Abstract:
:Dravet syndrome (DS) is a medically refractory epilepsy that onsets in the first year of life with prolonged seizures, often triggered by fever. Over time, patients develop other seizure types (myoclonic, atypical absences, drops), intellectual disability, crouch gait and other co-morbidities (sleep problems, autonomic dysfunction). Complete seizure control is generally not achievable with current therapies, and the goals of treatment are to balance reduction of seizure burden with adverse effects of therapies. Treatment of co-morbidities must also be addressed, as they have a significant impact on the quality of life of patients with DS. Seizures are typically worsened with sodium-channel agents. Accepted first-line agents include clobazam and valproic acid, although these rarely provide adequate seizure control. Benefit has also been noted with stiripentol, topiramate, levetiracetam, the ketogenic diet and vagal nerve stimulation. Several agents presently in development, specifically fenfluramine and cannabidiol, have shown efficacy in clinical trials. Status epilepticus is a recurring problem for patients with DS, particularly in their early childhood years. All patients should be prescribed a home rescue therapy (usually a benzodiazepine) but should also have a written seizure action plan that outlines when rescue should be given and further steps to take in the local hospital if the seizure persists despite home rescue therapy.
journal_name
CNS Drugsjournal_title
CNS drugsauthors
Knupp KG,Wirrell ECdoi
10.1007/s40263-018-0511-ysubject
Has Abstractpub_date
2018-04-01 00:00:00pages
335-350issue
4eissn
1172-7047issn
1179-1934pii
10.1007/s40263-018-0511-yjournal_volume
32pub_type
杂志文章,评审相关文献
CNS DRUGS文献大全abstract::Since 1989, several novel antipsychotic drugs have become available for use including clozapine, risperidone, olanzapine, quetiapine and ziprasidone. These agents represent a substantial improvement in the treatment of schizophrenia and related disorders and are considered to have a favourable adverse effect profile r...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200317050-00002
更新日期:2003-01-01 00:00:00
abstract::Biopharmaceuticals can induce antibodies, which interact with and neutralize the therapeutic effect of such drugs and are therefore termed neutralizing antibodies (NAbs). In the treatment of multiple sclerosis, NAbs against interferon (IFN)-beta and natalizumab have been recognized. The prevalence of NAbs against diff...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200923050-00003
更新日期:2009-01-01 00:00:00
abstract::A number of news items and case reports describing complex behaviours (e.g. sleep driving, sleep cooking, sleep eating, sleep conversations, sleep sex) associated with the use of hypnosedative medications have recently received considerable attention. Regulatory agencies examining these reports have subsequently issue...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/0023210-200822120-00005
更新日期:2008-01-01 00:00:00
abstract::Female hypoactive sexual desire disorder (HSDD) may occur in up to one-third of adult women in the US. The essential feature of female HSDD is a deficiency or absence of sexual fantasies and desire for sexual activity that causes marked distress or interpersonal difficulty. The evaluation of female HSDD generally requ...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200216110-00003
更新日期:2002-01-01 00:00:00
abstract::Disability, characterised by the loss of ability to perform activities of daily living (ADL), is a defining feature of dementia that results in growing caregiver burden and the eventual need for alternative care or nursing home placement. Functional decline in patients with dementia can also result from causes other t...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200418130-00003
更新日期:2004-01-01 00:00:00
abstract::Numerous in vitro and in vivo experimental animal studies have demonstrated that gangliosides, particularly GMI ganglioside (siagoside), may stimulate or accelerate the repair of peripheral and central nervous system neurons after various types of damage. Clinical studies of GMl in peripheral neuropathies and stroke, ...
journal_title:CNS drugs
pub_type: 杂志文章
doi:10.2165/00023210-199401030-00006
更新日期:1994-03-01 00:00:00
abstract::In humans, age-related memory impairments begin in mid-life and cognitive weakening continues with advancing age. An important aspect of defining memory decline is the distinction between dementia as a result of neurological diseases, such as Alzheimer's disease, and memory loss not specifically associated with diseas...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200620020-00006
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND:The molecules that provide access to activated T cells in the CNS, including chemokines, have been considered to be a crucial step in the pathogenesis of multiple sclerosis (MS). AIMS:In this study, we investigated serial serum chemokine levels in patients with relapsing-remitting MS over 1 year and the ass...
