Clinica Miastenia

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EXPERIMENTAL AND THERAPEUTIC MEDICINE 9: 1363-1368, 2015

Clinical characteristics and therapeutic evaluation


of childhood myasthenia gravis
ZHIXIAO YANG1, KAILI XU1 and HUI XIONG2
1

Department of Neurology, Children's Hospital of Zhengzhou, Zhengzhou, Henan 450053;


Department of Paediatrics, Peking University First Hospital, Beijing 100034, P.R. China

Received April 29, 2014; Accepted December 5, 2014


DOI: 10.3892/etm.2015.2256
Abstract.
This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia
gravis (MG) and address the prognosis through follow-up. The
clinical data of 135children with MG were grouped according
to clinical type and therapeutic drugs, retrospectively analyzed
and prospectively monitored. Of the 135MG patients, 85.2%
had type I (ocular type), with only 4.2% progressing to
systemicMG; 13.4% had typeII (general type); and 1.5% had
typeIII (fulminating type). Relapse occurred in 46.1% of the
102patients that were followed up. The positive rate for the
primary acetylcholine receptor antibody was 40.19%, without
significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by
electromyography was 37.97%. Decreased expression of CD4+,
CD8+, or CD3+ was present in 71% of the patients. Thymic
hyperplasia was present in 5.93% of the patients, while 1.48%
had thymoma. Steroid treatment was effective in the majority
of the patients. Ocular type MG was common in this cohort
of patients. The incidence and mortality of myasthenia crisis
were low, the presence of concurrent thymoma was rare and
only a limited number of children developed neurological
sequelae.
Introduction
Myasthenia gravis (MG) is an autoimmune disease primarily
mediated by the anti-acetylcholine receptor antibody
(AchRAb), which is associated with a variety of immune
molecules and occurs in neuromuscular junctions(1,2). MG
is characterized by skeletal muscle weakness, in parts of or
the entire body following continuous repetitive motion, fatigability and fluctuating levels of muscle weakness, as well as
other symptoms, including diplopia, ptosis and difficulties in

Correspondence to: Dr ZhiXiao Yang, Department of Neurology,

Children's Hospital of Zhengzhou, 255 Gangdu Street, Zhengzhou,


Henan 450053, P.R. China
Email: yangzhixiaocn@126.com

Key words: children, myasthenia gravis, treatment, prognosis

mastication and swallowing. It was previously demonstrated


that genetic factors play an important role in the pathogenesis
of MG(3). In addition, viral or bacterial infections, such as
poliovirus and Escherichia coli, may be involved in the pathogenesis of MG(4,5). However, there is no evidence indicating
a definitive MG pathogenesis. Anti-cholinesterase drugs,
nonspecific immunosuppressive agents, thymectomy and
plasmapheresis are currently the main treatment options(6-8).
MG is more common among Chinese children, who exhibit
significantly different clinical characteristics compared to
those observed in adults. The aim of this study was to analyze
the clinical characteristics, classification and treatment of MG
in children and understand the prognosis through follow-up, in
order to determine the optimal treatment options and improve
the patients' quality of life. In addition, we present the conclusions drawn from the age-related clinical characteristics,
treatment and prognosis.
Materials and methods
Experimental subjects. A total of 135children diagnosed with
MG, who were admitted to the Children's Hospital of Zhengzhou
between January,2008 and January,2014, were included in
this study. The patients included 57males and 78females. The
age at onset ranged between 4months and 15years, with an
average age at onset of 3years and 2months. Threepatients
were aged <1year, 65were aged 4months3years, 41were
aged 47years, 20were aged 812years and 6patients were
aged 1315 years. The age at the time of the doctor's visit
ranged between 4months and 15years. A total of 81patients
were newly diagnosed and 54received irregular treatment or
experienced recurrence following treatment.
This study was conducted in accordance with the principles of the Declaration of Helsinki and received approval
from the Ethics Committee of the Peking University First
Hospital. Written informed consent was obtained from all the
participants or their legal custodians.
Diagnostic criteria and classification. The following clinical
symptoms were considered indicative of MG: muscle fatigue;
muscle weakness mitigated in the morning and worsening
in the evening; and progressive muscle weakness following
continuous or repeated contractions, mitigated with rest or
cholinesterase inhibitor use.

