Abstract
Objective: To explore the clinical manifestation, diagnosis, therapy, and mechanism of hemichorea associated with non-ketotic hyperglycemia (HC-NH) so as to enhance awareness and avoid misdiagnosis or missed diagnosis of the disease.Methods: A case of HC-NH was reported and reviewed in terms of the clinical features, diagnosis and treatment.Results: Hemichorea associated with non-ketotic hyperglycemia is a rare complication of diabetes mellitus, which is commonly seen in elderly women with poorly-controlled diabetes. The condition is characterized by non-ketotic hyperglycemia, unilateral involuntary choreiform movements, and contralateral basal ganglia hyper-intensity by T1-weighted MR imaging or high density on CT scans. Blood glucose control is the basal treatment, in combination with dopamine receptor antagonists and benzodiazepine sedative, in controlling hemichorea.Conclusion: In clinical practice, the possibility of unilateral chorea should be considered for diabetic patients with poor blood glucose control.
Highlights
Hemichorea is usually associated with a contralateral lesion in the central nervous system and can result from infection, genetic mutation, neoplasms, neurodegeneration, stroke, metabolic disease, drug-exposure, and autoimmune disease [1, 2]
Hemichorea associated with non-ketotic hyperglycemia (HC-NH) is a rare complication of diabetes mellitus [3], which is commonly seen in elderly diabetic women with poor blood glucose control
HC-NH mostly occurs in non-ketotic diabetic patients with poor blood glucose control in the past [3], sometimes in ketotic diabetic patients [7], and occasionally in adolescents with newly-diagnosed diabetes [8]
Summary
Hemichorea is usually associated with a contralateral lesion in the central nervous system and can result from infection, genetic mutation, neoplasms, neurodegeneration, stroke, metabolic disease, drug-exposure, and autoimmune disease [1, 2]. Hemichorea associated with non-ketotic hyperglycemia (HC-NH) is a rare complication of diabetes mellitus [3], which is commonly seen in elderly diabetic women with poor blood glucose control. HC-NH was first described in 1960 [4] and is characterized by non-ketotic hyperglycemia, unilateral involuntary choreiform movements, and contralateral basal ganglia hyper-intensity on T1-weighted MR images or high density on CT scans [5]. HC-NH mostly occurs in non-ketotic diabetic patients with poor blood glucose control in the past [3], sometimes in ketotic diabetic patients [7], and occasionally in adolescents with newly-diagnosed diabetes [8]. Most of the hemichorea is found in the course of diabetes in HC-NH patients, and some of the first symptoms precede the discovery of diabetes [9]. Dance movements usually occur on the upper and lower limbs of the ipsilateral side
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