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fatigabilitate pronuntata. Miastenia a fost descrisa pentru prima data de catre Thomas Willis n 1972.
Miastenia are la baza sinteza de anticorpi ndreptati contra receptorului de acetilcolina situat pe
membrana fibrei musculare striate si care participa la contractia musculara.
Cuprins
Generalitati
Patogenia
Etiologie
Tabloul clinic
Diagnostic
Diagnostic diferential
Tratament
Generalitati
Miastenia gravis este o maladie relativ rara si poate debuta la orice vrsta. Femeile sunt afectate
putin mai frecvent dect barbatii cu un raport de 6:4. Incidenta mai mare a miasteniei la femei se
observa n jurul vrstei de 30 de ani, n timp ce incidenta maxima la barbati este atinsa la vrsta de
50-60 de ani.
Patogenia
Pentru a ntelege patogenia miasteniei gravis este necesar de a cunoaste mecanismul de generare
a contractiei musculare. Fibra musculara striata si primeste inervatia prin intermediul jonctiunii
neuromusculare. Aceasta jonctiune este alcatuita din membrana presinaptica care este butonul
terminal al axonului neuronului motor periferic, membrana postsinaptica care este membrana fibrei
musculara si fanta sinaptica. Pe membrana presinaptica se situeaza veziculele cu acetilcolina, iar pe
membrana postsinaptica receptorii acetilcolinei (ACh). La propagarea unui impuls spre membrana
presinaptica are loc eliberarea veziculelor n fanta presinaptica si acetilcolina difuzeaza spre
receptorii ACh provocnd deschiderea canalelor ionice si generarea potentialului de actiune
muscular. Dupa realizarea actiunii sale, acetilcolina este hidrolizata de catre acetilcolinesteraza.
n miastenie, sistemul imun devine ostil fata de receptorii ACh si ncepe sa sintetizeze anticorpi antiAChR. Ca rezultat are loc reducerea numarului de receptori ACh si respectiv actiunea acetilcolinei
este prejudiciata. Anticorpii ACh sunt prezenti n aproximativ 80-90% din cazuri de miastenie gravis.
Pacientii cu miastenie nsa fara anticorpi anti-AChR sunt recunoscuti ca fiind seronegativi (miastenia
gravis seronegativa). La o buna parte din acesti pacienti nsa se depisteaza alti anticorpi cum ar fi
anticorpii anti-MuSK.
Natura autoimuna a miasteniei este confirmata si prin observatiile unei asocieri frecvente dintre
miastenie si alte maladii autoimune (tiroidita autoimuna, lupusul eritematos de sistem, artrita
reumatoida, vitiligo s.a.)
Etiologia
Cauza miasteniei gravis este necunoscuta. Penicilamina este cunoscuta a declansa diferite tulburari
autoimune, printre care si miastenia gravis. Sunt cunoscute o serie de medicamente care pot
precipita si agrava miastenia gravis si care trebuie evitate la acesti pacienti:
Litiul
Procainamida
Verapamil
Quinidina
Clorochina
Prednisolonul
Anticolinergicele (trihexifenidil)
Tabloul clinic
Principala manifestare a miasteniei gravis este slabiciunea musculara. Slabiciunea poate afecta
toate grupurile musculare si n acest caz vom vorbi despre miastenia gravis generalizata. n cazul
formei oculare a miasteniei, slabiciunea este limitata doar la musculatura oculara extrinseca.
Musculatura bulbara este afectata n special n formele seronegative ale miasteniei. Slabiciunea n
membre are un caracter idiopatic, fiind mai pronuntata n segmentele proximale.
Variabilitatea slabiciunii este trasatura a miasteniei. Cunoasterea acesteia este obligatorie pentru o
evaluare corecta a pacientilor. Astfel, slabiciunea musculara n miastenie este mai pronuntata seara
si poate lipsi n orele diminetii. Acest fapt poate cauza erori de diagnostic.
Slabiciunea musculaturii faciale este prezenta practic n toate cazurile si determina o expresie
faciala de masca. Implicarea muschilor extraoculari va determina ptoza si diplopie. Frecvent
pacientul si ncreteste fruntea pentru a compensa ptoza palpebrala. Slabiciunea musculaturii
palatului moale va determina un aspect nazonat al vocii ct si un risc de regurgitatii si aspiratii
pulmonare. Procesul de masticatie deasemenea poate fi ngreunat, uneori pacientii fiind nevoiti sa-si
sprijine mandibula cu maina.
