Commentary: Uremic Striatopallidal Syndrome Manifesting as Acute Onset Chorea

Abstract

This is the case of a 50-year-old African American male with diabetes, hypertension, and chronic kidney disease attributed to membranous nephropathy stable under treatment with peritoneal dialysis. He presented acutely with generalized chorea and worsening of renal function. Interestingly, a computed tomography scan was initially normal, but 2 weeks later magnetic resonance imaging (MRI) demonstrated profound abnormalities, including swelling with ventricular compression involving the striatum (caudate and putamen), the globus pallidus (GPI and GPE), and the region of the substantia nigra. The presentation and imaging changes were considered diagnostic of uremic striatopallidal syndrome (USPS), and the patient was treated immediately with hemodialysis. He began to improve with the first round of hemodialysis, and by the third round his movement disorder had resolved completely. Despite the clinical improvement, 9 months later repeat MRI still demonstrated basal ganglia abnormalities. As with most patients with USPS, this patient had diabetes; however, it was felt that membranous nephropathy was the cause of his renal failure. In addition, most reported patients have been Asian with diabetes-related kidney disease already on hemodialysis. Patients generally present with either parkinsonism or chorea with the most prominent imaging changes seen in the GPI and GPE, respectively. This differential involvement was not evident here. Treatment involves more intensive hemodialysis (and in this case, switching from peritoneal to hemodialysis) usually with more rapid resolution of chorea than parkinsonism; the latter may show persistent features when there are residual cystic changes in the globus pallidus or putamen. This patient’s chorea quickly resolved; however, on subsequent follow-up, after the case report was accepted, he developed parkinsonism probably related to the persistent changes in the basal ganglia evident on imaging at 9 months. Movement disorders neurologists should be aware of this uncommon syndrome affecting patients with chronic kidney disease and should immediately encourage their nephrology colleagues to increase the intensity of the dialysis treatment first before entertaining pharmacological treatment of the abnormal movements.