Objective: To diagnose and characterize Non-ketotic Hyperglycemic Hemichorea as a first symptom of type 2 Diabetes using phenomenology and imaging

Background: Non-Ketotic Hyperglycemic Hemichorea is a rare movement disorder associated with uncontrolled diabetes mellitus. It has been reported in type 2 diabetes, and much rare in type 1 diabetes mellitus. It is characterized by hemichorea-hemiballism that resolves with glycemic control, though some cases may be unremitting. It has hardly been reported as the presenting complaint in a new diagnosis of diabetes mellitus. 

Method: We hereby discuss a case of NKHCB as a first complaint in a 52 year old lady not previously known to have diabetes. She presented to our hospital with 1 month history of involuntary movements of the right upper and lower limbs. She had no history of trauma, stroke or hypertension, and she was alert. The movements interfered with feeding and ambulation. Her examination revealed right-sided periodic choreiform and ballistic movements, suppressed by sleep. She had normal motor power, reflexes and preserved sensation over all dermatomes.

Results: Chemisty revealed normal sodium of 136mmol/l, BUN of 7.0mmol/L, calcium of 2.30mmol/L, and creatinine of 81umol/L. Her random blood glucose was 33.0mmol/L, and HbA1c of 11.0%. Her blood count showed white blood cell of 7.5 with no left shift, hemoglobin of 14.2g/dl and ESR of 14mm/hr. She had normal thyroid and hepatic function panels. CSF studies revealed normal proteins and glucose. The CT scan did not reveal any abnormality. An MRI showed a T1 hyperintensity on the left lentiform nucleus, with normal attenuation in T2 and DW sequences. Our patient was initiated on regular insulin with dose titration until euglycemia was achieved. She was also started on haloperidol due to the distressing abnormal movements, but this was withdrawn after 3 days because of adverse effects. The symptoms gradually improved with a complete resolution at 6 weeks. Her glycemic control remains excellent on lantus (glargine) insulin and metformin.

Conclusion: In conclusion, non-ketotic hyperglycemic hemichorea is a rare but important differential to consider in patients presenting with hemichorea-hemiballism. Prompt diagnosis carries an excellent diagnosis. All patients with this presentation should be screened for diabetes mellitus.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/non-ketotic-hyperglycemic-hemichorea-as-an-initial-manifestation-of-type-2-diabetes/