Commentary: Uremic Striatopallidal Syndrome Manifesting as Acute Onset Chorea

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.1 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. (1) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique. A.E.L.: 1A E.F.: 1B P.A.V.: 1B J.E.D.: 1B J.F.M.: 1B K.D.S.: 1B The authors confirm that approval of an institutional review board was not required for this work. Informed written consent for publication was obtained from the patient. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. No specific funding was received for this work. The authors report no conflicts of interest relevant to this work. A.E.L. has served as an advisor for Abbvie, Acorda, AFFiRis, Biogen, Denali, Janssen, Lilly, Lundbeck, Maplight, Paladin, Retrophin, Roche, Sun Pharma, Sunovion, Theravance, and Corticobasal Degeneration Solutions; received honoraria from Sun Pharma, AbbVie and Sunovion; received grants from Brain Canada, Canadian Institutes of Health Research, Corticobasal Degeneration Solutions, Edmond J Safra Philanthropic Foundation, The Michael J. Fox Foundation, the Ontario Brain Institute, Parkinson Foundation, Parkinson Canada, and W. Garfield Weston Foundation; and received publishing royalties from Elsevier, Saunders, Wiley-Blackwell, Johns Hopkins Press, and Cambridge University Press. E.F. and P.A.V. have nothing to disclose. J.E.D. receives research support from the Department of Veterans Affairs, the National Institutes of Health, The Michael J. Fox Foundation, and the Lewy Body Dementia Association and Innervace, Inc. He has received honoraria from the International Parkinson and Movement Disorder Society. J.F.M. receives research support from the department of Veterans Affairs and GE Healthcare and has received honoraria from The Michael J. Fox Foundation. K.D.S is a consultant for Acorda and Neurocrine and has served as an expert witness in welding and metoclopramide litigation.

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