Diabetic striatopathy-Does it exist in non-Asian subjects?

Background: Diabetic striatopathy (DS) is a rare complication of diabetes mellitus (DM). The syndrome appears in patients with uncontrolled DM and is characterized by abrupt onset of movement disorder, mainly hemichorea and accompanied by specific findings on brain imaging. It is believed that DS is unique to the Asian population and affects mainly elderly women with uncontrolled DM.

Methods: In order to define existence and characterization of DS in Western population, we reviewed the medical records of all patients admitted to the Chaim Sheba Medical Center between 2004 and 2014 and identified those with documented elevated HbA1c (>10%). The charts and imaging studies of those with elevated HbA1c and undiagnosed neurological symptoms were reviewed to diagnose DS.

Results: Out of 697 patients with HbA1c>10%, 328 patients had unknown neurological diagnosis. Among them, we identified 4 patients (3 women, mean age 73 and mean HbA1c of 14.8%) with hemichorea or choreoathetosis and brain imaging findings compatible with the diagnosis of DS. Only one out of the 4 patients was diagnosed during hospitalization with DS. All patients were treated with insulin with improvement of their symptoms during hospitalization. However, there was a recurrence in 2 of them and 1 died during the second episode.

Conclusion: Diabetic striatopathy exists but underdiagnosed in the Western population. It is important to increase the awareness for this clinical syndrome in order to treat those patients properly.