[Anesthesia considerations for deep-brain stimulation in a patient with type-2 pantothenate kinase deficiency (Hallervorden-Spatz disease)].

Abstract

Neurodegeneration associated with pantothenate kinase deficiency is an autosomal recessive condition caused by mutations in the pantothenate kinase 2 gene (PANK2). Clinical characteristics include progressive motor impairment and dementia. Medical treatment is limited and the dystonia tends to be refractory, making stereotactic surgery with placement of deep-brain electrodes an option that is being adopted with greater frequency in these patients. We report the case of a 32-year-old woman with severe dystonia associated with PANK2 protein deficiency. The patient was scheduled for stereotactic bilateral placement of electrodes in the medial globus pallidus, guided by computed tomography and under general anesthesia, to treat the debilitating dystonia and generalized stiffness associated with her condition. Anesthesia was maintained with propofol, rocuronium and remifentanil in perfusion during the intervention, which was uneventful. After the procedure, the patient was transferred to the intensive care unit and sedation was provided with remifentanil to allow slow, gradual emergence from anesthesia. The patient was discharged from hospital after placement of the implanted pulse generator, and subsequent follow-up showed improvement of the dystonia.

• Authors: P Hurtado, L Salvador, E Carrero, J Rumia, N Fàbregas
• Journal: Revista espanola de anestesiologia y reanimacion (Rev Esp Anestesiol Reanim) Vol. 56 Issue 3 Pg. 180-4 (Mar 2009) ISSN: 0034-9356 [Print] Spain
• Vernacular Title: Consideraciones anestésicas en una paciente con déficit de pantotenato cinasa tipo 2 (enfermedad de Hallervorden-Spatz) para estimulación cerebral profunda.
• PMID: 19408784 (Publication Type: Case Reports, Journal Article)

Chemical References

• Androstanols
• Neuromuscular Nondepolarizing Agents
• Piperidines
• Phosphotransferases (Alcohol Group Acceptor)
• pantothenate kinase
• Remifentanil
• Rocuronium
• Propofol

Topics

• Adult
• Androstanols (administration & dosage)
• Anesthesia, Intravenous (methods)
• Deep Brain Stimulation
• Dystonic Disorders (etiology, therapy)
• Female
• Globus Pallidus
• Humans
• Intubation, Intratracheal
• Muscle Rigidity (etiology, therapy)
• Neuromuscular Nondepolarizing Agents (administration & dosage)
• Pantothenate Kinase-Associated Neurodegeneration (complications, genetics)
• Phosphotransferases (Alcohol Group Acceptor) (deficiency)
• Piperidines (administration & dosage)
• Preanesthetic Medication
• Propofol (administration & dosage)
• Radiography, Interventional
• Remifentanil
• Rocuronium