Intrathecal Morphine Therapy in the Management of Status Dystonicus in Neurodegeneration Brain Iron Accumulation Type 1

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorLOPEZ, William Omar Contreras
dc.contributor.authorSCHROEDER, Humberto Kluge
dc.contributor.authorNEVILLE, Iuri Santana
dc.contributor.authorTEIXEIRA, Manoel Jacobsen
dc.contributor.authorBARBOSA, Danilo Costa
dc.contributor.authorMONACO, Bernardo Assumpcao de
dc.contributor.authorFONOFF, Erich Talamoni
dc.date.accessioned2015-10-26T16:28:32Z
dc.date.available2015-10-26T16:28:32Z
dc.date.issued2014
dc.description.abstractNeurodegeneration 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
dc.description.indexMEDLINE
dc.identifier.citationPEDIATRIC NEUROSURGERY, v.50, n.2, p.94-98, 2014
dc.identifier.doi10.1159/000370005
dc.identifier.eissn1423-0305
dc.identifier.issn1016-2291
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/11704
dc.language.isoeng
dc.publisherKARGER
dc.relation.ispartofPediatric Neurosurgery
dc.rightsrestrictedAccess
dc.rights.holderCopyright KARGER
dc.subjectNeurodegeneration with brain iron accumulation type 1
dc.subjectStatus dystonicus
dc.subjectDeep brain stimulation
dc.subjectIntrathecal baclofen therapy
dc.subjectIntrathecal morphine therapy
dc.subject.otherhallervorden-spatz-syndrome
dc.subject.otherkinase-associated neurodegeneration
dc.subject.otherspasticity
dc.subject.otherbaclofen
dc.subject.otherstimulation
dc.subject.otherdisease
dc.subject.otherpallidotomy
dc.subject.othertolerance
dc.subject.wosClinical Neurology
dc.subject.wosPediatrics
dc.subject.wosSurgery
dc.titleIntrathecal Morphine Therapy in the Management of Status Dystonicus in Neurodegeneration Brain Iron Accumulation Type 1
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.author.externalSCHROEDER, Humberto Kluge:Univ Sao Paulo, Sch Med, Inst Psychiat, Dept Neurol,Div Funct Neurosurg, BR-01060970 Sao Paulo, Brazil
hcfmusp.author.externalBARBOSA, Danilo Costa:Univ Sao Paulo, Sch Med, Inst Psychiat, Dept Neurol,Div Funct Neurosurg, BR-01060970 Sao Paulo, Brazil
hcfmusp.citation.scopus5
hcfmusp.contributor.author-fmusphcWILLIAM OMAR CONTRERAS LOPEZ
hcfmusp.contributor.author-fmusphcIURI SANTANA NEVILLE RIBEIRO
hcfmusp.contributor.author-fmusphcMANOEL JACOBSEN TEIXEIRA
hcfmusp.contributor.author-fmusphcBERNARDO ASSUMPCAO DE MONACO
hcfmusp.contributor.author-fmusphcERICH TALAMONI FONOFF
hcfmusp.description.beginpage94
hcfmusp.description.endpage98
hcfmusp.description.issue2
hcfmusp.description.volume50
hcfmusp.origemWOS
hcfmusp.origem.pubmed25896138
hcfmusp.origem.scopus2-s2.0-84983187685
hcfmusp.origem.wosWOS:000355932100009
hcfmusp.publisher.cityBASEL
hcfmusp.publisher.countrySWITZERLAND
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