Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPLOPEZ, William Omar ContrerasSCHROEDER, Humberto KlugeNEVILLE, Iuri SantanaTEIXEIRA, Manoel JacobsenBARBOSA, Danilo CostaMONACO, Bernardo Assumpcao deFONOFF, Erich Talamoni2015-10-262015-10-262014PEDIATRIC NEUROSURGERY, v.50, n.2, p.94-98, 20141016-2291https://observatorio.fm.usp.br/handle/OPI/11704Neurodegeneration with brain iron accumulation type 1 (NBIA-1) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. NBIA-1 encompasses typical iron brain accumulation, mostly in the globus pallidus with secondary dementia, spasticity, rigidity, dystonia, and choreoathetosis. Treatment remains mostly symptomatic and is challenging. We present the case of a 14-year-old boy diagnosed with NBIA-1, presenting intractable progressive generalized dystonia leading to unresponsive status dystonicus (SD). The patient received a SynchroMed II (model 8637) programmable system pump (Medtronic (R), Inc.) implant with an Ascenda intrathecal catheter for intrathecal morphine therapy (IMT). The initial dose of morphine was 1.0 mg/day. Overall, we observed no complications with IMT treatment and important improvement of the patient's motor function with stabilization of his incapacitating dystonia and his quality of life. On the Global Dystonia Severity Rating Scale, he presented 52% improvement, 30% improvement on the Unified Dystonia Rating Scale, and 38% improvement on the Fahn-Marsden Rating Scale after 10 months, when the dose was 1.7 mg/day. IMT should be considered as a potential palliative treatment in the management of intractable dystonia and SD secondary to NBIA-1. (C) 2015 S. Karger AG, BaselengrestrictedAccessNeurodegeneration with brain iron accumulation type 1Status dystonicusDeep brain stimulationIntrathecal baclofen therapyIntrathecal morphine therapyhallervorden-spatz-syndromekinase-associated neurodegenerationspasticitybaclofenstimulationdiseasepallidotomytolerancearticleCopyright KARGER10.1159/000370005Clinical NeurologyPediatricsSurgery1423-0305