Objective:

We describe a patient who presented with right painful hemidystonia secondary to uremic encephalopathy. We describe their presentation, clinical course and management with accompanying video. 

Background:

Uremic encephalopathy has been associated with basal ganglia abnormalities that cause movement disorders to include chorea, tremor, and asterixis. To date, hemidystonia secondary to uremic encephalopathy, particularly in the pediatric population, has not been described.  

Design/Methods:

A ten-year-old girl with Goldenhar syndrome (oculo-auriculo-vertebral syndrome) was admitted with acute respiratory failure which progressed to cardiac arrest leading to multiorgan failure including end stage renal disease requiring continuous renal replacement therapy (CRRT). After a brief period of recovery following extubation, she developed action tremor of the hands followed by painful right hemidystonia. Imaging following the onset of symptoms revealed new parenchymal signal abnormalities with increased T2 hyperintensities affecting the bilateral (left greater than right) caudate nuclei, globus pallidus, putamen, and left external capsule. Review of lab results revealed a correlation between the development of symptoms and a significant increase in urea and creatinine levels from baseline. She was treated with oral clonazepam and targeted botulinum neurotoxin injections with clinically meaningful benefit and closer regulation of electrolytes. Over the coming weeks, her cardiac function and renal function continued to decline, and care was redirected. 

Results:

Painful hemidystonia, although rare, can be a presentation of uremic encephalopathy due to basal ganglia involvement. This patient showed symptomatic improvement with oral benzodiazepines and botulinum neurotoxin injections.  

Conclusions:

Uremic encephalopathy can present with various movement disorders including chorea, tremor, asterixis and painful hemidystonia with basal ganglia involvement and should be considered in patients with worsening renal function.