Professional Documents
Culture Documents
SECOND
EDITION
OXFORD
V!YJVJtSITY RESS
Oxford University Press. Inc .. pnbhshc ...' ....
orks th:elffurther
~t:\\'
\'vrk
Auckland
(;~'I)("1()\\,n
KU<I1HLumpur
tvlttdrill
Dar es
'r,opc:!
(long
Saleem
Melbourne
~tcxic() City
Kong Karachi
Ntli]'(ILi
'rl)fVllh_,
11ft
ill
a rt:lricVitl $\':;tCIII,or
trnusmtttcd.
~
electronic,
11l1.!<:ht)lIi(._ ...
rll:~)fbe reproduced.
in anv" loon or bv nil\'
~I,
J'll(_'t'>(:vp)~ng,rccordlug. or otherwise,
1l1C'UIS.
~)f
(.;(l)lgl'e::;$ (:l,I{:I)oging-Su.Ptlb1it;~,ti,)U Dot
di'''(lrcJf'I'sl [~~dil"o,~d
hyJ !\ ndrew (;, Rlig(:1.-2.ncl ed.
(Contcmporery 11curol~)' series : S I}
luvludes l>il>1io~rilplu('al references .. and Index.
~I y:~th'~l,iil gl'l'IviS ;Iud myasthenk-
I.),: em. -
IS1~N 07S-0-1fJ-f)73S61-0
(hardcover ~elk. paper]
I. Engel, :\Itdre\v,
l J Series: C<JO!(!IIIj)or':uy flt~lIr(llog\' sertcs : 8.1, OOGtJ-9446
r1>NI,..~1: t. ~'Iya::;tl\cn.l;, t;I'~1\1:<. Z. Neuromuscular juucrion Dlseascs. \\lJ (;()7(i9N \',3120.12/\"):;
LC clussiiication not as....i~ncd
r;16.7'442-- clc23
20 J I ()3!i;().,
5?'t51
Tlle science of utc..heine is a rapidl)' c}I<lllgillg field, As new research uud ("lini(''<llexperience broaden OUT knowledge.
ch:,ugP.'Sin h'(::ltUl(,lIt and dl11g therapy 1)('(;111', The author end publisher of this work h;l\'(~checked with sources h('lip.v('d
to be reliable in the-ir efforts to provide information th;)! is accurate and complete. arid ill accordance with the standards
accepted ut tho ttme (.11'publlcntion Hoc-ever, in lj~ht of the possil'ilit} of human error Or (,hallgc. ....in Llll' practlce
or 'i,cdlcia)e. uenber rho <1~llb()r. '1(1" the ptcb1i:d,(,,',rl(I,' {III)' ether p:'II1:)' \\'bo has been ill\'(llved ifI the preparatlo or
puhlicntion of this work warrants that tho iufcrmaticn contained herein is in CVCl), r<'sll(:ct accurate or complete. Readers
urc encouraged to coufinn the tnfcrmatton containedherein \\'illl .'lht'r rclteble S(JHrCC$. end are slrongly edvtscd to check
rhc PI'(l(!I,I(;lInfornuitton sJI(J('1pruvklcd by the ph:n In:'(:IJI,lli~,1<':<Jrnp:II\}' I(u' (::.1(.:11
drug IhPJ' pl:)~Jto :'l<iluinh;ter,
9.57654321
Prtnte c! ;11 rbe lJuile<t St;Ite":.;{)f t\n,(-ril.::l
011:l(:id-frc(~ p;_lpcr
Contents
NEUROi\fUSCULAR JUNCTION
A",lrew C. Engel
BASIC CONCEPTS
:3
Channels of the Presynaptic Membrane Endocytotic Events and the Formation of Ne....v S}/(l<'l>ticVesicles
15
16
The junctional Folds und Thelr Cytoskeletal Components .... \Chl~s on the junctional Folds
"11(' Sall.trati"'g Disk Model (If Ncurornusculur Trensnussiou R-.:gllh\!i()u (If IJI(' Distribution
and Subunit Composition of !\C'hll The Postsynaptic Cytoskeleton The J unctional
Snrcoplasu :\Chl~ Synthesis und Dogrndsrion
SICNALING MECI-IANISMSArTIIE
NEUHOMUSCULAHJUNCTIOK
25
:\grin, ~'luSK, and LI~r>4 1)()k~7and Its Downstream Activators Crk. Crk ..L, and Tid.l
TIl(:: NetJregtditl/}\ (lIt\ Sign(,Jillg r'atll\\'il)'
2. ELECTlIODIACNOS(S
C. Mtcbel ttarper
INTHODUC-rfON
on j\IYASTHENICDlSOH])HS
3,
37
J\(~I'iOI)
38
Potent ials
Contents
NEEDLE
ELECl'ROMYOGHAPII Y 46
The Properties of' Motor Unit Potentials ~'IUl' Changes Observed in Myasthenic
Disorders
Singlc:-I"i1:)(:r lO:I.(;(;trO'tl}'()gr~'l)h}'
ELECTHOD1.AGNOSTJC
FlNDJNGS
IN SPECIFIC
3. THE IM~IUNOPATHOGENESIS
MYASTHENIC DlSOHDERS
Syndrome
OF MYASTHENIA GRAVIS
INTHODUCTION
(;0
JMMUNOLOGICAL
Basic Properties
>
The Intrathymic
O1n(1
Autotmmunity
so
61
PHINCIPLES
47
Tolerance
- :\rlligell Recognition by
T Cells
B CF.LL IlF.SI'ONSES
TN MYASTHENII\
GRAVIS
78
General Features of :\ntj,.\Chl{ Antibodies Epitopes l{e<:ognized by Anti~t\ChR Autibodies
,1\ III i-St riatioual J\nliI.)I )(lie:-- "'~'IIiSK-p<)sitj\,('" and "SI~r()II(;g<)Livc" ivt}':-..slbonia
>
COOPEHATION
PATHOGENIC
HET\VEEN
SUMMARY
EFFECTS
OF THE i\NTI-i\CHR
ANTIBODIES
82
83
84
,I.
