Case report of hyperglycemic nonketotic chorea with rapid radiological resolution.

BACKGROUND

Hemichorea is a rare manifestation of nonketotic hyperglycemia that usually affects elderly Asian women with poor glycemic control. Non-contrast computerized Tomography and T1- weighted Magnetic Resonance Imaging shows characteristic hyperintense basal ganglia lesions.

CASE PRESENTATION

A Fifty-seven year old Sri Lankan female presented with a two-day history of right upper limb chorea. She had been diagnosed with diabetes mellitus one year ago, but was not on any treatment and did not have any micro vascular or macro vascular complications. Random blood sugar was 420 mg/dl and full blood count, liver function tests, renal function tests, inflammatory markers, thyroid function tests, Urine protein / creatinine ratio, electrocardiogram and 2D Echo were normal. Arterial blood gas did not show acidosis and ketone bodies were not detected in urine. Non-contrast computerized Tomography brain on day 1 showed left side hyperdense lentiform and caudate nuclei and MRI on day 3 showed slightly high signal intensity of left side basal ganglia on T1- weighted images and low signal intensity on T2-weighted and Fluid-attenuated inversion recovery images. She was started on insulin and a low dose of clonazepam and glycemic control was achieved on day 3. Two days later, the chorea completely disappeared. CT brain was repeated 4 days and 10 days following glycemic control, which showed rapid resolution of CT changes. Clonazepam was stopped in 2 weeks and chorea did not recur.

CONCLUSION

This is a rare manifestation of diabetes in Sri lanka and diagnosing this rare entity will direct clinicians to achieve optimum glycemic control as the treatment which will lead to rapid clinical response without any other medications. In this case report we high light that with the clinical improvement, repeating a CT scan even after a very short period like 2 weeks will show rapid radiological resolution. This repeat imaging can also be useful to confirm the diagnosis, which will minimize unnecessary investigations and treatments. Further cases of hyperglycemic nonketotic chorea with brain imaging performed within short intervals is needed to evaluate the nature of rapid radiological changes, which will be useful to understand the pathology of this condition.