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Subtle hyperdensity of the right lentiform nucleus (arrow). In the patient's context, it may be related to a chorea hyperglycaemia basal ganglia syndrome-related abnormality.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Non-ketotic hyperglycaemic chorea-ballismus, also known as chorea hyperglycaemia basal ganglia (CHBG), described in 1960, is a rare manifestation of poorly controlled diabetes mellitus that occurs most commonly in women of East Asian descent, with a mean age of 71 years<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> It is characterised by a triad of acute or subacute non-ketotic, hyperglycaemic, chorea-ballismus state and hyperintense striatopathy on MRI or CT scan, most commonly the putamen.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Hemichorea and hemiballismus are hyperkinetic movements affecting the contralateral side to striatal hyperintensity on neuroimaging. Therefore, we report a case of non-ketotic hyperglycaemic chorea-ballismus and discuss the relevant features of its presentation and treatment in the emergency department.</p><p id="par0010" class="elsevierStylePara elsevierViewall">This is a 78-year-old female patient with a personal history of type 2 diabetes mellitus, anticoagulated paroxysmal atrial fibrillation and stage 2–3b chronic kidney disease of mixed vascular aetiology. Basically, the patient was active and independent in all activities of daily living. She presented to the emergency department with a 5-day history of abnormal left-limb movements in the context of elevated blood glucose levels (even >600 mg/dl). On assessment in the emergency department, the patient showed left mandibular movements, chorea and athetosis of the left limbs, with no other significant anomalies (Appendix Video of Supplementary material 1). Blood tests showed glucose 411 mg/dl, Cr 1.62 mg/dl, Na 124 mEq/l, K 5 mEq/l. All other parameters were normal. An imaging study was requested (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) which showed a subtle hyperdensity of the right lenticular nucleus which, in the context of the patient, could be related to an alteration secondary to CHBG syndrome. The patient was admitted to the internal medicine department, with neurological follow-up. The patient's glycaemic control improved during admission. At discharge, symptomatic improvement was maintained, but movement disorders persisted. The patient was symptom-free at the 3-month neurology outpatient follow-up visit.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">CHBG syndrome is a rare hyperkinetic movement disorder characterised by unilateral, involuntary, rapid jerky movements of one or both limbs.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Basal ganglia infarction accounts for the majority of cases, but CHBG syndrome is now recognised as the second most common aetiology.4 The exact pathogenesis of this disease is unknown, but it is thought to involve delayed hyperglycaemic ischaemia with disruption or depletion of thalamic gamma-aminobutyric acid input, striatal microhaemorrhages and cerebral malperfusion.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The prevalence of CHBG syndrome is estimated at less than one in 100,000, the average onset age is 70 and is most commonly described in female patients of East Asian descent.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In addition to hyperglycaemia, there are other serious disorders that can cause hemichorea-hemiballism and should be ruled out in the ED. These aetiologies include haemorrhagic and ischaemic stroke, carbon monoxide poisoning, infectious diseases, neurodegenerative disorders and neoplasms. The diagnosis of CHBG syndrome is made by CT scan, which shows unilateral hyperdensity of the putamen contralateral to the symptomatic side, with or without hyperattenuation of the caudate nucleus and absence of the internal capsule. Due to its hyperdensity on CT, the findings of CHBG syndrome may be confused with other pathological processes. Its unilaterality excludes microcalcification, Fahr's disease, Wilson's disease and manganese deposition. Involvement confined to the basal ganglia and the absence of mass effect exclude haemorrhage.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Upon recognition, CHBG syndrome should be treated with aggressive glucose control usually leading to resolution of hemichorea similar to our case, as well as imaging findings. Refractory cases may require treatment with postsynaptic dopamine receptor antagonists such as haloperidol or risperidone.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Early identification and treatment of CHBG syndrome leads to excellent patient outcomes and likely resolution of symptoms. As patients with diabetes are increasingly seeking care in the emergency department, clinicians must become familiar with their multiple complications and presentations and be prepared to differentiate them from other acute conditions. This case highlights the importance of including uncontrolled diabetes in the differential diagnosis of new-onset hemichorea-hemiballism. Furthermore, it highlights the value of diabetic patient education in the ED and in outpatient follow-up to mitigate the burden of critical complications.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical considerations</span><p id="par0035" class="elsevierStylePara elsevierViewall">Informed consent has been obtained from the patient.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Funding</span><p id="par0040" class="elsevierStylePara elsevierViewall">No funding has been received.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflict of interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">There is no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethical considerations" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Funding" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflict of interest" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<p id="par0055" class="elsevierStylePara elsevierViewall">The following is Supplementary data to this article:<elsevierMultimedia ident="upi0005"></elsevierMultimedia></p>" "etiqueta" => "Appendix A" "titulo" => "Supplementary data" "identificador" => "sec0025" ] ] ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1778 "Ancho" => 1591 "Tamanyo" => 201423 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Cranial CT scan. Subtle hyperdensity of the right lentiform nucleus (arrow). 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Evers Smith" 1 => "K.K. Chaurasia" 2 => "D.C. Dekoski" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.7759/cureus.25073" "Revista" => array:4 [ "tituloSerie" => "Cureus" "fecha" => "2022" "volumen" => "14" "numero" => "5" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Non-ketotic hyperglycemia chorea-ballismus and intracerebral hemorrhage: a case report and literature review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "M. Dong" 1 => "EJY" 2 => "L. Zhang" 3 => "W. Teng" 4 => "L. Tian" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3389/fnins.2021.690761" "Revista" => array:3 [ "tituloSerie" => "Front Neurosci." 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Journal Information
Vol. 161. Issue 7.
Pages 316-317 (October 2023)
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Vol. 161. Issue 7.
Pages 316-317 (October 2023)
Letter to the Editor
Chorea hyperglycemia basal ganglia syndrome
Síndrome de corea hiperglucémica de ganglios basales
Joaquín Valle Alonso
, Leandro Noblia Gamba, Esther Montoro Jorquera
Corresponding author
Servicio de Urgencias, Hospital Universitario Santa Lucia, Cartagena, Spain
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