Location
Suwanee, GA
Start Date
17-4-2026 12:00 PM
End Date
17-4-2026 1:00 PM
Description
Introduction
Uremic striatopallidal syndrome (USPS) is a rare neurologic complication of end-stage renal disease (ESRD) characterized by bilateral basal ganglia dysfunction and hyperkinetic movement disorders, which may manifest as chorea, dystonia, or parkinsonian features. The underlying pathophysiology is not fully understood but is thought to involve metabolic derangements, uremic toxin accumulation, and disruption of basal ganglia function. Because its presentation can mimic medication-induced symptoms, primary neurodegenerative conditions, or other metabolic encephalopathies, USPS may be underrecognized and misdiagnosed, leading to delays in appropriate management.
Methods
A retrospective review of clinical findings, laboratory data, imaging studies, and hospital course was performed for a patient admitted with progressive involuntary movements. The diagnostic evaluation included metabolic, autoimmune, toxicologic, and neurologic assessments to determine the etiology of the patient’s symptoms.
Results
A 44-year-old female with a history of hypertension, hyperlipidemia, diabetes mellitus, and ESRD on hemodialysis presented with eight days of progressively worsening involuntary movements involving the face and extremities. The movements were constant, uncontrollable, and not associated with loss of consciousness, seizures, headache, or recent medication changes. On examination, the patient demonstrated tachycardia, choreoathetoid movements, and preserved mental status. Initial laboratory studies revealed elevated creatine kinase and severe renal dysfunction. CT imaging of the head showed no acute intracranial abnormalities. Trials of benzodiazepines and diphenhydramine in the emergency department provided minimal improvement. Further evaluation demonstrated positive antinuclear antibodies and anti-SSA antibodies, with otherwise unrevealing autoimmune and metabolic workup. MRI of the brain revealed symmetric signal abnormalities within the bilateral basal ganglia with mild diffusion restriction and subtle T2/FLAIR hyperintensity. In the context of end-stage renal disease and acute movement disorder, these findings were most consistent with uremic striatopallidal syndrome. The patient was treated with continued hemodialysis and symptomatic management including risperidone and clonazepam, resulting in gradual clinical improvement during hospitalization.
Discussion
This case highlights the importance of considering uremic striatopallidal syndrome as a potential cause of acute movement disorders in patients with renal failure. USPS may initially be mistaken for medication-induced extrapyramidal symptoms or other neurologic disorders, particularly when initial imaging studies such as CT scans are unremarkable. Recognition of the characteristic MRI findings and clinical context is essential for appropriate management, including dialysis optimization, metabolic correction, and symptomatic pharmacologic therapy. Increased awareness of this condition among clinicians may help facilitate timely diagnosis.
Embargo Period
6-1-2026
Included in
Uremic Striatopallidal Syndrome: A Rare Cause of Hyperkinetic Movement Disorder in End-Stage Renal Disease
Suwanee, GA
Introduction
Uremic striatopallidal syndrome (USPS) is a rare neurologic complication of end-stage renal disease (ESRD) characterized by bilateral basal ganglia dysfunction and hyperkinetic movement disorders, which may manifest as chorea, dystonia, or parkinsonian features. The underlying pathophysiology is not fully understood but is thought to involve metabolic derangements, uremic toxin accumulation, and disruption of basal ganglia function. Because its presentation can mimic medication-induced symptoms, primary neurodegenerative conditions, or other metabolic encephalopathies, USPS may be underrecognized and misdiagnosed, leading to delays in appropriate management.
Methods
A retrospective review of clinical findings, laboratory data, imaging studies, and hospital course was performed for a patient admitted with progressive involuntary movements. The diagnostic evaluation included metabolic, autoimmune, toxicologic, and neurologic assessments to determine the etiology of the patient’s symptoms.
Results
A 44-year-old female with a history of hypertension, hyperlipidemia, diabetes mellitus, and ESRD on hemodialysis presented with eight days of progressively worsening involuntary movements involving the face and extremities. The movements were constant, uncontrollable, and not associated with loss of consciousness, seizures, headache, or recent medication changes. On examination, the patient demonstrated tachycardia, choreoathetoid movements, and preserved mental status. Initial laboratory studies revealed elevated creatine kinase and severe renal dysfunction. CT imaging of the head showed no acute intracranial abnormalities. Trials of benzodiazepines and diphenhydramine in the emergency department provided minimal improvement. Further evaluation demonstrated positive antinuclear antibodies and anti-SSA antibodies, with otherwise unrevealing autoimmune and metabolic workup. MRI of the brain revealed symmetric signal abnormalities within the bilateral basal ganglia with mild diffusion restriction and subtle T2/FLAIR hyperintensity. In the context of end-stage renal disease and acute movement disorder, these findings were most consistent with uremic striatopallidal syndrome. The patient was treated with continued hemodialysis and symptomatic management including risperidone and clonazepam, resulting in gradual clinical improvement during hospitalization.
Discussion
This case highlights the importance of considering uremic striatopallidal syndrome as a potential cause of acute movement disorders in patients with renal failure. USPS may initially be mistaken for medication-induced extrapyramidal symptoms or other neurologic disorders, particularly when initial imaging studies such as CT scans are unremarkable. Recognition of the characteristic MRI findings and clinical context is essential for appropriate management, including dialysis optimization, metabolic correction, and symptomatic pharmacologic therapy. Increased awareness of this condition among clinicians may help facilitate timely diagnosis.