Movement Disorders Clinical Practice | 2021
Uremic Striatopallidal Syndrome Manifesting as Acute Onset Chorea
Abstract
A 50-year-old African-American man with chronic kidney disease (CKD) on peritoneal dialysis presented to our movement disorders clinic for evaluation of sudden onset, progressively worsening, and involuntary movements of the trunk and lower limbs that began 8 days prior. The movements stopped during sleep, but otherwise were not suppressible and there was no family history of abnormal movements. In addition to CKD, he had a history of well-controlled diabetes mellitus, hypertension, anemia, depression, and gout. His CKD presented 9 years earlier as nephrotic syndrome secondary to membranous nephropathy and had been stably managed with peritoneal dialysis for the last 13 months. An emergency department evaluation 3 days after onset revealed an unremarkable head computed tomography (CT), blood urea nitrogen (BUN) of 70 mg/dL, and serum creatinine (Cr) of 11.1 mg/dL; he was urgently referred to our clinic where he was noted to have generalized chorea without other focal findings (Video 1). A magnetic resonance imaging (MRI) brain was obtained 2 weeks later that demonstrated bilateral, symmetric, marked T2, and fluid attenuated inversion recovery (FLAIR) hyperintensities associated with restricted diffusion throughout the basal ganglia. Lesions appeared expansile with edema and mild compression of the ventricles (Fig. 1A). He was diagnosed with uremic striatopallidal syndrome (USPS) and admitted to his local hospital for initiation of hemodialysis. At follow-up in our clinic 6 weeks later, he reported that the movements started to improve after his first round of hemodialysis and resolved by his third treatment. On examination, the chorea had resolved (Video 1). A follow-up MRI 9 months later revealed decreased, but not resolved, basal ganglia signal abnormalities when his BUN was 18 mg/dL and Cr 6.5 mg/dL (Fig. 1B).