SAT-647 Dancing with Diabetes: Unmasking Diabetic Striatopathy as a Rare Cause of Hemichorea

Abstract Disclosure: S. Iftikhar: None. K. Zulqadar: None. T. Jain: None. R. Kalas: None. V. Pao: None. Introduction: Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus(DM), characterized by hemichorea/ hemiballism—jerky, involuntary movements affecting one side of the body. It is typically linked to severe hyperglycemia without ketonemia and elevated HbA1c. Imaging findings include striatal hyperdensity on CT and T1-weighted hyperdensity on MRI. Due to its resemblance to other neurological conditions, DS is often misdiagnosed. Management with insulin and hydration usually leads to significant symptom improvement. Case Description: A 72-year-old woman with uncontrolled type 2 diabetes, hypertension, and hyperlipidemia presented to the emergency department with disabling right-sided jerking movements that began the night before. Her vital signs were stable except for elevated blood pressure of154/73 mm Hg. Examination revealed intermittent choreiform movements of the right arm and leg, with otherwise intact neurological function. Laboratory tests showed a blood glucose of 474 mg/dL, an anion gap of 13.5, bicarbonate of 26.5, and negative blood acetone levels, effectively ruling out diabetic ketoacidosis (DKA). Her HbA1c was significantly elevated at 15.8%, reflecting poorly controlled diabetes. Head CT revealed hyperintensity in the left caudate nucleus, raising initial concern for a left basal ganglia stroke. She was started on aspirin and atorvastatin and admitted to the stroke unit. However, an MRI on day 2 confirmed T1 hyperintensity in the left basal ganglia, consistent with DS, with no evidence of infarction or hemorrhage. The patient was managed with hydration and insulin therapy. Initial treatment for hemichorea with a GABA-receptor agonist was ineffective, and she declined haloperidol due to concerns about side effects. Clonazepam was subsequently initiated, resulting in an improvement of her choreiform movements alongside strict glucose control. Repeat MRI a week later revealed diminished T1 hyperintensity compared to the previous MRI study. Discussion: Diabetic striatopathy (DS) is a rare condition with an estimated prevalence of 1 in 100,000. It is often underdiagnosed due to limited physician awareness and can be misinterpreted as intracerebral hemorrhage due to hyperintensity on CT imaging. The putamen is most commonly affected, alone or in combination with the caudate nucleus. MRI is a more sensitive imaging modality for detecting DS, with a sensitivity of 95.33% compared to 78.9% for CT. The prognosis is excellent, with one case series reporting that 74% of patients achieved complete resolution of chorea within a period ranging from 1 hour to 10 months following appropriate glycemic management. This case report aims to raise awareness of a potential differential diagnosis for movement disorders, particularly in patients with poorly controlled diabetes. Presentation: Saturday, July 12, 2025

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Journal of the Endocrine Society
Published
2025-10-01
DOI
https://doi.org/10.1210/jendso/bvaf149.1170
Primary Topic
Neurological and metabolic disorders
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article
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article

SAT-647 Dancing with Diabetes: Unmasking Diabetic Striatopathy as a Rare Cause of Hemichorea

Vivian Y Pao, Rama Kalas, Kainat Zulqadar, Tanvi Jain et al.
Journal of the Endocrine Society
Neurological and metabolic disorders
article

SAT-647 Dancing with Diabetes: Unmasking Diabetic Striatopathy as a Rare Cause of Hemichorea

Vivian Y Pao, Rama Kalas, Kainat Zulqadar, Tanvi Jain, Sara Iftikhar
article en

Abstract

Abstract Disclosure: S. Iftikhar: None. K. Zulqadar: None. T. Jain: None. R. Kalas: None. V. Pao: None. Introduction: Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus(DM), characterized by hemichorea/ hemiballism—jerky, involuntary movements affecting one side of the body. It is typically linked to severe hyperglycemia without ketonemia and elevated HbA1c. Imaging findings include striatal hyperdensity on CT and T1-weighted hyperdensity on MRI. Due to its resemblance to other neurological conditions, DS is often misdiagnosed. Management with insulin and hydration usually leads to significant symptom improvement. Case Description: A 72-year-old woman with uncontrolled type 2 diabetes, hypertension, and hyperlipidemia presented to the emergency department with disabling right-sided jerking movements that began the night before. Her vital signs were stable except for elevated blood pressure of154/73 mm Hg. Examination revealed intermittent choreiform movements of the right arm and leg, with otherwise intact neurological function. Laboratory tests showed a blood glucose of 474 mg/dL, an anion gap of 13.5, bicarbonate of 26.5, and negative blood acetone levels, effectively ruling out diabetic ketoacidosis (DKA). Her HbA1c was significantly elevated at 15.8%, reflecting poorly controlled diabetes. Head CT revealed hyperintensity in the left caudate nucleus, raising initial concern for a left basal ganglia stroke. She was started on aspirin and atorvastatin and admitted to the stroke unit. However, an MRI on day 2 confirmed T1 hyperintensity in the left basal ganglia, consistent with DS, with no evidence of infarction or hemorrhage. The patient was managed with hydration and insulin therapy. Initial treatment for hemichorea with a GABA-receptor agonist was ineffective, and she declined haloperidol due to concerns about side effects. Clonazepam was subsequently initiated, resulting in an improvement of her choreiform movements alongside strict glucose control. Repeat MRI a week later revealed diminished T1 hyperintensity compared to the previous MRI study. Discussion: Diabetic striatopathy (DS) is a rare condition with an estimated prevalence of 1 in 100,000. It is often underdiagnosed due to limited physician awareness and can be misinterpreted as intracerebral hemorrhage due to hyperintensity on CT imaging. The putamen is most commonly affected, alone or in combination with the caudate nucleus. MRI is a more sensitive imaging modality for detecting DS, with a sensitivity of 95.33% compared to 78.9% for CT. The prognosis is excellent, with one case series reporting that 74% of patients achieved complete resolution of chorea within a period ranging from 1 hour to 10 months following appropriate glycemic management. This case report aims to raise awareness of a potential differential diagnosis for movement disorders, particularly in patients with poorly controlled diabetes. Presentation: Saturday, July 12, 2025

Journal of the Endocrine SocietyVol. 9(Supplement_1)
Greater Baltimore Medical Center (US)
Openalex Percentile: Top 59%
Neurological and metabolic disorders
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