ICNC Abstracts, ICNC 2018

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Management of Status Dystonicus in Children: Reporting of 3 cases with videos.
Kanij Fatema

Last modified: 2018-09-09

Abstract


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 emergency.  Marked, rapid exacerbation of dystonia requires prompt intervention and admission in the hospital.  Clinical features of dystonic storm include fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failure. It is critical to recognize early and differentiate dystonic storm from other hyperkinetic movement disorder emergencies. SD may be refractory to standard drug therapy. Besides  ,as a consequence of the intense muscle activity, metabolic complications such as rhabdomyolysis, leading to acute renal failure, may ensue.

 

We report 3 cases of  who developed severe dystonia culminating in SD necessitating management in hospital setting. We are also reporting the videos of three children for identifying this very debilitating disorder. One patient was treated in intensive care unit but the rest 2 was treated in ward as due to financial constraints. In brief 1st case was a 5 year boy with dyskinetic CP who was treated with trihexiphenidyl (THP), beclofen and midazolam infusion. Second case was a 15 month old boy, diagnosed case of mitochondrial encephalopathy (Leigh disease) who was treated with THP, beclofen, 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.

 

 


Keywords


Status dystonicus, Dystonia, Treatment, Early recognition

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