Aicardi Syndrome Treatment & Management

Updated: Nov 30, 2018
  • Author: Ravi Sunderkrishnan, MD; Chief Editor: Maria Descartes, MD  more...
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Treatment

Medical Care

There is no cure for Aicardi syndrome. Management of the different symptoms and manifestations is indicated in affected patients.

Seizures are treated with multiple antiepileptic medications; their effectiveness varies with each patient. According to one survey, the most effective treatment for seizures were ketogenic diet, vigabatran, lamotrigine, and topiramate.

Spasticity can result in contractures or limited range of motion that affects not only mobility but also hygiene care. Patients may benefit from specialized care with physical medicine and rehabilitation specialists as well as physical, occupational, and speech therapists.

Ongoing gastrointestinal issues and constipation can be managed under the care of a pediatrician or pediatric gastroenterologist.

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Surgical Care

Palliative epilepsy surgery in the form of corpus callosectomy of a partial corpus callosum and the use of vagal nerve stimulation have been reported to be of variable benefit. [20]

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Consultations

Consultation with a pediatric ophthalmologist and neurologist is generally required. As noted earlier, patients also benefit from physical and occupational rehabilitation and speech therapy.

Consultation with an orthopedic, pulmonary, or gastroenterologic specialist may be required if complications arise from scoliosis, pulmonary function, or feeding or aspiration difficulties.

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Diet

Use of the ketogenic diet has been tried, to some effect, to control seizures associated with this condition, specifically infantile spasms.

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