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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.

Background: Electrical Status Epilepticus in Sleep (ESES) is an epileptic encephalopathy syndrome characterized by infrequent clinical seizures and prominent interictal burden during slow wave sleep associated with cognitive deficits and behavioral dysfunction. Medical treatment with anti-epileptic drugs is often unsuccessful. Resective surgery may be a valuable option in carefully selected patients. This case series aims to describe the indications, long term results and utility of resective surgery for ESES.Methods: Information on 14 patients who underwent surgery for epilepsy and ESES at the Children's Hospital of Wisconsin between 2007 and 2017 is included. Clinical, electrographic and neuropsychological features and outcomes are described in detail.Results: The most common pathology was encephalomalacia due to perinatal middle cerebral artery stoke (5/14). Twelve patients had imaging findings of perinatal pathologies; however, two patients had normal magnetic resonance imaging. Surgery was performed to control refractory epilepsy in eight patients. Six patients had no clinical seizures for 1–6 years prior to surgery, one of which had no known clinical seizures at all. All showed cognitive declines (6/14) or impairment (8/14) on neuropsychological assessments, and surgery was suggested to minimize further cognitive declines. The most common surgical procedure was hemispherotomy (10/14). Temporo-parieto-occipital disconnection, frontal lobectomy, parieto-occipital resection, and limited corticectomy were also used, with good outcomes for the first three procedures. Clinical follow up mean was 4.4 years and 12 patients had excellent seizure outcome. Electroencephalography (EEG) follow up mean was 3 years and ESES resolved in 12/14 patients. All patients completed post-surgical neuropsychological evaluation with mean follow-up of 17.46 months.Conclusions: Resective surgery is an effective treatment for selected cases of ESES, producing long term seizure freedom, resolution of ESES and stabilization of cognitive and behavioral functioning in most patients. Our case series is the largest single center cohort description addressing resective surgery for ESES. Outcomes in this sample suggest that good long-term seizure, EEG and cognitive/behavioral outcomes can be achieved in patients with normal brain imaging and in limited lobar or multi-lobar resections. Moreover, patients with ESES and very infrequent clinical seizures can benefit from surgery with stabilization of cognitive and behavioral functioning.

Gelastic seizures (GS) are typically associated with hypothalamic hamartomas and present during childhood. However it is now known that GS can be found in focal epilepsies arising from other regions in the brain, including mesial and neocortical frontal, temporal and parietal regions. GS have rarely been described as the presenting manifestation of New Onset Refractory Status Epilepticus (NORSE). In this article we describe a previously healthy 3-year-old who presented with an explosive onset of GS that were refractory to multiple anti-seizure medications. These seizures arose from the right frontal region. An extensive metabolic and immunological evaluation was negative. Her brain magnetic resonance imaging (MRI) was negative, however the Positron Emission Tomography (PET) scan showed a hypermetabolic region in the right frontal inferior gyrus. She underwent a depth electrode evaluation that revealed a widespread irritative zone involving the PET “lesion” as well as mesial and neocortical regions in the right frontal lobe. The seizure onset zone was widespread and non-localizable. However the GS were associated with a clear ictal epileptiform discharge on invasive EEG arising from the depth of the superior frontal gyrus, which was not overlapping with the PET hypermetabolic region. She underwent a right frontal lobectomy sparing the primary motor region in the pre-central gyrus. She has remained seizure free for 15 months since. The pathological analysis showed focal cortical dysplasia type II in the region of the PET scan hypermetabolism. This case expands the clinical spectrum of GS to include cases of NORSE. Additionally the case highlights the role of resective surgery in GS presenting as NORSE and the potentially excellent outcome that can be achieved by early intervention.