Hemichorea-hemiballismus as the presenting manifestation of nonketotic hyperglycemia in an adolescent with undiagnosed type 2 diabetes mellitus

Nonketotic Hyperglycemic Chorea in a 10-Year-Old Asian Boy with Diabetes Mellitus

“…5,6 There were six previous case reports involving children and adolescents (►Table 2). 2,5,[7][8][9][10] The cases were predominantly in adolescents with abnormalities on imaging studies.…”

Pediatric Hemichorea-Hemiballismus Associated with Nonketotic Hyperglycemia: A Case Report and Review of the Literature

“…1,2,5,6 Including our case, HNKH has been reported as an initial manifestation of DM in five (71.4%) of the seven reported pediatric cases to date. 3,4 The mean age of the pediatric HNKH patients was 13.71 ± 2.92 years (Table 1). 3,4 Our case was diagnosed 3 weeks after the onset of abnormal involuntary movements.…”

“…3,4 The mean age of the pediatric HNKH patients was 13.71 ± 2.92 years (Table 1). 3,4 Our case was diagnosed 3 weeks after the onset of abnormal involuntary movements. HNKH is commonly misdiagnosed on the initial evaluation, 6 with a delay of up to 6 months reported.…”

“…[1][2][3][4][5][6] Haloperidol, clonazepam and valproate are commonly used drugs. [1][2][3][4][5][6] Including our case, five (71.4%) of the seven pediatric cases reported amelioration of involuntary movements within a month of onset. 3,4 It remains unclear which clinical factors could predict the final clinical outcome.…”

Chorea associated with nonketotic hyperglycemia (diabetic striatopathy) in an elderly male

“…Hemichorea-hemiballismus, as a manifestation of NKH, is a rarely described entity, typically seen in elderly Asian women with type 2 diabetes, though very rarely it has also been reported in type 1 diabetes, those with diabetic ketoacidosis and in children. [23] It is characterized by high signal on T1-weighted MRI, which is likely due to accumulation of lipid-laden macrophages, confined to the striatum. Most accepted pathophysiological mechanism in diabetic striatopathy involves hyperviscosity leading to local tissue hypoperfusion, depletion of gamma-Aminobutyric acid, and accumulation of manganese-containing gemistocytes in the basal ganglia which typically appear as T1 hyperintense lesions.…”