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Nonketotic Hyperglycemic Hemichorea

Published:September 25, 2022DOI:https://doi.org/10.1016/j.amjmed.2022.09.008
      To the Editor:

      Case Report

      A 72-year-old man with type 2 diabetes mellitus (DM) and hyperlipidemia presented with a fall. He reported 1 year of medication noncompliance. He was normotensive and afebrile. Examination revealed bilateral forearm abrasions and normal findings of his neurological, cardiovascular, respiratory, and abdominal systems.
      He was hyperglycemic (serum glucose 37.4 mmol/L) and calculated effective serum osmolality was 296 mOsm/kg. Venous blood gas showed pH 7.4, and urine was negative for ketones. Glycated hemoglobin exceeded range at >15%. Chest radiograph showed new left lower zone consolidation. Brain computed tomography (CT) showed increased attenuation of the right globus pallidus and putamen (Figure).
      Figure
      FigureAxial noncontrast computed tomography (A) and T1-weighted magnetic resonance imaging (B) of the brain demonstrates increased attenuation and hyperintensity respectively of the right globus pallidus and putamen (arrows).
      He was given oral antibiotics for left lower zone pneumonia, started on subcutaneous insulin, and discharged.
      One month later, he presented with 1 day of continuous left-sided involuntary movements. Examination showed involuntary random jerking movements of the left upper limb and lower limb, with variable speed, timing, and direction. Investigations showed hyperglycemia (serum glucose 13 mmol/L) and repeat glycated hemoglobin was 12.7%. Electrolytes, renal, thyroid, and liver function tests were within normal ranges. Electroencephalography showed no ictal or interictal discharges. Brain magnetic resonance imaging (MRI) showed T1-weighted hyperintensity of the right globus pallidus and putamen (Figure).
      He was diagnosed with nonketotic hyperglycemic hemichorea and treated with clonazepam and risperidone and compliance to insulin was reinforced. Two months later, the hemichorea and blood glucose control improved.

      Discussion

      Our patient had an uncommon initial presentation because he presented radiographically before the clinical symptom of hemichorea. This alerts clinicians to consider past medical history when presented with a patient with hemichorea.
      Nonketotic hyperglycemic hemichorea, also known as diabetic striatopathy, is an uncommon condition of hyperglycemia associated with chorea or ballism and unique imaging abnormalities of the basal ganglia on CT or MRI, with prevalence of 1 in 100,000.
      • Chua CB
      • Sun CK
      • Hsu CW
      • Tai YC
      • Liang CY
      • Tsai IT.
      "Diabetic striatopathy": clinical presentations, controversy, pathogenesis, treatments, and outcomes.
      Pathogenesis is hypothesized to be depletion of gamma-aminobutyric acid (GABA) due to upregulation of an alternative anaerobic pathway in the Krebs cycle, leading to disinhibition of the subthalamus and basal ganglia associated with hyperkinetic movement.
      • Oh SH
      • Lee KY
      • Im JH
      • Lee MS.
      Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.
      It occurs predominantly in elderly females with type 2 diabetes mellitus.
      • Abe Y
      • Yamamoto T
      • Soeda T
      • et al.
      Diabetic striatal disease: clinical presentation, neuroimaging, and pathology.
      Chorea or ballism may affect arm, leg, or face either unilaterally or bilaterally, with arm-leg combination most frequently affected.
      • Chua CB
      • Sun CK
      • Hsu CW
      • Tai YC
      • Liang CY
      • Tsai IT.
      "Diabetic striatopathy": clinical presentations, controversy, pathogenesis, treatments, and outcomes.
      Neuroimaging findings include hyperdense unilateral or bilateral lesions of the basal ganglia on CT and hyperintense signals in the putamen on T1-weighted MRI and of variable intensity on T2-weighted MRI, usually contralateral to the clinical symptom.
      • Hegde AN
      • Mohan S
      • Lath N
      • Lim CC.
      Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus.
      Unlike cerebral hemorrhage, there is absence of internal capsule involvement, edema, and space-occupying effect.
      • Zheng W
      • Chen L
      • Chen JH
      • et al.
      Hemichorea associated with non-ketotic hyperglycemia: a case report and literature review.
      Chorea and imaging anomalies do not appear concomitantly in 9% of patients, and 2% may show striatal involvement without clinical manifestation of chorea
      • Chua CB
      • Sun CK
      • Hsu CW
      • Tai YC
      • Liang CY
      • Tsai IT.
      "Diabetic striatopathy": clinical presentations, controversy, pathogenesis, treatments, and outcomes.
      such as in our patient initially.
      Management entails correction of hyperglycemia and may require anti-]chorea medications including antipsychotics, GABA-receptor agonists, selective serotonin reuptake inhibitors, and dopamine-depleting agents.
      • Das L
      • Pal R
      • Dutta P
      • Bhansali A.
      "Diabetic striatopathy" and ketoacidosis: report of two cases and review of literature.
      Deep brain stimulation
      • Son BC
      • Choi JG
      • Ko HC.
      Globus pallidus internus deep brain stimulation for disabling diabetic hemiballism/hemichorea.
      has also been reported.

      References

        • Chua CB
        • Sun CK
        • Hsu CW
        • Tai YC
        • Liang CY
        • Tsai IT.
        "Diabetic striatopathy": clinical presentations, controversy, pathogenesis, treatments, and outcomes.
        Sci Rep. 2020; 10: 1594https://doi.org/10.1038/s41598-020-58555-w
        • Oh SH
        • Lee KY
        • Im JH
        • Lee MS.
        Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.
        J Neurol Sci. 2002; 200: 57-62https://doi.org/10.1016/s0022-510x(02)00133-8
        • Abe Y
        • Yamamoto T
        • Soeda T
        • et al.
        Diabetic striatal disease: clinical presentation, neuroimaging, and pathology.
        Intern Med. 2009; 48: 1135-1141https://doi.org/10.2169/internalmedicine.48.1996
        • Hegde AN
        • Mohan S
        • Lath N
        • Lim CC.
        Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus.
        Radiographics. 2011; 31: 5-30https://doi.org/10.1148/rg.311105041
        • Zheng W
        • Chen L
        • Chen JH
        • et al.
        Hemichorea associated with non-ketotic hyperglycemia: a case report and literature review.
        Front Neurol. 2020; 11: 96https://doi.org/10.3389/fneur.2020.00096
        • Das L
        • Pal R
        • Dutta P
        • Bhansali A.
        "Diabetic striatopathy" and ketoacidosis: report of two cases and review of literature.
        Diabetes Res Clin Pract. 2017; 128: 1-5https://doi.org/10.1016/j.diabres.2017.03.008
        • Son BC
        • Choi JG
        • Ko HC.
        Globus pallidus internus deep brain stimulation for disabling diabetic hemiballism/hemichorea.
        Case Rep Neurol Med. 2017; 20172165905https://doi.org/10.1155/2017/2165905