Patients with alternating hemiplegia can suffer from painful repetitive muscle contractions that last hours. Researchers may now have found a simple treatment option for this symptom.
Alternating hemiplegia is a rare neurological disorder that affects both sides of the body, but in an alternating pattern. It usually appears in early childhood and can significantly impair the quality of life. The symptoms can manifest in episodes of paralysis that can last from minutes to days, involving one or alternating sides of the body. The repetitive muscle contractions called dystonia can cause excruciating pain and last for hours. The disorder is associated with a mutation in the ATP1A3 gene.
There is currently no cure for this condition and treatment is mainly focused on managing symptoms. This may include physical therapy or speech therapy. Other forms of supportive care may also be helpful in managing symptoms. Pharmacological treatment is usually ineffective, leaving physicians with no choice but to turn to sedatives for relief. However, a recent breakthrough has been discovered in the form of oxygen therapy, which has been shown to mitigate the debilitating symptoms of dystonia attacks.
“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 – nitrous oxide – is usually administered with 50 % oxygen. As it is impossible to give nitrous oxide in daily treatment, we wanted to test whether oxygen was responsible for stopping the attack.”
Further research showed that oxygen therapy delivered during an attack could stop the dystonic episodes within 15 minutes, improving the quality of life for those suffering from the disorder.
The researchers hypothesized that cortical spreading depression (CSD), a wave of electrical disturbance in the brain associated with a rapid phase of neuronal hyperactivity and a slower phase of electrical silence, might be responsible for the symptoms of alternating hemiplegia. They suggested that this wave is linked to hypoxia or reduced oxygen availability and that rapid administration of oxygen during an attack could interrupt or mitigate this phenomenon.
While promising, further research is needed to determine how to optimize oxygen therapy for home use and to understand how it could benefit other patients under similar conditions. Nonetheless, this discovery has the potential to significantly reduce the use of potent sedatives, making a world of difference for those suffering from this perplexing disorder.
Image source: Jeremy Bishop, Unsplash