Ca regula progresia maladiei are loc la interval de saptamni sau luni. Daca maladia se prezinta
initial cu simptome oculare, slabiciune progreseaza ulterior distal, implicnd musculatura faciala,
bulbara, tronculara si a membrelor inferioare. Mai rar slabiciunea se poate limita la musculatura
oculara pentru perioade de luni sau ani de zile.
De o importanta majora n miastenie este afectarea musculaturii respiratorii care poate fi letala n
caz de exacerbari. Criza miastenica poate fi precipitat de infectii intercurente sau de un sir de
medicamente care au fost mentionate mai sus. n caz de dezvoltare a crizei miastenice cu
insuficienta respiratorie se recurge imediat la intubare oro-traheala.
Remisiile spontane ca regula sunt rare. Cele mai multe remisii sub actiunea tratamentului survin n
primii 3 ani ai maladiei.
Diagnostic
Pentru diagnosticul miasteniei gravis se utilizeaza o serie de analize imunologice, metode imagistice
ct si examenul electrofiziologic.
Analizele imunologice
Depistarea anticorpilor n serul pacientilor cu miastenie gravis este o metoda de diagnostic
valoroasa, dat fiind prezenta acestor anticopri la 80-90% din pacienti.
Anticorpii contra receptorului de acetilcolina (anti-AChR) - testul este pozitiv la 74% din pacienti.
Anti-AChR sunt mai frecvent depistati la pacientii cu miastenie generalizata (80%) si doar la 50% din
pacientii cu forma oculara pura. Rezultatele fals pozitive pot fi obtinute n ca de sindrom LambertEaton, timom fara miastenie, cancer pulmonar cu celule mici, artrita reumatoida tratata cu
penicilamina ct si la 1-3% din populatia de peste 70 de ani.
Anticorpii contra fibrei striate - sunt prezenti la 84% din pacientii cu timoma mai tineri de 40 de ani si
mai rar la pacientii fara timoma.
Anticorpii contra tirozin kinazei musculare - Aproximativ jumatate din pacientii seronegativi (fara antiAChR) sunt pozitivi pentru anti-MuSK. Acesti pacienti pot reprezenta un grup distinct al miasteniei,
demonstrnd unele particularitati clinice. Pacientii cu anti-MuSK pozitivi tind a avea simmptome
bulbare mai severe asociata cu atrofie faciala si a limbii. n plus, ei nu beneficiaza de pe urma
inhibitorilor colinesterazei, aceste preparate putndu-le chiar agrava situatia.
Investigatiile imagistice
Tomografia computerizata a mediastinului este mandatorie pentru identificarea timomului, n special
la batrni, la care radiografia simpla poate fi negativa. RMN cerebrala si orbitala nu este
recomandata spre a fi efectuata de rutina, ea fiind necesara doar n cazurile neclare pentru
excluderea unor formatiuni de volum orbitare sau tronculare sau a sclerozei multiple.
Examenul electrofiziologic
Electromiografia poate detecta defectul de transmitere la nivelul jonctiunii musculare prin intermediul
pe doua: prin stimularea nervoasa repetitiva si prin electromiografia de fibra unica. Electromiografia
de fibra unica se considera a fi mai sensibila n diagnosticul miasteniei gravis dect stimularea
nervoasa repetitiva.
Testul cu edrofoniu (Tensilon)
Edrofoniul (Tensilon) este un inhibitor al acetilcolinesterazei cu actiune scurta care amelioreaza forta
musculara la pacientii cu miastenie gravis. Acesta este motivul pentru care se utilizeaza ca metoda
de diagnostic n miastenie. Pentru realizarea testului este necesar de a aprecia forta musculara
(ptoza sau strngerea minii) nainte si dupa administrarea edrofoniului. Dat fiind riscul de
bradicardie sinusala, se cere a avea la dispozitie o ampula cu atropina. Pentru test se utilizeaza 1 ml
solutie de edrofoniu de 10 mg/ml. Initial se administreaza 0,1 ml din doza. Daca nu survin careva
reactii adverse se administreaza restul de 0,9 ml.
n evaluarea testului este necesar de avut n consideratie ca aceste preparate pot ameliora forta
musculara si n caz de alte maladii asa ca scleroza laterala amiotrofica, polimiozita si unele forme de
polineuroneuropatii.
