Chorea due to diabetic hyperglycemia and uremia: Distinct clinical and imaging features

Abstract

This study was undertaken to describe the clinical and imaging characteristics of patients with chorea associated with nonketotic hyperglycemia (C-NKH) in comparison with patients with chorea associated with uremia (C-URE). We retrospectively analyzed the clinical data of consecutive 10 C-NKH and five C-URE patients who were treated between January 1, 2001 and January 31, 2013. Women were more frequently affected by C-NKH (70% vs. 30%) and C-URE (80% vs. 20%) compared with men. The C-NKH patients demonstrated T1-hyperintense and inhomogeneous lesions in the basal ganglia, whereas C-URE patients demonstrated T2-hyperintense and homogeneous lesions in the basal ganglia. The mean time for chorea resolution after treatment was significantly shorter in C-NKH patients than in C-URE patients (4.4 ± 2.6 d vs. 73.8 ± 14.2 d, respectively; P = 0.005). The clinical and imaging features are remarkably different between C-NKH and C-URE patients, suggesting distinct pathogenic mechanisms.

One Side of the Story; Clues to Etiology in Patients with Asymmetric Chorea

“…Both diseases shared T2 hyperintensities in the basal ganglia, however in the uremic patients these lesions were more edematous and had a rim of strong T2 hyperintensity not seen in the hyperglycemic patients. There also appeared to be a larger proportion of patients presenting with generalized (presumably symmetric) versus asymmetric chorea in the uremic patients compared to the hyperglycemia patients, albeit with a small sample size [ 69 ].…”

Recurrent hemichorea with different etiologies in one patient

“…In hypoglycemia, T2 hyperintensity was noted at the basal ganglia [ 9 ]. In uremia, T2 hyperintensity lesions with edematous and a rim enhancement were noted [ 10 ]. Therefore, the MRI is a useful tool for the differential diagnosis of the causes of hemichorea.…”

Review of Hereditary and Acquired Rare Choreas

“…Chorea has been reported in patients with uremia [ 117 ], and is more commonly seen among people of East Asian ancestry, for reasons which are unknown. It can be difficult to differentiate whether the chorea is secondary to nonketotic hyperglycemia, which is usually present, or uremia; however, the observation of hyperintense lesions involving the basal ganglia associated with marked surrounding edema on MRI T2-weighted sequences, and the relatively younger age of the patient can facilitate the diagnosis.…”