Abstract:
Background:Lennox-Gastaut syndrome (LGS) is a typical intractable form of epilepsy that most often occurs between the second and sixth year of life. This study aimed to evaluate the clinical efficacy and safety of ketogenic diet therapies (DTs) for LGS with mitochondrial dysfunction. Methods:This was a retrospective study involving 20 LGS patients with mitochondrial dysfunction who received several DTs from 2004 to 2014 at a single tertiary care center. Seizure reduction rate, cognitive function, retention rate, electroencephalography (EEG) changes, and adverse effects were examined before and after DTs. Results:The retention rates at 1 and 2 years after initiation of DTs were 45% and 40%, respectively. After 1-year follow up, we observed seizure freedom in two patients, 75% seizure reduction in two patients, 50% reduction in three patients, and 25% reduction in one patient. After 2-year follow up, the outcomes were seizure freedom in two patients, 90% seizure reduction in one patient, 75% reduction in two patients, and 50% reduction in two patients. EEG findings improved in nine patients. Nine patients were treated with DTs for 1 year; all patients demonstrated improved cognitive status. Eight patients were treated with DTs for 2 years, of whom seven had improved cognitive status. Poor tolerability of DTs was due to poor oral intake and gastrointestinal problems. Conclusions:We demonstrate that, in LGS with mitochondrial dysfunction, improvement of seizures and cognitive function are not inferior to those in other patients treated with DTs. This study showed that DTs are efficacious and feasible for LGS patients with mitochondrial dysfunction and can significantly improve their prognosis.
journal_name
Ther Adv Neurol Disordjournal_title
Therapeutic advances in neurological disordersauthors
Na JH,Kim HD,Lee YMdoi
10.1177/1756286419897813subject
Has Abstractpub_date
2020-02-06 00:00:00pages
1756286419897813eissn
1756-2856issn
1756-2864pii
10.1177_1756286419897813journal_volume
13pub_type
杂志文章abstract::Spinal muscular atrophy (SMA) is a progressive, recessively inherited neuromuscular disease, characterized by the degeneration of lower motor neurons in the spinal cord and brainstem, which leads to weakness and muscle atrophy. SMA currently represents the most common genetic cause of infant death. SMA is caused by th...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285618754501
更新日期:2018-02-05 00:00:00
abstract:OBJECTIVES:The cumulative safety and efficacy measures of percutaneous transluminal angioplasty and stenting (PTAS) for secondary stroke prevention in patients with symptomatic intracranial arterial stenosis (sICAS) have not previously been evaluated using a meta-analytical approach. METHODS:We conducted a systematic ...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285616650357
更新日期:2016-09-01 00:00:00
abstract:Introduction:Wernicke encephalopathy (WE) and Wernicke-Korsakoff syndrome (WKS) are well-known disorders caused by thiamine deficiency. In addition to the classical concept of these diseases, some literature data suggest a connection between mitochondrial dysfunction and WE/WKS. Psychotic disorders and WKS seem to run ...
