Abstract:
OBJECTIVES:There are very few data available in the literature on the use of stiripentol in adults with Dravet syndrome (DS). DS cases are increasingly recognized in adulthood, and more children with DS now survive to adulthood. The aim of the study was to document the effectiveness and tolerability of stiripentol in adults with DS. MATERIAL AND METHODS:We conducted an observational clinical audit in the epilepsy service of the National Hospital for Neurology and Neurosurgery, London (UK). RESULTS:We included 13 adult subjects with DS (eight females, five males). The responder (defined as more than 50% reduction in all seizure types) rate was 3/13 (23%) at 36 months. The following other outcomes were reported: seizure exacerbation (3/13, 23%), no change (3/13, 23%), less than 50% reduction in seizures (2/13, 15%), more than 50% reduction in generalized tonic-clonic seizures but no other seizure types (1/13, 8%), undefined response (1/13, 8%). The retention rate was 62% after 1 year and 31% after 5 years. Adverse effects were reported in 7/13 (54%): the most frequent were anorexia, weight loss, unsteadiness and tiredness. Withdrawal due to adverse effects occurred in 3/13 (23%). CONCLUSIONS:Compared with previous studies on children with DS, our results show a lower responder rate and a similar tolerability profile. Stiripentol can be effective with a good tolerability profile. Our audit is small, but supports the use of stiripentol in adults with DS when first-line treatments are ineffective or not tolerated, in keeping with published guidelines.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Balestrini S,Sisodiya SMdoi
10.1111/ane.12611subject
Has Abstractpub_date
2017-01-01 00:00:00pages
73-79issue
1eissn
0001-6314issn
1600-0404journal_volume
135pub_type
杂志文章abstract::The clinical features of multiple sclerosis (MS) in north-east Scotland have been examined along with an epidemiological study to determine the prevalence of the disease on 1st December, 1970. Patient information was collected from all available diagnostic indices, both retrospectively and prospectively from 1965. Eve...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1979.tb02973.x
更新日期:1979-10-01 00:00:00
abstract::Clinical measurement of intracranial pressure (ICP) is often performed to aid diagnosis of hydrocephalus. This review discusses analysis of ICP and its components' for the investigation of cerebrospinal fluid (CSF) dynamics. The role of pulse, slow and respiratory waveforms of ICP in diagnosis, prognostication and man...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/ane.12541
更新日期:2016-09-01 00:00:00
abstract::In 30 ischemic stroke patients, divided into 2 groups depending on the side of their hemispheric cerebral lesion, the authors evaluated the levels of CSF homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA). The changes of these metabolites in CSF samples collected 3, 14 and 25 days after stroke have been c...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1986.tb04603.x
更新日期:1986-06-01 00:00:00
abstract:OBJECTIVES:When the vascular disorder of the cochleovestibular system is mentioned, the diagnosis is based on exclusion of other diseases. Since arteries of the cochleovestibular system cannot be directly visualized, physicians must deduce from the vascular risk factors and the vascular lesion of other territories to t...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.104002068.x
更新日期:2001-08-01 00:00:00
abstract:BACKGROUND:One of recent interesting hypotheses of transient global amnesia (TGA) pathophysiology is the preexisting vulnerability of the memory network in patients with TGA. AIM OF THE STUDY:To verify the hypothesis that patients with recurrent amnestic attacks may have more disrupted structural connectivity than pat...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12518
更新日期:2016-08-01 00:00:00
abstract:BACKGROUND:Mild malformation of cortical dysplasia (mMCD) with oligodendroglial hyperplasia (MOGHE) is an epilepsy-related pathologic entity highlighted in post-surgical specimens of frontal lobe epilepsy (FLE) patients. AIMS OF THE STUDY:We present two temporal lobe epilepsy (TLE) cases with MOGHE and discuss clinica...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13142
更新日期:2019-10-01 00:00:00
abstract::Three patients are presented who developed conduction aphasia after a small infarction almost exclusively confined to the arcuate fasciculus. All of them were diagnosed as conduction aphasia within a week after the stroke and showed a rapid amelioration. On the basis of the 3 patients and conduction aphasics in the li...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1987.tb03597.x
更新日期:1987-12-01 00:00:00
