Alternating hemiplegia

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Alternating hemiplegia is a rare neurological disorder.

Onset

It develops in childhood, most often before the child is 18 months old.

Clinical features

  • The disorder is characterized by recurrent episodes of paralysis that involve one or both sides of the body, multiple limbs, or a single limb.
  • The paralysis may affect different parts of the body at different times and may be brief or last for several days.

Oftentimes these episodes will resolve after sleep.

Abnormal movements

Affected children may also have abnormal movements involving stiffening or "dance-like" movements of a limb, as well as walking and balance problems.

Seizures

Some children have seizures. Children may have normal or delayed development.

Benign and serious forms

There are both benign and more serious forms of the disorder.

Cause

  • Alternating hemiplegia is primarily caused by mutations in the ATP1A3 gene.
  • Occasionally, a mutation in the ATP1A2 gene is involved in the condition.

Prognosis

Children that have a milder form have relative good prognosis while those with a more severe form have bad prognosis.

Treatment

Verapamil has been used to help reduce severity of the disease.

Sources and references


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Contributors: Prab R. Tumpati, MD