Endocrine Abstracts

Introduction: Diabetes striatopathy (DS) is an extremely rare hyperglycaemic complication of diabetes with a prevalence reported as 1 in 100,000¹. Though DS is commonly associated with a non-ketotic hyperglycaemic hyperosmolar state (HHS), it is occasionally reported in diabetic ketoacidosis (DKA). DS is known as non-ketotic hemichorea-hemiballismus due to its presentation with hyperkinetic movements but rarely presents as stroke-mimic². Here we present an extremely rare case of DKA-associated DS in a patient who presented with unilateral hemiparesis and focal seizures.

Case report: 78 M, known T2DM, presented with acute right-sided weakness, facial droop, slurring of speech, and right-sided focal seizures along with acute decompensation of glycemic control (HbA1C 6.1%-->17%). His blood tests showed DKA (CBG>47, PH-7.23) with ketones of 5.4. CT head showed left-sided diabetes striatopathy. He was treated as per DKA protocol and was started on Humulin I twice daily. His neurological deficits and focal seizures got resolved in 3 days and he got discharged on regular insulin.

Conclusion: The term Diabetes striatopathy (DS) was introduced in 2009 to denote a condition with the radiological finding of striatal hyperintensity, and contralateral movement disorder in poorly controlled diabetes patients. DS is commonly known as non-ketotic hemichorea/hemiballismus, predominantly reported in elderly Asian females with poorly controlled T2DM³. Literature reviews have shown its varied presentation from hyperkinesia to hemiparesis. While hyperkinesis was mostly reported in nonketotic hyperglycaemic conditions, hemiparesis was found in patients with ketosis. Hyperglycaemia promotes cerebral autoregulation failure, hypoperfusion, and activation of anaerobic metabolism, resulting in depletion of GABA in the basal ganglia in non-ketosis hyperglycemia⁴. However, in hyperglycaemia with ketosis, the inhibitory neurotransmitter GABA is re-synthesized from acetoacetate and hence hyperkinetic movements are controlled. DS is a reversible condition with normalisation of hyperglycaemia. DS should be suspected in patients with uncontrolled diabetes and abnormal basal ganglia imaging.