Deep brain stimulation as a mode of treatment of early onset pantothenate kinase-associated neurodegeneration

Mikati, Mohamad A.; Yehya, Amin; Darwish, Hala; Karam, Pascale E.; Comair, Youssef G.

doi:10.1016/j.ejpn.2008.01.006

Cited by 85 publications

(36 citation statements)

References 11 publications

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“…Within the 1st year of continuous postoperative stimulation, therapeutic efficacy was maintained, and BFMDRS improved from 112 before surgery to 22.5 one year after surgery. Other authors including Chacko et al [14] , Mikati et al [15] , Isaac et al [16] and Shields et al [17] , all performed bilateral pallidal stimulation for NBIA1 patients and got similar results. The patients' symptoms greatly improved after DBS surgery.…”

Section: Discussionmentioning

confidence: 66%

Bilateral Subthalamic Nucleus Stimulation in the Treatment of Neurodegeneration with Brain Iron Accumulation Type 1

Ge¹,

Zhang²,

Ma³

et al. 2011

Stereotact Funct Neurosurg

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Background: Neurodegeneration with brain iron accumulation type 1 (NBIA1), previously called Hallervorden-Spatz disease, is a rare neurodegenerative condition with abnormal brain iron accumulation. There have been some reports of deep brain stimulation (DBS) in the treatment of NBIA1. However, the target was usually the globus pallidus internus or thalamus. Objectives/Methods: We present a case of NBIA1 in a 16-year-old male who was treated with bilateral subthalamic nucleus (STN)-DBS and explored its efficacy in the treatment of NBIA1. The patient presented with severe generalized dystonia and marked dysarthria and had previously had unsuccessful ablation surgery. MRI confirmed the diagnosis of NBIA1. Results: He had significant improvement postoperatively, and the benefit of surgery was maintained for 3 years during follow-up. Burke-Fahn-Marsden Dystonia Rating Scale score was 114/120 preoperatively, and dropped to 60/120 at 14 days, 35/120 at 1 month, 28/120 at 3 months, 14/120 at 1 year and 18/120 at 3 years postoperatively. Conclusions: Our results suggest that bilateral STN-DBS might be considered as an effective treatment for selective NBIA1 patients.

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Section: Discussionmentioning

confidence: 66%

Bilateral Subthalamic Nucleus Stimulation in the Treatment of Neurodegeneration with Brain Iron Accumulation Type 1

Ge¹,

Zhang²,

Ma³

et al. 2011

Stereotact Funct Neurosurg

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“…There are reports of favorable (65-91%) responses (Castelnau et al, 2005;Clement et al, 2007;Krause et al, 2006;Mikati et al, 2009;Umemura et al, 2004), but others reported only 20-30% improvement (Isaac et al, 2007;Shields et al, 2007). A single case report of STN DBS on a NBIA patient showed 84% improvement at 3 years after surgery (Ge et al, 2011).…”

Section: Nbiamentioning

confidence: 99%

Dystonia and DBS: The Jury Arrives

Kim¹,

Jeon²

2012

Dystonia - The Many Facets

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“…DBS has been shown to improve intractable epilepsy, cephalgias, restless legs syndrome, multiple sclerosis, pantothenate kinase-associated neurodegeneration, Tourette syndrome, major depressive disorders, obsessive compulsive derangements and other movement disorders, including essential tremor, and posttraumatic tremor. [2][3][4][5][6][7][8] Although DBS has not been approved in the United States for treatment of refractory pain, success in this area in Great Britain and Europe may well prompt a rethinking. With such a wide range of disorders, many of which resistant to multiple medications for which DBS may prove beneficial, anesthesiologists may soon be called on to assist in different situations and in patients with a host of pathologies.…”

mentioning

confidence: 95%

Deep Brain Stimulation—Surgery for Movement Disorders and Parkinson's Disease

Frost

Osborn

2009

International Anesthesiology Clinics

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CaseA 66-year-old woman with Parkinson's disease (PD) was scheduled for insertion of electrodes for deep brain stimulation (DBS). She was right handed, English speaking, and of normal intelligence. Her memory was good but a marked tremor interfered with her quality of life. Her current medications, levodopa (L-dopa) (Sinemet), bromocriptine (Parlodel), selegiline (Eldepryl), pramipexole (Mirapex), and amantadine (Symmetrel), afforded only fair movement control. She was very depressed and sertraline hydrochloride (Zoloft) had been prescribed. She had a history of hypertension treated with hydrochlorothiazide and diltiazem and type 2 diabetes controlled with diet and glyburide.'

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Deep brain stimulation as a mode of treatment of early onset pantothenate kinase-associated neurodegeneration

Cited by 85 publications

References 11 publications

Bilateral Subthalamic Nucleus Stimulation in the Treatment of Neurodegeneration with Brain Iron Accumulation Type 1

Bilateral Subthalamic Nucleus Stimulation in the Treatment of Neurodegeneration with Brain Iron Accumulation Type 1

Dystonia and DBS: The Jury Arrives

Deep Brain Stimulation—Surgery for Movement Disorders and Parkinson's Disease

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