Coma secondary to cerebral fat embolism syndrome due to sickle cell disease fully recovering following red cell exchange transfusion

Abstract

A 51-year-old woman known for sickle cell disease presented with 2 weeks of headache and bilateral lower limb pain. During admission, she suffered from multiple generalised tonic-clonic seizures but had an unremarkable CT of the brain. Incidentally, she had worsening baseline renal function. She was admitted to the intensive care unit with an acute confusional state. A bedside electroencephalogram showed triphasic waves and diffuse slow activity suggestive of encephalopathy with no epileptiform discharges. She remained obtunded despite appropriate medical therapy of hydration, antiepileptic and pain control. Lumbar puncture failed to identify an infectious cause. An urgent MRI of the brain was done and revealed features compatible with fat embolism syndrome (FES). Her haemoglobin S was 84.2%. Urgent red cell exchange transfusion was done, and within 3 days she fully regained her orientation and motor function. This represents the first case of such profound obtundation due to FES with a complete response to exchange transfusion.