Category: Disorders and Conditions

Treatment Of Partial Epilepsy

Symptomatic or Cryptogenic Partial Epilepsy Initial treatment of partial epilepsy is typically with a ‘narrow-spectrum’ anti-epileptic drug, but some broad-spectrum anti-epileptic drugs can also be used. The strongest evidence supports oxcarbazepine as initial monotherapy. Among the traditional anti-epileptic drugs, there is evidence to support the use of valproate, carbamazepine, phenobarbital and phenytoin. However, these anti-epileptic drugs may have more side-effects than some of the newer medications. Clobazam, levetiracetam, lamotrigine and topiramate can also be used in the initial treatment of childhood partial epilepsy. The patient’s seizure frequency, seizure severity, co-morbid conditions, ability to swallow the dosing formulation and insurance status/prescription plan all influence the choice …

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Treatment Of Epileptic Encephalopathies

Infantile Spasms West syndrome is the triad of infantile spasms, profoundly abnormal interictal electroencephalogram (hypsarrhythmia), and developmental arrest or regression. This is one of the classic catastrophic epilepsies of childhood. The prognosis and treatment of a child with infantile spasms often depends on the underlying aetiology of the disorder. For many infants, an extensive diagnostic work-up is required. While prompt treatment of infantile spasms is typically viewed as important, there are remarkably few data available to support the various treatment choices. A recent practice parameter by the American Academy of Pediatrics and the Child Neurology Society concluded that adrenocorticotropic hormone is ‘probably effective’ for the short-term treatment of infantile spasms …

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Treatment Of Severe Epilepsies With Onset Under 2 Years Of Age

The incidence of childhood epilepsy is highest in the first year of life. Most published series document poor long-term outcome with continuing seizures and neurodevelopmental impairment in 40-60%. Structural brain abnormalities are common, seen in 42-60%, and aetiologies encompass a wide spectrum of developmental brain malformations, acquired brain injuries, and genetic and metabolic conditions. Despite the previously reported poor clinical outcome, there are infants whose epilepsy takes a more benign course. Several idiopathic epilepsy syndromes have been delineated and are now included in the recently proposed international classifications. These …

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Vitamin Supplementation

A number of treatable metabolic disorders, including vitamin-responsive conditions, present with medically resistant seizures in the first year of life. Response to empirical treatment may have to guide further investigative work-up. Disorders of vitamin B6 metabolism Pyridoxal 5′-phosphate (PLP) is the B6 vitamer, which has co-factor activity and acts with several enzymes that are involved in the metabolism of neurotransmitters (e.g. dopamine, serotonin, glutamate and y-aminobutyric acid [GABA]). Progress in the understanding of disorders caused by deficiency of cerebral PLP has been made recently. Increased consumption of PLP through inactivation or defects of its biosynthesis in liver and brain involving pyridox(am)ine …

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Infantile Spasms And West Syndrome

Natural or synthetic adrenocorticotropic hormone, glucocorticosteroids and vigabatrin are the main pharmacological agents currently suggested for the treatment of infantile spasms. In addition, conventional and newer anti-epileptic drugs, as well as vitamin B6, are used. There is no agreement which agent should be commenced first line. Protocols differ in dosing regimes as well as duration of treatment courses, depending on geographical area and availability of these agents. vigabatrin is not licensed in the US, natural adrenocorticotropic hormone has been replaced by synthetic adrenocorticotropic hormone in some European countries and Japan, and different types of oral steroids are in use. Despite a …

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Severe Myoclonic Epilepsy In Infancy (Dravet Syndrome)

Severe myoclonic epilepsy in infancy, first described by Dravet, is an early-onset epilepsy syndrome with catastrophic course and poor seizure as well as cognitive outcome. Onset is in the first year of life. Developmentally normal infants present with atypical febrile convulsions (focal features, prolonged) and episodes of febrile as well as afebrile status epilepticus. The occurrence of afebrile multiple type seizures including myoclonus from the second year of life onwards is accompanied by developmental plateauing or regression. Whilst the interictal electroencephalogram is initially normal, later in the course with expression of afebrile seizures generalized, multi-focal and focal epileptiform abnormalities are seen. Photosensitivity has been observed in up to 42% of patients …

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The Ketogenic Diet

The ketogenic diet has been used in the treatment of epilepsy for almost 100 years. This is a high-fat diet, designed to mimic the effects on starvation in the production of ketones. Several studies have shown benefit in older children with drug-resistant epilepsy, in the use of both the classical diet (where the diet is based on the ratio of long-chain fat to carbohydrate including protein) and the medium-chain triglyceride diet. Criticism has been directed at the high dietetic resource required, and the unpalatability of the diet, but neither should be consideration to non-use. The availability of liquid preparation in the use of either diet (medium-chain triglyceride emulsion or liquigen with …

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Rheumatoid Arthritis: Emerging Therapies

The launch of highly effective biological therapies in the late 1990s — in particular, the tumor necrosis factor-alpha (tumor necrosis factor-alpha) inhibitors — has dramatically improved the therapeutic options available for the treatment of rheumatoid arthritis (rheumatoid arthritis). The availability of these effective, albeit expensive, agents has significantly raised the bar for emerging therapies in the rheumatoid arthritis market. Despite the steep competition in developing therapies for this market, a vast number of agents, many of them biologies, are under development for the treatment of rheumatoid arthritis. This discussion focuses on emerging therapies that …

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Interleukin-Based Therapies

Overview Despite the relatively low efficacy of the interleukin (IL)-lβ antagonist anakinra and the relative lack of success of this agent in the rheumatoid arthritis market, companies continue to study IL-1 and other interleukins in the hope of finding a novel drug for rheumatoid arthritis. Three interleukin-based therapies that have advanced to comparatively late-stage development are discussed here, including Chugai/Roche’s atlizumab (MRA) and Amgen’s AMG-714 (HuMax IL-15), which target IL-6 and IL-15 respectively. Abbott’s ABT-874, an anti-IL-12 mAb currently in Phase II studies, is not discussed owing to a lack of clinical data in rheumatoid arthritis. Regen-eron’s IL-1 inhibitor RGN 303 was also in clinical …

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Lymphocyte Modulators

Overview A new class of biological agents has emerged that specifically target T and B lymphocytes, the main cells involved in an adaptive immune response. Most currently available biological agents target the products of activated macrophages, cytokines. However, T cells help activate macrophages, and both types of lymphocytes have been implicated in the inflammation and joint destruction found in rheumatoid arthritis. The rheumatoid synovium contains activated T and B cells, and immunoglobulin produced by B cells, such as rheumatoid factor and antinuclear or anticytoplasmic autoantibodies, serve as diagnostic markers for rheumatoid arthritis that may also be involved in the disease process. This section discusses one emerging therapy that …

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