難治頻回部分発作重積型急性脳炎

Self-inflicted oral injuries in children with neurological disorders represent a significant clinical concern due to their multifactorial impact on oral and systemic health. Neurological disorders, such as febrile infection-related epilepsy syndrome (FIRES) and glutaric aciduria type I, may cause dystonia, involuntary orofacial movements, and self-inflicted biting, resulting in oral trauma. Management typically requires an interdisciplinary approach combining behavioral and pharmacologic strategies with protective intraoral appliances and, in selected cases, to prevent further trauma and preserve function. Three pediatric cases with neurological disorders in this series presenting with self-inflicted oral injuries were successfully managed with a customized intraoral appliance. All children showed mucosal healing within 7-10 days and no recurrence of trauma during the periodic follow-ups. Customized appliances provided effective protection, durability, and hygiene compatibility.

RATIONALE: This case series discusses the nursing care and clinical outcomes of 3 pediatric patients with febrile infection-related epilepsy syndrome (FIRES) and acute-phase complications, focusing on critical care interventions such as seizure management, respiratory support, and nutritional therapy. PATIENT CONCERNS: Three male patients, aged 10, 5, and 7, presented with altered consciousness, seizures, and recurrent fever. Diagnostic tests, including magnetic resonance imaging, electroencephalogram (EEG), and cerebrospinal fluid analysis, revealed abnormal EEG findings, suspected meningeal inflammation, and cerebrospinal fluid abnormalities. DIAGNOSES: All patients were diagnosed with FIRES, accompanied by acute neurological deterioration, supported by EEG and magnetic resonance imaging. INTERVENTIONS: All patients required intensive care unit mechanical ventilation. Two underwent tracheostomy during prolonged ventilator dependence, whereas 1 was extubated to low-flow nasal oxygen after approximately 14 days without tracheostomy. Seizure management included anticonvulsants and a ketogenic diet, with individualized adjustments according to clinical response. Multidisciplinary care involved specialists in neurology, respiratory medicine, and rehabilitation. OUTCOMES: Short-term outcomes differed. One tracheostomized patient was later decannulated and discharged clinically improved, with recovery of communication, oral intake, and independent ambulation. The non-tracheostomized patient remained off invasive ventilation but continued inpatient neurologic and nutritional management, while the other tracheostomized patient still required ventilatory and neurologic management in the latest available record. LESSONS: Coordinated multidisciplinary nursing, early nutritional planning, and structured monitoring may help organize acute-phase FIRES care while supporting individualized respiratory, seizure, and complication management.

Febrile infection-related epilepsy syndrome (FIRES) is a rare cause of new-onset refractory status epilepticus following a febrile illness. We report the case of a previously healthy three-year-old boy who developed focal seizures in the context of a confirmed Influenza A infection, rapidly progressing to super-refractory status epilepticus. Initial investigations, including cerebrospinal fluid analysis and neuroimaging, were unremarkable. Despite early multimodal therapy with multiple antiseizure medications, continuous anesthetic infusions, immunomodulatory treatments, and a ketogenic diet, seizure control remained transient. Continuous EEG confirmed persistent super-refractory status epilepticus, while cytokine analysis demonstrated elevated interleukin-6 and interleukin-8 levels. Follow-up MRI showed bilateral claustrum and hippocampal involvement, later progressing to diffuse cerebral atrophy. Despite escalation of therapy, including intrathecal corticosteroids and vagal nerve stimulation, the patient showed no sustained improvement. Care was ultimately redirected toward palliation, and the patient died after 50 days of intensive care. This case highlights the fulminant and refractory nature of FIRES, supports a potential role of cytokine-driven neuroinflammation, and suggests a possible association with a preceding viral infection.

OBJECTIVES: Super-refractory status epilepticus (SRSE) is a neurological emergency with high morbidity and mortality. Cenobamate, a novel antiseizure medication, may be helpful in managing SRSE, but evidence is limited. METHODS: This retrospective case series reports the use of cenobamate as add-on therapy in the management of three cases of SRSE. RESULTS: Median age at status epilepticus (SE) onset was 28 years (range 27-75). The etiologies of SRSE included one case of febrile infection-related epilepsy syndrome (FIRES), one patient with a probable genetic etiology (SCN7A variant of uncertain significance), and one case of unknown etiology. Cenobamate was introduced at a median of 27 days (range 13-103) after SE onset. Resolution of SRSE was observed after a median of 7 days (range 3-30) once cenobamate was started. Median dose of cenobamate at SRSE cessation was 25 mg/day (range 12.5-50 mg/day), and the median maintenance dose at the time of discharge was 200 mg/day (range 100-400 mg/day). Two patients died and one patient achieved functional independence. No severe medication side effects, specifically drug reaction with eosinophilia and systemic symptoms (DRESS), were observed. DISCUSSION: Cenobamate may have a role in the management of SRSE. Further studies are needed to define the optimal timing of initiation, titration strategy, and target dose of cenobamate in the context of SE.

BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) is a severe epileptic encephalopathy with limited treatment options. Neuroinflammation, particularly involving IL-1β and IL-6, is a key driver. Evidence on sequential use of IL-6 receptor blockade (tocilizumab) followed by IL-1 receptor blockade (anakinra) is scarce, especially when IL-1β levels are normal. CASE PRESENTATION: A previously healthy 6-year-old boy developed super-refractory status epilepticus six days after influenza A infection. He failed multiple antiseizure medications, anesthetics, corticosteroids, intravenous immunoglobulin, ketogenic diet, and mild therapeutic hypothermia. Cerebrospinal fluid (CSF) and serum cytokine testing showed elevated IL-6 but normal IL-1β. After initial improvement with tocilizumab, seizures relapsed. Tocilizumab retreatment and plasma exchange were ineffective. Switching to daily subcutaneous anakinra led to rapid seizure control within five days. He was weaned off anesthetics, extubated, and eventually discharged with mild cognitive dysfunction (PCPC score 2). At one-month follow-up, he had rare brief seizures and attended school. CONCLUSION: This case demonstrates that anakinra can be effective in tocilizumab-refractory FIRES even when CSF and serum IL-1β levels are normal. Anakinra should be considered as a second-line option in selected patients, regardless of IL-1β biomarker status.