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.