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YANG et al: CHILDHOOD MYASTHENIA GRAVIS

The diagnosis is confirmed with a positive result on one of the


following tests(9): fatigue test or sulfuric acid neostigmine test
(every 0.0250.05mg/kg, no improved muscle weakness after
1530min, restitution after 1.5h); repetitive nerve stimulation
frequency test (low-frequency repetitive stimulation of muscle
action potential amplitude, decreasing attenuation>10%); or
positive determination of serum antiAchR antibody.
The MG classification was determined using the modified
Osserman method as follows: typeI (ocular type MG; OMG)
with only extraocular muscle involvement, including typeIa
(ptosis) and typeIb (ptosis and eye fixation); typeII (general
type MG; GMG) including typeIIa (eye and limb muscle
weakness) and typeIIb (general weakness during mastication
or swallowing and dysarthria); typeIII (fulminating type) with
sudden weakness and myasthenic crisis; typeIV (flaccid type),
which progresses from typeI orII and peaks at 23years; and
typeV (amyotrophic type)(4).
Methods. The clinical characteristics, affiliated examinations,
treatment and prognosis of the patients were retrospectively
analyzed and prospectively followed up.
Statistical analysis. Data analysis was performed using
SPSS14.0 software (SPSS Inc., Chicago, IL, USA). Continuous
data are expressed as meansstandard deviation or minimum
and maximum. Enumeration data are expressed as the number
of patients and frequency. Groups based on the enumeration
data are expressed using the number of patients and percentage
of patients within each group. The comparisons among the
enumeration data groups were performed using Chi-square
tests or Fisher's exact tests. P<0.05 was considered to indicate a
statistically significant difference.
Results
Clinical manifestations and classification. The treatment duration was <1month in 55patients, 13months in 29patients,
46 months in 12 patients, 712 months in 7 patients and
113years in 32patients. The majority of the patients with a
disease course of 6months were newly diagnosed patients,
while the majority of the patients with a disease course
of>6months received irregular treatment or experienced relapse
following treatment. All the patients experienced manifestations
involving clinical muscle weakness during hospitalization:
only 61patients (45.2%) had ptosis; 54patients (40.0%) had a
combination of ptosis and eye movement disorders; 14patients
had ocular symptoms with associated limb weakness; 4patients
had a combination of bulbar muscle involvement, such as facial,
tongue, throat and mastication muscle weakness; and 2patients
had respiratory muscle involvement. Type I was present in
115patients (85.2%), typeII in 18patients, typeIII in 2patients
(1.5%) and none of the patients had typeIV orV (TableI).
A 12-year-old female patient developed hyperthyroidism
following multiple disease recurrences. A 2.3-year-old female
patient also had selective IgA deficiency. A total of 6patients
experienced a myasthenic crisis during the course of the
disease, including 4patients who underwent ventilatorassisted
respiratory therapy and 2patients who succumbed to severe
pulmonary infection. An 18-year-old patient was misdiagnosed with muscular dystrophy; 4patients were misdiagnosed

