Introduction
Type 1 diabetes (T1D) is a common chronic disease in childhood. In 2015, the incidence of T1D in youth in the United States was 22.3 per 100,000[1] and the incidence has been reported to by increasing by 2-3% every year [2]. Most of the patients with T1D present with typical symptoms of hyperglycemia like polyuria and polydipsia, however around 30% present in diabetic ketoacidosis (DKA)[3]. Neurologic complications are relatively rare but can be present at initial diagnosis like lethargy, decreased level of consciousness, and coma. Other rare neurologic presentations that are more commonly seen in adult patients with T1D include cerebellar ataxia, hemiballismus-chorea, peripheral neuropathy, hyperosmolar coma, and stroke at the time of diagnosis; however these tend to be rarely reported in children[4]. Furthermore, the metabolic derangements and dehydration associated with the severity of DKA have been reported to affect brain structure resulting in different neurological sequelae [5, 6]. While nonketotic hyperglycemic hemichorea-hemiballismus (NHHH) has been reported in adults as a neurologic complication of uncontrolled type 2 diabetes (T2D), it’s rather rarely reported in children where T1D is much more common than T2D. NHHH, also known as diabetic striatopathy, was first described in 1960 [7] and is a movement disorder characterized by a typical triad of involuntary movements, striatal abnormalities on neuroimaging and hyperglycemia in the setting of uncontrolled or newly diagnosed diabetes mellitus[8]. This condition is rare and has been described in the elderly population with T2D with an estimated incidence of less than 1:100,000 [9]. NHHH has only been reported to occur in a few pediatric patients [10]. We describe the case of an adolescent boy who presented to our institution with NHHH after a recent diagnosis of T1D.