Alternate hemiplegia
- Article
- 2021-03-14
Alternating hemiplegia is a rare neurological condition that develops in childhood, usually before the child is 18 months old. The condition is characterized by recurrent episodes of paralysis involving one or both sides of the body, multiple limbs, or a single limb. The paralysis can affect different parts of the body at different times and can be short-lived or last for several days. Often these episodes disappear after sleeping. Affected children may also have abnormal movements with stiffening or "dance-like" movements of a limb, as well as walking and balance problems. Some children have seizures. Children can have normal or delayed development. There are both benign and more serious forms of the condition. Alternating hemiplegia is mainly caused by mutations in the ATP1A3 gene. Occasionally, a mutation in the ATP1A2 gene has been implicated in the condition. These genes provide instructions for making very similar proteins. Mutations in these genes reduce the activity of the enzyme Na + / K + ATPase, which affects the signals that control muscle movement. However, it is not yet clear how the reduced enzyme activity leads to the symptoms of the condition.
Therapy
Drug therapy, including verapamil, can help reduce the severity and duration of paralysis attacks associated with the more severe form of alternating hemiplegia.
Prognosis
Children with the benign form of alternating hemiplegia have a good prognosis. Those who experience the more severe form have a poor prognosis because intellectual and mental capacities do not respond to drug treatment and balance and gait problems persist. Over time, walking without assistance becomes difficult or impossible.
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