Status dystonicus: Diagnosis and management of a rare and challenging entity

: We report the case of a six-year-old girl with Moyamoya disease who presented with bilateral internal carotid artery malignant infarct following encephaloduroarteriosynangiosis (EDAS). During her neurorehabilitation, she developed gradually worsening dystonic spasms with opisthotonic posturing, tachycardia, tachypnea and desaturation. This rare life threatening movement disorder was diagnosed as status dystonicus based on the history and clinical presentation. Status Dystonicus occurs commonly in children and the etiology is often diverse. It occurs in patients with preexisting dystonia or following an acute central nervous system insult of varied etiology. Status dystonicus is usually precipitated by one or more triggering factors. Rarity and lack of objective criteria for diagnosis often delays the management thereby increasing the risk of mortality and morbidity. Here, we discuss the challenges faced in the diagnosis and management of a child with denovo status dystonicus.