Concomitant medical products: product id: 3387, lot #: unknown, product type: lead.Other relevant device(s) are: product id: 3387, serial/lot #: unknown.(b)(4).Age or date of birth: patient date of birth is month and year valid.Date of event: please note that this date is based off of the date of publication of the article as the event dates were not provided in the published literature.Description of problem or event: it was not possible to ascertain specific device information from the article or to match the events reported with previously reported events.Correspondence has been sent to the author of the article inquiring about individual patient information and additional information regarding the reported events.If information is provided in the future, a supplemental report will be issued.
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Benato a, carecchio m, burlina a, et al.Long-term effect of subthalamic and pallidal deep brain stimulation for status dystonicus in children with methylmalonic acidemia and gnao1 mutation.J neural transm (vienna).2019.Doi: 10.1007/s00702-019-02010-2.Summary: 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.Reported events: 1 patient was implanted with a bilateral gpi-dbs.After one week of stimulation, the patient's involuntary movements improved and they could be extubated, gradually returning to their pre-crisis neurological state.At discharge from the hospital, their neurological examination showed mild involuntary movements of the limbs, diminished compared to their pre-sd baseline.During the post-operative follow up, stimulation parameters had been progressively adjusted to following double monopolar configuration: l-gpi: 4v/120 s/210 hz, contacts 0-, 1-; r-gpi: 4 v/120 s/210 hz, contacts 10-, 11-.Three months after the surgery, the patient presented an erosion of the skin above the left electrode skull borehole, which was successfully managed with a cutaneous cap.Unfortunately, the complication did result in the dislocation of the left electrode.During the five year follow-up, the patient experienced two more episodes of dystonic storm, both involving exclusively the right side of the body.In addition, they also presented some hyperkinetic involuntary movements in their right hemibody during the day.These recurrences were attributed to the displacement of the left electrode, leading to reduced effectiveness of the left gpi stimulation.Five years after the first operation, the patient had a left electrode revision surgery.One month later, at the last follow-up, the hyperkinetic movements in their right side of their body had completely disappeared.The patient suffers from profound intellectual disability; and their mmrs score was four.
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