Intrathecal morphine therapy in the management of status dystonicus in neurodegeneration brain iron accumulation type 1

William Omar Contreras Lopez, Humberto Kluge Schroeder, Iuri Santana Neville, Manoel Jacobsen Teixeira, Danilo Costa Barbosa, Bernardo Assumpçao De Mônaco, Erich Talamoni Fonoff

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

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.

Original languageEnglish
Pages (from-to)94-98
Number of pages5
JournalPediatric Neurosurgery
Volume50
Issue number2
DOIs
StatePublished - 1 Jun 2015
Externally publishedYes

Keywords

  • Deep brain stimulation
  • Intrathecal baclofen therapy
  • Intrathecal morphine therapy
  • Neurodegeneration with brain iron accumulation type 1
  • Status dystonicus

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