abstract:OBJECTIVES:Post-stroke cognitive impairment (PSCI) has considerable impact on patients and society. However, long-term studies on PSCI are scarce and may be influenced by assessment methods and selection bias. We aimed to (i) assess the prevalence of long-term PSCI; (ii) compare two common cognitive assessment instrume...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12709
更新日期:2017-09-01 00:00:00
abstract::In a previous study we found that nine of 47 siblings to multiple sclerosis (MS) patients with a normal neurological examination carry an intrathecal oligoclonal immunopathy with limited specificity, a condition we termed MS immunopathic trait. The purpose of this study is to further characterize the MS immunopathic t...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00197.x
更新日期:2004-02-01 00:00:00
abstract::3-methoxy-4-hydroxyphenylglycol (MHPG) and 5-hydroxyindoleacetic acid (5-HIAA) as well as homovanillic acid (HVA) and cyclic AMP were estimated in the morning urine of 41 drug-free parkinsonian patients and 25 hospitalized controls. In 29 patients, the estimations were repeated after 2 weeks treatment with L-dopa plus...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1982.tb04524.x
更新日期:1982-08-01 00:00:00
abstract:BACKGROUND:Thirty patients with glioblastoma multiforme (GBM) were treated by boron neutron capture therapy (BNCT) at the Studsvik facility in Sweden, in a clinical trial exploring a procedure in which 900 mg p-boronophenylalanine (BPA) per kilo body weight was infused in 6 h. OBJECTIVE:The present study was designed ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2007.00837.x
更新日期:2007-09-01 00:00:00
abstract:OBJECTIVES:Comorbidities of idiopathic normal pressure hydrocephalus (iNPH), such as Alzheimer's disease (AD) and Parkinson's spectrum (PS) disorder, can affect the long-term prognosis of cerebrospinal fluid (CSF) shunting. Therefore, it is important to be able to predict comorbidities in the early stage of the disease...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13306
更新日期:2020-12-01 00:00:00
abstract::Brain perfusion was studied interictally with 99mTc-HM-PAO SPECT in 47 adult patients with partial epilepsy and normal brain CT. Epilepsy was classified as secondarily generalized in 24 patients, as complex partial in 17 patients and as simple partial in 6 patients. In 24 patients good seizure control was not achieved...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb05486.x
更新日期:1992-12-01 00:00:00
abstract:OBJECTIVES:To evaluate the activity of cortico-cortical (intracortical) inhibitory circuits within the motor cortex in patients with progressive asymmetric limb-kinetic apraxia. MATERIALS AND METHODS:We studied 4 patients with progressive limb-kinetic apraxia whose clinical diagnosis was corticobasal degeneration (CBD...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2000.102004244.x
更新日期:2000-10-01 00:00:00
abstract:OBJECTIVE:To evaluate the epidemiological and clinical features of motor neuron disease (MND) in a region (835,000 inhabitants) of south-western Greece. PATIENTS AND METHODS:The medical records of all patients diagnosed with adult-onset MND at the Department of Neurology of the University Hospital of Patras from 1990 ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2004.00362.x
更新日期:2005-02-01 00:00:00
abstract:OBJECTIVES:We sought to assess the efficacy of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD)-associated trunk posture abnormalities retrospectively analyzing data from 101 patients reporting mild-to-severe trunk posture abnormalities of a cohort of 216 PD patients treated with STN-DBS...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12889
更新日期:2018-05-01 00:00:00
abstract:OBJECTIVES:Focal brain ischemia induced in rodents by occlusion of the middle cerebral artery (MCA) is a widely used paradigm of human brain infarct. The objective of this study is to compare the effectiveness and reproducibility of MCA filament occlusion model in rats and mice. MATERIALS AND METHODS:A total of 140 ro...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00010.x
更新日期:2003-04-01 00:00:00
abstract::Decompressive craniectomy is a neurosurgical technique in which a portion of the skull is removed to reduce intracranial pressure. The rationale for this procedure is based on the Monro-Kellie Doctrine; expanding the physical space confining edematous brain tissue after traumatic brain injury will reduce intracranial ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0404.2010.01397.x
更新日期:2011-04-01 00:00:00
abstract:OBJECTIVES:The common prion protein gene (PRNP) codon 129 polymorphism modifies the susceptibility to and the phenotype of prion diseases. However, no truly representative normal population-based data, or data stratified according to age or gender are available on the distribution of this polymorphism. MATERIAL AND ME...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00199.x
