Objective: Autonomic and peripheral neurologic manifestations of hyperglycemia are commonly seen in clinical practice; acute hyperglycemia-induced involuntary movements (HIIM) are rarer and less well known. In this article we describe 2 patients with HIIM and review the scientific literature to better characterize the clinical and pathophysiologic features of these disorders.Methods: A literature search was performed using the PubMed database. Whenever possible, the most recent publication on a topic was utilized, with a 20-year cutoff time since publication. Articles were selected based on the quality of presented data or citations utilized.Results: HIIM can present in a variety of ways including seizures, hemichorea-hemiballismus (HCHB) and, very rarely, tremor. While HIIM are more commonly seen in patients with long-standing, uncontrolled diabetes, they can also be the initial presentation of diabetes. The precise pathophysiology of HIIM remains unknown; however, at least for HCHB, it is hypothesized that hyperglycemia increases the metabolism of the inhibitory neurotransmit-Abbreviations: HCHB = hemichorea-hemiballismus; HIIM = hyperglycemia-induced involuntary movements; GABA = gamma aminobutyric acid; EPC = epilepsia partialis continua; MRI = magnetic resonance imaging; NKH = nonketotic hyperglycemia