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A 7-year-old boy, weighing 18 kg, was diagnosed with maple syrup urine disease (MSUD). He suffered from spasticity of the lower limbs and pain that did not respond to oral medications. Injections of botulinum toxin A (BTX-A) at 10 sites and epidural analgesia with 0.125% bupivacaine were used to treat spasticity with good results. We conclude that BTX-A combined with epidural analgesia may be a useful treatment option for incapacitating, painful spasticity related to MSUD. This treatment modality allowed a comprehensive rehabilitation program to be completed and it lasted longer than 9 months.

The use of botulinum toxin and epidural analgesia for the treatment of spasticity and pain in a patient with maple syrup urine disease