journal_title:CNS drugs
pub_type: 杂志文章
doi:10.2165/11595060-000000000-00000
更新日期:2011-11-01 00:00:00
abstract::The development of an intervention to slow or halt disease progression remains the greatest unmet therapeutic need in Parkinson's disease. Given the number of failures of various novel interventions in disease-modifying clinical trials in combination with the ever-increasing costs and lengthy processes for drug develo...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-018-0548-y
更新日期:2018-08-01 00:00:00
abstract::Bulimia nervosa is an eating disorder characterised by recurrent episodes of binge eating and associated efforts to purge the ingested calories through self-induced vomiting, laxative or diuretic abuse, fasting or intensive exercise. The aetiopathogenesis and pathophysiology of the disorder are currently unclear. Biol...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200115020-00004
更新日期:2001-01-01 00:00:00
abstract::The coronavirus 2019 (COVID-19) pandemic is expected to linger. Decisions regarding initiation or continuation of disease-modifying therapy for multiple sclerosis have to consider the potential relevance to the pandemic. Understanding the mechanism of action and the possible idiosyncratic effects of each therapeutic a...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-020-00756-y
更新日期:2020-09-01 00:00:00
abstract::Frontotemporal lobar degeneration (FTLD) describes a spectrum of clinically, pathologically and genetically heterogeneous neurodegenerative disorders of unknown aetiology. FTLD spectrum disorders collectively represent a leading cause of early-onset dementia, with most cases presenting between 45 and 64 years of age. ...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/11640070-000000000-00000
更新日期:2012-10-01 00:00:00
abstract::There is escalating interest in cell-based therapies to restore lost dopamine inputs in Parkinson's disease. This is based upon the rationale that implanting dopamine progenitors into the striatum can potentially improve dopamine-responsive motor symptoms. A rich body of data describing clinical trials of previous cel...
journal_title:CNS drugs
pub_type: 杂志文章
doi:10.1007/s40263-020-00727-3
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:Earlier commencement of clozapine has been related to a better response in treatment-resistant schizophrenia. OBJECTIVES:To identify variables that predict clozapine use after a first episode of schizophrenia (FES). METHODS:Patients with FES and ≤15 days of lifetime antipsychotic treatment were followed up...
journal_title:CNS drugs
pub_type: 杂志文章
doi:10.1007/s40263-016-0358-z
更新日期:2016-10-01 00:00:00
abstract::As in all patient populations, epilepsy is common in pregnant women. Consequently, approximately 1 in 200 pregnancies is exposed to antiepileptic drugs (AEDs). Although exposure to AEDs in utero has been associated with an increased risk of major fetal malformations, most women with epilepsy require medication through...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200620100-00001
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Patients with focal seizures recruited into adjunctive antiepileptic drug (AED) trials have become more refractory and severe over time; concurrently, placebo responses have increased. To attempt to account for heterogeneity among trials, propensity-score weighted patient-level data were used t...
journal_title:CNS drugs
pub_type: 杂志文章,随机对照试验
doi:10.1007/s40263-017-0462-8
更新日期:2017-10-01 00:00:00
abstract::Much work has been carried out in recent years showing that elevated glutamate levels in the extracellular environment of the central nervous system play a pivotal role in neurodegeneration in acute CNS injuries. With the elucidation of the mechanism governing glutamate excitotoxicity, researchers are devising therape...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-015-0225-3
更新日期:2015-02-01 00:00:00
abstract::Attention-deficit/hyperactivity disorder, or ADHD, is the most frequently occurring neurobiological disorder in childhood and is defined by symptoms of inattention and/or hyperactivity and impulsivity that are excessive when compared with other individuals at the same developmental level. ADHD can be successfully trea...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-013-0130-6
更新日期:2014-02-01 00:00:00
abstract::Mitoxantrone (Novantrone), a synthetic anthracenedione derivative, is an antineoplastic, immunomodulatory agent. Its presumed mechanism of action in patients with multiple sclerosis (MS) is via immunomodulatory mechanisms, although these remain to be fully elucidated. Intravenous mitoxantrone treatment improved neurol...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200418060-00010
更新日期:2004-01-01 00:00:00
abstract::It is difficult to determine the relative efficacy of atypical antipsychotics for the treatment of schizophrenia, based on the available literature. The purpose of this article is to review and compare the efficacy of two atypical antipsychotics: olanzapine and ziprasidone.This review focused on randomised trials in w...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200519060-00003
更新日期:2005-01-01 00:00:00
abstract::Multiple stimulant and non-stimulant medications are approved for the treatment of attention-deficit/hyperactivity disorder (ADHD), one of the most prevalent childhood neurodevelopmental disorders. Choosing among the available agents and determining the most effective ADHD medication for a given child can be a time-co...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-020-00702-y
更新日期:2020-04-01 00:00:00
abstract::Glutamatergic, noradrenergic, serotonergic, and cholinergic systems play a critical role in the basal ganglia circuitry. Targeting these non-dopaminergic receptors remains a focus of ongoing research to improve Parkinson's disease (PD) motor symptoms, without the potential side effects of dopamine replacement therapy....
