ESTADO EPILÉPTICO DE INICIO RECIENTE SUPERREFRACTARIO ASOCIADO A PROBABLE ENCEFALITIS AUTOINMUNE TRATADO CON ÉXITO MEDIANTE TERAPIA CETOGÉNICA.
Authors/Creators
- 1. Fundación Hospital San Pedro. San Juan de Pasto, Nariño, Colombia
- 2. Universidad de Nariño. Fundación Hospital San Pedro. San Juan de Pasto, Nariño, Colombia
-
3.
University of Nariño
- 4. Fundación Universitaria San Martín. Fundación Hospital San Pedro. San Juan de Pasto, Nariño, Colombia
- 5. Universidad Cooperativa de Colombia. Fundación Hospital San Pedro. San Juan de Pasto, Nariño, Colombia.
Description
Tipo de artículo: Caso clínico
El estatus epiléptico superrefractario asociado a New-onset refractory status epilepticus constituye una emergencia neurológica de elevada mortalidad y complejidad terapéutica, frecuentemente relacionada con mecanismos neuroinflamatorios e inmunomediados.
Se presenta el caso de una mujer de 26 años sin antecedentes neurológicos previos, quien desarrolló alteración neuropsiquiátrica subaguda seguida de crisis convulsivas refractarias que evolucionaron hacia estatus epiléptico superrefractario. Los estudios iniciales descartaron causas estructurales e infecciosas; el líquido cefalorraquídeo mostró perfil inflamatorio leve no supurativo y la resonancia magnética cerebral evidenció compromiso temporal bilateral compatible con encefalitis límbica de probable etiología autoinmune. El electroencefalograma documentó actividad epileptiforme focal persistente.
A pesar del manejo anticonvulsivante multimodal, sedación profunda, anestésicos intravenosos, corticoides y plasmaféresis, persistió recurrencia ictal. Debido a la refractariedad clínica se instauró dieta cetogénica enteral como estrategia terapéutica adyuvante, observándose disminución progresiva de la actividad convulsiva, retiro exitoso de anestésicos intravenosos y recuperación neurológica gradual.
Este caso resalta la importancia del reconocimiento temprano de encefalitis autoinmune asociada a NORSE y respalda el potencial papel inmunometabólico de la dieta cetogénica en escenarios de extrema refractariedad neurológica.
Abstract (English)
Article type: Clinical case
Super-refractory status epilepticus associated with New-onset refractory status epilepticus (NORSE) represents a neurological emergency with high mortality and major therapeutic complexity, frequently linked to neuroinflammatory and immune-mediated mechanisms.
We present the case of a 26-year-old woman without previous neurological history who developed subacute neuropsychiatric deterioration followed by refractory seizures that progressed to super-refractory status epilepticus. Initial studies excluded structural and infectious causes; cerebrospinal fluid analysis demonstrated a mild non-suppurative inflammatory profile, while brain magnetic resonance imaging revealed bilateral temporal involvement consistent with limbic encephalitis of probable autoimmune origin. Electroencephalography documented persistent focal epileptiform activity.
Despite multimodal anticonvulsant therapy, deep sedation, intravenous anesthetics, corticosteroids, and plasmapheresis, ictal recurrence persisted. Due to ongoing clinical refractoriness, enteral ketogenic diet therapy was initiated as an adjuvant therapeutic strategy, resulting in progressive reduction of seizure activity, successful withdrawal of intravenous anesthetics, and gradual neurological recovery.
This case highlights the importance of early recognition of autoimmune encephalitis associated with NORSE and supports the potential immunometabolic role of ketogenic diet therapy in scenarios of extreme neurological refractoriness.
Files
sanum_v10_n3_a20.pdf
Files
(341.8 kB)
| Name | Size | Download all |
|---|---|---|
|
md5:8e29aab8075727a8b74d14605ee44bd2
|
341.8 kB | Preview Download |
Additional details
Additional titles
- Translated title (English)
- SUPER-REFRACTORY NEW-ONSET STATUS EPILEPTICUS ASSOCIATED WITH PROBABLE AUTOIMMUNE ENCEPHALITIS SUCCESSFULLY MANAGED WITH KETOGENIC THERAPY
Dates
- Collected
-
2026-05-23manuscrito recibido
- Accepted
-
2026-07-04evaluación doble ciego
- Available
-
2026-07-29publicación en número de la revista
Software
- Repository URL
- https://revistacientificasanum.com/
References
- 1. Wickström R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, et al. the International NORSE Consensus GroupInternational consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection-related epilepsy syndrome (FIRES): Summary and clinical tools. Epilepsia. 2022;63:2827–2839. https://doi.org/10.1111/epi.17391 2. Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, Navarro V, Specchio N, Lagae L, Rossetti AO, Hocker S, Gofton TE, Abend NS, Gilmore EJ, Hahn C, Khosravani H, Rosenow F, Trinka E. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018 Apr;59(4):739-744. doi: 10.1111/epi.14016. Epub 2018 Feb 5. PMID: 29399791. 3. Gaspard N, Hirsch LJ, Sculier C, Loddenkemper T, van Baalen A, Lancrenon J, Emmery M, Specchio N, Farias-Moeller R, Wong N, Nabbout R. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia. 2018 Apr;59(4):745-752. doi: 10.1111/epi.14022. Epub 2018 Feb 24. PMID: 29476535. 4. Lattanzi S, Leitinger M, Rocchi C, Salvemini S, Matricardi S, Brigo F, Meletti S, Trinka E. Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies. Eur J Neurol. 2022 Feb;29(2):626-647. doi: 10.1111/ene.15149. Epub 2021 Nov 2. PMID: 34661330; PMCID: PMC9298123. 5. Uy CE, Binks S, Irani SR. Autoimmune encephalitis: clinical spectrum and management. Pract Neurol. 2021 Oct;21(5):412-423. doi: 10.1136/practneurol-2020-002567. Epub 2021 Jun 9. PMID: 34108243; PMCID: PMC8461404. 6. Steriade, C., Bauer, J. & Bien, C.G. Autoimmune encephalitis-associated epilepsy. Nat Rev Neurol 21, 312–326 (2025). https://doi.org/10.1038/s41582-025-01089-4 7. Melzer N, Rosenow F; Autoimmune-associated epilepsy – a challenging concept; Seizure - European Journal of Epilepsy, 2024; 128, 20-23. 8. Hanin A, Muscal E, Hirsch LJ. Second-line immunotherapy in new onset refractory status epilepticus. Epilepsia. 2024;65:1203–1223. https://doi.org/10.1111/epi.17933