VICI SYNDROME

Summarized by Plex Health
Last Updated: 03 May 2022

Vici syndrome is a severe disorder that starts early in life and influences many body systems. A characteristic feature of Vici syndrome is a brain irregularity called agenesis of the corpus callosum, in which the tissue that connects the left and right fifty percents of the brain falls short to create normally throughout the onset of growth before birth. Other brain problems can occur in Vici syndrome, consisting of underdevelopment of an area of the brain recognized as the pons and reduced myelin, which is a fatty substance that secures and covers nerve cells. Along with problems with brain advancement, malfunction of brain tissue may occur gradually, leading to an abnormally small head dimension. The brain troubles contribute to profound developing hold-up in people with Vici syndrome. A potentially life-threatening heart disease called cardiomyopathy prevails in children with Vici syndrome. Some affected children additionally have heart problems that exist from birth. Other key features of Vici syndrome include skin and hair that are lighter in color than that of family members and other individuals with the same ethnic history, and clouding of the lenses of the eyes or other eye abnormalities, which may lower vision. Because of the intensity of the problem, most individuals with Vici syndrome do not survive past age 5.

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