Malignant migrating partial seizures in infancy

SUMMARY The syndrome of malignant migrating partial seizures in infancy was first reported in 1995, and is now included among the childhood epilepsy syndromes in development in the proposal of the revision of the International League Against Epilepsy (ILAE) classification of the epilepsies and epilepsy syndromes. The main clinical features are seizure onset in the first 6 months of life, occurrence of almost continuous migrating polymorphous focal seizures, combined with multifocal ictal electroencephalography (EEG) discharges, and progressive deterioration of psychomotor development. Etiology is so far unknown. Seizures are markedly drug resistant and outcome is generally severe. Based on age at onset, migrating partial seizures in infancy (MMPEI) may be placed between early epileptic encephalopathies (early myoclonic encephalopathy [EME] and early infantile epileptic encephalopathy [EIEE]) and infantile spasms

Update on rufinamide in childhood epilepsy

Rufinamide is an orally active, structurally novel compound (1-[(2,6-difluorophenil1) methyl1]-1 hydro 1,2,3-triazole-4 carboxamide), which is structurally distinct from other anticonvulsant drugs. It was granted orphan drug status for the adjunctive treatment of Lennox-Gastaut syndrome (LGS) in the United States in 2004, and released for use in Europe in 2007. In January 2009, rufinamide was approved by the United States Food and Drug Administration for treatment of LGS in children 4 years of age and older. It is also approved for adjunctive treatment for partial seizures in adults and adolescents. Rufinamide’s efficacy mainly against atonic/tonic seizures in patients with LGS seems nowadays indubitable and has been confirmed both in randomized controlled trial and in open label extension studies. More recently, rufinamide was evaluated for the adjunctive treatment of childhood-onset epileptic encephalopathies and epileptic syndromes other than LGS, including epileptic spasms, multifocal epileptic encephalopathy with spasm/tonic seizures, myoclonic-astatic epilepsy, Dravet syndrome and malignant migrating partial seizures in infancy. This review updates the existing literature data on the efficacy and safety/tolerability of rufinamide in childhood-onset epilepsy syndromes

A Low Cost Amplifier And Acquisition System For Cortical-Electroncephalography In Non-Human Applications

A simple circuit is described to make an AC-amplifier and an analog-to-digital converter in a single, compact solution, for use in basic research, but not on humans. The circuit sends data to and is powered from a common USB port of modern computers; using proper firmware and driver the communication with the device is an emulated RS232 serial port

Lamotrigine versus valproic acid as first-line monotherapy in newly diagnosed typical absence seizures: an open-label,randomized, parallel-group study

Purpose: To compare the efficacy of lamotrigine (LTG) and valproic acid (VPA) in newly diagnosed children and adolescents with typical absence seizures. Methods: A randomized, open-label parallel-group design was used. After undergoing an awake video-EEG recording, which included one to two trials of 3 min of hyperventilation and intermittent photic stimulation, eligible patients were randomized to receive LTG or VPA. LTG was initiated at a daily dose of 0.5 mg/kg for 2 weeks in two divided doses, followed by 1.0 mg/kg/day for an additional 2 weeks. Thereafter, doses were increased in 1-mg/kg/day increments every 5 days until seizures were controlled, intolerable adverse effects occurred, or a maximum dose of 12 mg/kg/day had been reached. VPA was equally uptitrated according to clinical response, starting at 10 mg/kg and increasing by 5 mg/kg/24 h every 3 days, if required, to a maximum of 30 mg/kg/day in three divided doses. Patients were seen in the clinic every month for ≤12 months.The primary efficacy end point at each visit was seizure freedom, defined as lack of clinically observed seizures since the previous visit and lack of electroclinical seizures during ambulatory 24-h EEG testing and a video-EEG session with hyperventilation. Results: Thirty-eight children (17 boys, 21 girls), aged from 3 to 13 years (mean, 7.5 years), all newly diagnosed with childhood or juvenile typical absence seizures, were enrolled. After 1 month of treatment, 10 (52.6%) of 19 children taking VPA and one (5.3%) of 19 taking LTG were seizure free (p = 0.004). By the 3-month follow-up, 12 (63.1%) children taking VPA and seven (36.8%) taking LTG were controlled (p = 0.19). After 12 months, 13 children taking VPA (dose range, 20–30 mg/kg/day; mean serum level, 76.8 mg/L; range, 51.4–91 mg/L) and 10 taking LTG (dose range, 2–11 mg/kg/day; mean serum level, 8.1 mg/L; range, 1.1–18 mg/L) were seizure free (p=0.51). Side effects were mostly mild and transient and were recorded in two (10.6%) children treated with VPA and in six (31.8%) treated with LTG. Conclusions: Both VPA and LTG can be efficacious against absence seizures, although VPA shows a much faster onset of action, at least in part because of its shorter titration schedule. KeyWords: Lamotrigine—Valproic acid—Typical absences— Monotherapy. Valproic acid (VPA) and ethosuximide (ESM) have been shown to be equally effective as monotherapy for typical absence seizures (1,2), and, at present, they are generally considered first-choice drugs for this seizure type. VPA controls absences in∼75% of patients, in addition to being effective against generalized tonic–clonic seizures (70%) and myoclonic seizures (75%). However, its use may involve safety risks for postmenarchal women (3). ESM produces complete control of absences in 70% of treated patients (4,5), but it is unsuitable as monotherapy Accepted Ma

