ABSTRACT
BACKGROUND: Trihexyphenidyl and clonazepam are commonly used to treat dystonia in children with cerebral palsy (CP). However, there is a notable gap in the literature when it comes to studies that combine these first-line agents for the management of dystonia. METHODS: This open-label, randomized controlled trial aimed to compare the efficacy of adding oral clonazepam to trihexyphenidyl (THP + CLZ) versus using trihexyphenidyl alone (THP) in reducing the severity of dystonia, as measured by the Barry-Albright Dystonia (BAD) score. The study was conducted over a 12-week therapy period in children with dystonic CP aged two to 14 years. RESULTS: Each group enrolled 51 participants. The THP + CLZ group showed significantly better improvement in dystonia severity at 12 weeks compared with the THP group alone (-4.5 ± 2.9 vs -3.4 ± 1.7, P = 0.02). Furthermore, the THP + CLZ group exhibited superior improvement in the severity of choreoathetosis, upper limb function, pain perception by the child, and quality of life, with P values of 0.02, 0.009, 0.01, and 0.01, respectively. The number of participants experiencing treatment-emergent adverse events was comparable in both groups (P = 0.67). Importantly, none of the participants in any of the groups reported any serious adverse events. CONCLUSION: A combination of oral THP + CLZ proves to be more efficacious than using THP alone for the treatment of dystonic CP in children aged two to 14 years in terms of reducing the severity of dystonia.
ABSTRACT
Congenital rubella syndrome (CRS) usually occurs in a developing fetus of a pregnant woman who has contracted rubella, usually in the first trimester. With universal rubella vaccination, the incidence of CRS in developed countries has drastically reduced. However, in developing countries, CRS continues to be a public health menace in children. Sensorineural deafness, cataract, micropthalamia, patent ductus arteriosus and pulmonary stenosis are common clinical manifestations in a child affected with CRS. Other relatively rare manifestations are hepatosplenomegaly, thrombocytopenia and blueberry muffin spots. Although it is well known that CRS predisposes towards development of diabetes mellitus usually in adults, but in children only anecdotal case reports are available. Latent viral infection, immune mediated mechanism and HLA allele predisposition are various possible pathogenic mechanisms. Majority of affected persons require insulin therapy. Hereby we are demonstrating a case of seven year old boy with CRS, who presented with diabetic ketoacidosis and was successfully managed with insulin therapy. This report intends to aware clinicians regarding predisposition of children with CRS for developing diabetes, as timely diagnosing and instituting insulin therapy will help in avoiding complications and improving their quality of life.
