Aicardi Syndrome

Aicardi syndrome is a rare disorder. It affects only females. Its onset generally begins between the ages of 3 and 5 months. It is an inherited (genetic) disorder.

SYMPTOMS

Symptoms include:

  • Partial or complete absence (agenesis) of the corpus callosum. This is the structure that links the 2 hemispheres of the brain.

  • Infantile spasms (a form of childhood seizure).

  • Mental retardation.

  • Lesions (lacunae) of the retina of the eye. This is an eye abnormality.

  • Aicardi syndrome may be associated with other brain defects, such as:

  • Small brain (microcephaly).

  • Fluid-filled cavities or gaps in the brain (porencephalic cysts).

TREATMENT

There is no cure for Aicardi syndrome nor is there a standard course of treatment. Treatment is symptomatic. It generally involves:

  • Management of convulsions (seizures).

  • Intervention programs for mental retardation.

PROGNOSIS

The outcome for individuals with Aicardi syndrome varies according to the presence and severity of symptoms.