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A 72-year-old man with type 2 diabetes mellitus (DM) and hyperlipidemia presented with a fall. He reported 1 year of medication noncompliance. He was normotensive and afebrile. Examination revealed bilateral forearm abrasions and normal findings of his neurological, cardiovascular, respiratory, and abdominal systems.
He was hyperglycemic (serum glucose 37.4 mmol/L) and calculated effective serum osmolality was 296 mOsm/kg. Venous blood gas showed pH 7.4, and urine was negative for ketones. Glycated hemoglobin exceeded range at >15%. Chest radiograph showed new left lower zone consolidation. Brain computed tomography (CT) showed increased attenuation of the right globus pallidus and putamen (Figure).
FigureAxial noncontrast computed tomography (A) and T1-weighted magnetic resonance imaging (B) of the brain demonstrates increased attenuation and hyperintensity respectively of the right globus pallidus and putamen (arrows).
He was given oral antibiotics for left lower zone pneumonia, started on subcutaneous insulin, and discharged.
One month later, he presented with 1 day of continuous left-sided involuntary movements. Examination showed involuntary random jerking movements of the left upper limb and lower limb, with variable speed, timing, and direction. Investigations showed hyperglycemia (serum glucose 13 mmol/L) and repeat glycated hemoglobin was 12.7%. Electrolytes, renal, thyroid, and liver function tests were within normal ranges. Electroencephalography showed no ictal or interictal discharges. Brain magnetic resonance imaging (MRI) showed T1-weighted hyperintensity of the right globus pallidus and putamen (Figure).
He was diagnosed with nonketotic hyperglycemic hemichorea and treated with clonazepam and risperidone and compliance to insulin was reinforced. Two months later, the hemichorea and blood glucose control improved.
Discussion
Our patient had an uncommon initial presentation because he presented radiographically before the clinical symptom of hemichorea. This alerts clinicians to consider past medical history when presented with a patient with hemichorea.
Nonketotic hyperglycemic hemichorea, also known as diabetic striatopathy, is an uncommon condition of hyperglycemia associated with chorea or ballism and unique imaging abnormalities of the basal ganglia on CT or MRI, with prevalence of 1 in 100,000.
Pathogenesis is hypothesized to be depletion of gamma-aminobutyric acid (GABA) due to upregulation of an alternative anaerobic pathway in the Krebs cycle, leading to disinhibition of the subthalamus and basal ganglia associated with hyperkinetic movement.
Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.
Neuroimaging findings include hyperdense unilateral or bilateral lesions of the basal ganglia on CT and hyperintense signals in the putamen on T1-weighted MRI and of variable intensity on T2-weighted MRI, usually contralateral to the clinical symptom.
Chorea and imaging anomalies do not appear concomitantly in 9% of patients, and 2% may show striatal involvement without clinical manifestation of chorea
Management entails correction of hyperglycemia and may require anti-]chorea medications including antipsychotics, GABA-receptor agonists, selective serotonin reuptake inhibitors, and dopamine-depleting agents.
Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.
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