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Case Report: Dystonic Storm Following Japanese Encephalitis Virus Infection

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  • 1 Department of General Medicine, Burdwan Medical College, Burdwan, West Bengal, India;
  • | 2 Department of Neuromedicine, Bangur Institute of Neurosciences (BIN), Kolkata, West Bengal, India;
  • | 3 Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain;
  • | 4 Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain;
  • | 5 Department of Medicine, Universidad Complutense, Madrid, Spain
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ABSTRACT.

Dystonic storm (also called status dystonicus) is a neurological emergency characterized by sustained/intermittent involuntary generalized muscle contractions resulting in repetitive painful twisting movements and abnormal postures. It is commonly documented in patients with diagnosed primary dystonic syndromes or secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson’s disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs, among others). However, viral-induced dystonic storm cases have rarely been reported. We describe the case of an 11-year-old girl from rural West Bengal (India) with a dystonic storm after Japanese encephalitis. Generalized dystonic spasms lasted for about 10–20 minutes and occurred 20–30 times/day. They were associated with extreme pain, fever, exhaustion, sweating, tachycardia, tachypnea, pupillary dilatation, arterial hypertension, and mutism and were precipitated by a full bladder and relieved somewhat during sleep. When dystonic spasms abated, she had high-grade generalized rigidity of all four limbs and fixed cervical and truncal dystonia. She was put on invasive ventilation and deep intravenous sedation with continuous midazolam infusion and other supportive measures and had a good clinical recovery. During the 12 months of follow-up, she did not have any other episode of a dystonic storm. However, axial rigidity and intermittent appendicular (upper limb) dystonic posturing were observed. The authors also have briefly discussed the differential diagnoses and treatment plans for such a neurological emergency.

Author Notes

Address correspondence to Julián Benito-León, Department of Neurology, University Hospital “12 de Octubre”, Avenida de Córdoba S/N, 28041 Madrid, Spain. E-mail: jbenitol67@gmail.com

Financial support: Julián Benito-León is supported by the National Institutes of Health, Bethesda, MD (Grant no. NINDS #R01 NS39422), the European Commission (Grant no. ICT-2011-287739, NeuroTREMOR), the Ministry of Economy and Competitiveness (Grant no. RTC-2015-3967-1, NetMD—platform for the tracking of movement disorder), and the Spanish Health Research Agency (Grant nos. FIS PI12/01602 and FIS PI16/00451).

Authors’ addresses: Ritwik Ghosh, Department of General Medicine, Burdwan Medical College, Burdwan, West Bengal, India, E-mail: ritwikmed2014@gmail.com. Souvik Dubey and Shambaditya Das, Department of Neuromedicine, Bangur Institute of Neurosciences (BIN), Kolkata, West Bengal, India, E-mails: drsouvik79@gmail.com and drshambadityadas@gmail.com. Julián Benito-Leon, Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain, Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain, and Department of Medicine, Universidad Complutense, Madrid, Spain, E-mail: jbenitol67@gmail.com.

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