Diagnostic diferential
Diagnosticul diferential al miasteniei gravis se realizeaza cu urmatoarele maladii:
Scleroza multipla
Patologia tiroidiana
Dermatomiozita/polimiozita
Sindromul Tolosa-Hunt
Tratamentul
Inhibitorii acetilcolinesterazei si terapia imunomodulatoare reprezinta pilonii tratamentului miasteniei
gravis. n formele moderate inhibitorii AChE pot reprezenta terapia de baza, n timp ce n formele
mai severe poate fi necesara asocierea preparatelor imunosupresoare.
Preparatele din grupul inhibitorilor AChE utilizate n miastenia gravis sunt piridostigmina (Mestinon,
Kalimin) si neostigmina (Prostigmina). Prednisolonul, azatioprina, imunoglobulina intravenoasa,
ciclosporina, ciclofosfamida, micofenolat, rituximab sunt preparatele care pot fi utilizate atunci cnd
este necesara terapia imunomodulatoare.
O alternativa la tratamentul medicamentos este plasmafereza, care actioneaza prin eliminarea
anticorpilor si complexelor imune din serul bolnavilor. Plasmafereza se utilizeaza n special n
perioada pre- si postoperatorie la pacientii cu miastenie gravis sau n tratamentul exacerbarilor.
Daca miastenia nu raspunde la alte tratamente, plasmafereza poate fi utilizata saptamnal ca
tratament de baza.
Tratamentul chirurgical al miasteniei se utilizeaza la pacientii cu timom sau hiperplazie a timusului.
Timectomia trebuie realizata la toti pacientii cu timom si la cei fara timom nsa cu vrste ntre 10-55
ani si cu miastenie generalizata. Remisiile induse de timectomie sunt mai frecvente la pacientii tineri
tratati chirurgical. Rata de remisie creste odata cu timpul atingnd 40-60% la 7-10 ani dupa
interventie. Mai amanuntit despre timectomie vedeti (Timectomia).
Trebuie sa intelegem ca muschii nu inseamna numai cei care misca bratele si picioarele, cei cu care
mergem, alergam, urcam scari sau caram greutati, ci tot muschi sunt si cei care ne ajuta sa vorbim,
sa mestecam si sa inghitim mancarea, sa tinem ochii deschisi, sa vedem o singura imagine cu ambii
ochi, cei care ne coordoneaza mimica fetei ca si cei care ne coordoneaza respiratia. Bolnavii de
miastenie pot avea probleme cu toti acesti muschi, simultan sau pe rand.
Ciudat, nu? Nu seamana cu nicio boala "obisnuita".
Cum descriu bolnavii de miastenie ceea ce simt?
De cele mai multe ori si pentru bolnav e greu sa realizeze si sa descrie ce simte, mai ales cand
simptomele sunt discrete si apar si dispar aparent fara explicatie. Activitati simple pentru cei mai
multi oameni devin dificile sau imposibile pentru miastenic, cum ar fi mancatul, vorbitul sau rasul
...cand am ochii obositi, pleoapele imi sunt grele si se lasa peste ochi, incat nu-i mai pot deschide,
pe amandoi sau uneori doar pe unul din ei.
cand ma uit lateral sau in sus, vad dublu, uneori sunt doua obiecte in loc de unul sau imaginile
capata un al doilea contur
vorbesc cu voce tare si la un moment dat nu mai pot pronunta clar, vocea devine nazonata si nu
se mai intelege ce spun
uneori am o fata "lunga", parca fara expresie, colturile gurii imi sunt lasate, nu mai pot sa rad cu
toata gura ci doar schitez o grimasa
alteori nu mai pot sa tin gura inchisa, simt ca mandibula cade si trebuie sa o mentin cu mana
cand mananc ceva tare, un covrig sau biscuit, la un moment dat obosesc sa mestec si mancarea imi
ramane in obraji, ca la hamsteri...
ma inec cand inghit alimente solide, trebuie sa beau mereu apa in timpul mesei
ma inec si cu apa, tusesc si imi dreg mereu vocea, uneori apa imi iese pe nas...
scap obiectele din mana, nu mai pot sa tin nici pixul pe hartie, nu pot ridica bratele in sus sa ma
pieptan...
urc greu treptele, ma opresc des, nu mai pot sa ma ridic de pe scaun, din pat
cand merg, mi se moaie dintr-o data genunchii si chiar am cazut brusc pe strada sau pe scari, lumea
m-a etichetat drept betiv(a) sau drogat(a)....
am avut toate aceste simptome....
am ajuns in criza, nu am mai putut inghiti nici saliva si nici respira bine si m-au dus la spital unde
imediat m-au conectat la un aparat de respiratie artificiala......