EP[D~MLOLOCY
ONSET
Ka',I:;,lski
90
92
92
OCULAR MYASTHENL<\
9:3
Contents
XI
"
..
AGE-RELATED
FAMILIAL INCIDENCE
DISORDERS
Thymoma
OF MYASTHENIA GRAVIS
ASSOCIATED
Thyroid Disorders
SUMMARY
\VITH MYASTHENIA
too
101
GR/IVIS
10]
102
AND HISTOIUCAL
BACKGHOUND
l'HESENTATION
109
i'\'[}:~lsllu.:I)iaGr~~\;;s ~\'l(~Caused I)~'Autilxxlies
.L08
CLINICAL
1<)Muscle-Specific Protein
Kinase (i\'luSK-1'.'IG}
PllYSICAL FINDINGS
Ill.
Myasthenia Gravis
}\'luSK-j'vIC
DIFFERENTIAL
113
DIAGNOSIS
r)jagl'l()~i~
\o\IC:ftkl)t~S:s Diifcrcnfinl
DIAGNOSTIC
11(;
I'ATHOGEKSIS
Tests
130
130
1:30
OF MG
onrci
N OF MG
1.3.1
THEATMENTOFMG
132-
Prelimtnaries
Treatment StJ'illegies
THEATING
or
145
()rug Interactions
FUTURE
The
PROSPECTS
PUl'H(lc)X of
FOH TREATMENT
CONCLUSIONS
(")1'
OF ~fG
148
!\,tC Treatments
150
XII
Contents
(If STORY
1.5(;
CLIKICAL
FEATUHES
156
J5i
CANCER-ASSOCIATE!)
NON-CANCER
L,I~MS (CA-LE:MS)
157
ASS()CIATE;,I) OR AUTOIMMUr\E:
EI'IL)EM IOLOGY
LEMS (NCA-LEMS)
151>
159
PATHOPHYSIOLOGY
ELECTHODIAGNOSTIC
AND IMMUNOPATHOLOGY
l5!)
FI:-1DINGS
16l
Repetitive Nerve Stimulation Testing Needle ElectTolll)'ogr,lph)' (E;v[G) Si,ngJ(::.fll)er
1~1C!<:I'l"on)y()gl'<)I)lly Electl'()IYI~f(lgl'al)lljc
Qnantitation
SEHOLOGICAL TESTING
l65
\loll'lge.g~ll(.~(,1
Calcium Channel Autihodies SOXl
OTH EH TESTS
1136
DIFFEHENTJAL
l)J;\GNOSlS
MGn,ElvIS OVERLAP
TREATMENT
Cancer
;\Jlcib('ldic.::!'
l66
167
lmmunotherapy
SUMMARY
severity
)67
Svmptomatic Treatment
PROGNOSIS
(If
168
169
169
MYASTHENIC
SYNDHOMES
173
MECHANISMS COMPROMISING
THE SAFETY ~IARGIN OF
NEUROMUSCULAR
TRANSMISSION
li4
THE CLASSIF1CATION
THE INVESTIGATION
Clintcnl ()tISCI'Vilt-iUI)S
OF CONGENITAL
OF CONGENITAL
l\!J()tl)hc)i(I!,.,)'
MYASTHENIC SYNDROMES
li11
MYASTHENIC SYNDHOlvlES
),74
In vitro
Svndrouu-
SYr\AIYfIC BASAJ/LAMINA-ASSOCIATI!:D
eMS
lilt)
Endplate Acetylcholinesterase Deficiency Defects il) p2.L.~\lninin