Pediatric extensor truncal dystonia treatment with botulinum toxin

Introduction/Background Axial dystonia resulting in opisthotonus is observed in some children and adults with cerebral palsy and other brain disorders. This motor disorder results from involuntary activation of the trunk and cervical muscles and is associated with motor disability and pain. Usually opisthotonus does not respond to oral medications. The aim of this study is to examine the effect of botulinum toxin treatment in opisthotonus, and to objectify the patient's response and satisfaction. Material and method Six patients with opisthotonus were included in the study, the causes of dystonia being cerebral palsy (2 cases), traumatic brain injury (1 case), neurometabolic disease (2 cases) and autoimmune encephalitis (1 case). Interview with the patients and their caregivers determined the main problems in daily living resulting from opisthotonus and helped to define the goals of botulinum toxin therapy. The clinical examination performed by the injector identified the type of opisthotonus and the muscles to be injected in order to obtain the optimum effect. A set of goals by the patient, caregiver and rehabilitation team was decided for each patient. Each goal was rated on a five-point scale. Botulinum toxin was injected under electromyographic guidance into the chosen muscles of the neck and trunk (posterior and anterior muscles as needed). The Goal Attainment Scale (GAS) was used to evaluate treatment outcome. Results All individual baseline GAS scores were below 40. After BTX-A treatment, outcome GAS scores were above 60 in 5 cases and above 50 in one case. All individual GAS changes were positive, with a greater than 10 point-difference, which is considered to be of clinical importance. Conclusion Botulinum toxin treatment of selected muscles of the neck and trunk is a promising solution in pediatric dystonic opisthotonus. The GAS instrument can depict the positive changes for the patient provided by this treatment.