Objective: We describe two cases of Hemichorea-Hemiballism (HCHB) associated with nonketotic hyperglycemia.

Background: HCHB is defined as continuous, non-patterned, and involuntary movement involving one side of the body. A focal vascular lesion in the contralateral basal ganglia is the most common cause of this dyskinesia. In the absence of an identifiable focal vascular lesion, metabolic disease, brain neoplasm and infectious diseases of the central nervous system are considered to be the more common causes. HCHB has been associated with nonketotic hyperglycemia and particular neuroradiological, brain computed tomography (CT), magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) map findings.

Methods: We reviewed medical records of two patients who were hospitalized with the diagnosis of HCHB associated with hyperglycemia. Two patients with HCHB underwent Brain CT, MRI, DWI, ADC. Routine laboratory tests included measurement of serum glucose, electrolytes, urine ketones, serum ketones and Hemoglobin-A1c levels in two patients.

Results: All patient had elevated Hemoglobin-A1c levels, ranging from 14.5 to 14.6%, without evidence of ketosis. Case 1 and 2, T1-weighted MRI images and brain CT characteristically demonstrates hyperintensity in the contralateral corpus striatum. The DWI images were normal. The ADC values were decreased.

Conclusions: HCHB that accompanies hyperglycemia exhibits characteristic findings on the T1-weighted MRI. The authors report two cases of HCHB associated with non-ketotic hyperglycemia and basal ganglia hyperintensity on the T1-weighted brain MR imaging in 87-year-old-woman and 74-year-old man with non-controlled diabetes mellitus.

References: 1. Lee BC, Hwang SH, Chang GY. Hemiballismus-hemichorea in older diabetic women: a clinical syndrome with MRI correlation. Neurology. 1999;52(3):646-8. 2. Chu K, Kang DW, Kim DE, Park SH, Roh JK. Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome? Arch Neurol. 2002;59(3):448-52.

« Back to 2018 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/two-cases-of-hemichorea-hemiballism-in-nonketotic-hyperglycemia/