Main Article Content
Objective: Patients with primary and secondary dystonic syndromes occasionally develop severe episodes of generalized dystonia and rigidity which is known as status dystonicus (SD) or dystonic storm. This is a frightening hyperkinetic movement disorder and it is an emergency. Marked, rapid exacerbation of dystonia requires prompt intervention and admission in the hospital. It is critical to recognize early and differentiate dystonic storm from other hyperkinetic movement disorder as it may lead to metabolic complications such as rhabdomyolysis, leading to acute renal failure. This paper has been written to describe three cases of SD, all having secondary dystonia with different etiologies to highlight the mode of presentation, diagnosis, treatment and outcome.
Methodology: We report 3 cases of severe secondary dystonia culminating in SD necessitating management in hospital setting. All the three cases were admitted in a tertiary care hospital and evaluated.
Results: One patient was treated in intensive care unit. In brief 1st case was a 5 year boy with dyskinetic CP who was treated with trihexiphenidyl (THP), baclofen and midazolam infusion. Second case was a 15 month old boy, diagnosed case of mitochondrial encephalopathy (Leigh disease) who was treated with THP, baclofen, haloperidol, clonazepam and infusion midazolam. The third case was a 13 month old boy, diagnosed case of Molybdenum Cofactor deficiency who was treated with THP, tizanidine but they refused to take midazolam.
Conclusion: In this case series, three cases with SD with different etiology have been described with clinical features, modalities of treatment and outcome.
Fasano A, Ricciardi L, Bentivoglio AR, Canavese C, Zorzi G, Petrovic I, et al. Status dystonicus: Predictors of outcome and progression patterns of underlying disease. Mov Disord.2012;27:783–8.
Manji H, Howard RS, Miller DH, Hirsch NP, Carr L, Bhatia K, et al. Status dystonicus: The syndrome and its management. Brain.1998;121:243–52.
Teive HA, Munhoz RP, Souza MM, Antoniuk SA, Santos ML, Teixeira MJ, et al. Status Dystonicus: Study of five cases. ArqNeuropsiquiatr.2005;63:26 -9.
Jankovic J, Penn AS. Severe dystonia and myoglobinuria. Neurology. 1982;32:1195–7.
Buckley PF, Hutchinson M. Neuroleptic malignant syndrome. J Neurol Neurosurg Psychiatry. 1995;58:271-3.
Muirhead W, Jalloh I, Vloeberghs M. Status dystonicus resembling the intrathecal baclofen withdrawal syndrome: A case report and review of the literature. J Med Case Rep.2010;4:294. DOI: 10.1186/1752-1947-4-294.
Svetel M, Sternic N, Pejovic S, Kostic VS. Penicillamine-induced lethal status dystonicus in a patient with Wilson’s disease. Mov Disord. 2001;16:568–9.
Termsarasab P, Frucht SJ. Dystonic storm: A practical clinical and video review. Journal of Clinical Movement Disorders 2017;4:10. DOI: 10.1186/s40734-017-0057-z
Allen NM, Lin JP, Lynch T,King MD. Status dystonicus: A practice guide. Developmental Medicine & Child Neurology.2014;56:105–12.
Edevelopmental medicine & child neurology velopmental medicine & child. Int J Clin Pediatr. 2017;6(3-4):51-53.
Carmana KB, Yildirimb GK,KiralcE, Kocaka O , Gurlevika SL, Yarara C, et al. Status Dystonicus: A Rare Presentation of Molybdenum Cofactor Deficiency. Int J Clin Pediatr. 2017;6(3-4):51-53.
Atwal PS, Scaglia F. Molybdenum cofactor deficiency. Mol Genet Metab. 2016;117(1): 1-4.
Ichida K, Aydin HI, Hosoyamada M, Kalkanoglu HS, Dursun A, Ohno I, et al. A Turkish case with molybdenum cofactor deficiency. Nucleosides Nucleotides Nucleic Acids. 2006;25(9-11):1087-91.
Kikuchi K, Hamano S, Mochizuki H, Ichida K, Ida H. Molybdenum cofactor deficiency mimics cerebral palsy: Differentiating factors for diagnosis. Pediatr Neurol. 2012; 47(2):147-9.
Alkufri F, Harrower T, Rahman Y, Hughes E, Mundy H, Knibb JA, et al. Molybdenum cofactor deficiency presenting with a parkinsonism-dystonia syndrome. Mov Disord. 2013;28(3):399-401.
Badheka R, Barad NK, Sankhla CS. Pediatric movement disorders . Neurology India. 2018;66(7):59-67.
Walcott BP, Nahed BV, Kahle KT, Duhaime AC, SharmaN, Eskandar EN. Deep brain stimulation for medicallyrefractory life-threatening status dystonicus in children. J NeurosurgPediatr2012;9:99–102.
Lumsden DE, Lundy C, Fairhurst C, Lin JP. Dystonia severity action plan: A simple grading system for medical severity of status dystonicus and life-threatening dystonia. Dev Med Child Neurol. 2013;55: 671–2.
Kyriagis M, Grattan-Smith P, Scheinberg A, Teo C, Nakaji N, Waugh M. Status dystonicus and Hallervorden-Spatz disease: Treatment with intrathecal baclofen and pallidotomy. J Paediatr Child Health.2004;40:322–5.