Immunosuppressive therapy should be maintained throughout pregnancy, as this reduces the chance of neonatal muscle weakness, and controls the mothers myasthenia.About 10–20% of infants with mothers affected by the condition are born with transient neonatal myasthenia (TNM), which generally produces feeding and respiratory difficulties that develop about 12 hours to several days after birth. A child with TNM typically responds very well to acetylcholinesterase inhibitors, and the condition generally resolves over a period of three weeks, as the antibodies diminish, and generally does not result in any complications. Very rarely, an infant can be born with arthrogryposis multiplex congenita, secondary to profound intrauterine weakness. This is due to maternal antibodies that target an infants acetylcholine receptors. In some cases, the mother remains asymptomatic. Diagnosis
MG can be difficult to diagnose, as the symptoms can be subtle and hard to distinguish from both normal variants and other neurological disorders.Three types of myasthenic symptoms in children can be distinguished:
Transient neonatal myasthenia occurs in 10 to 15% of babies born to mothers afflicted with the disorder, and disappears after a few weeks. Congenital myasthenia, the rarest form, occurs when genes are present from both parents. Juvenile myasthenia gravis is most common in females.Congenital myasthenias cause muscle weakness and fatigability similar to those of MG. The signs of congenital myasthenia usually are present in the first years of childhood, although they may not be recognized until adulthood. Classification
When diagnosed with MG, a person is assessed for his or her neurological status and the level of illness is established.
Pneumocephalus is the presence of air or gas within the cranial cavity. It is usually associated with disruption of the skull: after head and facial trauma, tumors of the skull base, after neurosurgery or otorhinolaryngology, and rarely, spontaneously. Pneumocephalus can occur in scuba diving, but is very rare in this context. If there is a valve mechanism which allows air to enter the skull but prevents it from escaping, a tension pneumocephalus can occur (similar to what can happen in a tension pneumothorax). CT scans of patients with a tension pneumocephalus typically show air that compresses the frontal lobes of the brain, which results in a tented appearance of the brain in the skull known as the Mount Fuji sign. The name is derived from the resemblance of the brain to Mount Fuji in Japan, a volcano known for its symmetrical cone. In typical cases, there is a symmetrical depression near the midline (such as the crater of a volcano), due to intact bridging veins. Its occurrence seems to be limited to tension pneumocephalus (not occurring in pneumocephalus without tension). The sign was first described by a team of Japanese neurosurgeons.Pneumocephalus has also been shown to follow neurosurgical procedures such as deep brain stimulation and hematoma evacuation (e.g., chronic subdural hematoma), where while seemingly innocuous to the patient, may cause brain shift, subsequent stereotactic inaccuracy, and even another surgical intervention.