New-onset refractory status epilepticus (NORSE) is a rare neurological emergency in which a patient without epilepsy or overt structural or toxic/metabolic derangement develops status epilepticus refractory to treatment. Although seizures have been documented in patients who undergo blood transfusion and develop evidence of posterior reversible encephalopathy syndrome (PRES), this is the first documented case of NORSE following blood transfusion.

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A 46-year-old woman with distant history of thyroidectomy for papillary thyroid cancer presented with six months of intermittent menorrhagia, followed by one month of continuous menorrhagia and progressive lethargy. No other prodromal or neurological symptoms were evident. She was found to have hemoglobin 2.6 g/dL and was immediately transfused. After receiving her third unit of blood, she became tachycardic and confused, and then had a witnessed generalized tonic-clonic seizure. Continuous EEG revealed frequent right frontal seizures for which she received levetiracetam. Brain MRI showed focal T2 hyperintensity and diffusion restriction in the bilateral right greater than left frontal cortex and medial thalami. She had no documented hypotension to suggest anoxic injury. Lumbar puncture showed modest neutrophilic pleocytosis (5-10 WBCs) without oligoclonal bands. On the fourth hospital day, EEG showed non-convulsive status epilepticus (NCSE). Despite treatment with levetiracetam, lacosamide, phenytoin, clobazam, propofol, and midazolam infusions, she developed super-refractory NCSE and was placed in pentobarbital coma for burst suppression for 48 hours. She received high-dose intravenous methylprednisolone. By the eleventh hospital day, her EEG had improved dramatically, and she was weaned off pentobarbital with no further seizures. Extensive diagnostic workup for infectious, inflammatory/autoimmune, neoplastic/paraneoplastic, metabolic, and prion diseases was unrevealing.

Approximately 50% of NORSE cases are cryptogenic, and none have previously been attributed to blood transfusion. Rapid correction of severe anemia could lead to brain capillary leakage and cortical irritation, though refractory status epilepticus is unexpected.