Paediatric non-ketotic hyperglycaemic hemichorea–hemiballismus

Abstract

Non-ketotic hyperglycaemic hemichorea-hemiballismus (NHHH) is commonly seen among elderly Asian women with type 2 diabetes mellitus. Here, we present a case of a 16-year-old Filipina with type 1 diabetes mellitus who is poorly compliant to her medications and subsequently developed right hemichorea-hemiballismus (HH). She was initially admitted with hyperglycaemia but was negative for ketonuria or metabolic acidosis. Neuroimaging showed bilateral lentiform nuclei and left caudate hyperdensities on CT and T1-weighted hyperintensity on MRI. Blood glucose was controlled with insulin. Haloperidol and clonazepam were started for the HH with gradual resolution of symptoms in 6 weeks. This is the fifth reported case of NHHH seen among the paediatric age group. NHHH in the paediatric population is clinically and radiographically similar to NHHH seen among adults. Correction of hyperglycaemia results in clinical improvement and radiographic resolution of lesions but persistent cases may necessitate specific treatment targeted towards the abnormal movements.

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

“…[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. Neither baseline blood glucose nor clinical duration showed any clear association with the time to recovery or predicted neurologic sequelae.…”

“…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.…”

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.…”