryyoungadults,theprimarytumors
patients with advanced primary disease (Gavrilovic most likely to metastasize to the brain are sarcomas
andPosner,2005).Autopsyandclinicalstudiessuggest (osteogenic sarcoma, rhabdomyosarcoma, and
thatbrainmetastasesoccurin10–30%ofadultpatients Ewing’s sarcoma) and germ cell tumors (Kebudi
with systemic malignancies (Posner and Chernik, 1978; et al., 2005).
Schoutenetal.,2002),thusrepresentingbyfarthemost Lung cancers arethemostcommonsourceofbrain
frequent neurologic complication of systemic cancer metastases,accountingforatleastonehalfofthecases
*Correspondence to: Jaime Ga´llego Pe´rez de Larraya, M.D., Ph.D., Department of Neurology and Neurosurgery, Clı´nica
UniversidaddeNavarra,UniversidaddeNavarra,Avd.Pı´oXII,36,Pamplona31008,Spain.Tel:þ34-948-255400,Fax:þ34-948-
296500,E-mail:[email protected]
{ThischapterisdedicatedtothememoryofProfessorJerzyHildebrand