with oculomotor nerve palsy, including 1 patient that was


misdiagnosed for 4years; 5patients were misdiagnosed with
Guillain-Barr syndrome; and 1patient was misdiagnosed with
idiopathic epilepsy. Twopatients only exhibited limb-girdle
muscle weakness, with an early misdiagnosis of psychogenic
illness; and 2 patients were misdiagnosed with brain stem
encephalitis. These patients were ultimately diagnosed using
electromyography (EMG), serum AchR-Ab detection and
response to treatment.
Serum antibody. Serum AchR-Ab (Beijing-Hui Bogosian
Biotechnology Co., Ltd. Beijing, China), anti-ryanodine
receptor calcium release channel antibody (RyR-Ab)
(Shanghaikamaishu Biotechnology Co., Ltd., USA) and anticatenin antibody (Titin-Ab) (Shanghai Chemical Reagent Co.,
Ltd., Shanghai, China) were detected by ELISA and monitored
during follow-up. Serum AchR-Ab was positive in 43 (40.19%)
of the 107primarily diagnosed patients and the positivity rate
was not significantly different among the clinical subtypes.
Of the 28 primarily diagnosed patients in whom serum
RyR-Ab was detected, 3patients (10.71%) were also positive
for AchRAb Serum Titin-Ab was detected in 30 patients
and 12patients (40%) tested positive for both Titin-Ab and
AchRAb. Computed tomography (CT) did not reveal thymoma
in 9of these patients and chest radiography was normal in
3patients who did not undergo chest CT. Of the 18patients
with negative Titin-Ab, 7were positive for AchRAb, whereas
the remaining 11patients were negative. Following treatment
for 624months, 24patients underwent dynamic follow-up
of serum AchR-Ab and 9 of the 17patients (53%) who were
initially negative for AchR-Ab were found to be positive; in
2of these patients, AchR-Ab remained positive from the end
of treatment to clinical recovery during the follow-up. Of the
7patients with positive AchR-Ab, 4had negative AchR-Ab with
symptomatic remission.
EMG. EMG (Model ZET-100; Shanghai Zhong Ren Electronic
Instrument Co., Ltd.) was conducted in 79patients, with routine
inspection of the facial, ulnar, median and common peroneal
nerves; the skin over the muscles was routinely disinfected and
the needle poles were quickly inserted. The pin position was
selected as the center of the abdominal muscle or movement
points and a 1 or 2 needle electrode was inserted into every
muscle. The different (upper, middle, or lower) muscle insertion sites were selected when the parameters of the motor unit
potential were determined. The repetitive nerve stimulation
frequency test of the 30patients (37.97%) exhibited attenuation, whereas none of the ocular patients exhibited peripheral
nerve attenuation.
Immunological parameters. As regards the 63patients who
underwent immunoglobulin and the 101 who underwent
CDseries tests, the IgG, IgA and IgM results were all normal,
1patient exhibited decreased IgA and 71% of the patients had
CD series count abnormalities, with the majority expressing
decreased CD4 + and CD8+ and some patients expressing
increased CD19+ (TableI).
Imaging. Routine radiographic examination of the anteroposterior chest was performed in 131patients and 57patients

EXPERIMENTAL AND THERAPEUTIC MEDICINE 9: 1363-1368, 2015

1365

Table I. Clinical information of 135 cases of patients.


Characteristics
Gender
Male
Female
Upper respiratory infection before onset
Age at onset (5 months-15 years)
5 months3 years
47 years
812 years
1315 years
Clinical classification at onset (Osserman type)
Ia
Ib
IIa
IIb
III
Relapsing cases (times)
12
35
>5
Auxiliary examinations
AchR-Ab-positive
RyR-Ab-positive
Titin-Ab-positive
Negative Ab turned positive during follow-up
Abnormal RNS
Abnormal thyroid function
Abnormal immunoglobulin
Abnormal CD series
Thymic hyperplasia, thymoma

Patient no. (n=135)

57-59
78-76
36

40.0-43.7
60.0-46.3
26.7

68
41
20
6

50.4
30.4
14.8
4.4

61
54
14
4
2
54/106a
43/54
10/54
1/54

45.2
40.0
10.3
3.0
1.5
50.9
79.6
18.5
1.9

43/107b
3/28c
12/30d
9/17e
30/79f
17/86g
1/63h
71/101i
2/131j

40.2
10.7
40.0
53.0
38.0
26.1
1.6
71.0
1.5

A total of 29 patients were lost to follow-up. bPrimarily diagnosed patients. cNumber of patients who underwent the RYR-Ab test. dNumber
of patients who underwent the Titin-Ab test. ePatients initially negative for AchRAb. f,g,h,iNumber of patients who underwent the respective
tests. jPatients who underwent radiographic examination of the chest. AchRAb, anti-acetylcholine receptor antibody; RYR-Ab, anti-ryanodine
receptor calcium release channel antibody.
a

underwent simultaneous single-slice spiral CT of the thymus;


8patients (6.11%) exhibited thymic hyperplasia and 2patients
(1.5%) had thymoma that was pathologically diagnosed,
without Titin-Ab detection.
Other tests. Following blood biochemistry, muscle enzyme
and thyroid function tests, 1patient had hyperthyroidism with
positive thyroglobulin and anti-thyroid peroxidase antibodies.
The echocardiography results of all the patients were normal.
Treat ment. Oral pyr idinium brom ide neostigm ine
(57mgkgday, 3times/day) was administered to 39patients
for 6 months to 1 year, depending on the condition. Oral
prednisone was administered to 26patients, with transitional
reduction: the initial 1.52mgkgday was gradually reduced
to 7.510 mg every morning and with optimal symptom
control (46weeks later), the therapy was maintained for

