What is Xiborin?

  Xiponin, an irreversible GABA transaminase inhibitor that increases GABA levels in the brain and cerebrospinal fluid, has been used in Europe since the 1990s to treat infantile spasms [145] with better results than ACTH. in 2009, the FDA approved the drug for use in infantile spasms in the United States, and it is now listed as the In 2009, the FDA approved the drug for use in the United States for infantile spasms, and it is now listed as a first-line pharmacological treatment option for infantile spasms in both Europe and North America [143, 146, 147], and may also be effective for drug-refractory complex partial seizures in TSC patients.  There is no standardized dose or duration of application of Xiponin, but the dose reported in the literature is 18C200 mg/kg/day [25], and the starting dose recommended in the American TSC consensus [148-150] is 50 mg/kg/day, which is gradually increased to 100C150 mg/kg/day depending on the child’s response to the drug. The side effects of this drug include drowsiness, decreased muscle tone, weight gain, and insomnia, but its biggest drawback is its effect on visual field, where patients may develop irreversible centripetal visual field defects [143, 146, 147, 151-153]. The exact mechanism of this side effect is unknown and may be due to a decrease in the function of the optic cone cells and anaplastic cells. This side effect is more common in adult patients than in pediatric patients, with an incidence of approximately 25%C50 % in adult patients, approximately 15% in pediatric patients, and approximately 15%C31 % in infants and children [154]. The time from the start of treatment to remission of epilepsy is approximately 12-35 days, during which the probability of visual field disturbances is relatively low, and it is currently considered that if there is no significant effect after more than 12 weeks of Xiponin application, discontinuation of the drug can be considered; it is currently recommended that if TSC patients are taking the drug, periodic dynamic visual field examinations (kinetic perimetry) by an ophthalmologist are recommended to possible visual field defects in early stage patients. Electroretinography has also been recommended for routine testing, but this technique is not yet well established. In patients with TSC, the consequences of poorly controlled epilepsy are even more severe in terms of cognitive decline, so many physicians now view the side effects of Ciprofloxacin’s visual field narrowing as an unavoidable regret, otherwise the consequences would be even more severe. Because of the possibility of visual field narrowing with this drug, it has been suggested that if the child has been seizure-free for more than 6 months, the drug may be considered discontinued, but there is a possibility of seizure reoccurrence after discontinuation of Xiporin.