Objective:

To describe a rare but interesting neurologic complication of uncontrolled diabetes; diabetic striatopathy, and to discuss its presentation as an uncommon etiology of acute hyperkinetic movement disorders.

Background:

Diabetic striatopathy is described as a hyperglycemic state in conjunction with striatal abnormalities on imaging and or the presence of hyperkinetic movements such as chorea. Imaging abnormalities described include hyperdense appearance of the striatum on CT and hyperintensity of the striatum on T1 MRI sequence. Associated hyperkinetic movements are thought to be reversible with correction of hyperglycemia. Given its rarity and ease of being misdiagnosed, we present a case of a 74-year-old woman with imaging findings consistent with Diabetic striatopathy. She presented in-extremis from an outside hospital for neurosurgical evaluation of a left basal ganglia hemorrhage. Her past medical history included uncontrolled type 2 diabetes and hypertension.

Design/Methods:

Not Applicable

Results:

Physical examination revealed an intubated, comatose, hypotensive patient with an irregular pulse. Her Laboratory values suggested uncontrolled diabetes with a hemoglobin A1C of “> 17.0%” (beyond the laboratory's upper limit) and serum glucose of 611. She had profound diabetic ketoacidosis. A non-contrasted head CT revealed what was initially thought to be a left basal ganglia hemorrhage; same interpretation as the outside hospital but was later favored to likely represent “nonketotic hyperglycemic chorea” and less likely a basal ganglia hemorrhage by the attending radiologist. MRI confirmed this with T1 hyperintensity in the left striatum. 

Conclusions:

Our patient passed away from her severe metabolic derangements and no hyperkinetic movements were observed. However, her presentation highlighted to us an important and underrecognized differential of basal ganglia hemorrhage on imaging especially in the setting of uncontrolled diabetes. The paucity of information surrounding its pathophysiology, poses an area of potential research. Often cited as associated with hyperglycemic nonketotic state, it was enlightening to see it in diabetic ketoacidosis.