New antiepileptic drugs in the treatment of Lennox-Gastaut syndrome

Lennox–Gastaut syndrome is a childhood epileptic encephalopathy characterised by polymorphic seizures and neuropsychological decline. The most characteristic seizures are tonic fits, atypical absences and atonic seizures, in that order. Treatment options for patients with LGS are limited because of the resistance of seizures to pharmacological treatment. Owing to the many seizure types, many drugs are used in combinations that are mostly guided by anecdotal evidence or personal experience. Opinions towards treatment are further complicated because an antiepileptic drug might be of some benefit for the control of one type of seizure while aggravating another type. Concomitantly, polytherapy increases the potential for adverse events. The ultimate goal of epilepsy treatment is to achieve seizure control in a safe manner. Seizure freedom appears to be unrealistic in some refractory epilepsies, especially LGS. In this Review, we discuss newer antiepileptic drugs (Felbamate, Lamotrigine, Levetiracetam, Topiramate, Rufinamide, Vigabatrin, Zonisamide) in the treatment of Lennox-Gastaut syndrome. Investigation of the effects of newer medications might help to identify treatments that, when used in the early stages of the disorder, might have long-term beneficial effects on seizures and the associated comorbidities. Key words: antiepileptic drugs, Lennox- Gastaut syndrome, epileps

Unusual compulsive motor activity during treatment with clothiapine in a mentally retarded adolescent

Atypical antipsychotic agents, specifically those with a high hyposerotonergic activity such as clozapine and clothiapine, have been associated with de novo obsessive–compulsive symptoms. We report the case of a 16-year-old adolescent male with severe mental impairment and disruptive behaviour who developed a compulsive head and body turning disorder on clothiapine. Such a symptom had to be distinguished from epileptic partial seizures; it promptly disappeared with the drug discontinuation

Simultaneous onset of infantile spasms in monozygotic twins.

The clinical, electroencephalographic, and genetic findings are reported for three pairs of monozygotic twins who developed infantile spasms in their first year. In all three pairs, the spasms started on the same day in each member of the pair. Neither sequencing of the ARX and CDKL5 (alias STK9) genes nor array comparative genomic hybridization assessment revealed any abnormalities. The long-term outcome was poor in all twins, although with different severity in individual pairs. These findings suggest that genes other than those currently known likely play a role in predisposition to infantile spasms, and that genetic susceptibility is linked to a variable phenotypic expression, ranging from quite benign to very severe, in monozygotic twins with no other apparent risk factors

Gastaut type-idiopathic childhood occipital epilepsy and childhood absence epilepsy: a clinically significant association?

