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    Dystonia in neurodegeneration with brain iron accumulation: outcome of bilateral pallidal stimulation
    (OXFORD UNIV PRESS, 2010-01-01) Timmermann, L.; Pauls, K. A. M.; Wieland, K.; Jech, R.; Kurlemann, G.; Sharma, N.; Gill, S. S.; Haenggeli, C. A.; Hayflick, S. J.; Hogarth, P.; Leenders, K. L.; Limousin, P.; Malanga, C. J.; Moro, E.; Ostrem, J. L.; Revilla, F. J.; Santens, P.; Schnitzler, A.; Tisch, S.; Valldeoriola, F.; Vesper, J.; Volkmann, J.; Woitalla, D.; Peker, S.
    Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and bilateral pallidal deep brain stimulation. Patient details including gender, age at onset, age at operation, genetic status, magnetic resonance imaging status, history and clinical findings were requested. Data on severity of dystonia (Burke Fahn Marsden Dystonia Rating Scale-Motor Scale, Barry Albright Dystonia Scale), disability (Burke Fahn Marsden Dystonia Rating Scale-Disability Scale), quality of life (subjective global rating from 1 to 10 obtained retrospectively from patient and caregiver) as well as data on supportive therapy, concurrent pharmacotherapy, stimulation settings, adverse events and side effects were collected. Data were collected once preoperatively and at 2-6 and 9-15 months postoperatively. The primary outcome measure was change in severity of dystonia. The mean improvement in severity of dystonia was 28.5\% at 2-6 months and 25.7\% at 9-15 months. At 9-15 months postoperatively, 66.7\% of patients showed an improvement of 20\% or more in severity of dystonia, and 31.3\% showed an improvement of 20\% or more in disability. Global quality of life ratings showed a median improvement of 83.3\% at 9-15 months. Severity of dystonia preoperatively and disease duration predicted improvement in severity of dystonia at 2-6 months
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    Management of Pregnancy and Childbirth in a Cervical Dystonia Patient with an Implanted Deep Brain Stimulation System: A Case Report
    (WOLTERS KLUWER MEDKNOW PUBLICATIONS, 2022-01-01) Ozturk, Gulsah; Kadirogullari, Pinar
    Deep brain stimulation (DBS) can lead to psychosocial and functional improvement in medically refractory cervical, segmental, or generalized moderate to severe dystonia. After treatment with DBS in women with dystonia, pregnancy can be planned. However, in the literature, there are no standardized clinical guidelines for the management of movement disorder treated with DBS during pregnancy. Herein, we report a 24-year-old female patient with cervical dystonia (CD) who have an implanted bilateral globus pallidus intema (GPi)-DBS. The patient got pregnant during the 5-year follow-up period after DBS surgery and then delivered a healthy baby via cesarean section under general anesthesia. A patient with CD who have a DBS system with a rechargeable battery could be managed safely during pregnancy and childbirth.