244 research outputs found
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
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
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
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
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