Pallidotomy for medically refractory status dystonicus in childhood

Authors

  • Carlo Efisio Marras,

    1. Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Michele Rizzi,

    1. Department of Neurosurgery, IRCCS Fondazione Istituto Neurologico ‘Carlo Besta’, Milan, Italy
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  • Laura Cantonetti,

    1. Neurorehabilitation Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Erika Rebessi,

    Corresponding author
    1. Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
    • Correspondence to Erika Rebessi, Department of Neuroscience and Neurorehabilitation, Neurosurgery Unit, Bambino Gesù Children's Hospital, IRCCS 4, Piazza Sant'Onofrio, 00165 Rome, Italy. E-mail: erika.rebessi@opbg.net

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  • Alessandro De Benedictis,

    1. Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Francesco Portaluri,

    1. Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Franco Randi,

    1. Neurology Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Alessandra Savioli,

    1. Intensive Care Unit, Department of Emergency, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Enrico Castelli,

    1. Neurorehabilitation Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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  • Federico Vigevano

    1. Neurology Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital (BGCH), Rome, Italy
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Abstract

Aim

Status dystonicus is a rare and potentially fatal condition of continuous and generalized muscle contraction that can complicate dystonia. As status dystonicus is usually refractory to traditional pharmacological therapy, alternative and invasive strategies have been developed, but so far there are no guidelines on status dystonicus management. Pallidotomy has shown good results in status dystonicus treatment.

Method

We report indications, surgical strategy, and outcome of bilateral pallidotomy in four pediatric patients (four males; mean age at surgery 11y 5mo) with secondary dystonia, who developed refractory status dystonicus. Pallidotomy was performed in the area corresponding to the mid portion of the globus pallidus internus.

Results

This procedure allowed patients to recover the pre-status dystonicus condition, controlling dystonic postures and movements of trunk and limbs. Moreover oromandibular dystonia, which is resistant to conservative approaches and deep brain stimulation, was significantly reduced. No postoperative complications were registered.

Interpretation

Our study suggests pallidotomy as a feasible treatment in patients with secondary dystonia complicated by status dystonicus.

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