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Background: Status dystonicus (SD) is a severe episode of generalized dystonia, potentially complicated by respiratory and metabolic disruption. Triggers can be infection, medication, or metabolic disturbance. The prognosis is variable and mortality is approximately 10%.
Case Description: An 18 month old girl presented to the ER with clinical suspicion of a febrile status epilepticus and was evaluated according to APLS principles. Eventually, a SD became apparent, with generalized dystonic features at examination. Most likely, the episode was provoked by a single dose of metoclopramide. Her clinical state improved rapidly, possibly aided by administration of biperiden.
Conclusion: Treatment of SD encompasses elimination or treatment of the trigger, stabilization of vital functions, possible administration of sedatives and dystonia specific medication. Metoclopramide holds a relatively high risk for extrapyramidal complications (1-10%) and dystonia (0.1-1.5%), even within therapeutic range. The use of anti-emetics with less alarming side effect profiles, for example ondansetron, is recommended.
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BMC Neurol
August 2025
Department of Clinical Pediatrics Sciences, Unit of Child Neuropsychiatry, Member of the ERN Epicare Network, IRCCS Istituto Giannina Gaslini, Via G Gaslini 5, Genova, 16100, Italy.
Background: Herpes simplex virus 1 (HSV-1) encephalitis may result in relapsing neurological symptoms secondary to immune-mediated processes, including anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Refractory status dyskineticus (RSD), a severe subset of status dystonicus, is characterized by a hyperkinetic movement disorder phenotype alongside dystonic features. This critical condition presents substantial challenges in neurocritical care.
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August 2025
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Background: There is limited data regarding the role of pallidothalamic tract (PTT) lesioning after failure of pallidotomy.
Objectives: To report the role of unilateral PTT lesioning in refractory generalized dystonia following bilateral pallidotomy and hemi-dystonia following unilateral pallidotomy.
Methods: A single-center retrospective case series.
J Mov Disord
August 2025
Department of Neurology, National Institute of Mental Health and Neuro Sciences, Bengaluru, India.
Mov Disord Clin Pract
August 2025
Division of Biochemical Genetics, Department of Pediatrics, BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Background: Status dystonicus is a life-threatening movement disorder emergency characterized by frequent or prolonged severe episodes of generalized dystonia. The phenomenology, etiology, and outcome is heterogenous and poorly characterized, making implementation of a standardized management approach challenging.
Objectives: We characterized demographics of children with status dystonicus in a tertiary children's hospital pediatric intensive care unit (PICU), management patterns, and outcomes.
Neuromodulation
June 2025
European Reference Network for Rare Neurological Diseases, Tübingen, Germany; Movement Disorders Unit, Pediatric Neurology Department, Institut de Recerca, Hospital Sant Joan de Déu Barcelona, Barcelona, Spain; U-703 Center for Biomedical Research on Rare Diseases, Instituto de Salud Carlos III, B
Objectives: Deep brain stimulation (DBS) is an advanced treatment for individuals with GNAO1-related disorders (GNAO1-RD), which are characterized by severe movement abnormalities such as status dystonicus and dyskinetic crises. Decision-making surrounding DBS is complex and influenced by medical, emotional, and logistical factors. This study aimed to explore caregiver perspectives on the decision-making process, including influencing factors and family experiences.
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