Acute chorea and bilateral basal ganglia lesions in a hemodialysis patient

Abstract

CASE PRESENTATION A 71-year-old Hispanic woman presented to our medical center with a general sense of feeling ‘unwell.’ She suffered from multiple medical conditions, including long-standing history of diabetes, hypertension, hypercholesterolemia, depression, and end-stage renal disease (ESRD) for which she has been on dialysis for the last 3 years. Seven years prior to her current admission, the patient presented with an acute onset of left seventh nerve palsy associated with hypercalcemia and submandibular and perihilar lymphadenopathy. Submandibular lymph node biopsy showed infiltration with a polymorphous population of lymphocytes and granulomas, findings consistent with sarcoidosis. She was treated with a course of corticosteroids and methotrexate, with complete resolution of symptoms within 2 years of the diagnosis. Steroids were discontinued 6 years and methotrexate 4 years before her current admission. Her sarcoidosis remained in complete remission. Her past medical history was also significant for primary hyperparathyroidism; she underwent partial parathyroidectomy in the early 1980s. The patient’s kidney disease had been diagnosed 4 years prior, when she presented with a nephrotic syndrome and creatinine (Cr) of 1.5 mg per 100 ml (ref. 0.5–0.9 mg per 100 ml). The kidney biopsy performed at that time revealed advanced focal segmental glomerulosclerosis and she progressed to ESRD within 1 year of the diagnosis. Over the last 3 years, the patient has been maintained on regular thrice-weekly hemodialysis through a radial arteriovenous fistula with adequate clearance (urea reduction ratio ranging from 77 to 84%) and no major complications. The patient was brought to the emergency room by her family for unstable gait with ‘dance-like’ quality. The symptoms developed gradually over the period of 2 days. The patient neither missed dialysis nor had been ill, and had no sick contacts. Her medication regimen included aspirin, diltiazem, paroxetine, statin, glipizide, sevelamer, multivitamins, and stool softeners. She denied any exposure to antipsychotics or antiemetics. There was no family history of movement disorders, psychiatric disease, or renal disease. The patient used to work as a seamstress and retired several years ago. She did not smoke or drink and denied any illicit substance use. The patient’s pulse was 82–88 per min and the blood