Sunt povestile celor ca noi.
inShare
Astzi, pot fi controlate MG. Exist mai multe terapii disponibile pentru a ajuta la reducerea
slbiciune muscular. Cele mai multe persoane cu MG au rezultate bune la tratament. La
unele persoane MG, ca multe alte boli autoimune, poate merge n remisie (o perioad de
timp fr simptome) i slbiciune muscular pot disprea complet.
Remiterea sau mbuntirea pot aprea fr tratament, n unele cazuri. n conformitate cu
distrofie muscular de asociere, pn la 20 la sut din persoan cu MG poate avea
remiterea complet a simptomelor fr nici un tratament, i un alt 20 la sut pot mbunti
fr tratament. Aceste mbuntiri spontane sunt mult mai probabil s apar n timpul
stadii incipiente de MG.
Tratamentul MG pot include:
Thymectomy, ndeprtarea chirurgical timus (care este anormale n cele mai multe
persoane cu MG). Aceasta operatie este adoptat pentru persoanele cu MG care au
tumori, precum i pentru persoanele fr tumori. Aceasta mbuntete simptome n
mai mult de jumtate din persoanelor fr tumori. Acesta poate vindeca unii oameni
cu MG, eventual prin re-balancing sistemul imunitar.
Alte terapii uneori utilizat pentru a trata MG perioadele mai ales dificil de slbiciune includ:
Schimb plasmapheresis sau plasm. Aceasta este o procedur care elimin anticorpi
anormale din snge.
http://www.news-medical.net/health/Myasthenia-Gravis-Treatment-%28Romanian%29.aspx
http://archive.today/ainmd.org
Antibodies
Anti-AChR
MuSK
Striational
Other
General principles
Other diagnostic tests
Repetitive nerve stimulation
Single fiber EMG
Tensilon testing
Rationale
o As thymectomy or long term immunotherapy may be
necessary to treat MG, it is essential to establish a firm
diagnosis
o A firm diagnosis avoids inappropriate treatments, and
their side effects, in patients who do not have the
disease.
Tensilon tests
o May be readily performed at the bedside
Cogan
General
Drug properties
o Action
Inhibits acetylcholinesterase
Prolongs presence of neurotransmitter,
acetylcholine, in the NMJ
Results in enhanced muscle strength
o Duration: Lasts for a few minutes
o Response
In patients with NMJ dysfunction
Not specific for MG
o Time course: Minutes; Rapid-onset; Short-acting
Method
Initially
o Dosing: 2 mg of edrophonium is administered
intravenously as a test dose
o Monitoring heart rate: Bradycardia or ventricular
fibrillation may develop
Follow-up
o After observing for about 2 minutes, if no clear response
develops
o Up to 8 additional mg of edrophonium is injected
A double-blind protocol with a saline injection as placebo has
been advocated
Testing should be performed with patient free of all
cholinesterase-inhibitor medications
Cholinergic side effects of edrophonium
o May include increased salivation and lacrimation, mild
sweating, flushing, urgency & perioral fasciculations.
Muscle AChR
(Adult)
Serum antibodies vs Acetylcholine Receptors
o Thymoma without MG
o Immune liver disorders
o Lambert-Eaton syndrome (13%)
o Primary lung cancer: 3%
o Older patients (> 70 years): 1% to 3%
o Neuromyotonia
Antibody effects on AChRs
o Modulation
Mechanism
o Anti-AChR antibodies cross-link AChRs on the
post-synaptic membrane
o Endocytosis & degradation of AChRs are
accelerated
Test results
o May occur in a rare patient when anti-AChR
antibody binding is negative.
o MG: Usual loss = 20% to 90% of AChRs
(Normal < 20%)
o MG with thymoma: > 90% loss
o False positives: Hemolysis; Muscle relaxant
drugs; Serum heating
o Blocking binding site for ACh on AChR (positive = 26% to
100% blocking).
