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.
- haematology (incl blood transfusion)
- adult intensive care
- sickle cell disease
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Contributors NA has contributed to manuscript preparation, acquisition of pictures and review of the literature. AA-F has reviewed the draft manuscript and suggested alterations in conjunction with NA. AA-F and KA did critical revision of the content and final approval of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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