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Abstract Objective Therapeutic strategies for patients with febrile infection‐related epilepsy syndrome (FIRES) are limited, ad hoc, and frequently ineffective. Based on evidence that inflammation drives pathogenesis in FIRES, we used ex vivo stimulation of peripheral blood mononuclear cells (PBMCs) to characterize the monocytic response profile before and after therapy in a child successfully treated with dexamethasone delivered intrathecally six times between hospital Day 23 and 40 at 0.25 mg/kg/dose. Methods PBMCs were isolated from serial blood draws acquired during refractory status epilepticus (RSE) and following resolution associated with intrathecal dexamethasone therapy in a previously healthy 9‐year‐old male that presented with seizures following Streptococcal pharyngitis. Cells were stimulated with bacterial or viral ligands and cytokine release was measured and compared to responses in age‐matched healthy control PBMCs. Levels of inflammatory factors in the blood and CSF were also measured and compared to pediatric healthy control ranges. Results During RSE, serum levels of IL6, CXCL8, HMGB1, S100A8/A9, and CRP were significantly elevated. IL6 was elevated in CSF. Ex vivo stimulation of PBMCs collected during RSE revealed hyperinflammatory release of IL6 and CXCL8 in response to bacterial stimulation. Following intrathecal dexamethasone, RSE resolved, inflammatory levels normalized in serum and CSF, and the PBMC hyperinflammatory response renormalized. Significance FIRES may be associated with a hyperinflammatory monocytic response to normally banal bacterial pathogens. This hyperinflammatory response may induce a profound neutrophil burden and the consequent release of factors that further exacerbate inflammation and drive neuroinflammation. Intrathecal dexamethasone may resolve RSE by resetting this inflammatory feedback loop.

Summary Objective To investigate parental perception of FIRES outcomes, assess emotional states and related social media usage. Methods A survey‐based study of parents of children with FIRES participating in a FIRES‐specific Facebook group was performed. The survey collected information on medical aspects of their child's course in the acute, subacute, and chronic periods, emotional states, and social media usage. Child outcome was assessed utilizing the pediatric extended Glasgow outcome scale (GOS‐E). Parental emotional states were assessed utilizing the Depression, Anxiety and Stress Scale (DASS). Descriptive statistics were performed. Associations were described using the Spearman rank correlation. Open‐ended questions were included. Thematic analysis was performed. Results Twenty‐nine surveys were analyzed. All children were in the chronic phase at time of survey response, except for two who died. Mothers answered 22 surveys, and fathers answered seven. Median age at FIRES presentation was 5.6 years [IQR 4.2‐8.95], with a median number of 3 seizures per week [IQR 0‐10, range 0‐50], 4 daily anti‐seizure medicines [IQR 3‐5], and chronic GOS‐E of 6 [IQR 2‐8 range 2‐8]. Most parents reported none to mild levels of depression, anxiety, and stress. Higher seizure burden positively correlated with parental depression symptoms (r = .41 (95% CI 0.01, 0.70), P = .045). Most parents found social media helpful with coping and 96% desired FIRES research advertised. Twenty‐five parents shared their recommendations to fellow parents and the medical team in an open‐ended format. Themes included support, expertise, and medical advice. Significance Despite their children's significantly impaired functional outcome after FIRES and high rates of medically refractory epilepsy, the cohort demonstrated remarkable emotional resilience. They perceive social media as beneficial, are interested in social media‐advertised research, and share valuable advice. Social media may serve as an introductory platform to enhance the physician‐scientist‐parent/patient relationship.

Electrical status epilepticus of slow-wave sleep (ESES) is characterized by excessive interictal spike-wave discharges on EEG during sleep and can occur in the absence of overt clinical seizures. Continuous spike-wave during slow wave sleep (CSWS), an epilepsy syndrome associated with ESES, is associated with a plateau/decline in cognitive development and increases in behavioral and emotional dysregulation. Here we present a case in which neuropsychological (NP) evaluation initially ordered based on memory and attention concerns led to the identification of subclinical seizure activity and an evolving epileptic encephalopathy in an 11-year-old child with a history of remote neurological insult. The patient was referred for an initial NP evaluation at age 8 which revealed weaknesses in functions typically mediated by the dominant (usually left) hemisphere juxtaposed with her left hemiparesis. EEG was recommended which showed independent, multifocal spike and sharp wave discharges exacerbated by sleep. Follow-up NP evaluations over the following 26 months, during which time aggressive treatment was initiated, coincided with EEG findings of an evolving epileptic encephalopathy in the patient who continued to remain free from clinical seizures. This case highlights the importance of comprehensive epilepsy care and routine involvement of neuropsychology in the management of complex epilepsy patients.

Exercise-induced rhabdomyolysis has been described in military recruits, trained athletes and daily runners. Statin use, quail ingestion, infection by Epstein-Barr virus (EBV), and hypothyroidism, though rare, are risk factors for the development of rhabdomyolysis. We describe the case of a 15-year-old female who presented with myalgias, weakness, and pigmenturia following marching band practice. Laboratory tests confirmed an elevated creatine kinase (CK) level as well as a profound hypothyroid state. Muscle biopsy revealed severe muscle necrosis and myositis. Treatment with levothyroxine resulted in obtaining an euthyroid state and regain of muscle strength as well as decrease in CK levels. Although rare, hypothyroidism should be considered as a potential cause of rhabdomyolysis in pediatric patients undergoing a myopathy workup.