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Hyperglycemic Hemichorea: A Case Report
Joana Lopes1, Eulália Antunes1, Bárbara Oliveira1
1Internal Medicine, Hospital de Braga, Braga, PRT.
Insights
Non-ketotic hyperglycemic hemichorea-hemiballismus is a rare movement disorder linked to uncontrolled diabetes. Prompt diagnosis and metabolic control, including insulin therapy, can effectively resolve these involuntary movements.
Area of Science:
- Neurology
- Endocrinology
- Radiology
Background:
- Chorea and ballism are hyperkinetic movement disorders with diverse etiologies.
- Non-ketotic hyperglycemic hemichorea-hemiballismus (NHH) is a rare complication of poorly controlled diabetes mellitus.
- NHH presents with characteristic MRI findings in the basal ganglia.
Observation:
- A 74-year-old woman with type 2 diabetes presented with acute, involuntary movements of her left side.
- Her blood glucose was severely elevated (541 mg/dL) without ketosis, and HbA1c was 14%.
- Brain MRI revealed T1 hyperintensity in the right corpus striatum, consistent with NHH.
Findings:
- The patient's involuntary movements resolved after insulin therapy and haloperidol administration.
- Metabolic optimization was key to the successful treatment of the choreiform movements.
- The case highlights the diagnostic utility of neuroimaging in conjunction with clinical presentation.
Implications:
- Early recognition of NHH is crucial for timely intervention and management.
- Decompensated diabetes can manifest as a neurological emergency, necessitating prompt metabolic control.
- Raising awareness for NHH can improve diagnosis and patient outcomes in diabetic populations.
Abstract:
Chorea is a hyperkinetic movement disorder characterized by a continuous flow of rapid, random, and involuntary bodily contractions, usually affecting the distal limbs. When these movements are more proximal or assume a larger amplitude with a flinging or kicking character, they're referred to as ballism. These disorders can be associated with several causes, ranging from genetic and neurovascular to toxic, autoimmune, and metabolic. Non-ketotic hyperglycemic hemichorea-hemiballismus is a rare consequence of decompensated diabetes mellitus with a poorly understood pathogenesis but with characteristic MRI T1 and T2 hyperintense abnormalities in the contralateral basal ganglia. We present the case of a 74-year-old woman with a history of poorly controlled type 2 diabetes mellitus, dyslipidemia, and arterial hypertension who was admitted to the emergency room due to a two-day history of rapid non-stereotypical involuntary movements of the left side of her body. A neurological exam showed large amplitude and repetitive left-side body movements. Glycemia was 541 mg/dL with no ketosis. Her glycosylated hemoglobin was 14%. A brain CT excluded acute abnormalities. Brain MRI showed a discrete T1 hyperintense signal involving the right corpus striatum, compatible with non-ketotic hyperglycemic hemichorea-hemiballism syndrome. After metabolic optimization with insulin and haloperidol, the movements resolved. Early recognition and metabolic control are essential to the resolution of choreiform movements. Our aim is to raise awareness for hyperglycemic hemichorea-hemiballismus, in which decompensated diabetes is the early sign of diagnosis.
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