Anticonvulsive effect of anterior thalamic deep brain stimulation in super-refractory status epilepticus crucially depends on active stimulation zone—A single case observation

Super-refractory status epilepticus has a high mortality, and its treatment remains a challenge for clinical epileptologists. Deep brain stimulation (DBS) is successfully used in pharmacotherapy-resistant epilepsy and larger studies showed significant seizure reduction by high frequency stimulation in the anterior nucleus of the thalamus (ANT) [1]. Furthermore, several case reports indicate that DBS is also effective in the management of status epilepticus. In a recent, very comprehensive publication, Lehtimäki and co-workers reported resolution of a super-refractory non-convulsive status epilepticus with ...

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Patient and Family Perspectives of Paediatric Psychogenic Non-Epileptic Seizures: A Systematic Review

Psychogenic non-epileptic seizures (PNES) are noticeable changes in an individual’s consciousness or behaviour that share a similar appearance to epileptic seizures, with the key difference being that PNES are not accompanied by electrophysiological changes [1]. Although as many as 15 different names have been documented in describing this condition [2], PNES is the term that will be employed throughout this systematic review. Reuber [3] posits that these seizures are best understood through the lens of a biopsychosocial, etiologic model, whereby ...

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HUMAN NEOCORTICAL INTERICTAL EPILEPTIFORM DISCHARGES ARE INITIATED BY A LOW-VOLTAGE NEGATIVE POLARITY WAVE

Epilepsy is a disease that affects the electrical activity of the brain. Epileptic seizures are paroxysms of electrical discharges that disrupt the brain’s ability to process information. In between seizures, a brain with epilepsy generates isolated electrical discharges known as interictal epileptiform discharges (IEDs) (Stern and Engel, 2013). IED identification corroborates a diagnosis of epilepsy and enables classification of the underlying type of epilepsy (Stern and Engel, 2013). In some cases, IED waveforms correlate with underlying pathology (Noachtar et al., ...

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THE BEST EVIDENCE FOR PROGRESSIVE MYOCLONIC EPILEPSY: A PATHWAY TO PRECISION THERAPY

The Progressive Myoclonus Epilepsies (PMEs) are a group of uncommon clinically and genetically heterogeneous disorders (mainly autosomal recessive), characterised by myoclonus, generalized epilepsy, and progressive neurological deterioration, including dementia and ataxia [1]. PMEs are disorders with debilitating evolution, resistance to treatment and poor prognosis, and it is estimated that these diseases are responsible for up to 1% of epileptic syndromes in children and adolescents around the world.

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Tuberous Sclerosis Complex in Chinese patients: phenotypic analysis and mutational screening of TSC1/TSC2 genes

Tuberous sclerosis complex (TSC) is a rare autosomal dominant inherited disorder characterized by the growths of benign hamartomatous tumors in multiple organ systems, primarily in the brain, eyes, heart, kidney, skin and lungs [1]. The prevalence of the disease was estimated to range from 1/6000 to 1/10 000 [2]. Inactivating mutations in either of two genes, TSC1 on chromosome 9q34 (MIM 191100) and TSC2 on chromosome 16p13 (MIM 191092), were identified as responsible for TSC [3,4]. The TSC1 gene contains ...

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Using scalp EEG and intracranial EEG signals for predicting epileptic seizures: review of available methodologies

Epilepsy is a common neurological disorder in which patients suffer seizures. Being able to predict the onset of a seizure before it occurs is important since this may facilitate the prevention of accidents and injury that can occur during seizures and additionally may help with pre-seizure delivery of medication or other interventions [1]. Electrical activity in the brain can be monitored using electroencephalogram (EEG) signals [2], which can be recorded from the scalp of patients, referred to as scalp EEG ...

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Prognostic factors and seizure outcome in Posterior Reversible Encephalopathy Syndrome (PRES) in children with hematological malignancies and bone marrow failure: a retrospective monocentric study

Posterior reversible encephalopathy syndrome (PRES), as defined by Hinchey et al. (1996), is a phenomenon of transient cerebral vasogenic edema occurring preferentially in posterior circulation [1]. Clinically, PRES is characterized by headaches, seizures, reduced consciousness, and visual and other focal neurological symptoms [2,3]. PRES is a clinicoradiological syndrome; a characteristic radiologic finding is vasogenic edema in the bilateral parietal-occipital lobes, which might be related to the lower concentrations of sympathetic innervation of the posterior intracranial arteries in comparison with other ...

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Autism, ADHD and parent-reported behavioural difficulties in young children with epilepsy

Ddifficulties with neurodevelopment and behaviour are frequently reported in children with epilpesy [1]. The difficulties experienced by the children are greater than that experienced by children with other non-neurological conditions [2] and have often a greater impact on Health Related Quality of Life (HRQOL) than seizures [3]. In school-aged children the prevalence of behavioural comorbidities have been described [1]. There is however, limited data on behavior in younger children with epilepsy [4] including the presence of Autism Spectrum Disorder (ASD) ...

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Epileptic encephalopathy and brain iron accumulation due to WDR45 mutation

We present the case of a 10-year-old Chinese boy with profound developmental delay, spastic quadriparesis, and intractable epilepsy with tonic and atypical absence seizures. He was first noted to have a delay at 9 months. When evaluated at 17 months he had diffusely decreased tone, was only able to roll over but could not sit, hold his head, crawl or communicate. His seizures started at age 2 and consisted of atypical absence (hypomotor) and generalized tonic seizures occasionally preceded by ...

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Electrical stimulation mapping in children

Electrical stimulation mapping is a longstanding practice that aids in identification and delineation of eloquent cortex. Initially used to expand our understanding of the typical human cortex, it now plays a significant role in mapping cortical function in individuals with atypical structural and functional tissue organization undergoing epilepsy surgery. This review discusses the unique challenges that arise in the functional testing of the immature cortex of a child and the parameters of stimulation that optimize accurate results in conventional open ...

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