12 years. Seven patients were treated with pyridinium


bromide neostigmine combined with prednisone that was
transitionally reduced; for the 61patients treated with methylprednisolone pulse therapy at a dose of 1520mgkgday,
each week-long course consisted of a continuous administration for 3days and no treatment for 4days for 13courses,
followed by 1.52mgkgday oral prednisone, which was
reduced after 46 weeks and followed by a reduction of
0.5mg/kg oral prednisolone every two weeks in transitional
decrements. After it was decreased to 0.5mg/kg/day it was
maintained at that concentration for a period of 12years.
Two patients were routinely administered prednisone for
12years postoperatively following thymectomy. In addition,
10 patients were treated with 400 mgkgday intravenous
gamma globulin (IVIG) due to repeated recurrence of the
disease, once a day for 5days. A total of 9patients received
cyclosporinA, azathioprine and other immunosuppressive

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YANG et al: CHILDHOOD MYASTHENIA GRAVIS

Table II. Therapeutic and followup information of the patients (n=106).


Therapeutic schemes

Total no.

Ocular typea
Pyridostigmine group
Steroid group
General typeb
Pyridostigmine group
Steroid group
Course of treatment (years)
<1
12
>2

Cured, no. (%)

Relapse (no.)

88
43
45
21
7 (33)
14
67
36 (54)
31
18 9 9
6
4 (67)
2
12
5 (42)
7
30
74
2

10 (33)
40 (54)
2 (100)

20
34
0

=2.662, P=0.103. bExact probability test, P=0.620.

a 2

agents. The duration of improved muscle weakness, duration of maximum effect and recurrence (e.g., symptoms
reappearing following discontinuation of the drug) were
monitored during the follow-up for inpatients and outpatients.
Regarding the four types of treatment (based on the initial
treatment program), 39patients were classified to the single
pyridinium bromide neostigmine group, 26patients to the
transitional reduction of oral prednisone group, 7patients to
the pyridinium bromide neostigmine in combination with
reduction of prednisone group and 61patients to the methylprednisolone pulse therapy group. The mean treatment
onset time was 13(1017), 11(415), 8(112) and 2(16)days,
respectively. The mean duration of the maximum effect was
74(23212), 52(19174), 48(7125) and 18(354)days, respectively, with no significant differences among the subtypes.
The follow-up of the treatment lasted for 215years (until
January,2010), during which time 29patients were lost to
follow-up, with the remaining 106MG patients completing the
followup. During the treatment, relapse occurred12times in
43patients, 35times in 10patients and >5times in 1patient;
the onset of relapse occurred within 2 years for 84.6% of
the patients. The frequency of relapse was not significantly
different between the single pyridinium bromide neostigmine
and the steroid groups, but the lower rate of recurrence in the
steroid treatment group was statistically significant (TableII).
In the 10patients who were administered IVIG due to repeated
recurrence, their condition improved markedly in the first
1015days following the injection.
Due to frequent relapses or steroid dependence/resistance,
2patients received additional azathioprine and 1patient was
switched to cyclosporin A treatment due to bone marrow
suppression. A total of 9patients were administered cyclosporinA and 1 of these patients was switched to sulfur
azathioprine due to suboptimal efficacy.
Adverse reactions. All the early adverse reactions that developed in patients who underwent steroid therapy alone involved
different degrees of Cushing's syndrome, weight gain,
hypertension (n=1) and weakness exacerbation (n=2). Patients
who were administered pyridinium bromide neostigmine

