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.
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 NBIA1patients.
Authors: Marina Svetel; Aleksandra Tomić; Nataša Dragašević; Igor Petrović; Nikola Kresojević; Robert Jech; Dušan Urgošik; Isidora Banjac; Jelena Vitković; Ivana Novaković; Vladimir S Kostić Journal: J Neurol Date: 2019-08-29 Impact factor: 4.849
Authors: Susanne A Schneider; Petr Dusek; John Hardy; Ana Westenberger; Joseph Jankovic; Kailash P Bhatia Journal: Curr Neuropharmacol Date: 2013-01 Impact factor: 7.363
Authors: Maria Stamelou; Scarlett C Lai; Annu Aggarwal; Susanne A Schneider; Henry Houlden; Tu-Hsueh Yeh; Amit Batla; Chin-Song Lu; Mohit Bhatt; Kailash P Bhatia Journal: Mov Disord Date: 2013-06-04 Impact factor: 10.338