journal_title:Therapeutic advances in neurological disorders
pub_type:
doi:10.1177/1756286420938972
更新日期:2020-07-30 00:00:00
abstract::Alzheimer's dementia (AD) is increasingly being recognized as one of the most important medical and social problems in older people in industrialized and non-industrialized nations. To date, only symptomatic treatments exist for this disease, all trying to counterbalance the neurotransmitter disturbance. Three choline...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285612461679
更新日期:2013-01-01 00:00:00
abstract:Background:To investigate outcome and toxicity of high-dose systemic methotrexate (HDMTX)-based polychemotherapy and intracerebroventricular (ICV) chemotherapy via an Ommaya reservoir in elderly patients with primary central nervous system lymphoma (PCNSL). Methods:We performed a retrospective analysis on patients ⩾65...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286420951087
更新日期:2020-10-07 00:00:00
abstract::Key objectives in the treatment of multiple sclerosis (MS) include prevention of relapses, a reduction in the accumulation of disability and slowing of the brain volume loss that occurs from the earliest stages of the disease. Teriflunomide, a once-daily, oral immunomodulatory therapy, has demonstrated efficacy across...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285617722500
更新日期:2017-12-01 00:00:00
abstract::For patients with relapsing-remitting multiple sclerosis (RRMS), there are currently six approved medications that have been shown to alter the natural course of the disease. The approved medications include three beta interferon formulations, glatiramer acetate, natalizumab and mitoxantrone. Treating aggressive forms...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285609344375
更新日期:2009-11-01 00:00:00
abstract::The World Wide Web is increasingly used in therapeutic settings. In this regard, internet-based interventions have proven effective in ameliorating several health behaviors, amongst them physical activity behavior. Internet-delivered interventions have shown positive effects on physical activity and physical function ...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285616640684
更新日期:2016-07-01 00:00:00
abstract::Calcitonin gene related peptide (CGRP) monoclonal antibodies (mAbs) have been the first class of specifically developed preventive treatments for migraine. Clinical trials data suggest superiority of the CGRP mAbs to placebo in terms of prevention of migraine symptoms, migraine-specific quality of life and headache re...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286420927119
更新日期:2020-05-27 00:00:00
abstract:OBJECTIVES:To assess clinical outcomes and patient satisfaction in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) or multifocal motor neuropathy (MMN) who were switched from intravenous immunoglobulin (IVIG) to subcutaneous immunoglobulin (SCIG). METHODS:Eight consecutive patients, four...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285614563056
更新日期:2015-01-01 00:00:00
abstract::Impulse control disorders are a psychiatric condition characterized by the failure to resist an impulsive act or behavior that may be harmful to self or others. In movement disorders, impulse control disorders are associated with dopaminergic treatment, notably dopamine agonists (DAs). Impulse control disorders have b...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285613476127
更新日期:2013-05-01 00:00:00
abstract::Stroke is the fifth leading cause of death and the most frequent cause of disability worldwide. Currently, stroke diagnosis is based on neuroimaging; therefore, the lack of a rapid tool to diagnose stroke is still a major concern. In addition, therapeutic approaches to combat ischemic stroke are still scarce, since th...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286418789340
更新日期:2018-08-06 00:00:00
abstract::Multiple sclerosis (MS) is a chronic inflammatory disorder of the central nervous system. Demyelinization of nerve fibres not only affects the motor and sensory systems functionally, but may also cause psychopathological signs and symptoms. In addition to the psychiatric manifestations of MS, many patients have reacti...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285608100325
更新日期:2009-01-01 00:00:00
abstract:Background:Gastrointestinal (GI) events are common adverse events (AEs) associated with delayed-release dimethyl fumarate (DMF), an approved treatment for relapsing-remitting multiple sclerosis (RRMS). The objective of the TOLERATE study was to evaluate GI tolerability and GI mitigation via symptomatic therapies in pat...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286418768775
更新日期:2018-04-18 00:00:00
abstract::Alzheimer's disease (AD) is a neurodegenerative disease characterized by progressive memory loss and dementia. Accumulating evidence suggests that inflammation is involved in the pathogenesis of AD. Epidemiological studies suggest that use of anti-inflammatory drugs is associated with a lower incidence of AD. However,...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286418791107
更新日期:2018-08-09 00:00:00
abstract::The treatment of stroke caused by intracranial vessel occlusion with intravenous recombinant tissue plasminogen activator (rt-PA) was the only evidence-based treatment option for a long time. Nevertheless the response rate was disappointing in large vessel occlusions. Five studies that evaluated the efficacy of mechan...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285617742082
更新日期:2017-11-22 00:00:00
