Cerebral hemiatrophy and hemiparesis following hemiclonic status epilepticus in Dravet syndrome.

Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome.

Treatment of seizures in the neonate: Guidelines and consensus-based recommendations-Special report from the ILAE Task Force on Neonatal Seizures.

Seizures are common in neonates, but there is substantial management variability. The Neonatal Task Force of the International League Against Epilepsy (ILAE) developed evidence-based recommendations about antiseizure medication (ASM) management in neonates in accordance with ILAE standards. Six priority questions were formulated, a systematic literature review and meta-analysis were performed, and results were reported following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) 2020 standards.

Cannabidiol in children with treatment-resistant epilepsy with myoclonic-atonic seizures.

Purpose: This multicenter study aimed to evaluate the efficacy and tolerability of add-on cannabidiol (CBD) in treatment-resistant patients with epilepsy with myoclonic-atonic seizures (EMAtS) (n = 22) and Sturge Weber syndrome (SWS) with myoclonic-atonic seizures (n = 4).

Methods: Patients who met the diagnostic criteria of treatment-resistant EMAtS or SWS with myoclonic-atonic seizures were included. Cannabidiol was added in doses ranging from 8 to 40 mg/kg/day.

Use of sulthiame as add-on therapy in children with non-self-limited focal epilepsies of childhood.

Purpose: This retrospective study aimed to evaluate the efficacy and tolerability of sulthiame (STM) as an add-on treatment in 49 patients with non-self-limited focal epilepsies of childhood (non-SeLFE) resistant to other antiseizure medications (ASM) and/or non-pharmacological treatment.

Methods: Patients with non-SeLFE who had failed to respond to at least five previous ASM, alone or in combination, were included in the study. All patients underwent neurological examination, brain magnetic resonance imaging repeated prolonged electroencephalography (EEG) or video-EEG studies, and neurometabolic studies.

Use of sulthiame as add-on therapy in children with myoclonic atonic epilepsy: A study of 35 patients.

Purpose: The aim of this retrospective study was to evaluate efficacy and tolerability of sulthiame (STM) as add-on treatment in 35 patients with myoclonic atonic epilepsy (MAE) resistant to other antiseizure medications (ASMs) and/or non-pharmacological treatment.

Methods: Patients were selected according to the diagnostic definition of MAE and were resistant to at least four previous to ASM, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies as well as neurometabolic studies were performed in all cases.

Panayiotopoulos syndrome: Unusual clinical manifestations.

Purpose: We retrospectively analyzed the electroclinical features, treatment, and outcome of patients with Panayiotopoulos syndrome (PS) who presented with unusual clinical manifestations.

Method: A retrospective, descriptive, multicenter study was conducted evaluating 44 patients with PS who had seizures with an unusual semiology. Data from patients with PS seen at eight Argentine centers between April 2000 and April 2019 were collected.

More than one self-limited epilepsy of childhood in the same patient: A multicenter study.

Objective: We describe the evolution of the electroclinical picture of patients with different types of self-limited epilepsy of childhood (SLEC) occurring at the same or at different times with or without atypical evolutions as well as patients with SLEC associated with childhood absence epilepsy (CAE).

Material And Methods: A multicenter, retrospective, descriptive study was conducted evaluating patients with SLEC who had focal seizures of different types of SLEC including atypical evolutions as well as SLEC associated with absence epilepsy seen at eight Argentinian centers between April 2000 and April 2019. Of 7705 patients with SLEC, aged between 2 and 14 years (mean, 7.

Self-limited epilepsy of childhood with affective seizures: A well-defined epileptic syndrome?

Objective: Here we present cases of focal epilepsy with affective symptoms analyzing seizure characteristics, EEG pattern, treatment, and outcome.

Methods: A multicenter, descriptive, retrospective study was conducted evaluating 18 patients with self-limited epilepsy who presented with seizures with affective symptoms seen between April 2000 and April 2018 at eight Argentinian centers.

Results: Eighteen patients had focal seizures with affective symptoms; all of them had affective symptoms characterized by sudden fright or terror and screaming.

Self-limited epilepsy with centro-temporal spikes: A study of 46 patients with unusual clinical manifestations.

Purpose: We retrospectively analyzed the seizure characteristics, EEG pattern, treatment, and outcome in a series of patients with self-limited epilepsy with centrotemporal spikes (SLECTS) who presented with unusual clinical manifestations.

Method: A retrospective, descriptive, multicenter study was conducted evaluating 46 patients with SLECTS who had seizures with an unusual semiology. We collected data from patients with SLECTS seen at eight Argentine centers between April 1998 and April 2018.

Telemedicine and epilepsy: A patient satisfaction survey of a pediatric remote care program.

Purpose: The aim of this study was to assess parent satisfaction three years after the implementation of a pediatric epilepsy telemedicine program.

