Myasthenia in children: about 5 cases observed in Abidjan, Ivory Coast

Abstract

Background:
Myasthenia is a rare autoimmune disease in children that is poorly described in African literature.
Method:
We reported 5 cases of myasthenia gravis in children aged from 0 to 15 years observed in a 3 years period (October 2015 to December 2018) at outpatient’s child neurology consultation and ophthalmology in Abidjan, Ivory Coast. the diagnosis of myasthenia gravis was made on the basis of clinical, electroneuromyographic and immunological signs (anti-achR antibodies).
Results:
The mean age of patients was 5.8 years. The sex ratio was 1.5. Ptosis was the common presentation in all cases. The ice pack test and anti-AChR antibodies were performed in 3 patients. ENMG showed a significant decrement of more than 10% in all patients. Only one patient had thymic involvement. All patients received anticholinesterase drugs. As adjunctive treatment, 2 patients received steroids and one patient received azathioprine. There was an improvement in 40% (2/5) of the patients, a deterioration in 20% (1/5) and death occurred in 2 patients (40%). The average time to diagnosis was 13 months.
Conclusion:
This study shows a delay and wandering in the diagnosis. We recommend that myasthenia gravis should be suspected in the presence of any single or bilateral, asymmetrical and / or fluctuating ptosis in children. Moreover, whenever possible, an ice pack and pharmacological tests should be performed, and anti-achR dosage levels as well.