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Aicardi syndrome



Aicardi syndrome 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.


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.


Aicardi syndrome 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-Gouti?res which is a distinct disorder should not be confused with Aicardi syndrome,


Aicardi syndrome 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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