Clinica Miastenia
Clinica Miastenia
Clinica Miastenia
1364
1365
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
1366
Total no.
Ocular typea
Pyridostigmine group
Steroid group
General typeb
Pyridostigmine group
Steroid group
Course of treatment (years)
<1
12
>2
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
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
1367
1368
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.
References
1. Zouvelou V, Rentzos M, Toulas P and Evdokimidis I:
AchRpositive myasthenia gravis with MRI evidence of early
muscle atrophy. J Clin Neurosci19: 918919, 2012.
2. McGrogan A, Sneddon S and de Vries CS: The incidence
of myasthenia gravis: a systematic literature review.
Neuroepidemiology34: 171183, 2010.
3. Giraud M, Vandiedonck C and Garchon HJ: Genetic
factors in autoim mune myasthenia gravis. Ann N Y
AcadSci1132:180192,2008.
4. CavalcanteP, BarberisM, CannoneM, etal: Detection of
poliovirusinfected macrophages in thymus of patients with
myasthenia gravis. Neurology74: 11181126, 2010.
5. Stefansson K, Dieperink ME, Richman DP and Marton LS:
Sharing of epitopes by bacteria and the nicotinic acetylcholine
receptor: a possible role in the pathogenesis of myasthenia gravis.
Ann NY Acad Sci505: 451460, 1987.