combined with steroid therapy exhibited no early exacerbation


of weakness. Upon symptom improvement, the steroid dose
was reduced. During the maintenance period, the majority of
the patients exhibited no other long-term adverse effects, such
as severe infections, fractures, abscess formation or gastrointestinal hemorrhage.
Followup. The longest follow-up period was 15 years in
1patient, who is currently 27years old, without symptoms of
muscle weakness. A total of 4patients experienced sequelae
in the form of ocular movement disorders and 2patients
succumbed to the disease.
Discussion
MG is an acquired autoimmune neuromuscular transmission
disorder. The pathophysiology involves the production of
AchR-Ab and the inhibition of AchR, reducing the number of
functional AchR in the neuromuscular junction, which leads
to an abnormal decline in the motor endplate membrane
potential. Studies conducted in Europe and the United
States have reported a prevalence of 141.5/1.0x105, with
an uncommon MG onset during childhood (1015%)(10).
However, a recent study conducted in China indicated that
the onset of MG in children is not rare in China(11), with
similar rates to those in Japan(12), indicating that pediatric
MG is relatively common in Asia. The results of the present
study are consistent with those available in the literature
and demonstrated a relatively early age at onset (50.3% of
the patients were aged <3years), with a marginally higher
rate among females (56.3%), common relapse (50.9%) and a
higher relapse rate within the first 2years. A higher number
of children with MG in European countries progress to GMG
within 2years. Research in Japan has indicated that 75% of
patients with OMG had recessive GMG(13), i.e., no clinical
symptoms were present, but EMG detected repetitive nerve
stimulation frequency attenuation in other nerves in addition
to the facial nerve in patients who were diagnosed with latent
GMG. Our patients rarely progressed to GMG and EMG did
not suggest an attenuation phenomenon in other neurological

EXPERIMENTAL AND THERAPEUTIC MEDICINE 9: 1363-1368, 2015

examinations of children with OMG (rare recessive GMG).


However, detailed history, increased number of fatigue tests
and EMG sites and long-term follow-up of the patients should
be performed in order to increase the accuracy of typing.
Spalek etal(14) investigated 768adult MG patients and
found that 112patients (14.15%) also suffered from other autoimmune diseases. In the present study, one 12year-old female
patient experienced repeated recurrence of bulging eyes over
the first 3years following treatment. The initial examination for
AchR-Ab was negative, but was positive at recurrence; testing
for antithyroid antibodies was also positive. Graves' disease is
reportedly rare in patients with MG (0.2%), whereas 510%
of patients with MG have autoimmune thyroid diseases(15).
Antithyroid antibodies are more common in OMG and reportedly more common in Asian patients(16). In the patients in the
present study, concurrent Graves' ophthalmopathy was not as
common. It is difficult to determine when proptosis and ptosis
appear, but serum antibody tests may help with the diagnosis,
particularly in children with atypical clinical manifestations
and no definitive diagnosis. Therefore, routine testing for antithyroid antibodies and thyroid function should be performed
and other autoimmune diseases should be considered.
Of the patients who were initially AchRAbnegative, 53%
became positive during treatment or following relapse, which
may be a reaction during a different period of the same disease
or may be due to differences in individual immunity and
associated with the level of antibody titers and differences in
secretion in various tissues and organs(17). Dynamic follow-up
may increase the antibody positivity rate in children with MG;
however, as a causative factor, AchRAb was long-standing in
certain patients, indicating that long-term treatment may lower
the risk of recurrence.
It was previously demonstrated that, in addition to being
AchR-Ab mediated, MG pathogenesis also involves other
antibodies or molecules, such as Titin-Ab, which were recently
found to be highly relevant with thymomarelated MG (MGT).
The Titin-Ab positivity rate in MGT was as high as 8090%,
which is of high diagnostic value for MGT. The level of serum
Titin-Ab was also found to be significantly correlated with
MGT severity and AchR-Ab levels. The detected positivity
rate of Titin-Ab in the present group was 40% and the positive
patients were also positive for AchR-Ab, although the imaging
studies revealed no evidence of thymoma. However, follow-up
of the Titin-Ab-positive patients was necessary, particularly
for diagnosis by enhanced CT. Foreign studies have reported
that positive RyR-Ab is associated with GMG, particularly
with severe oropharyngeal muscle weakness that often occurs
during myasthenic crises(12); however, no such correlation
was found in the 3positive patients who were followed up. In
this group, 71% of patients had abnormal CD series, with the
majority displaying decreased CD4+ and CD8+, indicating that
immune cells also play a role in the pathogenesis of MG.
MG is usually mitigated in the morning and worsens in
the evening and patients may experience incomplete paralysis
in the later stages. Two patients with upper eyelid, limb and
bulbar muscle involvement experienced sustained myasthenia.
Fatigue and drug testing was negative and the patients were
diagnosed with brain stem encephalitis. Other patients were
misdiagnosed with oculomotor nerve palsy, muscular dystrophy,
Guillain-Barr syndrome, mitochondrial diseases, cardiomyop-

