Reversible syndrome of extrapyramidal movement disorders with bilateral basal ganglia lesions in uremia: a case series and review of the literature

Abstract

Background: The distinct clinicoradiological syndrome of reversible basal ganglia lesions associated with extrapyramidal movement disorders in uremic patients has rarely been described in the literature. There have been no reported cases from Africa.

Methods: This study is a retrospective analysis of uremic patients presenting with extrapyramidal movement disorders in Durban, South Africa from 2003 to 2016. A review of all published studies was also undertaken.

Results: Seven patients who presented with this syndrome were identified. An additional 41 cases were reported in the literature. Our seven cases showed similar characteristics to those previously reported. All patients were of Asian ethnicity and had dialysis dependent renal failure, 86% (6/7) due to diabetic nephropathy. The most frequent presentation was parkinsonism (5/7) followed by chorea (1/7) and dystonia (1/7). Typical neuroimaging findings included bilateral symmetrical basal ganglia abnormalities that were hypodense on computed tomography scan, and T1 hypointense and T2 hyperintense on magnetic resonance imaging. A key feature of this syndrome is its reversibility with supportive treatment; Clinical improvement was observed in 86% (6/7), which was accompanied by radiological regression of lesions in two patients who underwent follow-up imaging.

Conclusions: The syndrome of acute extrapyramidal movement disorders in uremic patients with bilateral basal ganglia lesions presents with typical clinical and radiological findings. Awareness of this syndrome especially in Asian diabetic patients with renal failure is important for early recognition and appropriate supportive management to aid its resolution.

Keywords: Basal ganglia, diabetic nephropathy, extrapyramidal, magnetic resonance imaging, renal failure