Sometimes children, usually under the age of 18 months, experience paralysis of one half of the body, sometimes even on both sides. This can be explained by what is called alternating hemiplegia of the child (AHT). If until now we only knew one gene that explained this pathology, we now know that another gene may be involved, underlines Inserm in a press release.
“Alternating hemiplegia of childhood (HAE) is a very rare neurodevelopmental disease characterized by periodic hemiplegia, persistent developmental delay and cognitive deficit”, defines Inserm. It is a rare childhood disease it affects less than one in 100,000 children per year. It is usually declared via a paralysis that can last a few minutes or last for several days and which follow events that may have caused stress, for example, but this can also be triggered by swimming, a variation in brightness, physical activity, etc.
A protective brain barrier no longer works
“Scientists from Dublin and Paris have identified the CLDN5 gene, as being responsible for alternating hemiplegia in children in two unrelated cases of HAE in France. The protein produced by this gene, claudin-5, is essential for maintaining the integrity of the blood-brain barrier that isolates and protects the brain from the rest of the body’s functioning.
In other words, an element that serves to isolate the brain so that it is not attacked by external events no longer works. Rather than being a “barrier”, it becomes a channel. Fact, the brain overreacts to everyday stimuli. This is an essential discovery to understand the functioning of this pathology.
Source: Inserm
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