Title : Chorea-hyperglycemia basal ganglia syndrome in a 66-year-old female with uncontrolled type 2 diabetes mellitus: A case report
Abstract:
Introduction. Brain is highly vulnerable to damage from a range of metabolic disorders. Chorea-Hyperglycemia Basal Ganglia Syndrome is a rare and poorly recognized entity that manifests as hyperkinetic movement disorder in a setting of uncontrolled diabetes mellitus. Strict control of blood sugar is the mainstay of treatment.
Case Description. A 66-year-old female with a known history of type 2 diabetes mellitus and hypertension presented with three-week history of bilateral involuntary movements of the extremities characterized as rapid, irregular, semi-purposeful and non-rhythmic, which dissipate during sleep. Neurological examination was unremarkable except for the involuntary movements. 16-channel electroencephalogram and slit-lamp examination was unremarkable. Uncontrolled blood glucose without ketonuria and acidosis was noted. Baseline and repeat cranial computed tomography scan plain showed a faint calcification in bilateral basal ganglia and focal area of hypodensity in the anterior limb of the right internal capsule. Hyperintense signals in bilateral lentiform nuclei in both T1W and T2W/FLAIR was seen on cranial magnetic resonance imaging, which is typical of chorea-hyperglycemia basal ganglia syndrome.2 Patient was initially given a trial of risperidone for the severe involuntary movements however, no noticeable improvement and was subsequently started with insulin therapy to achieve a good glycemic control, which led to a dramatic resolution in her choreiform movements.
Conclusion. This case report highlights the metabolic cause of chorea known as Chorea-Hyperglycemia Basal Ganglia Syndrome (CHBGS). It also emphasizes that this condition can present as bilateral chorea, instead of the more common unilateral presentation. Early recognition and proper treatment of this reversible syndrome can significantly reduce hospital stay and improve the patient's quality of life.
