Intrathecal Morphine Therapy in the Management of Status Dystonicus in Neurodegeneration Brain Iron Accumulation Type 1
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Citações na Scopus
5
Tipo de produção
article
Data de publicação
2014
Título da Revista
ISSN da Revista
Título do Volume
Editora
KARGER
Autores
SCHROEDER, Humberto Kluge
BARBOSA, Danilo Costa
Citação
PEDIATRIC NEUROSURGERY, v.50, n.2, p.94-98, 2014
Resumo
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 (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, Basel
Palavras-chave
Neurodegeneration with brain iron accumulation type 1, Status dystonicus, Deep brain stimulation, Intrathecal baclofen therapy, Intrathecal morphine therapy
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