SynthesisJournal of neural transmission (Vienna, Austria : 1996)2019
Long-term effect of subthalamic and pallidal deep brain stimulation for status dystonicus in children with methylmalonic acidemia and GNAO1 mutation.
Synthesis in Journal of neural transmission (Vienna, Austria : 1996), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 38 citations in OpenAlex.
- Consensus-based follow-up and treatment registry for GNAO1-associated disorder.Developmental medicine and child neurology · 2026Article
- Status dystonicus is a distinct state characterized by pallidal beta-band activity.Nature communications · 2025Article
- Exploring the Impact of Dyskinetic Crises in GNAO1-Related Disorders: A Survey for Parents and Caregivers.Movement disorders clinical practice · 2025Article
- Dyskinetic crisis inFrontiers in neurology · 2024Article
- Deep Brain Stimulation of the Globus Pallidus Internus in a Child with Refractory Dystonia due to L2-Hydroxyglutaric Aciduria.Stereotactic and functional neurosurgery · 2024Article
- GNAO1-related neurodevelopmental disorder: Literature review and caregiver survey.Epilepsy & behavior reports · 2023Article
- Surgical treatment of movement disorders in neurometabolic conditions.Frontiers in neurology · 2023Review
- Phenotypes in children withFrontiers in pediatrics · 2023Article
- Restoration of the GTPase activity and cellular interactions of GαScience advances · 2022Article
- DBS emergency surgery for treatment of dystonic storm associated with rhabdomyolysis and acute colitis in DYT-GNAO1.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Article
- Gonadal mosaicism in GNAO1 causing neurodevelopmental disorder with involuntary movements; two additional variants.Molecular genetics and metabolism reports · 2022Article
- Mouse models characterize GNAO1 encephalopathy as a neurodevelopmental disorder leading to motor anomalies: from a severe G203R to a milder C215Y mutation.Acta neuropathologica communications · 2022Article
- Both subthalamic and pallidal deep brain stimulation are effective forTherapeutic advances in neurological disorders · 2022Article
- Deep brain stimulation in a young child withSurgical neurology international · 2022Article
- Arching deep brain stimulation in dystonia types.Journal of neural transmission (Vienna, Austria : 1996) · 2021Review
- Pallidal Deep Brain Stimulation for Monogenic Dystonia: The Effect of Gene on Outcome.Frontiers in neurology · 2020Review
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Status dystonicus (SD) is a rare and potentially life-threatening condition requiring intensive care management. Deep brain stimulation (DBS) has emerged as an effective treatment for SD refractory to medical management, but its application in this field is still limited. Here, we report the long-term outcome of four pediatric patients treated with DBS at the University Hospital of Padua, Italy, for SD refractory to medications. In addition, we present the results of a systematic literature review aimed at identifying published cases of SD treated with DBS, with focus on motor outcome. In our cohort, two children were affected by methylmalonic acidemia and suffered acute basal ganglia lesions, while the other two carried a pathogenic mutation in GNAO1 gene. DBS target was subthalamic nucleus (STN) in one case and globus pallidus internus (GPi) in three. All patients experienced SD resolution within 8-19 days after surgery. Mean post-operative follow-up was 5 years. We identified in the literature 53 additional SD cases treated with DBS (median age at DBS implantation: 12 years) with reported positive outcome in 51 and resolution of SD in a mean of 17 days after surgery. Our findings indicate that DBS is an effective treatment for SD refractory to medications, even in patients with acute basal ganglia lesions; STN can be an appropriate target when GPi is damaged. Moreover, data from long-term follow-up show that SD recurrences can be significantly reduced in frequency or abolished after DBS implantation.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.