First case of X‐linked dystonia‐parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation

Evidente, Virgilio Gerald H.; Lyons, Mark; Wheeler, Mark; Hillman, Renee; Helepolelei, Luann; Beynen, Froukje M.; Nolte, Dagmar; Müller, Ulrich; Starr, Philip A.

doi:10.1002/mds.21420

Cited by 76 publications

(42 citation statements)

References 15 publications

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“…The features of these cases as well as our case are all summarized in table 1 . The first case reported by Evidente et al [5] was a 45-year-old man who first presented with shuffling gait at age 31. He later developed handwriting difficulty, then lower limb dystonia, dysarthria with jaw opening dystonia, and facial tremor.…”

Section: Discussionmentioning

confidence: 99%

“…The disabling symptoms have also been surgically refractory in the limited available reports in the literature where thalamotomy was employed [3,4] . To date, there are two existing case reports on bilateral globus pallidus internus deep brain stimulation (GPi-DBS) to address XDP symptoms [5,6] . We report our experience with GPi-DBS in XDP, and we discuss the varying responses of parkinsonism and dystonia to DBS.…”

Section: Introductionmentioning

confidence: 99%

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Differential Response of Dystonia and Parkinsonism following Globus Pallidus Internus Deep Brain Stimulation in X-Linked Dystonia-Parkinsonism (Lubag)

Oyama¹,

Fernández²,

Foote³

et al. 2010

Stereotact Funct Neurosurg

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Background: X-linked dystonia-parkinsonism (XDP; DYT3; Lubag) is an adult-onset hereditary progressive dystonia/parkinsonism which is typically minimally responsive to pharmacological treatment. Case Report: We report a 63- year-old man with a diagnosis of XDP who underwent bilateral globus pallidus internus deep brain stimulator (GPi-DBS) placement. His course initially began with right hand tremor and dystonia at age 57 and progressed to also include bradykinesia and rigidity. The patient tolerated the procedure without significant complications. GPi-DBS improved his right hand dystonia, but did not significantly improve his parkinsonism. Conclusion: DBS may be a therapeutic option for select cases of XDP, but its specificindications must be carefully discussed, as the available cases have had mixed responses. Whether other targets may be more effective is not known.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Differential Response of Dystonia and Parkinsonism following Globus Pallidus Internus Deep Brain Stimulation in X-Linked Dystonia-Parkinsonism (Lubag)

Oyama¹,

Fernández²,

Foote³

et al. 2010

Stereotact Funct Neurosurg

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“…Evidente et al [24] reported the first case of XDP treated with bilateral pallidal DBS in 2007. Several other centers have reported excellent outcomes in single patients (Table 1), but the average follow-up has been short.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Outcomes of Bilateral Pallidal Deep Brain Stimulation for X-Linked Dystonia and Parkinsonism

Kilbane

Witt

Galifianakis

et al. 2018

Stereotact Funct Neurosurg

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Background: X-linked dystonia parkinsonism (XDP) causes adult-onset progressive dystonia and parkinsonism, which may not respond to pharmacotherapy. Objective: Previous case reports have reported beneficial effects from bilateral pallidal (GPi) deep brain stimulation (DBS). Here, we report the long-term clinical outcomes of 3 patients treated at our center. Methods: All patients presented with medication refractory dystonia and parkinsonism. They were followed prospectively. Clinical evaluations included the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and the Unified Parkinson’s Disease Rating Scale (UPDRS). Adverse events were recorded. Results: The average length of follow-up was 45.7 months. No serious adverse events occurred. All patients experienced an immediate and sustained improvement in dystonia. Mean percentage improvement in motor subscores of BFMDRS was 63.5% at the last follow-up visit. Parkinsonism was less responsive to neuromodulation, with a mean improvement in UPDRS-III of 39.5%. Standard pallidal stimulation parameters were used. Freezing of gait developed after DBS therapy in 2 patients, stimulation-induced in one and due to disease progression in the other. Conclusion: Bilateral pallidal DBS resulted in significant and sustained improvement in dystonia and moderate improvement in parkinsonism. Pallidal DBS represents an important treatment option for XPD for the management of motor symptoms.

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“…In the literature, 5 case reports of GPi DBS on X-linked dystonia parkinsonism are available (Aguilar et al, 2011;Evidente et al, 2007;Martinez-Torres et al, 2009;Oyama et al, 2010;Wadia et al, 2010). All cases showed improvement in dystonia.…”

Section: X-linked Dystonia Parkinsonism (Dyt3 'Lubag')mentioning

confidence: 99%

Dystonia and DBS: The Jury Arrives

Kim¹,

Jeon²

2012

Dystonia - The Many Facets

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First case of X‐linked dystonia‐parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation

Cited by 76 publications

References 15 publications

Differential Response of Dystonia and Parkinsonism following Globus Pallidus Internus Deep Brain Stimulation in X-Linked Dystonia-Parkinsonism (Lubag)

Differential Response of Dystonia and Parkinsonism following Globus Pallidus Internus Deep Brain Stimulation in X-Linked Dystonia-Parkinsonism (Lubag)

Long-Term Outcomes of Bilateral Pallidal Deep Brain Stimulation for X-Linked Dystonia and Parkinsonism

Dystonia and DBS: The Jury Arrives

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