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-020-00754-0
更新日期:2020-10-01 00:00:00
abstract::The aim of this review is to provide clinicians with a picture of the mechanisms by which: histamine and histaminergic agonists act on the vestibular system both peripherally and centrally; and histaminergic agonists and antagonists interfere with the recovery process after peripheral vestibular lesion. We have focuse...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200115110-00004
更新日期:2001-01-01 00:00:00
abstract::New formulations of stimulant medications for the treatment of attention-deficit hyperactivity disorder (ADHD) have been an important focus for pharmaceutical industry research and development over the past decade. In this article, we review and assess the therapeutic potential of five new stimulant formulations (one ...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200418140-00005
更新日期:2004-01-01 00:00:00
abstract::Duloxetine (Cymbalta(R)) is a potent serotonin and noradrenaline (norepinephrine) reuptake inhibitor (SNRI) in the CNS. It is indicated for the treatment of generalized anxiety disorder (GAD) as well as other indications. In patients with GAD of at least moderate severity, oral duloxetine 60-120 mg once daily was effe...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200923060-00006
更新日期:2009-01-01 00:00:00
abstract::Alcoholic patients suffer from harmful allostatic neuroplastic changes in the brain causing an acute withdrawal syndrome upon cessation of drinking followed by a protracted abstinence syndrome and an increased risk of relapse to heavy drinking. Benzodiazepines have long been the treatment of choice for detoxifying pat...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-015-0240-4
更新日期:2015-04-01 00:00:00
abstract::gamma-Aminobutyric acid (GABA) is considered to be the major inhibitory neurotransmitter in the brain and loss of GABA inhibition has been clearly implicated in epileptogenesis. GABA interacts with 3 types of receptor: GABAA, GABAB and GABAC. The GABAA receptor has provided an excellent target for the development of d...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200115050-00001
更新日期:2001-01-01 00:00:00
abstract::In recent years, several new antiepileptic drugs (AEDs) have been licensed: felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, topiramate, vigabatrin and zonisamide. These drugs have proven efficacy as add-on therapy in patients with difficult-to-treat partial epilepsy, as 20-50% of patients ...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.2165/00023210-200317060-00003
更新日期:2003-01-01 00:00:00
abstract:INTRODUCTION:The gabapentinoids pregabalin and gabapentin are being increasingly prescribed for a range of clinical conditions. Recently, although gabapentinoids at therapeutic dosages may present with low addictive liability levels, cases of misuse and rising numbers of related fatalities have been reported. OBJECTIV...
journal_title:CNS drugs
pub_type: 杂志文章
doi:10.1007/s40263-016-0359-y
更新日期:2016-07-01 00:00:00
abstract::Statins, or 3-hydroxy-3-methyl-glutaryl coenzyme A reductase inhibitors, such as lovastatin, atorvastatin, simvastatin, pravastatin, fluvastatin, rosuvastatin and pitavastatin, are cholesterol-lowering drugs used in clinical practice to prevent coronary heart disease. These drugs are generally well tolerated and have ...
journal_title:CNS drugs
pub_type: 杂志文章,评审
doi:10.1007/s40263-013-0135-1
更新日期:2014-03-01 00:00:00