Diabetic uremic syndrome is a concept that has been increasingly recognized since it was proposed in 2003. It differs from uremic encephalopathy. 

Hereby, we describe the first case of North African Arab descent with end-stage renal disease and diabetes who developed acute Parkinsonism with bilateral lenticular fork sign and uremia that partially resolved after extensive dialysis therapy.

Case Description:

65 years old man with end-stage renal disease on peritoneal dialysis and newly diagnosed diabetes millets presented with a 3-day history of unsteady gait, generalized weakness, mild dysarthria, mild dysphagia, drowsiness, and changes in concentration.

Examination revealed a mask face, dysphagia, absent gag reflex, dysphonia, generalized motor weakness more prominent in lower limbs, bradykinesia, absent knee reflexes, and myoclonic jerks. Laboratory studies showed urea 25 mmol/L, Creatinin 922 umol/L,  arterial pH 7.25, and normal lactate. The lumbar puncture study was unremarkable. CT head showed swollen hypodense bilateral lentiform nuclei with a hypodense rim delineating the basal ganglia and giving the appearance of a lentiform fork sign. MRI showed bilateral T2 and FLAIR putamen and globus pallidus hyperintensity and significant lactate peak in the bilateral basal ganglia on MR spectroscopy. EMG study was evident of axonal polyneuropathy.

IVIG course was commenced, and motor weakness improved. The peritoneal dialysis was intensified, and later treatment was switched to hemodialysis.

Acidemia resolved, and uremia significantly improved. Two weeks post-admission, the fork sign on MRI significantly regressed, and the patient’s Parkinsonian symptoms partially improved.

Development of acute or subacute extrapyramidal movement disorder in end-stage renal disease associated with diabetes should promote the possibility of diabetic uremic syndrome with bilateral basal ganglia lesions forming fork sign appearance that occurs early on brain imaging. Usually, extensive dialysis, preferable hemodialysis, leads to a total resolution of brain imaging and partial resolution of clinical findings.