Abstract
Myasthenia gravis refers to a group of diseases affecting the neuromuscular junction
resulting in muscle weakness. The clinical hallmark of this condition is skeletal
muscle weakness that worsens with sustained or repetitive muscle activation, caused
via disruption of neuromuscular transmission by antibodies directed most commonly
against the nicotinic acetylcholine receptor. Transient neonatal myasthenia gravis
is caused by the crossing of antibodies from a myasthenic mother to her developing
child, while juvenile myasthenia gravis involves the production of antibodies by the
child's own immune system against neuromuscular junction components. Ptosis and extraocular
muscle weakness are common presenting symptoms of juvenile myasthenia gravis and may
be accompanied by, or progress to, generalized weakness. Diagnosis of myasthenia gravis
relies on careful neurological examination, supported by additional diagnostic modalities
which may include the patient's response to short-acting cholinesterase inhibitors,
serum testing for causative antibodies, or electromyography. Management options include
cholinesterase inhibitors for symptomatic treatment, immunosuppression with corticosteroids
or steroid-sparing immunosuppressants, and thymectomy when a thymoma is identified
on imaging.
Keywords
myasthenia gravis - children - ocular - diagnosis - treatment