We report an unusual association between idiopathic occipital epilepsy and childhood absence epilepsy in 2 pediatric patients. At first clinical and electroencephalographic evaluation, the patients presented the peculiar signs of idiopathic occipital epilepsy Gastaut type: focal sensory visual seizures, migrainelike symptoms (only in one patient) and unilateral spike–wave discharges over occipital regions. Both children were treated with valproic acid and their seizures were rapidly controlled. After a seizure-free period, the patients presented typical absence with ictal electroencephalographies showing 3 cycles/s generalized and symmetrical spike–wave complexes. We discuss the possible association between these two epileptic syndromes and its common pathophysiological mechanisms

Cognitive and linguistic abnormalities in benign childhood epilepsy with centrotemporal spikes

Aim: To assess the cognitive function and language ability in children with benign partial epilepsy with centrotemporal spikes. Methods: Twenty-five patients with benign partial epilepsy with centrotemporal spikes were included. They were divided into two subgroups. Group I: 10 patients with rolandic focus who were not treated. Group II: 15 patients with rolandic focus receiving treatment. A third Group of 12 healthy subjects have been studied. All children underwent standardized neuropsychological testing: electroencephalogram recording, Wechsler Intelligence Scale for Children-revised, Peabody Picture Vocabulary Test-III (PPVT-III) and Boston Naming Test (BNT), both during active disease (T1) and 2 years after recovery from epilepsy (T2). Results: At T1 evaluation, no significant differences in group I and II patients about general intelligence, when compared with controls, were found. Group I and II patients were impaired with respect to controls in the receptive and expressive vocabulary evaluated with PCVT-III and BNT, respectively. At T2 evaluation, group I and II patients showed a normalization of the language abnormalities. Conclusion: Deficits of speech-related abilities can be detected in children with this type of epilepsy: these dysfunctions seem to be independent of the effects of antiepileptic treatment and are reversible after remission of epilepsy

Monitoring and Managing Depressive Symptoms in Adolescents with Epilepsy

Depressive disorders are the most frequent psychiatric disturbances associated with epilepsy in adolescents and include a broad and heterogeneous spectrum of conditions that share hallmark features and symptoms such as sadness, irritability, decreased motivation or interests, fatigue, withdrawal, hopelessness, anhedonia, changes in appetite and weight, and sleep disturbances that are persistent and pervasive most days for at least 2 weeks. Using generic self-report depression surveys and current diagnostic codes, clinical and surveillance studies have revealed prevalence rates of 20–25% for depression in youth with epilepsy, with adolescents showing particular vulnerability. Furthermore, 20% of youth with epilepsy endorse suicidal ideation, and youth endorsing suicidal ideation do not necessarily have clinical symptoms of depression. Considering that depression in youth with epilepsy is a common comorbidity, characterized by poorer psychosocial and healthy-related outcomes and increased risk of suicide, a brief, free measure of specific depressive symptoms in youth with epilepsy would be beneficial. Recently, the NDDI-E-.Y inventory has been developed from the adult NDDI-E, and validated in many countries. NDDI-E-Y showed reliable and construct validity, being a brief screening tool (12 items) that can be easily included in routine epilepsy care.For the management of depressive symptoms in adolescents, interventions can be distinguished in non-pharmacological and pharmacological. The first includes psychoeducation which should clarify to adolescents and parents the main features of epileptic disorder, side effects of antiepileptic drugs, treatment modalities, how to cope with learning and social difficulties, in order to improve quality of life. Concurrently, a cognitive-behavioral therapy (CBT) including individual therapy, supportive and family therapy and school services, should be carried on. Psychopharmacology for depressive symptoms should be deserved to moderate to severe depressive symptomatology, only after deep assessment of prior and current antiepileptic and/or psychopharmacologic treatment. SSRIs including fluoxetin, sertraline, fluvoxamine and escitalopram should be first considered. Data coming from experimental studies in animals and humans seem to confirm no decrease of seizure threshold by SSRI adjunctive therapy