Objective: To present a unique case of a patient with hyperglycemic associated chorea showing no improvement during a long-term follow-up, despite a strict glycemic control.

Background: Chorea hyperglycemia basal ganglia syndrome (CHBS) is a rare disorder accounting for only 1% of the total chorea-ballism cases. The knowledge of this disorder is based on case reports and case series sharing distinctive clinical and radiological features. It is characterized by acute or subacute, most often unilateral, evolvement of chorea/ballism after nonketotic hyperglycemic episode, MRI characteristic features mainly of T1-hyperintense signal in the basal ganglia, and alleviation of symptoms as glycemic control is achieved. The pathophysiology of CHBS is poorly understood.

Method: We report a poorly controlled diabetic patient who developed permanent CHBS.

Results: A 76-year-old poorly controlled diabetic female, presented with ataxia, dysarthria and nonketotic hyperglycemia, her blood glucose level was up to 700 mg/dl, basic HbA1C was 12.5%. CT scan demonstrated chronic ischemic changes in the left parietal lobe not thought to be contributing to her symptoms. Several days after her admission she developed choreiform movements more prominent on the lower limbs, rigid tone, hypomimia and cognitive decline. MRI scan demonstrated T2 widespread white matter hyperintensity, T1 hyperintense and T2 hypointense abnormalities in the basal ganglia bilaterally consistent with a metabolic disorder. An extensive workup was unremarkable. A gradual control of her blood sugar was started achieving HbA1C of 7%. During 18-months of follow-up no improvement in symptoms including chorea was seen despite a strict glycemic control.

Conclusion: The emergence of chorea in uncontrolled diabetic patient should raise the suspicion of CHBS in the setting of characteristic T1 MRI hyperintensity in the basal ganglia. Although mostly a benign disorder improving as the glucose levels are controlled, in accordance to this case, we conclude that CHBS might be complicated by a permanent chorea and extrapyramidal features attributed to an irreversible damage to the basal ganglia. Therefore, an early recognition and treatment, mainly strict glycemic control, is of major importance in order to avoid complications.

References: 1. Ryan C, Ahlskog JE, Savica R. Hyperglycemic chorea/ballism ascertained over 15 years at a referral medical center. (2018) Parkinsonism Relat Disord; 48:97-100. 2. Zhou Y. Hemichorea in nonketotic hyperglycemia: Putamenal and cerebellum lesion on MR imaging. (2012) World Journal of Neuroscience. 02 (02). 3. Wintermark M, Fischbein NJ, Mukherjee P et-al. Unilateral putaminal CT, MR, and diffusion abnormalities secondary to nonketotic hyperglycemia in the setting of acute neurologic symptoms mimicking stroke. (2004) AJNR Am J Neuroradiol. 25 (6): 975-6. 4. Lai PH, Tien RD, Chang MH et-al. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. (1996) AJNR Am J Neuroradiol. 17 (6): 1057-64.

« Back to 2019 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/permanent-chorea-in-76-years-old-female-with-poorly-controlled-diabetes/