更新日期:2003-11-01 00:00:00
abstract::We compared orosomucoid levels in cerebrospinal fluid (CSF) in patients with aseptic meningitis, multiple sclerosis, ischemic and hemorrhagic stroke, CNS-affecting leukemia/lymphoma, and CNS-tumor with the levels in a reference group not having neurologic diseases. Because of possible blood brain barrier damage, we co...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1987.tb05454.x
更新日期:1987-05-01 00:00:00
abstract::The level of urinary taurine and 17-hydroxycorticosteroids were estimated in 19 cases of epilepsy. These levels were then compared with those of normal healthy subjects. The urinary taurine levels were significantly higher in epilepsy and have diagnostic as well as prognostic significance. ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1983.tb04853.x
更新日期:1983-12-01 00:00:00
abstract::The effects of L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS), an unnatural norepinephrine precursor, on alpha 2-adrenergic receptors in platelet membranes were investigated in a patient with familial amyloidotic polyneuropathy, two patients with multiple system atrophy, and two patients with Parkinson's disease. Ea...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1991.tb04901.x
更新日期:1991-07-01 00:00:00
abstract:OBJECTIVES:To assess predictors of headache in patients who had sustained a head injury 22 years earlier. MATERIALS AND METHODS:A questionnaire about headache was sent to 361 subjects hospitalized for head injury in 1974-1975. RESULTS:A total of 249 patients (69%) responded to the questionnaire. The prevalence of hea...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2005.00344.x
更新日期:2005-07-01 00:00:00
abstract:OBJECTIVE:To assess hemispheric differences in the duration of focal onset seizures and its association with clinical and demographic factors. METHODS:A retrospective analysis was performed on adult patients with drug-resistant unifocal epilepsy, who underwent intracranial EEG recording between 01/2006 and 06/2016. Se...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13356
更新日期:2020-10-04 00:00:00
abstract:OBJECTIVES:We investigated cardiovascular and renin responses to head-up tilt tests in patients with Parkinson's disease (PD), multiple system atrophy (MSA), and in controls to determine variables for the assignment of parkinsonism to the potential underlying neurologic condition. PATIENTS AND METHODS:Sequential chang...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1997.tb00270.x
更新日期:1997-10-01 00:00:00
abstract::Hypothyroidism induced by anti-epileptic drug treatment gave rise to thyroid function test studies in patients treated with carbamazepine (CBZ) only. In 42 patients on long-term CBZ treatment thyroxine (T4), free T4-index (FT4I), and triiodothyronine (T3) concentrations in serum were significantly lower than in contro...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1981.tb00754.x
更新日期:1981-02-01 00:00:00
abstract::The relation between angiographically determined cerebral vasospasm following a subarachnoid hemorrhage and regional cerebral blood flow (CBF) was studied in 63 investigations of 45 patients. The CBF was measured using the intra-arterial 133-Xe clearance technique within one hour of angiography. A positive correlation...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1990.tb01598.x
更新日期:1990-08-01 00:00:00
abstract:INTRODUCTION:Cerebral small vessel disease (SVD) contributes to dementia and disability in the elderly, and may negatively affect stroke outcomes. We aimed to evaluate to what extent single features and global burden of SVD detected with magnetic resonance (MR) are associated with worse outcomes in patients with ischae...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12745
更新日期:2017-07-01 00:00:00
abstract:OBJECTIVES:To determine headache characteristics in the obstructive sleep apnea syndrome (OSAS) and in insomnia. PATIENTS AND METHODS:Fifty-six OSAS patients and 50 insomnia patients were assessed in the same time period. Patients in both groups underwent an accurate interview to investigate headache occurrence and it...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2005.00372.x
更新日期:2005-05-01 00:00:00
abstract::Transitional progressive multiple sclerosis (MS) is quite an unusual form of presentation and course of the disease. A case with this progressive form is presented and brain MRI and MTI findings are discussed in relation to the possible insight they may provide for understanding the mechanisms that determine progressi...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1995.tb01036.x
更新日期:1995-08-01 00:00:00