dystonia is a neurologic disorder characterized by sustained muscle contractions producing torsional and repetitive movements or abnormal postures . it can be classified as focal , multifocal , segmental , hemi - dystonia , or generalized . in children generalized dystonia midazolam is a short - acting benzodiazepine used for induction and maintenance of general anesthesia and for sedation in palliative care . several adverse reactions have been reported after midazolam use , which include agitated excitement , mental confusion , and extrapyramidal reactions , such as dystonia , athetoid movements , and tremor . midazolam - induced acute dystonia was rarely reported in the literature.[35 ] we report midazolam - induced acute dystonia in a 6-year - old girl reversed by diazepam . this is possibly the youngest case reported in the literature that developed acute dystonic reaction after midazolam injection and the first case reversed by diazepam . a previously healthy 6-year - old girl was given intravenous ( iv ) midazolam ( 0.2 mg / kg ) as a sedative prior to removing foreign body from external ear canal , without any other premedication . the dystonic contractions persisted despite administration of flumazenil ( 0.01 mg / kg ) and biperiden lactate ( 2.5 mg ) iv twice . there were no history of consanguinity , psychiatric disorder or neurological disorders , including dystonia , in the family . the vital signs of the patient were in normal ranges and she was alert and oriented . she was in opisthotonus posture and there were dystonic contractions in lower and upper extremities . the complete blood count , liver and renal function tests , electrolytes and glucose concentrations were normal . the patient was given diazepam 0.2 mg / kg iv , since dystonic contractions persisted despite flumazenil ( 0.01 mg / kg ) and biperiden lactate ( 2.5 mg ) administration two times . diazepam was repeated at 4 hours intervals , because dystonic contractions relapsed 4 hours after the first diazepam administration . two days after the recovery from the dystonic contractions diazepam was stopped . by the fourth day of the hospitalisation serum ck decreased to normal range . secondary dystonia was excluded with the normal images at basal ganglia on mri [ figure 1 ] . primary dystonia ( dyt-1 , dyt-6 , and dyt-7 dystonia ) is attributed to genetic causes . secondary dystonia may be caused by metabolic diseases , trauma , toxins , infections , and drugs . primary dystonia was excluded because there was no history of consanguinity and neurological disorders including dystonia in the family , and brain mri of the patient were normal . midazolam is a short - acting , water - soluble benzodiazepine used for induction and maintenance of general anesthesia and for sedation in palliative care . its use is associated with some untoward reactions including mental confusion , agitation , uncooperativeness , dystonic contractions , muscle tremor , athetosis , and laryngospasm.[35 ] there is limited data about the extrapyramidal side effects of midazolam in the literature.[47 ] acute dystonic reaction following iv sedation with midazolam , prior to upper gastrointestinal endoscopy , has been reported in an 14-year - old boy . extrapyramidal side effects following the subcutaneous injection of midazolam has been reported in an 82-year - old man . in both the cases , during the dystonic reactions , the patient remained alert and oriented but in an opisthotonus posture and there were dystonic contractions in lower and upper extremities . age , sex , obesity , drugs , and ethnicity have been shown to affect the half - life of midazolam . extrapyramidal side effects have been reported to disappear completely in a few days after the cessation of midazolam treatment . the half - life of midazolam may have been prolonged in our patient due to an unestablished factor . facilitation of the inhibitory effects of gama - aminobutyric acid ( gaba ) or anticholinergic effects in the central nervous system may be the underlying mechanisms of the extrapyramidal reactions induced by midazolam . gabaa has many subunits , which indicate the structural heterogeneity of the gabaa receptors.[1012 ] flumazenil is a potent gaba antagonist , and is used to antagonize the midazolam - induced extrapyramidal side effects.[47 ] we used diazepam in our patient since acute dystonic reaction persisted despite flumazenil administration . although diazepam does not act directly on the gabaa receptor , benzodiazepine receptor is part of the same ionophore as the gabaa receptor and facilitates the opening of the chloride channel . the antidyskinetic effect of diazepam may be explained by the structural heterogeneity of gabaa receptors . in conclusion although it remains to be shown how diazepam successfully treated it , diazepam may be an effective treatment option in patients who are unresponsive to flumazenil .
midazolam can induce acute dystonia in childhood . 
 we report the development of acute dystonia in a 6-year - old girl after receiving midazolam as a sedative . 
 dystonic contractions persisted despite flumazenil and biperiden lactate injections and the patient was treated with diazepam . 
 acute dystonia was rapidly abolished after the administration of diazepam intravenously . 
 diazepam may be an effective treatment option in patients who are unresponsive to flumazenil .