Hemichorea/Hemiballism Associated with Hyperglycemia: Report of 20 Cases

Hemichorea-Hemiballism as a Manifestation of Hyperglycemia

“…Hemichorea-hemiballism associated with hyperglycemia (HHAH) was first described in 1960. However, no more than 200 cases have been reported ever since [ 1 ]. HHAH is characterized by the sudden occurrence of hemichorea, or its more severe expression hemiballism, in patients with non-ketotic hyperglycemia [ 2 ].…”

“…Non-ketotic hyperglycemia is the second most frequent cause of the hemichorea-hemiballism syndrome, only after vascular origin [ 3 , 4 ]. The typical triad of HHAH consists of involuntary movements, striatal abnormalities on neuroimaging, and non-ketotic hyperglycemia with known or previous unrecognized diabetes [ 1 , 4 ]. Usually, HHAH occurs unilaterally, but in some cases, it can occur bilaterally [ 5 ].…”

“…Type 2 and less frequently type 1 diabetes are associated with HHAH. Diabetes was newly diagnosed in several cases of HHAH [ 1 ]. Laboratory findings in reviewed literature include a series of 53 patients with HHAH from Oh et al with mean serum glucose level upon admission of 481.5 mg/dL (ranging from 169 to 1264 mg/dL) and mean glycated hemoglobin (HbA1c) of 14.4% (ranging from 9.9% to 19.2%) [ 6 ].…”

“…The main treatment of HHAH is the normalization of blood glucose. Hemichorea-hemiballism slowly improves in the days after serum glucose correction [ 1 , 6 , 9 ]. In addition, neuroleptics are often used to expedite symptomatic resolution, as the symptoms of HHAH can cause discomfort to the patients and, in extreme cases, lead to rhabdomyolysis and renal failure.…”

“…In addition, neuroleptics are often used to expedite symptomatic resolution, as the symptoms of HHAH can cause discomfort to the patients and, in extreme cases, lead to rhabdomyolysis and renal failure. In some series, different combinations of glycemia control, neuroleptics, dopamine-depleting agents, benzodiazepines, and anticonvulsants were used [ 1 , 9 ].…”