EPILEPSIA MIOCLONICA PROGRESIVA PDF

Introduction: Lafora disease is autosomal recessive progressive myoclonus epilepsy with late childhood-to teenage-onset caused by loss-of-function mutations in either EPM2A or EPM2B genes encoding laforin or malin, respectively. Development: The main symptoms of Lafora disease, which worsen progressively, are: myoclonus, occipital seizures, generalized tonic-clonic seizures, cognitive decline, neuropsychiatric syptoms and ataxia with a fatal outcome. Pathologically, Lafora disease is characterized by the presence of polyglucosans deposits named Lafora bodies , in the brain, liver, muscle and sweat glands. Diagnosis of Lafora disease is made through clinical, electrophysiological, histological and genetic findings. Currently, there is no treatment to cure or prevent the development of the disease. Traditionally, antiepileptic drugs are used for the management of myoclonus and seizures.

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In , the European Commission approved Zebinix based on data showing that it reduces seizure frequency and improves health-related quality of life. Epilepsy is one of the most common neurological diseases, affecting up to , people in Spain[2] - and the successful treatment of partial-onset seizures the most common type of epilepsy remains a challenge.

Zebinix, developed by BIAL, is the outcome of its longstanding scientific commitment to CNS research and development, and its launch in Spain now offers a new therapeutic option which is shown to decrease seizure frequency and improve quality of life in those patients with poor seizure control," commented Mark Duffy, Business Development Director, BIAL.

By increasing our European footprint, we are able to bring valuable treatment options to more patients with epilepsy. The launch of Zebinix in Spain is a clear example of our dedication to this therapeutic area," commented Dr. In its first year Zebinix has had over 9, months of patient exposure. Epilepsy is a chronic neurological disease characterised by abnormal discharges of neuronal activity causing seizures.

Clinically, these manifest as convulsions or jerking of muscles. Depending on the seizure type, seizures may be limited to one part of the body, or may be generalised to involve the whole body.

Patients may also experience abnormal sensations, altered behaviour or altered consciousness. Epilepsy is a disorder with many possible causes. Often the cause of epilepsy is unknown. However, anything that disturbs the normal pattern of neuron activity - from illness to brain damage to abnormal brain development, can lead to seizures. Epilepsy is characterised by abnormal firing of impulses from nerve cells in the brain. In partial-onset seizures, these bursts of electrical activity are initially focused in specific areas of the brain, but may become more generalised; the symptoms vary according to the affected areas.

Nerve impulses are triggered via voltage-gated sodium channels in the nerve cell membrane. Furthermore, central nervous system related adverse events, such as lightheadedness dizziness , somnolence sleepiness , and cognitive slowing attention and memory deficits , are highly prevalent with existing anti-epileptic agents. Hence, there is a need for new anti-epileptic agents that offer effective reduction in seizure frequency combined with a favourable safety profile.

Zebinix is indicated as adjunctive therapy in adults with partial-onset seizures with or without secondary generalisation. Patients in phase III had a history of at least four partial seizures per month despite treatment with up to three concomitant anti-epileptic drugs. During the trials, patients were randomised to various dosages of Zebinix or placebo and after a 2-week titration period, were assessed over a week maintenance period, with continued follow-up over a one year open-label period.

Over the week maintenance period, Zebinix mg and mg once-daily significantly reduced seizure frequency, and was significantly more effective than placebo. In the Phase II and III clinical trials adverse events mainly occurred during the first 6 weeks of treatment and the majority of patients experienced adverse events of mild to moderate intensity.

After 6 weeks of treatment, there were no observed differences in the incidence of side effects between patients treated with Zebinix and the placebo group. Eisai is committed to developing and delivering highly beneficial new treatments to help improve the lives of people with epilepsy.

The development of anti-epileptic drugs AEDs is a major strategic area for Eisai in the European market. With operations in the U. Founded in , BIAL is an international pharmaceutical group with products available in more than 40 countries throughout four continents.

BIAL is a privately held Portuguese research based pharmaceutical company and the largest Portuguese pharmaceutical company, based in S. Mamede do Coronado, Portugal, responsible for the research and development of eslicarbazepine acetate Zebinix. It is the partner of choice for many companies, having a strong presence in the Iberian peninsula as well as in over 10 countries in Latin America and in around 20 French or Portuguese speaking African countries.

Key research areas for BIAL are the central nervous system, the cardiovascular system and allergen immunotherapy. BIAL currently has several other innovative programs under development, which the company expects to bring to the market within the next years, thereby strengthening its position throughout Europe.

Zebinix eslicarbazepine acetate : summary of product characteristics. New England Journal of Medicine ; Efficacy and safety of eslicarbazepine acetate as adjunctive treatment in adults with refractory partial-onset seizures: A randomized, double-blind, placebo-controlled, parallel-group phase III study. Epilepsia ; 50 3 Eslicarbazepine acetate BIA Eslicarbazepine acetate as adjunctive therapy in adult patients with partial epilepsy; Epilepsy Research ; Efficacy and safety of eslicarbazepine acetate as add-on treatment in adults with refractory partial-onset seizures: BIA Study.

Improvement in quality-of-life and depressive symptoms during long term treatment with eslicarbazepine acetate: BIA study Abstract No. Improvement in quality-of-life and depressive symptoms during long-term treatment with eslicarbazepine acetate: BIA study Abstract No. Quality-of-life improvement during long-term treatment with eslicarbazepine acetate Abs tract No.

Depressive symptoms improvement during long-term treatment with eslicarbazepine acetate Abstract No. Abstract No. Long-term-treatment of partial epilepsy with eslicarbazepine acetate ESL : results of a one-year open-label extension to study BIA Abstract No. Long-term treatment of partial epilepsy with eslicarbazepine acetate ESL : results of a one-year open-label extension of study BIA Abstract No. Elger et al.

Zebinix, developed by BIAL, is the outcome of its longstanding scientific commitment to CNS research and development, and its launch in Spain now offers a new therapeutic option which is shown to decrease seizure frequency and improve quality of life in those patients with poor seizure control," commented Mark Duffy, Business Development Director, BIAL "Eisai's mission to regard patients and their families as the most important participants in the healthcare process is demonstrated by our continued commitment to epilepsy in Europe.

Zebinix R is under license from Bial. About epilepsy, partial-onset seizures and their treatment Epilepsy is a chronic neurological disease characterised by abnormal discharges of neuronal activity causing seizures. About Zebinix eslicarbazepine acetate Zebinix is indicated as adjunctive therapy in adults with partial-onset seizures with or without secondary generalisation. Efficacy Over the week maintenance period, Zebinix mg and mg once-daily significantly reduced seizure frequency, and was significantly more effective than placebo.

About Eisai Europe in Epilepsy Eisai is committed to developing and delivering highly beneficial new treatments to help improve the lives of people with epilepsy. For further information please visit our web site www. Data on file.

COMPUTATIONAL GEOMECHANICS VERRUIJT PDF

Zebinix, Novel Once-Daily Anti-Epileptic Treatment, Launches in Spain

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ANDERTON CIRCLIP PDF

[Lafora Disease: A Review of the Literature]

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