abstract::Blepharospasm is a focal (although usually bilateral) dystonia of the orbicularis oculi muscles, producing excessive eye closure. This produces significant disability through functional blindness. Botulinum neurotoxins (BoNT) have become the treatment of choice for blepharospasm; the impressive response rate and the t...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285614557475
更新日期:2015-03-01 00:00:00
abstract::Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that largely affects optic nerves and spinal cord. Recent studies have identified an elevation of serum anti-aquaporin 4 antibody as a hallmark of NMO. Typical cases of NMO significantly differ from multiple sclerosis (MS) in immunological markers, hi...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285608093978
更新日期:2008-07-01 00:00:00
abstract::B cells play a central role in the pathogenesis in multiple sclerosis (MS), being involved in the activation of proinflammatory T cells, secretion of proinflammatory cytokines, and production of autoantibodies directed against myelin. Hence, the usage of B-cell-depleting monoclonal antibodies as therapy for autoimmune...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285615601933
更新日期:2016-01-01 00:00:00
abstract::Chronic migraine (CM) is a severely disabling neurological condition characterized by episodes of pulsating unilateral or bilateral headache. The United States Food and Drug Administration (FDA) approved onabotulinumtoxinA (Botox®) for the prophylactic treatment of CM in 2010. It has been shown that onabotulinumtoxinA...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285616677005
更新日期:2017-02-01 00:00:00
abstract::In the past few years, acquired demyelinating syndromes of the central nervous system associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) have evolved into a new inflammatory disease entity distinct from neuromyelitis optica spectrum disorders or multiple sclerosis. The meticulous clinical des...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286420945135
更新日期:2020-07-31 00:00:00
abstract::Until the early 1990s, a limited number of antiepileptic drugs (AEDs) were available. Since then, a large variety of new AEDs have been developed and introduced, several of them offering new modes of action. One of these new AED families is described and reviewed in this article. Levetiracetam (LEV) and brivaracetam (...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286419873518
更新日期:2019-09-09 00:00:00
abstract::Experimental models of Parkinson's disease (PD) are of great importance for improving the design of future clinical trials. Various neurotoxic models are available, including 6-hydroxydopamine (6-OHDA), 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), paraquat and rotenone. However, no model is considered perfect;...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285611413004
更新日期:2011-11-01 00:00:00
abstract::Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl p...
journal_title:Therapeutic advances in neurological disorders
pub_type:
doi:10.1177/1756286420909973
更新日期:2020-06-03 00:00:00
abstract:Background:Network science provides powerful access to essential organizational principles of the brain. The aim of this study was to investigate longitudinal evolution of gray matter networks in early relapsing-remitting MS (RRMS) compared with healthy controls (HCs) and contrast network dynamics with conventional atr...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286419838673
更新日期:2019-04-24 00:00:00
abstract::Brain imaging is increasingly used to support clinicians in diagnosing multiple sclerosis (MS) and monitoring its progression. However, the role of magnetic resonance imaging (MRI) in MS goes far beyond its clinical application. Indeed, advanced imaging techniques have helped to detect different components of MS patho...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286419859722
更新日期:2019-06-27 00:00:00
abstract:Background:To compare the long-term clinical outcomes of different antihypertensive drugs in stable patients after acute hemorrhagic stroke (HS). Methods:From January 2001 to December 2013, patients with first-ever primary HS were identified in the National Health Insurance Research Database, Taiwan. Patients with tra...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286418802688
更新日期:2018-09-28 00:00:00
abstract::Since the approval in 2010 of fingolimod 0.5 mg (Gilenya; Novartis Pharma AG, Basel, Switzerland) in the USA as an oral therapy for relapsing forms of multiple sclerosis, long-term clinical experience with this therapy has been increasing. This review provides a summary of the cumulative dataset from clinical trials a...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285616628766
更新日期:2016-03-01 00:00:00
abstract:BACKGROUND:Efficacy of interferon beta in multiple sclerosis (MS) can be dampened in patients who develop neutralizing antidrug antibodies (NAbs). Peginterferon beta1a is an interferon conjugated with a polyethylene glycol (PEG) moiety. Pegylation increases a drug's half life and exposure, and may also reduce immunogen...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285616633967
更新日期:2016-07-01 00:00:00
abstract::More than 30% of epilepsy patients remain refractory despite the advent of new antiepileptic drugs (AEDs) over two decades. Although a small percentage of these refractory patients may become seizure free when a new AED is added, combined administration of AEDs or the application of novel AEDs is the most common thera...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285609355850
更新日期:2010-03-01 00:00:00