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Deep brain stimulation for pantothenate kinase-associated neurodegeneration.

Garcia-Ruiz PJ, Ayerbe J, Vela Desojo L, Feliz CE, Del Val Fernandez J - Case Rep Neurol Med (2015)

Bottom Line: Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty.This result has been maintained up to the present.GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.

ABSTRACT
Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

No MeSH data available.


Related in: MedlinePlus

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Mentions: Due to her severe writing problems (which had made it impossible for her to write a sentence) and given that the previous medical treatment had been unsuccessful and based on previous cases, treatment with GPi DBS was proposed. After informed written consent was obtained, the patient underwent bilateral implantation of a quadripolar electrode into the GPi. The surgical procedure was performed by frame-based stereotactic technique while the patient remained under generalized anesthesia. The target was bilaterally defined by CT/MR image fusion with the standard coordinates for GPi (2 mm anterior to the midcommissural point and 20 mm lateral and 4 mm ventral to the intercommissural line). Neurophysiologic target verification was performed intraoperatively by simultaneous multielectrode microrecordings, microstimulation, and visual evoked potentials. After determining the definite bilateral coordinates, a permanent quadripolar electrode (DBS 3387, Medtronic) was implanted in each side. During the procedure, the electrodes were connected to a dual-channel pulse generator (Kinetra, Medtronic) subcutaneously placed in the right subclavicular area. The positioning of the electrodes in the caudal ventral portion of GPi was verified with MRI (Figures 1 and 2).


Deep brain stimulation for pantothenate kinase-associated neurodegeneration.

Garcia-Ruiz PJ, Ayerbe J, Vela Desojo L, Feliz CE, Del Val Fernandez J - Case Rep Neurol Med (2015)

© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4352941&req=5

Mentions: Due to her severe writing problems (which had made it impossible for her to write a sentence) and given that the previous medical treatment had been unsuccessful and based on previous cases, treatment with GPi DBS was proposed. After informed written consent was obtained, the patient underwent bilateral implantation of a quadripolar electrode into the GPi. The surgical procedure was performed by frame-based stereotactic technique while the patient remained under generalized anesthesia. The target was bilaterally defined by CT/MR image fusion with the standard coordinates for GPi (2 mm anterior to the midcommissural point and 20 mm lateral and 4 mm ventral to the intercommissural line). Neurophysiologic target verification was performed intraoperatively by simultaneous multielectrode microrecordings, microstimulation, and visual evoked potentials. After determining the definite bilateral coordinates, a permanent quadripolar electrode (DBS 3387, Medtronic) was implanted in each side. During the procedure, the electrodes were connected to a dual-channel pulse generator (Kinetra, Medtronic) subcutaneously placed in the right subclavicular area. The positioning of the electrodes in the caudal ventral portion of GPi was verified with MRI (Figures 1 and 2).

Bottom Line: Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty.This result has been maintained up to the present.GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.

ABSTRACT
Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

No MeSH data available.


Related in: MedlinePlus