Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis

Meenakshisundaram, S.; Sankaranarayanan, Muthukani; Jeyaraman, Murugan; Ayyappan, C; Karthik, Somalinga Nagendran; Pandi, Suresh

doi:10.1002/epi4.12454

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…Five out of 19 studies viz. Mewasingh et al, Liaqat et al, Caranzano et al, Basha et al and Meenakshi-Sundaram et al reported on recurrence of seizures either during hospitalization (10 patients) or during follow up (1 patient) after ketamine treatment [ 17 , 20 , 21 , 24 , 30 ]. Among ten patients, further treatment with either ketamine or propofol or surgical intervention led to resolution of seizures.…”

Section: Resultsmentioning

confidence: 99%

Use of ketamine in Super Refractory Status Epilepticus: a systematic review

Adhikari,

Yadav,

Nepal

et al. 2024

Neurol. Res. Pract.

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Objective This review specifically investigates ketamine’s role in SRSE management. Methods PubMed, EMBASE, and Google Scholar databases were searched from inception to May 1st, 2023, for English-language literature. Inclusion criteria encompassed studies on SRSE in humans of all ages and genders treated with ketamine. Results In this systematic review encompassing 19 studies with 336 participants, age ranged from 9 months to 86 years. Infections, anoxia, and metabolic issues emerged as the common causes of SRSE, while some cases had unknown origins, termed as NORSE (New Onset RSE) or FIRESs (Febrile Infection-Related Epilepsy Syndrome). Most studies categorized SRSE cases into convulsive (N = 105) and non-convulsive (N = 197). Ketamine was used after failed antiepileptics and anesthetics in 17 studies, while in others, it was a first or second line of treatment. Dosages varied from 0.5 mg/kg (bolus) and 0.2-15 mg/kg/hour (maintenance) in adults and 1-3 mg/kg (bolus) and 0.5-3 mg/kg/hour (maintenance) in pediatrics, lasting one to 30 days. Ketamine was concurrently used with other drugs in 40–100% of cases, most frequently propofol and midazolam. Seizure resolution rate varied from 53.3 to 91% and 40–100% in larger (N = 42–68) and smaller case series (N = 5–20) respectively. Seizure resolution occurred in every case of case report except in one in which the patient died. Burst suppression in EEG was reported in 12 patients from two case series and two case reports. Recurrence was reported in 11 patients from five studies. The reported all-cause mortality varied from 38.8 to 59.5% and 0-36.4% in larger and smaller case series., unrelated directly to ketamine dosage or duration. Significance Ketamine demonstrates safety and effectiveness in SRSE, offering advantages over GABAergic drugs by acting on NMDA receptors, providing neuroprotection, and reducing vasopressor requirement.

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Section: Resultsmentioning

confidence: 99%

Use of ketamine in Super Refractory Status Epilepticus: a systematic review

Adhikari,

Yadav,

Nepal

et al. 2024

Neurol. Res. Pract.

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“…Haemophagocytic lymphohistiocytosis (HLH) is a rare haematological syndrome of severe immune activation with macrophage and T-cell infiltration resulting in multiorgan damage. The association of HLH and FIRES has been described in paediatric patients by Farias-Moeller et al and Meenakshi-Sundaram et al 2 3. We describe the management of an adult patient with FIRES and HLH.…”

Section: Introductionmentioning

confidence: 68%

“…Of the remaining two patients, one had moderate cognitive impairment, and the other was dependent on all activities of daily living. Meenakshi-Sundaram et al reported a 14-year-old with FIRES and secondary HLH, with viral illness 9 days before admission 2. The patient died despite chemotherapy and immunotherapy.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory epilepsy (FIRES) and haemophagocytic lymphohistiocytosis (HLH): an adult case

et al. 2023

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We describe a man in his 30s with haemophagocytic lymphohistiocytosis (HLH), secondary to an upper respiratory tract infection, with subsequent febrile infection-related epilepsy syndrome. He had a prolonged hospital admission, during which he was treated with chemotherapy for HLH and antiepileptic medications for refractory seizures. He was discharged fully dependent to a care facility and died from aspiration pneumonia 11 months later. This case report highlights his management and discusses these conditions’ pathophysiology and future management.

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Hemophagocytic lymphohistiocytosis in an adult patient with super‐refractory status epilepticus

Haanpää,

Kämppi,

Kantonen

et al. 2024

Epilepsia Open

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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 SummaryThis 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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Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis

Cited by 3 publications

References 18 publications

Use of ketamine in Super Refractory Status Epilepticus: a systematic review

Use of ketamine in Super Refractory Status Epilepticus: a systematic review

Inflammatory epilepsy (FIRES) and haemophagocytic lymphohistiocytosis (HLH): an adult case

Hemophagocytic lymphohistiocytosis in an adult patient with super‐refractory status epilepticus

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