Endocrine Abstracts

Background: Diabetic striatopathy (DS) is a rare dyskinetic syndrome associated with acute decompensated hyperglycemia that commonly presents as hemichorea-ballism. Its natural history following resolution of the hyperglycemic crisis is not well delineated.

Methods: The study was a prospective evaluation of the long-term clinico-radiological outcomes of patients presenting with DS. Neuroimaging (CT/MRI) was performed at baseline and follow-up (>3 months). Ancillary work-up included calcium profile, slit-lamp examination, and FDG-PET selectively.

Results: There were 7 patients; 2 young males with T1DM presenting with diabetic ketoacidosis (DKA) and 5 post-menopausal females with T2DM presenting with hyperosmolar hyperglycemic syndrome (HHS). All had acute-onset left-sided hemichorea-ballism. DS was the presenting manifestation of DM in 2 patients. Mean HbA1c at admission was 14.2 ± 3.1 %. The most common pattern on CT was bilateral striatal hyperdensities (67%) and on MRI was bilateral T1, T2 hyperintensities (67%). Discrepancy between CT and MRI was seen in 16.7% of patients. Clinico-radiological discordance was noted in 28.5% of patients, in terms of either laterality of lesions or persistent dyskinesia with normal MRI. Resolution of dyskinesia was seen with glycemic optimization alone in 33% and additional therapy in the rest (tetrabenazine (50%), clonazepam (17%)). One patient, who did not receive any specific therapy, had persistent symptoms. The duration of resolution of dyskinesia was earlier in patients who presented with DKA (<1week) than with HHS (median 5 (2-15) months). Follow-up imaging (>3 months) revealed loss of striatal volume and dilation of the frontal horn of the lateral ventricles in 50% of patients, mixed intensity lesion (residual hyperintensity and new-onset hemosiderin-related hypointensity) in 16%, and new-onset hemosiderin hypointensity alone in 34%.

Conclusion: Clinical resolution of DS is common with the restoration of euglycemia but may require additional medical therapy. Prospective imaging reveals unilateral ventricular dilation, focal gliosis, or hypointensity suggestive of hemosiderin deposition.