Category: Choreas (Non-Huntington's Disease)
Objective: To present a case of uremic striatopallidal syndrome manifested by both hypo-and hyperkinetic extrapyramidal movements.
Background: UE is an uncommon neurologic complication encountered in patients with acute or chronic renal failure. We report the case of a 42-year-old man of Romany descent with end-stage renal disease (ESRD) who developed symmetrical bilateral basal ganglia syndrome manifesting as an acute bulbar stroke.
Method: The patient presented with a 4-days history of sudden onset of generalized weakness, dysphagia, dysarthria, right hemiparesis, and an unsteady gait. He suffered from long-standing uncontrolled diabetes mellitus on insulin therapy, hypertension, tobacco use and ESRD secondary to diabetic nephropathy on regular hemodialysis for the last three years. The patient had no history of a recent infection but reported frequent hypoglycemic episodes. Neurological examination revealed hypophonia, dysarthria, hypomimia, bradykinesia, and a short-step gait.
Results: Laboratory investigations demonstrated increased renal function tests and glycemia of 20 mg/dl. Other metabolic and electrolyte tests were normal. The acute clinical presentation of a patient with vascular risk factors made us consider a bulbar stroke. Brain MRI revealed a bilateral lentiform fork sign. The patient was diagnosed with “bilateral basal ganglia syndrome,” triggered by hypoglycemia. He was discharged from hospital without significant changes in the neurological status. Three months later, he presented with generalized choreiform movements. The involuntary movements were so severe that caused him an inability to stay stable during hemodialysis. They were associated with episodes of hypoglycemia and improved with relatively increased glucose levels.
Conclusion: In conclusion, we highlight that it is of paramount importance to avoid glycemic fluctuations in diabetic uremic patients in order to prevent the syndrome of acute basal ganglia lesions and progression of clinical symptomatology.
To cite this abstract in AMA style:
B. Qazimllari, A. Kuqo, J. Tana, A. Rroji, A. Rama, A. Verlaku, J. Kruja. Uremic encephalopathy (UE) mimicking acute bulbar stroke with progression from bradykinesia to choreic movements in a diabetic patient undergoing hemodialysis: A case report [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/uremic-encephalopathy-ue-mimicking-acute-bulbar-stroke-with-progression-from-bradykinesia-to-choreic-movements-in-a-diabetic-patient-undergoing-hemodialysis-a-case-report/. Accessed January 18, 2025.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/uremic-encephalopathy-ue-mimicking-acute-bulbar-stroke-with-progression-from-bradykinesia-to-choreic-movements-in-a-diabetic-patient-undergoing-hemodialysis-a-case-report/