Congenital hemiparesis, unilateral polymicrogyria and epilepsy with or without status epilepticus during sleep: a study of 66 patients with long-term follow-up.

Aim: We retrospectively analysed the electroclinical features, treatment, and outcome in patients with unilateral polymicrogyria (PMG), focussing on epileptic syndrome with or without encephalopathy, with status epilepticus during sleep (ESES) or continuous spikes and waves during slow sleep (CSWS) syndrome.

Methods: From June 1990 to December 2012, 39 males and 27 females, aged 5-26 years, were studied. We did not include patients with bilateral PMG or cases with unilateral PMG associated with other cerebral lesions.

Electroclinical overlap of two types of epileptic encephalopathy occurring in the same children in a certain age period?

In this study, we describe three patients who each had an electroclinical overlap of two different epileptic encephalopathies (EE), with onset in a certain age period. Patient 1 had electroclinical features compatible with continuous spikes and waves during slow sleep (CSWSS) syndrome that changed into Lennox-Gastaut syndrome (LGS) (symptomatic, cause porencephalic cyst) at the age of 8.5 years.

Vagus nerve stimulation: effectiveness and tolerability in 64 paediatric patients with refractory epilepsies.

Aim: We discuss the effectiveness, tolerability, and safety of vagus nerve stimulation (VNS) as adjunctive therapy in 64 paediatric patients with refractory epilepsies.

Materials And Methods: Sixty-four patients (34 male and 30 female) implanted with VNS for refractory epilepsy were analysed. Electroclinical features were compatible with Lennox-Gastaut syndrome in 46 patients, focal epilepsies in 10 patients, Dravet syndrome in three patients, epilepsy with myoclonic-astatic seizures in three patients, and West syndrome in two.

Vagus nerve stimulation: effectiveness and tolerability in patients with epileptic encephalopathies.

Purpose: We discuss the effectiveness, tolerability, and safety of vagus nerve stimulation (VNS) as adjunctive therapy in 26 patients with refractory epileptic encephalopathies (EEs).

Material And Methods: Twenty-six patients (17 male and 9 female) with electroclinical features compatible with Lennox-Gastaut syndrome (LGS) in 20 patients, Dravet syndrome (DS) in 3 patients, and epilepsy with myoclonic-astatic seizures (EMAS) in 3 patients implanted with the NCP system were analyzed.

Results: In our series of patients with LGS, 17 cases showed a significant improvement in seizure control, with a reduction in seizure frequency of at least 50%.

Childhood occipital epilepsy of Gastaut: a study of 33 patients.

Purpose: To characterize the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G).

Methods: Children with electroclinical criteria of COE-G were retrospectively identified and followed-up clinically, and with sleep and awake EEGs between 1990 and 2007.

Results: We identified 33 patients with COE-G.

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.

Ketogenic diet in patients with myoclonic-astatic epilepsy.

For more than 80 years, the ketogenic diet has been used as an alternative to antiepileptic drugs for patients with refractory epilepsy. Myoclonic-astatic epilepsy in early childhood is one of the malignant epilepsy syndromes that often proves refractory to antiepileptic drugs treatment. Objective.

Late-onset, "Gastaut type", childhood occipital epilepsy: an unusual evolution.

We report on two girls and one boy with clinical and electroencephalographic features of late-onset childhood epilepsy with occipital paroxysms of the "Gastaut type", showing an unusual evolution. Neurological examination and brain imaging were normal in all three. At the age of 7.

Ketogenic diet in patients with Dravet syndrome.

Purpose: The ketogenic diet (KD) has been used as a therapeutic alternative to antiepileptic drugs (AEDs) for refractory epilepsy. Severe myoclonic epilepsy in infants or Dravet syndrome (DS) is one of the most malignant epileptic syndromes. In this retrospective study, we evaluated the efficacy and tolerability of the KD in patients with diagnostic criteria of DS.

Benign focal seizures of adolescence: a prospective study.

Purpose: To characterize the clinical and EEG findings and evolution of the syndrome of benign focal seizures of adolescence (BFSA), as described by Loiseau et al.

Methods: A prospective study was performed in adolescents with normal clinical and neurologic examinations and normal neuroradiologic studies who had focal seizures that occurred isolated or in a cluster, with or without secondary generalization in the first 24 to 48 h after onset. None of the patients was treated with antiepileptic drugs (AEDs).

Symptomatic occipital lobe epilepsy following neonatal hypoglycemia.

This study reports on the clinical, electrophysiologic, and neuroradiologic aspects of patients with epilepsy secondary to neonatal hypoglycemia. Fifteen patients with epilepsy and/or posterior cerebral lesions, and neonatal hypoglycemia were studied in the epilepsy clinic between February 1990 and March 2003. The mean age was 12 years.

Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria.

We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51 children with either unilateral PMG or closed-lip SCHZ were selected through neuroradiological analysis for investigation at our service. We evaluated the frequency of epilepsy, electroclinical features and evolution.

Idiopathic occipital and absence epilepsies appearing in the same children.

Our aim is to report the association between idiopathic occipital epilepsy and childhood absence epilepsy in the same children. Six children met the diagnostic criteria for both idiopathic occipital epilepsy and childhood absence epilepsy, five patients with idiopathic occipital epilepsy Gastaut type and another with Panayiotopoulos type. All patients were monitored for 2 to 10 years with repeated electroencephalograms when awake and during sleep.

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.

Benign familial infantile seizures: further delineation of the syndrome.

Benign familial infantile seizures are an autosomal dominant epilepsy disorder that is characterized by convulsions, with onset at age 3 to 12 months and a favorable outcome. Benign familial infantile seizures have been linked to chromosome 19q whereas infantile convulsions and choreoathetosis syndrome, in which benign familial infantile seizure is associated with paroxysmal choreoathetosis, has been linked to chromosome 16p 12-q12. Many additional families from diverse ethnic backgrounds have similar syndromes that have been linked to the chromosome 16 infantile convulsions and choreoathetosis syndrome region.