Case description

A 44-year-old man presented with generalised weakness, shakiness, loss of appetite, fatigue, flank pain and vertigo for several days. He had vomited several times. On examination, he was somnolent, with psychomotor slowing and disorientation, but with no additional focal neurological deficit. Laboratory findings identified acute kidney injury (creatinine 1389 µmol/L, normal<115). Blood gas analysis showed acidosis with pH 7.29 (normal 7.35–7.45), reduced bicarbonate (15 mmol/L; normal 22–26), a negative base excess (−10 mmol/L; normal −2 to 2) and elevated anion gap (28 mmol; normal 3–11). Ultrasound scan of abdomen showed enlarged kidneys. MR scan of brain showed extensive signal changes bilaterally in the basal ganglia and brainstem as well as in temporomesial areas (figure 1A,B). The cerebrospinal fluid (CSF) showed a granulocytic pleocytosis (164 cells/µL; normal <4) with elevated CSF protein (2.3 g/L; normal <0.45). No bacterial or viral pathogen was detected in the PCR multiplex, nor any oxalate crystals or etylene glycol in a later assay. A broad autoimmune …