De almeida marcelino, a.L., mainka, t., krause, p.Et al.Deep brain stimulation reduces (nocturnal) dyskinetic exacerbations in patients with adcy5 mutation: a case series.J neurol (2020).Doi: 10.1007/s00415-020-09871-8 other relevant device(s) are: product id: 37085-95, serial/lot #: (b)(4), ubd: 25-oct-2015, implanted: (b)(6) 2011, explanted: (b)(6) 2017, udi#: (b)(4).This event was initially reported under manufacturer's report # 2182207-2020-00757.Additional information was received which included product serial numbers, which prompted the creation of this report.Any additional information received regarding this event will be submitted under this report.If information is provided in the future, a supplemental report will be issued.
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Summary: mutations in the adcy5 gene can cause a complex hyperkinetic movement disorder.Episodic exacerbations of dyskinesia are a particularly disturbing symptom as they occur predominantly during night and interrupt sleep.The authors present the clinical short- and long-term effects of pallidal deep brain stimulation (dbs) in three patients with a confirmed pathogenic adcy5 mutation.Patients were implanted with bilateral pallidal dbs at the age of 34, 20 and 13 years.Medical records were reviewed for clinical history.Pre- and postoperative video files were assessed using the ¿abnormal involuntary movement scale¿ (aims) as well as the motor part of the ¿burke fahn marsden dystonia rating scale¿ (bfmdrs).All patients reported subjective general improvement ranging from 40 to 60%, especially the reduction of nocturnal episodic dyskinesias (80¿90%).Objective scales revealed only a mild decrease of involuntary movements in all and reduced dystonia in one patient.Dbs-induced effects were sustained up to 13 years after implantation.The authors demonstrate that treatment with pallidal dbs was effective in reducing nocturnal dyskinetic exacerbations in patients with adcy5-related movement disorder, which was sustained over the long term.Reported event: a (b)(6) male patient implanted with gpi-dbs for hyperkinetic movement disorder experienced severe worsening of dystonic features and/or nocturnal attacks that led to hospital admission through emergency services.Both extensions were found to be broken and were replaced.
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