What Is An Aicardi-Goutières Syndrome?

March 02 22:12 2019 Print This Article

It is defined as a disorder in which there is an involvement of the genetics. It is also referred as the cree encephalitis along with the pseudo torch syndrome. They were considered to be separate identities. This is a type of leuko dystrophy and is quite lethal within the first few years. It is autosomal recessive in the nature and occurs within the first few weeks of life.

What are the causes of Aicardi-Goutières syndrome?

It includes the mapping of this syndrome with the chromosome 3p 21. It suggested that the two along with a false TORCH which is referred as the toxoplasmosis, rubella, cyto megalo virus along with the different types of herpes simplex viruses. This condition has been described along with a TREX 1. As the mutations in the gene are seen hardly and are autosomal recessive in the nature. The cases are left alone as consan guineous families. It is also known as the auto zygosity disease.

What is the history of Aicardi-Goutières syndrome?

It includes the description by J Aicardi along with the F Goutieres. It was described in the year 1984. It was based on the early cases of early onset increase in the familial and along with the calcification. It occurs along with the basal ganglia. One also observes a lymphocytosis in case of chronic cerebero spinal fluid. There is a familial encephalopathy also. There were clinical studies also which included the different cases of early onset of encephalopathy. It occurred in a cree community in Canada. It was described in the year 1988. It was given the name as Cree encephalitis.

What are the signs and symptoms of Aicardi-Goutières syndrom?

It includes the lymphocytosis in case of chronic cerebero spinal fluid. There is a familial encephalopathy also.

What is the treatment of Aicardi-Goutières syndrome?

It includes the supportive as well as the management of seizures. It is seen along with the spasticity.

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