title: Intrathecal morphine therapy in the management of status dystonicus in neurodegeneration brain iron accumulation type 1.
abstract: Neurodegeneration 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®, 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.
authors.affiliation: Division of Functional Neurosurgery, Institute of Psychiatry, Department of Neurology, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: William Omar Contreras
authors.initials: WO
authors.lastname: Lopez
authors.forename: Humberto
authors.initials: H
authors.lastname: Kluge Schroeder
authors.forename: Iuri
authors.initials: I
authors.lastname: Santana Neville
authors.forename: Manoel
authors.initials: M
authors.lastname: Jacobsen Teixeira
authors.forename: Danilo
authors.initials: D
authors.lastname: Costa Barbosa
authors.forename: Bernardo
authors.initials: B
authors.lastname: Assumpçao de Mônaco
authors.forename: Erich
authors.initials: E
authors.lastname: Talamoni Fonoff
mesh_terms: D000293:Adolescent; D020821:Dystonic Disorders / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D015918:Infusion Pumps, Implantable; D008297:Male; D009020:Morphine / Q000008:administration & dosage / Q000494:pharmacology*; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000150:complications / Q000188:drug therapy*
chemical_list: D009020:Morphine