Methods: The program was developed with support from the Ministry of Health through Hospital Nacional de Pediatría J.P.

Rufinamide as add-on therapy in children with epileptic encephalopathies other than Lennox-Gastaut syndrome: A study of 34 patients.

Objective: Here, we present a multicenter series of patients with developmental and epileptic encephalopathies (DEE) and related electroclinical patterns (REP) other than Lennox-Gastaut syndrome (LGS) who were treated with rufinamide as add-on therapy.

Methods: Medical records of 34 patients with DEE and REP other than LGS treated with add-on rufinamide seen at four pediatric neurology centers in Argentina between May 2014 and March 2019 were retrospectively analyzed.

Results: We evaluated 34 patients (18 males, 16 females), aged between 2 and 15 years with a mean and median age of 6 and 8 years, respectively.

Benign infantile seizures and paroxysmal dyskinesia: a well-defined familial syndrome.

Unlabelled: The aim of this study was to analyze the electroclinical features and evolution in patients with benign infantile seizures (BIS) associated with paroxysmal dyskinesia (PD).

Patients And Methods: Among 198 patients with BIS (78 of whom were familial cases), we evaluated 12 unrelated patients with BIS and PD seen at two pediatric neurology departments from January 1990 to February 2009.

Results: The patients were eight boys and four girls, one of whom was not a familial case.

Benign infantile seizures associated with gastroenteritis and benign infantile seizures alone in the same patients: Related syndromes or coincidental entities?

We report two patients, the first one had benign infantile seizures (BIS) associated with gastroenteritis (BISG), followed by BIS without gastroenteritis, in the second patient BIS was followed by BISG related to anti-rotavirus vaccine. After 12 and 6 months of follow-up, both patients had normal psychomotor development. BISG may be a situation-related seizure, a coincidental entity associated with BIS.

Benign infantile seizures: a prospective study.

Introduction: One idiopathic focal epileptic syndrome with onset during infancy is recognized, the benign infantile seizures (BIS).

Objective: To analyze the electroclinical features and evolution in patients with BIS and assess the difference between familial and non-familial infantile cases.

Patients And Methods: We performed a prospective follow-up study in 41 patients seen at our department between September 2002 and March 2004, with BIS from 2 to 12 months of age.

Benign infantile seizures with mild gastroenteritis: study of 22 patients.

Purpose: To analyze the electroclinical features, aetiology and outcome in patients with normal neurological examination and psychomotor development who presented seizures during a mild gastroenteritis (MG).

Patients And Methods: Evaluation of the clinical charts of 22 patients who were assessed in the Neurology Department, Hospital Nacional de Pediatría Prof. Dr.

Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a study of nine cases.

Purpose: We present a series of nine patients with early-onset hydrocephalus who had seizures and continuous spikes and waves during slow sleep (CSWS) associated with neurocognitive and motor deterioration.

Methods: Six boys and three girls aged 9-16 years (mean 11.3 years) were studied.

Myoclonic status in nonprogressive encephalopathies: study of 29 cases.

Purpose: Myoclonic status in nonprogressive encephalopaties (MSNE) is characterized by recurrence of long-lasting myoclonic status appearing in infants and young children with nonprogressive encephalopathy. Here, we describe the electroclinical features and evolution of MSNE.

Methods: Between February 1, 1990 and July 31, 2005, 29 patients who met diagnostic criteria of MSNE were enrolled in the study at our department and have been followed up to the present time.

[Angelman syndrome: the electroclinical characteristics in 35 patients].

Aims: The purpose of this study is to report on 35 patients with Angelman syndrome (AS) in whom we evaluated the electroclinical characteristics and the progression of their epilepsy.

Patients And Methods: The following factors were evaluated: sex, family background, neurological examination, age at onset and semiology of the epileptic seizures, EEG, types of epilepsy according to the international classification and response to therapy. We investigated the karyotype, and conducted FISH and methylation tests for AS.

Benign familial and non-familial infantile seizures: a study of 64 patients.

Introduction: The recently proposed diagnostic scheme for people with epileptic seizures and with epilepsy (Epilepsia 2001) includes two idiopathic focal epileptic syndromes with onset during the first year of life, the benign familial and non-familial infantile seizures.

Objectives: To analyze the electroclinical features and evolution in patients with benign familial and non-familial infantile seizures.

Patients And Methods: Sixty-four patients (36 males and 28 females) were evaluated at the Neurology Department of the J.

Bilateral posterior agyria-pachygyria and epilepsy.

We analyzed the electroclinical findings in two patients with bilateral posterior agyria-pachygyria. Both patients presented with mental retardation, mild motor deficit and epilepsy. The electroclinical findings were characterized by frequent tonic or atonic generalized seizures with occasionally simple or complex partial seizures.