چكيده لاتين :
Objective
This stud y was undertaken to evalu ate the clini cal spectrum of myasthenia
gravis in children and determine factors th at help the clini cian in his/her
diagnosis and management.
Materials & Methods
A retrospective review was performed on all pedi atri c patients suffering from
myasthenia gravis (M .G) admitted in the department of pedia tri c neurology
of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and
2002 .
Results
Of the thirty-two children wi th M.G. enrolled in our study, seven were suffering
from the congenital type while the remaining (25 cases) had the juvenile M.G.
Initial symptoms of congenital M.G were ptosis (7/7), limitation of eye movement
(2/7) and mild generalized weakness (6/ 7). Althou gh the Tensilon test was
positive in 85% of congenital M. G cases, no myasthenia crisis or spontan eous
remi ssion was observed in any of them. In chi ldren with juvenile M. G, the age
of presentation was 1.2 to 12.5 years, mean age 5.7 ±4.2 years (1 5 girls and
10 boys). The most common presenting symptoms in juvenile group were
ptosis in 96% and generalized weakness in 76%. Eight of them (32%) had had
at least one myasthenia crisis. EMG was di agnostic in 83% and one case the
tensilon test was positive in 84%. One patient had hyperth yroidism and had
already been di agnosed with hypothyroidism; two of them were epi lept ics.
Eight patients underwent thym ectomy microscopi cally; in specimens examined,
five (62%) showed thymic folli cular hyperpla sia wh ile in remaining three results
were normal. O ne pati ent (1 2.5%) recovered completely aft er thymectomy
with no need for medi cation during the follow up. Four patients (50%) showed
relative improvem ent and in three cases (37%) improvem ent was negligibl e.
Conclusion
The results showed a fem ale to male ratio of 1.5/1 which was co rrelated to
adult M.G. The most common presenting symptoms consisted of ophtalmoplegia,
with bil ateral pto sis being the most significant . Althou gh thi s study revealed
that thymectomy lacks any remarkable prognostic influence, all patients had
thymectomy after two years of disease onset. Some reports have indicated
positive results if surgery was performed within two years of onset of disease.
