Aicardi Syndrome Follow-up

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

Complications

See the list below:

  • Early sudden death is typical in severely affected individuals and is usually caused by pulmonary complications such as pneumonia.

  • Seizures or their treatments (eg, adrenocorticotropic hormone [ACTH]) may also be associated with increased morbidity (eg, hypertension, diabetes, infections) and death.

  • The aforementioned malignancies may develop, and choroid plexus papillomas may cause obstructive hydrocephalus.

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Prognosis

See the list below:

  • Prognosis is uniformly poor, with most children unable to walk or communicate; those who can are not high-functioning and require continuous care for their needs.

  • Survival rates have been improving, with an estimated survival of 62% at age 27 years.

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Patient Education

For patient education resources, see the Brain and Nervous System Center and Children's Health Center, as well as Epilepsy and Seizures in Children.

Patients may also be referred to the Aicardi Syndrome Foundation.

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