Neuropediatrics 2012; 43 - WS27_02
DOI: 10.1055/s-0032-1307180

Myasthenia gravis in young children and adolescents: clinical signs and treatment options

A Della Marina 1, H Trippe 1, S Lutz 1, G Shamdeen 2, U Schara 1
  • 1Pädiatrische Neurologie, Universitätskinderklinik Essen, Essen, Germany
  • 2Klinik für Kinder- und Jugenheilkunde, Bad Hersfeld, Germany

Introduction:

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against the postsynaptic membrane of the neuromuscular junction. Clinical signs in young children and adolescents range from isolated ocular symptoms to general muscular weakness and respiratory insufficiency.

Methods: Since 2008 nine patients (7w/2m) diagnosed with childhood and JMG have been treated in our clinic. At the time of diagnosis 8/9 patients were pre-pubertal, and one patient was peri-pubertal.

Results: Four of 9 patients showed first symptoms at the age between 18 months to 5 years, in two of them they were manifested by an isolated ocular form (the most frequent presentation), and one patient showed an atypical generalised form of muscular weakness. One patient with primary ocular symptoms developed, after a symptom-free interval, a generalised muscle weakness.

Five of 9 patients manifest at the age between 7 to 11 years the generalised muscular weakness and bulbar symptoms. One patient from this group with a delayed diagnosis showed pronounced muscle weakness leading to myasthenic crisis.

Antibodies to the nicotinic acetylcholine receptor (AChR) were found in 8/9 patients and all patients responded to pyridostigmine. Immunosuppression included corticosteroid therapy in 8/9 and azathioprine in 5/8 patients, one patient received additionally mycophenolate mofetil and plasmapheresis. 4/8 had to undergo thymectomy which subsequently led to an improvement in their symptoms.

Conclusion: MG in young children and adolescents shows distinct features of clinical signs when compared to adults. Young children may show general muscular weakness already during the second year of life, and in this group antibodies to AChR can only be slightly increased. Because of existing therapeutic options, an early diagnosis is important.

Until now, there has been no evidence-based data for children and adolescents with childhood and JMG and the diagnosis and management should urgently be made by taking into account specificities of this age group.

juvenile myasthenia gravis - clinical symptoms - therapy