1367

athy and other symptoms. These patients were all diagnosed by


positive AchR-Ab and EMG and recovered during the follow-up
after treatment; therefore, several differential diagnoses should
be considered in the clinical setting. Early onset should also be
distinguished from congenital myasthenic syndrome. Another
9patients were had negative neostigmine test results, but the
AchR-Ab were present or the results of the EMG were positive,
and steroid treatment was effective. Therefore, acquired autoimmune MG should also be considered.
Although the pathogenesis of MG is clear, the currently
available treatments are not immune or antigenspecific. The
international and domestic treatment standards are currently
not uniform, including hormone measurements, course of treatment and selection of second-line drugs. Acetylcholinesterase
inhibitors were suitable for all MG patients, except those undergoing cholinergic crisis, and may significantly reduce muscle
weakness and impede AchR repair; therefore, they should not
be used as a single agent in the long term. Many advocate the
use of steroid therapy in the early stages(18) to prevent transiently increased weakness. Corticosteroids, which are the most
commonly used first-line immunomodulatory therapy for MG,
exert multiple effects on Tcell, Bcell and macrophage function. Kupersmith(18) reported that prednisone may delay and
reduce the incidence of GMG after >4years of follow-up and
effectively control recurrence.
The following advantages of methylprednisolone pulse
therapy were observed: i)satisfactory effect in the short term,
with rapidly improving symptoms; ii)ability to reduce or discontinue cholinesterase inhibitor use in the short term may simplify
treatment; iii) relatively few adverse reactions, effectively
controlled during hospitalization; and iv)ability to return to
normal routine as soon as possible and continue with outpatient
observation and treatment. However, a retrospective analysis
regarding MG crisis indicated that it may be associated with
inappropriate methylprednisolone pulse therapy(19). Our data
demonstrated that the average time to onset and duration of the
maximum effect in the steroid pulse group was superior to the
transitional reduction of the oral prednisone group and the pyridinium bromide neostigmine in combination with reduction of
prednisone group. Except for the two MG patients who received
steroids alone, the remaining children did not experience MG
exacerbation or crisis. Steroid treatment of short duration resulted
in early relapse; a treatment duration of2years was considered
more appropriate. In addition to Cushing's facies, weight gain
during the early stages of medication and high blood pressure
occurred in 1patient, but there were no severe adverse drug
reactions in the majority of the patients who were administered
steroid therapy. However, regular referrals to ophthalmology,
blood glucose monitoring, blood pressure monitoring, a low-salt
diet, use of cod liver oil supplements and monitoring of calcium
and bone density are recommended. If the illness recurs during
treatment, the steroid dose may be restored to pre-reduction
levels; alternatively, cholinesterase inhibitors, IVIG, or other
immunosuppressive agents may be added in the short-term if
the manifestations are severe.
Azathioprine, which is the most common immunosuppressant used long-term to treat MG in addition to corticosteroids,
was proven to be effective through randomized controlled trials.
CyclosporineA is the only treatment confirmed to be effective in
improving clinical symptoms and decreasing anti-AchR serum

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YANG et al: CHILDHOOD MYASTHENIA GRAVIS

levels compared to a control group in a randomized, doubleblind, controlled trial. In the Children's Hospital of Zhengzhou,
cyclosporineA treatment was used for 1.5years in children with
repeated relapses with steroid-resistant or dependent disease,
on the basis of observed severe side effects with azathioprine,
reports on the effectiveness of cyclosporineA treatment(20)
in addition to our own experience with cyclosporineA and the
lack of severe adverse events with cyclosporineA treatment.
Other clinically applied drugs and treatment methods, such as
cyclophosphamide, mycophenolate mofetil, tacrolimus, plasmapheresis and thymectomy were also used. Recently, rituximab
and antigenspecific plasma exchange were also used, but more
experience is required. Thymectomy in pediatrics should be
cautiously performed. In addition, the use of drugs that aggravate muscle weakness, such as aminoglycoside and macrolide
antibiotics, should be avoided; MG exacerbation may occur
during surgery(21). When the non-ionic contrast iohexol is used
in enhanced CT, no aggravated weakness is observed.
In summary, MG in children is more common in China.
OGM is common and there are fewer patients who progressed
to GMG, fewer patients with concurrent hyperthyroidism and
a low incidence of thymoma. The overall prognosis is good,
with fewer neurological sequelae, although visual impairment
should also be considered. The significance of integrated
management, long-term follow-up, standardized treatment,
education for children and parents and psychological support
should be emphasized.
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