New-onset refractory status epilepticus with a hyperferritinemic hyperinflammatory phenotype in Kyasanur Forest...

Kyasanur Forest Disease (KFD) is a tick-borne flaviviral infection endemic to India. While neurological manifestations are recognized, new-onset refractory status epilepticus (NORSE) has not been previously reported. We describe NORSE in the setting of severe KFD-associated hyperinflammation and multiorgan dysfunction.

A 53-year-old previously healthy woman with virologically confirmed KFD developed generalized tonic-clonic seizures that progressed to NORSE during a severe systemic illness. She had profound cytopenias, severe hepatitis, coagulopathy, myocarditis, acute kidney injury, and marked hyperferritinemia, with a peak ferritin concentration of 14,466 ng/ml, consistent with a macrophage activation-like hyperinflammatory phenotype. Neurological evaluation demonstrated normal brain magnetic resonance imaging and non-inflammatory cerebrospinal fluid. CSF reverse-transcription polymerase chain reaction for KFD virus was negative, and no alternative evidence of central nervous system infection was identified. The patient required mechanical ventilation, multiple anti-seizure medications, continuous anesthetic therapy, ketamine, and corticosteroids for control of refractory status epilepticus. Seizure burden subsequently declined in parallel with improvement in systemic inflammation and multiorgan dysfunction. Ferritin decreased from 14,466 to 834 ng/ml, accompanied by progressive neurological and organ recovery.

NORSE may represent a rare neurological manifestation of severe KFD. In this case, the absence of demonstrable central nervous system infection, together with the temporal association between systemic hyperinflammation and seizure activity, raises the possibility that KFD-associated immune dysregulation contributed to the development of NORSE. Recognition of this potential association may be important when evaluating neurological deterioration in patients with severe KFD and hyperinflammatory multiorgan disease..