Hemophagocytic lymphohistiocytosis in an adult patient with super-refractory status epilepticus.
Epilepsia Open
; 2024 Aug 16.
Article
in En
| MEDLINE
| ID: mdl-39151036
ABSTRACT
This case report presents a 38-year-old male patient who, after a febrile infection, developed super-refractory status epilepticus and multiorgan failure, and died in 2 weeks despite the best possible intensive care. Autopsy revealed findings suggestive of hemophagocytic lymphohistiocytosis (HLH). This case shows that a rare immunological cause such as HLH may cause febrile infection-related epilepsy syndrome (FIRES), and complications of intensive care can mask the physiological and laboratory changes in HLH. PLAIN LANGUAGE SUMMARY:
This case report presents a 38-year-old man who, after a febrile infection, developed intractable epileptic activity requiring intensive care treatment. During the intensive care, the patient showed signs of multiple organ damage and died in 2 weeks despite the best possible treatment. Autopsy revealed findings suggestive of hemophagocytic lymphohistiocytosis (HLH), which is a rare immune system regulation disorder leading to persistent inflammatory state and organ damages. This case shows that an immunological disorder like HLH may underlie treatment resistant fever-related epileptic seizures.
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Collection:
01-internacional
Database:
MEDLINE
Language:
En
Journal:
Epilepsia Open
Year:
2024
Document type:
Article
Affiliation country: