Aicardi syndrome

Below you will find more information about Aicardi syndrome from Medigest. If you believe that you are suffering from any of the symptoms of Aicardi syndrome it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Aicardi syndrome and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Aicardi syndrome comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Aicardi syndrome is an unusual abnormality syndrome distinguished by the lack of a major structure in the brain referred to as corpus callosum, the existence of abnormalities in the retina adn seizures in the form of immature spasms. An X-linked dominant trait is inherited in Aicardi syndrome that is fatal in males.

Historical Background

In 1965, Jean Aicardi who is a French neurologist initially recognized the disorder as a distinctive syndrome. The explanation of the syndrome is written in the review article of Dr. Aicardi, the article entitles as Aicardi J, Aicardi syndrome: old and new findings, Int Pediatr. 1998;14(1):5-8. Aicardi-GoutiΦres which is a distinct disorder should not be confused with Aicardi syndrome,

Epidemiology

About 500 cases of Aicardi syndrome have accounted for worldwide particularly in Japan. Apart from that the syndrome is very usual, its accurate frequency in the population is around 1,000 which started in 1825.

Prognosis

Depending on the case of the patient the prognosis varies. Although, all individuals accounted with Aicardi syndrome to date have suffered developmental interruption of a considerable degree usually results in modest to intense mental retardation. The age spectrum of the individuals accounted with Aicardi syndrome starts from birth to the mid 40's.

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