Miastenia dziecięca

Barbara Ryniewicz

Streszczenie
Myasthenia gravis is an acquired autoimmunological disease, with fluctuating weakness and fatigability of skeletal muscles after exertion. Myasthenic symptoms are caused by disturbances of neuro-muscular transmission as a result of acting of anti-AchR antibodies in neuro-muscular junction.
We observed different types of myasthenia in childhood, such as transient neonatal myasthenia, familial myasthenia, pure ocular myasthenia and generalized juvenile myasthenia. Transient neonatal myasthenia is due to placental transfer of anti AchR antibodies from mother to the child, the symptoms last to 40 days. In our material it was present in 16% of neonates born from the myasthenic mothers. In 3% of patients there is more than one member of family affected.
10-16% of patients with generalized myasthenia have the onset during childhood.
In generalized myasthenia extraocular (ptosis, diplopia), bulbar (dysartria, dysphagia) and extremity muscles are involved.
In diagnosis of myasthenia pharmacological test with Tensilon or Neostigmine and electrophysiological study (repetitive nerve stimulation, SFEMG) should be done.
In treatment of myasthenia we use cholinesterase inhibitors, thymectomy and immunosuppressive drugs. In our material of 146 children after thymectomy, we observed improvement in 88% and full remission in 41%.

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