Rare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome

Abstract

An 83-year-old woman presented with acute-onset haemichorea and haemiballism particularly affecting the left side of the body. She was known to have type 2 diabetes, which was poorly controlled with sitagliptin. She was hyperglycaemic but not ketotic or acidotic. After she was started on insulin and good glycaemic control was achieved, her abnormal movements dramatically improved. MRI of the brain showed a T1-weighted hyperintense lesion on the right basal ganglia, which is typical of chorea-hyperglycaemia-basal ganglia syndrome. Other causes of chorea, for example, Huntington's disease, Sydenham chorea, Wilson's disease, malignancy, systemic lupus erythematosus, haemorrhage/infarction, thyroid dysfunction, drug-induced chorea and antiphospholipid syndrome, were excluded or deemed less likely given her rapid response to achieving near euglycaemia.

A case of chorea-hyperglycemia-basal ganglia syndrome: a rare case of movement disorder

“…3 As per another hypothesis, hyperglycemia induced hyper viscosity causes blood-brain barrier damage resulting in ischemia of striatal neurons. 4 Patients reported are usually elderly with long standing Type 2 diabetes with poor control of blood glucose levels. 1 Involuntary movements resolve with prompt normalisation of blood glucose levels.…”

Hemichorea-hemiballismus due to diabetic striatopathy a serious complication of uncontrolled diabetes

“…These terms include DS, hyperglycaemic nonketotic hemichorea/hemiballismus, hyperglycaemic chorea and chorea-hyperglycaemia-basal ganglia syndrome. [5][6][7] Patients who present with hemiballismus and hyperglycaemia without findings on imaging may be categorised as having 'clinically isolated DS'. Conversely, 'radiologically isolated DS' describes striatal changes in neuroimaging in patients with hyperglycaemia who do not have symptoms of a movement disorder.…”