Oxygen Therapy: A New Promising Treatment for a Rare Childhood Disease

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Alternating Hemiplegia is a debilitating neurodevelopmental disorder that stems from a mutation in the ATP1A3 gene. This condition affects children at an early age and has a major impact on their quality of life. During episodes of paralysis, which can range from a few minutes to several days, one o

r alternating sides of the body may become paralyzed. Additionally, patients may experience repetitive muscle contractions, known as dystonia, which can last for hours and cause significant pain.

“We don’t know how to help these patients. Therefore, we remain particularly attentive to the sometimes-unexpected factors that could relieve them,” explains Emmanuel Flamand-Roze, neurologist and expert in abnormal movements. “Recently, a 25-year-old man told us that after receiving laughing gas during an episode of dystonia, his symptoms subsided within minutes. We tried to identify the reason, knowing that this gas –– is usually administered with 50% oxygen.

Team member Quentin Welniarz proposed a hypothesis to explain the effect of oxygen. According to him, a phenomenon called cortical spreading depression , which corresponds to a wave of electrical disturbance in the brain, plays an essential role in triggering the symptoms of alternating hemiplegia. This wave consists of a rapid phase of neuronal hyperactivity and a slower phase of electrical silence in the cortex cells. It’s also associated with hypoxia – i.e., reduced oxygen availability.

 

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