Efficacy of the ketogenic diet in patients with Dravet syndrome: A meta-analysis

Dravet syndrome (DS), otherwise known as severe myoclonic epilepsy of infancy (SMEI), is a severe and rare drug-resistant seizure disorder with an incidence of 1 in 20,000 to 1 in 40,000 [1–2]. Between 70% and 80% of patients with DS carry sodium channel α1 subunit gene (SCN1A) abnormalities [3]. The characteristics of DS include early onset (before 1 year of age). Infants with DS often present with prolonged, hemiconvulsive seizures triggered by fever; other seizure types, including focal with impaired awareness, ...

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Sirolimus for seizure control in children with Tuberous sclerosis: Is it really a wonder drug?

We read with great interest the recently published article titled “Sirolimus improves seizure control in pediatric patients with tuberous sclerosis: A prospective cohort study” by Wen He et al, which concludes sirolimus can be used as a first-line antiepileptic drug in children with tuberous sclerosis (TSC) and can cause significant gain in development milestones [1]. However, we wish to add a few comments.

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Chrysostomos (Tomis) P. Panayiotopoulos

I was privileged to meet Tomis when I was a registrar at St Thomas’ Hospital in 1992, fresh from early training at the National Hospital for Neurology, Queen Square. Tomis’ enthusiasm for his specialities, clinical neurophysiology and epileptology, was infectious. His personal charm was immediately apparent, which together with his smile and charming Greek accent gave him an air of approachability. He was invariably polite in the face of ignorance, and encouraged all around him by the strategy of talking ...

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The value of rapid eye movement sleep in the localization of epileptogenic foci for patients with focal epilepsy

Epilepsy is one of the common neurological diseases, characterized by abnormally synchronized discharges of brain neurons [1]. There are more than 50 million epileptic patients worldwide, 30% of whom are refractory [2], and the poor therapeutic effects contribute heavy psychological and economic burdens on patients and their own families. Epileptic surgery can sometimes have seizure free or reduce the frequency of seizures, of which the premise is to accurately localize the epileptogenic foci. Currently, the localization of epileptogenic foci involves ...

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Epileptogenesis-Induced Changes of Hippocampal-Piriform Connectivity

Human experience [1,2] and many animal models [3,4] of epilepsy follow a classic scenario of an inciting epileptogenic event followed by a latency phase before emergence of spontaneous recurrent seizures (SRS). There is evidence that tissue remodeling occurs during the latency period, which facilitates the initiation and propagation of epileptic seizures [5–7]. Biomarkers of such remodeling can be of value for diagnosis and assessment of response to therapeutic interventions. Cerebro-cerebral evoked potentials (CCEPs) recorded in the seizure network in response ...

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Caregiver burden in psychogenic non-epileptic seizures

Psychogenic non-epileptic seizures (PNES) semiologically mimic epileptic seizures (ES) but lack their neurobiological and neurophysiological basis.[1] PNES represent 10-30% of referrals to epilepsy specialists,[2,3] and their prevalence in the general population is estimated at 2 to 33 per 100,000.[3] They constitute a significant public health issue with an estimated lifetime cost per patient cohort year in the US ranging from $110-920 million.[4]

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Neonatal neuroimaging and neurophysiology predict infantile onset epilepsy after perinatal hypoxic ischemic encephalopathy

Hypoxic-ischemic encephalopathy (HIE) due to perinatal asphyxia occurs in approximately 2,5 per 1000 live full-term births [1], and is one of the leading causes of neonatal deaths and severe developmental and neurological compromise [2]. HIE is also one of the most common causes of infantile spasms syndrome (IS) [3] accounting for 8-10% of all IS cases [4,5]. HIE is currently treated with therapeutic hypothermia, which despite its favorable effect on the overall outcome, does not affect the rate of postneonatal ...

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Slow Wave Activity during NREM Sleep in Patients with Electrical Status Epilepticus in Sleep

We share our research findings regarding slow-wave activity (SWA) evaluation in patients with Electrical Status Epilepticus in Sleep (ESES) compared to sex-matched controls. Slow-wave sleep plays an important role in memory consolidation and learning processes1,2. Many patients with ESES present with neurocognitive and learning difficulties3. Impaired SWA in sleep, in particular downscaling of slow wave activity during the course of the night, may therefore in part be related to neurocognitive difficulties in patients with ESES3.

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