Prevalence: Repeated arthrogryposis; 52% of
generalized MG; 30% of ocular
1% of patients with no binding or blocking
antibodies
o Complement binding
RNS: General
From: M Al-Lozi
o Time course
Onset: Immediate
Duration: ~2 minutes
o Degree of repair: Partial or Complete
Post-exercise exhaustion
o Definition: Post exercise appearance, or
exacerbation, of decremental response
o Protocol
Stimulus: Muscle exercise for 1 minute
Repeat RNS after 1, 2, 3 and 4 minutes
o Time course
Onset: Maximal 3 to 5 minutes after
exercise
Disappears by: 10 minutes after exercise
o RNS is positive in about 75% of patients with generalized
MG, if:
Proximal & Clinically involved muscles are tested
Muscle is warm: Cooling reduces size of decrement
More than one muscle is tested: Strong muscles
often have less decrement
o Diagnostic issues
Sensitivity of RNS for MG: Greatly reduced when
distal muscles are tested
RNS is positive in only 50% of patients with ocular
MG.
Diagnostic specificity
Normal SFEMG
Abstract
Patients with autoimmune myasthenia gravis (MG) should be further
classified before initiating therapy, as treatment response varies for
1. Introduction
Myasthenia gravis (MG) has a prevalence of 150 per million, with
nearly one million MG patients worldwide. The yearly incidence is 10
15 per million per year [1]. Before any treatment was available the
prognosis was severe, with an expected 50% 10-years mortality. With
modern treatment facilities such as immunotherapy, thymectomy, and
intensive care facilities available, population-based studies show that
MG and non-MG individuals have the same life expectancy [2], but still
often with reduced physical abilities, reduced quality of life, and risk of
complications.
There are three key aspects of MG which define the therapeutic
opportunities.(i)MG is a well-defined autoimmune disease and thus
responds to immunoactive treatment.(ii)MG is caused by impaired
acetylcholine receptor (AChR) stimulation in the postsynaptic skeletal
muscle membrane and thus responds to an increase in AChR activity.
(iii)MG has muscle weakness as the only symptom, and consequently
should respond to measures that increase muscle function and
counteract muscle weakness.
MG treatment is firmly established as the domain of neurologists.
Neurologists should be in charge even if the target organ is skeletal
muscle, disease mechanisms are systemic, thymus is a target organ
for diagnostic, therapeutic and scientific approach, hypoventilation is a
life-threatening symptom, and diplopia often the most troublesome
symptom. Ten percent of MG patients have another autoimmune
disorder in addition, further supporting the need for complementary
medical competence. Close cooperation with other fields of medicine
provides knowledge regarding new immunoactive drugs, thus
expanding the therapeutic opportunities for MG.
2. MG Classification
The various subgroups of autoimmune MG respond differently to
treatment. Thus, before deciding any treatment, all individual MG
patients should be defined according to subgroups. Classification
aspects reflect the investigations of each patient that are necessary to
undertake [3]:(1)early-onset MG: age at onset <50 years. Thymic
hyperplasia;(2)late-onset MG: age at onset >50 years. Thymic atrophy;
(3)thymoma-associated MG;(4)MG with anti-MuSK antibodies;(5)ocular
MG: symptoms only from periocular muscles;(6)MG with no detectable
AChR and MuSK antibodies;
The MG group with no detectable antibodies is heterogeneous. Some of
these patients have low-affinity AChR antibodies that are not
detectable by the routine assays and sometimes also thymic
hyperplasia [4]. Some may similarly have undetectable MuSK
antibodies, and some most probably have autoantibodies against other
antigen(s) in the postsynaptic membrane. There are not yet any
commercial tests available for the low-affinity AChR antibodies [5]. MG
patients with a thymoma have nearly always detectable AChR
antibodies in serum. Necessary investigations include tests for AChR
and MuSK autoantibodies and CT/MR of the anterior mediastinum. Titin
and ryanodine receptor antibodies may be helpful for classification. For
patients with no AChR and MuSK antibodies, it is necessary with
thorough examinations to exclude other causes for their muscle
weakness,
including
nonautoimmune
myasthenic
syndromes.
Neurophysiological examinations with repetitive nerve stimulation and
responding to plasma exchange and IvIg are not necessarily the same.
Thus, if one treatment fails, the other may well be tried. It should be
more convenient to add IvIg after plasma exchange than doing the
procedure the other way around, this is to avoid washing away all
therapeutic immunoglobulin just given to treat the patient.
For severe MG and in an acute situation, high-dose parenteral
corticosteroids can be given and also in addition to plasma exchange
or IvIg [10]. An early exacerbation can be seen after initiation of
corticosteroids, but with pharmacological doses, a therapeutic effect
often appears very early.
