Hemichorea Hyperglycaemia Basal Ganglia Syndrome in a Patient with Type 1 Diabetes Mellitus: A Rare Case Report

Abstract

Hyperglycaemia induced involuntary movements are uncommon in clinical practice though Chorea Hyperglycaemia Basal Ganglia syndrome (CHBG) or Non-ketotic Hyperglycaemic Hemichorea (NHH) is being reported with increasing frequency due to the clinical awareness and widespread availability of neuroimaging. Prompt recognition of CHBG is essential, since correction of hyperglycaemia usually leads to early resolution of the involuntary movements. It is usually seen in elderly patients with uncontrolled Type 2 diabetes mellitus who present acutely with hemichorea or hemiballismus. It is rarely reported in Type 1 diabetes mellitus. Here, the author presents a 25-year-old male patient diagnosed with Type 1 diabetes mellitus with persistent chorea involving the left upper and lower extremity in whom the movement disorder persisted despite correction of hyperglycaemia.

Chorea Hyperglycaemia Basal Ganglia Syndrome in an Uncontrolled Diabetic Patient

“…CHBG warrants awareness as a complication of long standing poorly controlled diabetes mellitus, usually occurring in type 2 diabetes mellitus, with few case reports in type 1 diabetes patients also described [1,2] .Most of the available case reports are from Asian population and elderly females which probably indicates a genetic predisposition [3] . It is usually reported in elderly females with hyperglycaemic hyperosmolar state and rare in the setting of diabetic ketoacidosis [4] .…”

“…There are various causes like haemorrhagic or ischemic stroke, neoplasm, SLE, Huntington's disease, Sydenham's chorea, HIV, hyperthyroidism, trauma and drug toxicity. CHBG can occur both in patients with long standing uncontrolled diabetes mellitus or as an initial manifestation of hyperglycaemia leading to the diagnosis of diabetes mellitus [3] . Our patient was diagnosed as CHBG after ruling out the above causes.…”

“…In nonketotic hyperglycaemia, insufficient ketoac-etate for production of GABA and acetylcholine resynthesis can lead to lack of these neurotransmitters. Depletion of GABA and acetylcholine within the basal ganglia may lead to the onset of chorea [2,3] . Some studies also suggest the possibility that the metabolic disturbances unmask previous established asymptomatic striatum vasculopathy.…”

“…[5] The radiological findings in CHBG syndrome include hyperintensities in striatum and Globus-pallidum in T1 weighted MRI. In a study of 20 cases of chorea associated with hyperglycaemia, almost all the patients had striatal abnormalities on neuroimaging which was either contralateral or bilateral in few cases [3] . These patients usually have partial or complete resolution of symptoms with adequate blood sugar control recovery usually occurring within 48hrs, however can take 4-6 weeks for improvement of symptoms.…”

“…Brain is susceptible to injury by various metabolic disorders, one of them being uncontrolled blood sugar levels which affect the basal ganglia leading to involuntary movements. [2] Chorea Hyperglycaemia Basal Ganglia syndrome (CHBG) is a rare condition that occurs in uncontrolled Type 2 diabetes mellitus and characterised by hemichorea/hemiballismus with non-ketotic hyperglycaemia [3] . The main importance of identifying this cause is, it is reversible with adequate glycaemic control.…”