ArticleThe Journal of clinical endocrinology and metabolism2026
Case Series of 46 Patients With Nonketotic Hyperglycemia-associated Chorea: A Retrospective Follow-up Study.
Article in The Journal of clinical endocrinology and metabolism, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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4 citing papers in PubMed.
- Hemichorea associated with nonketotic hyperglycemia: A case report with unremarkable neuroimaging at presentation.The Journal of international medical research · 2026Article
- Acupuncture Treatment for Ipsilateral Hemichorea Associated With Non-Ketotic Hyperglycemia: A Case Report.Clinical case reports · 2026Article
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Abstract
backgroundNonketotic hyperglycemia-associated chorea (NKHC) is a rare but underrecognized complication of diabetes mellitus, with an unclear pathogenesis and variable clinical presentations. Recent studies highlight the role of metabolic disturbances and cerebrovascular pathophysiology in its development. RESEARCH DESIGN AND
methodsTo investigate the clinical, imaging, and therapeutic characteristics of 46 patients with NKHC, integrating multimodal neuroimaging and long-term follow-up data from 2013 to 2022. This multicenter retrospective study enrolled patients with acute-onset hemichorea/hemiballismus, nonketotic hyperglycemia (random blood glucose ≥11.1 mmol/L), and basal ganglia abnormalities on magnetic resonance imaging (MRI). Clinical outcomes, metabolic profiles, and neuroimaging features (including T1-weighted hyperintensity, dynamic contrast-enhanced-MRI, and magnetic resonance spectroscopy) were systematically analyzed.
resultsThe cohort exhibited a mean age of 68.6 ± 10.4 years (range 48-89), with equal sex distribution. Hyperglycemia (mean glycated hemoglobin 11.4% ± 3.8%) and T1-weighted hyperintensity in the contralateral basal ganglia were universal (91.3% on MRI). Notably, 26.1% of patients presented with normoglycemia at admission yet exhibited typical chorea, challenging the "hyperglycemia-first" paradigm. Follow-up MRI revealed dynamic changes in T1 signal intensity, with 2 cases demonstrating symptom exacerbation despite signal resolution. NKHC manifests heterogeneous clinical and imaging phenotypes, suggesting a multifactorial pathogenesis involving metabolic derangement, cerebrovascular insufficiency, and blood-brain barrier disruption. Normoglycemic presentations and paradoxical imaging changes warrant further mechanistic exploration.
conclusionTo our knowledge, this study represents the largest case series of NKHC to date, offering valuable insights into the clinical and imaging characteristics of this condition.
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