4. Drug Treatment
4.1. General
Patients with the diagnosis of MG should always be considered for
symptomatic as well as immunoactive drug treatment. Nearly all
patients need some treatment, at least in periods where the disease
shows clinical activity with permanent or intermittent muscle
weakness. Symptomatic drugs have a short-lasting activity both
regarding effect and side effects. Dosage can be rapidly changed and
the treatment is flexible. Immunoactive drugs have an effect linked to
pathogenesis, and the effect usually needs some time before it
becomes manifest. Side effects are relevant and should be considered
in a long-term perspective. Immunoactive drugs need special attention
in children and MG women of childbearing age [6]. Thus, the
considerations for patient and doctor are different for symptomatic and
immunoactive drug treatment.
4.2. Symptomatic Drugs
Acetylcholine esterase inhibition at the neuromuscular junction has a
symptomatic effect in myasthenia and especially in autoimmune MG
[3, 10, 1416]. Optimal dosage is adjusted according to effect and side
effects. Side effects appear from the nonneuromuscular cholinergic
synapses in the autonomic system, which are overstimulated.
Alternative ways to increase the amount of acetylcholine at the
neuromuscular end plate have been tried, but with less effect than
inhibiting the degradation. Acetylcholine esterase inhibitors have a
stable and predictable effect, apparently unchanged over years. No
scientific comparisons have been undertaken between the various
esterase inhibitors. The most commonly used is pyridostigmine and
also the faster acting neostigmine. Ambenonium is used in some
countries. Some MG patients with anti-MuSK antibodies are
hypersensitive to an increase in acetylcholine concentration.
5. Thymectomy
Thymectomy should be undertaken in all the 1015% of MG patients
with a thymoma. MG improvement sometimes occurs in such patients,
but less consistently than in patients with a hyperplastic thymus. The
main reason for thymectomy in thymoma patients is to remove a
potentially infiltrating tumour [24]. In some patients with no or very
mild MG symptoms, a severe exacerbation of MG with an increase in
6. Accessory Treatment
During acute MG exacerbations, intensive care therapy with respiratory
support is life saving. Infections should be treated rigorously. The
marked reduction in MG mortality is for a large part due to modern
intensive care therapy [2], although also pharmacological treatment
and thymectomy represent cornerstones in MG therapy.
Physical training, weight control, and sensible life style modifications
should be discussed with all MG patients [10]. Seasonal flu vaccination
should be recommended. Symptomatic ophthalmological treatment
may be helpful for ocular MG with troublesome diplopia.
7. Treatment Principles
MG should be treated early and with vigour, after classification of
subtype and MG severity. Moderate or severe myasthenic weakness
represents an immediate and permanent challenge. Treatment at an
early stage with thymectomy and/or immunoactive drugs improve
long-term outcome. With a lack of initial response, it is not sufficient to
have tried only 2-3 alternative drug options. Drugs can be combined.
Longitudinal measurement of AChR antibodies can be helpful in
evaluating treatment effect and also in the differentiation between MG
and non-MG symptoms experienced by the patient [5]. The clinical
response and evaluation is most important, but there tends to be a
correlation between MG severity/activity and AChR antibody
concentration in the individual patient. There are no studies
systematically and prospectively examining the usefulness of repeated
antibody examinations in established MG. Non-MG drugs given to
patients with MG should always be checked for potential adverse
neuromuscular effects.
Most MG patients are in the need of long-term therapy. For patients in
a stable remission when on immunoactive drugs, a conservative policy
regarding full drug withdrawal is recommended. A low-dose
prednisolone, azathioprine, or other immunoactive drugs can in such
patients be sufficient to maintain the stable condition, but also
necessary to avoid new exacerbations. Younger patients in particular,
not least after thymectomy, can obtain a full clinical remission without
any need for continued drug treatment. Late-onset MG patients and
thymoma MG patients usually need life-long treatment.
In 10% of MG patients, the onset is before age 18 years [6]. The
disease is very rare in infancy. In Asian populations, up to 50% of cases
present in adolescence [9]. Most children with MG have AChR
antibodies. Those without antibodies should be thoroughly checked for
non-MG myasthenic syndromes. The response to thymectomy is
usually very favourable, and thymectomy should be done early.
8. Future Treatment
It is a paradox that MG treatment is still so unspecific. MG is the best
characterised autoimmune disease with well-defined pathogenetic
antibodies that impair function through the destruction and inhibition
of muscle cell AChR. Still our therapeutic immunosuppression is aimed
http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD006986/pdf
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