Clinical and neuroimaging spectrum of hyperglycemia-associated chorea-ballism: Systematic review and exploratory analysis of case reports

Hyperglycemia-associated chorea-ballism (HCB) is an infrequent neurological syndrome occurring predominantly in elderly females and in the setting of non-ketotic hyperglycemia (NKH). A systematic review was conducted in accordance with the PRISMA statement. Studies published between 1980 and 2018 that reported demographic, clinical, laboratory and imaging features from patients with HCB were screened. 136 studies describing 286 patients were included in the analysis. The patients included had a median age of 72 years; those with ketotic hyperglycemia (KH) were older (p<0.001). Women and NKHpatients were the most frequently affected (63% and 92%, respectively). The median glucose level at admission was420 mg/dL (IQR 328-535), and was significantly higher in KH (p=0.009). Moreover, the absence of a clear lesion on imaging studies and the finding of bilateral imaging evidence of lesions were each more frequent in the KH group (p=0.036 and p=0.008, respectively). 48 cases (19.4%) presented with bilateral CT/MRI lesions, having higher values of plasma osmolarity compared with the patients with unilateral lesions (p=0.011). Every patient received hypoglycemic treatment, but only 174 (60.84%) were prescribed neuroleptics. 213 patients (84.86%) showed a total recovery, after a median of 14 days (IQR 3-31). Bilateral chorea-ballism was supported by bilateral imaging evidence of involvement in only 60% of the cases (positive predictive value). Patients not prescribed neuroleptics, with negative lentiform nucleus involvement, and age within the third tertile (≥ 78 years) had an odds ratio of 6.6 (CI 95% 1.18-141.10) for a complete clinical recovery. Significant differences were identified between types of hyperglycemia and regarding the clinical and imaging laterality features. Furthermore, the predictor variables evaluated showed potential utility for assessing the prognosis of HCB patients.