A 77 year old female with a history of gastric bypass surgery complicated by copper deficiency, and alcoholic liver cirrhosis was hospitalized for failure to thrive and encephalopathy. Initial MRI brain showed non-specific white matter hyperintensities. Mental status continued to worsen despite treatment with IV thiamine, copper (low 41 mcg/dL, normal 80-155), and antibiotics for presumed peritonitis.
Repeat MRI brain showed T2 signal abnormality within the central pons and midbrain with sparing of the anterior pontine periphery. Serum sodium on presentation was 138 mEq/L (normal 135-145) and fluctuated during hospitalization between 133-150. She subsequently developed hypoxemic respiratory failure requiring intubation and kidney failure requiring hemodialysis. Despite all aggressive measures she remained severely encephalopathic with repeat MRI brain revealing new confluent large areas of T2 hyperintensity in the centrum semiovale and corona radiata. T2 hyperintensity in the midbrain and central pons remained unchanged. The family declined lumbar puncture and pursued comfort measures.
Autopsy was performed which revealed extensive demyelination in the midbrain tegmentum with relative preservation of axons; capillary and arterial cerebral amyloid angiopathy most prominent in the cerebellar leptomeninges, extending into the cerebellar cortex; and moderate Alzheimer’s type 2 astrocytosis in the basal ganglia consistent with prior history of hepatic encephalopathy.