Objective: To highlight uremic striatopathy as a reversible metabolic cause of acute hyperkinetic movement disorder in patients with end stage renal disease.

Background: Uremic striatopathy is a rare metabolic complication of advanced renal failure characterized by acute or subacute movement disorders. Clinical manifestations include chorea, ballism, and parkinsonism.

Method: We report the clinical course and neuroimaging findings of a patient with end-stage renal disease on maintenance haemodialysis who developed acute hyperkinetic movement disorder due to inadequate dialysis.

Results: A 74-year-old man with well controlled diabetes and end stage renal disease on maintenance haemodialysis, presented with progressively worsening involuntary movements over the last three days. On examination he was confused, disoriented and distressed due to the involuntary movements. While seated, negative myoclonus (asterixis) were observed in hands and arms, and on standing with support, the negative myoclonus led to sudden head drops and flexion at trunk leading to near-falls. Additionally, there was generalized chorea affecting the face, neck, trunk and limb muscles. Metabolic workup was normal except for elevated serum creatinine 11.2 mg/dL (normal 0.7 to 1.3 mg/dL) and single-pool Kt/V 0.73 (suggesting inadequate creatinine clearance, target >1.2). MRI brain showed swelling and hyperintense signal in both putamen on FLAIR sequences. T1W images did not show changes of hyperglycemia. Considering the diagnosis of uremic striatopathy the frequency and intensity of haemodialysis were increased. Over the next three days confusion and myoclonus resolved. However, the chorea worsened. Tetrabenzine (75 mg/day) was given for symptomatic relief. By the 5th day of admission, the chorea improved and the patient could walk with support. One month later, on continued hemodialysis the chorea had completely resolved and tetrabenazine could be withdrawn.

Conclusion: In this video case-report we highlight the clinical course and neuroimaging findings of the rare syndrome of uremic striatopathy. Our report reiterates that symptoms may resolve by correction of uremia.

« Back to 2026 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/reversible-uremic-striatopathy-presenting-as-acute-generalized-myoclonus-and-chorea-in-a-patient-with-end-stage-renal-disease-a-video-case-report/