"Diabetic striatopathy": clinical presentations, controversy, pathogenesis, treatments, and outcomes

Abstract

Diabetic striatopathy (DS) is a rare medical condition with ambiguous nomenclature. We searched PubMed database from 1992 to 2018 for articles describing hyperglycemia associated with chorea/ballism and/or neuroimages of striatal abnormalities. Descriptive analysis was performed on demographic/clinical characteristics, locations of striatal abnormalities on neuroimages, pathology findings, treatment strategies, and outcomes. In total, 176 patients (male:female = 1:1.7) were identified from 72 articles with mean age 67.6 ± 15.9 (range, 8-92). Among them, 96.6% had type 2 DM with 17% being newly diagnosed. Average blood glucose and glycated hemoglobin concentrations were 414 mg/dL and 13.1%, respectively. Most patients (88.1%) presented with hemichorea/hemiballism. Isolated putamen and combined putamen-caudate nucleus involvements were most common on neuroimaging studies with discrepancies between CT and MRI findings in about one-sixth of patients. Unilateral arm-leg combination was the most frequent with bilateral chorea in 9.7% of patients. Chorea and imaging anomalies did not appear concomitantly in one-tenth of patients. Successful treatment rates of chorea with glucose-control-only and additional anti-chorea medications were 25.7% and 76.2%, respectively, with an overall recurrence rate being 18.2%. The most commonly used anti-chorea drug was haloperidol. To date, four out of six pathological studies revealed evidence of hemorrhage as a probable pathogenesis.

Figure 1

Selection of eligible literature for the present study.

Figure 2

Typical images from (A) computed tomography (CT) and (B) T1-weighted magnetic resonance imaging (MRI) showing striatal anomalies of putamen (white arrows) and caudate nucleus (white arrowheads). Note the hyperdensity over globus pallidus on CT image but lack of hyperintensity on MRI (black arrows), defined as “incompatibility” in the present study.

Figure 3

Retrospective review of treatment regimens of patients with hyperglycemic chorea.

Figure 4

Number of patients with successful anti-chorea treatments using (A) monotherapy, and (B) combined regimens. (C) Overview of frequency of use of individual anti-chorea agent and effectiveness (either as monotherapy or part of combined regimens). Alp, alprazolam; Bac, baclofen; Car, carbamazepine; Chl, chlorpromazine; Clo, clonazepam; Clor, clorazepate; Dia, diazepam; Flu, fluphenazine; Gab, gabapentin; Hal, haloperidol; Lev, levodopa; Leve, levetiracetam; Lor, lorazepam; Phe, phenytoin; Phen, phenobarbital; Pim, pimozide; Qua, quatiapine; Res, reserpine; Ris, risperidone; Sul, sulpiride; Tet, tetrabenazine; Tia, tiapride; Val, valproate.