A 20-year old lady with poorly controlled type 1 diabetes mellitus diagnosed since 9 years old, comorbidities of hyperlipidemia and steatohepatitis as well as a history of smoking, presented with an acute 2-day history of involuntary, writhing movements of her left upper and lower limbs. Physical examination confirmed left mild hemiparesis with hemichorea-athetosis. Emergent MRI brain demonstrated abnormal signal in the right caudate nucleus and lentiform nucleus (low T2W/FLAIR signal and high T1W signal) suggestive of diabetic striatopathy. There is corresponding marked non-ketotic hyperglycemia (?any levels) with a glycated haemogloblin (HbA1c) of 12%. Incidentally, MR angiography brain revealed severe stenoses of both terminal internal carotid arteries with multiple collaterals, suggestive of severe bilateral Moyamoya syndrome. A hypercapnoea challenge demonstrated exhausted vasodilatory reserves with paradoxical reduction of flow velocities. Extensive investigations for the aetiology of the Moymoya syndrome that included autoimmune cerebral vasculitis, were unremarkable. The patient was managed with aggressive glycemic control and symptomatic treatment for her movement disorder, with full resolution of her symptoms after 4 weeks. She was started on aspirin and advised for vascular bypass surgery for her Moyamoya syndrome. Diabetic striatopathy presenting with acute hemichorea and/or hemiballimus, can occur in type 2 diabetic patients (usually female, Asian and elderly) with non-ketotic hyperglycemia. Diabetic striatopathy rarely occurs in a young patient and in Type 1 diabetes. It is postulated that chronic hyperglycemia leads to hyperviscosity, in turn causing cerebral hypoperfusion, anaerobic metabolism and reduced γ-aminobutyric acid levels. This in turn leads to increased thalamocortical activity, causing abnormal movements. Moyamoya syndrome is a rare condition of which diabetes is a possible aetiology. Chorea is a rare presenting symptom, secondary to ischaemic dysfunction and imbalance in the complex basal ganglia circuitry. We describe the first reported case of a young adult with poorly controlled type 1 diabetes, hyperlipidemia and smoking, who presented with acute hemichorea. Given the risk factors, our initial differential was that of a thrombotic stroke. She was eventually found to have 2 rare diabetes-related central nervous complications of hyperglycemia-induced striatopathy and severe Moyamoya syndrome, both of which could result in acute movement disorder. MRI and MRA brain are important investigations to help differentiate the three causes. Aggressive glycemic control often results in resolution of the movement disorder in diabetic striatopathy. However, it is important to ensure there are no concomitant diabetes-related vascular stenoses, that would increase stroke risk and necessitate different management.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology