Abstract:
OBJECTIVES:Myotonic dystrophy type 1 (DM1) is the most common adult-onset muscular dystrophy. It is associated with motor symptoms but patients also display non-motor symptoms such as particular personality traits. Studies have reported mixed results about personality characteristics which may be attributable to small sample sizes, different disease severity of groups studied, and use of different questionnaires or method. This study aimed to describe the psychological characteristics of a large cohort of patients with DM1, to characterize those at risk of developing a psychiatric disorder, and to compare characteristics between two DM1 phenotypes, a mild and more severe adult-onset phenotype. METHODS:Two hundred patients with DM1 (152 adult-onset; 48 mild) were asked to complete questionnaires assessing personality traits, psychological symptoms, self-esteem, and suicidal risk. Neurological and neuropsychological assessments were performed to compare personality characteristics to clinical and cognitive measures. RESULTS:Patients with DM1 globally showed personality traits and psychological symptoms in the average range compared to normative data, with normal levels of self-esteem and suicidal ideation. However, 27% of patients were found to be at high risk of developing a psychiatric disorder. Moreover, psychological traits differed across phenotypes, with the most severe phenotype tending to show more severe psychological symptoms. The presence of higher phobic anxiety and lower self-esteem was associated with lower education, a higher number of CTG repeats, more severe muscular impairment, and lower cognitive functioning (P < 0.001). CONCLUSIONS:Different phenotypes should thus be taken into account in clinical settings for individual management of patients and optimizing therapeutic success.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Bertrand JA,Jean S,Laberge L,Gagnon C,Mathieu J,Gagnon JF,Richer Ldoi
10.1111/ane.12356subject
Has Abstractpub_date
2015-07-01 00:00:00pages
49-58issue
1eissn
0001-6314issn
1600-0404journal_volume
132pub_type
杂志文章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:OBJECTIVES:To characterise a Swedish family with a rare hereditary myopathy with unique sarcoplasmic inclusion bodies in the muscle biopsy. MATERIALS AND METHODS:Part of the pedigree was described in 1980. Nine new members of the included and the phenotype further characterised through clinical, neurophysiological and...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2005.00475.x
更新日期:2005-10-01 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::We present clinical and virological data on 9 patients, 7 women and 2 men aged 31-56 years, with recurrent aseptic meningitis (Mollaret's meningitis). Polymerase chain reaction detected Herpes simplex virus type 2 DNA in cerebrospinal fluid samples from all patients collected during their latest attacks of meningitis....
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1998.tb07296.x
更新日期:1998-09-01 00:00:00
abstract::Current understanding on the historic, epidemiologic, genetic, clinical, neuropathologic, neurochemical, diagnostic and therapeutic aspects of progressive supranuclear palsy (PSP) are revisited. In addition, new research directions are described. ...
journal_title:Acta neurologica Scandinavica
pub_type: 更正并重新发布的文章,杂志文章,评审
doi:
更新日期:1998-12-01 00:00:00
abstract::Among industrial solvents in present use trichloroethylene belongs to those which have been the concern of most neurological and occupational investigations since the beginning of this century. Reports on a broad spectrum of neurotoxicity are however mostly based on case-studies and accidental circumstances. The objec...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1993.tb04101.x
更新日期:1993-03-01 00:00:00
abstract::N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, MPTP, is a neurotoxic substance known to induce a parkinsonian syndrome in primates. The distribution of intravenously injected 11C-labelled MPTP (11C-MPTP) in the head of Rhesus monkeys was studied by means of positron emission tomography, PET. The influence of pretreatme...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1986.tb04618.x
更新日期:1986-07-01 00:00:00
abstract::The role of metabolic abnormalities in the development of diabetic neuropathy is controversial. To investigate the peripheral nerve function and the influence of hyperglycemia on nerve conduction in insulin-dependent diabetes, a one-year neurophysiological study was carried out in 30 type 1 diabetic patients ranging i...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1989.tb03905.x
更新日期:1989-11-01 00:00:00
abstract:OBJECTIVES:Biomarkers with the potential for longitudinal measurements are needed in multiple sclerosis (MS). Urine is easy to collect, and repeated sampling is possible. METHODS:39 paired CSF and urine samples were taken. Oligoclonal bands (OCBs) were measured in CSF. Kappa and lambda free light chain (FLC), neopteri...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12119
更新日期:2013-11-01 00:00:00
abstract::Carotid stenoses of ≥50% account for about 15-20% of strokes. Their degree may be moderate (50-69%) or severe (70-99%). Current diagnostic methods include ultrasound, MR- or CT-angiography. Stenosis severity, irregular plaque surface, and presence of microembolic signals detected by transcranial Doppler predict the ea...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0404.2012.01672.x
更新日期:2012-11-01 00:00:00
abstract::We report 20 cases of right unilateral spatial neglect caused by lesions in the left cerebral hemisphere. Differences in neuropsychological symptoms and lesions sites are discussed in connection with handedness. Of the right-handed patients, 6 had severe aphasia, 4 had Gerstmann's syndrome, and 1 had pure agraphia, bu...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb06040.x
更新日期:1992-06-01 00:00:00
abstract:OBJECTIVES:To evaluate the contribution of the demographical, clinical, analytical and genetic factors to brain signal intensity changes in T2-weighted MR images in amyotrophic lateral sclerosis (ALS) patients and controls. METHODS:Susceptibility-weighted and FLAIR sequences were obtained in a 3T MR scanner. Iron-rela...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12863
更新日期:2018-02-01 00:00:00
abstract:UNLABELLED:Soluble APO-1 (sAPO-1) may prevent apoptosis of lymphocytes induced by activation of the APO-1/Fas receptor. OBJECTIVES:To determine sAPO-1 levels in the serum of multiple sclerosis (MS) patients and controls in order to investigate if abnormal lymphocyte apoptosis occurs in this disease. METHODS:Serum sam...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1997.tb00100.x
更新日期:1997-04-01 00:00:00
abstract:BACKGROUND:For most major antiepileptic drugs, neuropsychological side effects have been reported. Healthy volunteer studies have found that both carbamazepine and valproate impair aspects of verbal memory. OBJECTIVE:The aim of this study was to assess the effects of carbamazepine and valproate on verbal memory, in a ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,随机对照试验
doi:10.1111/j.1600-0404.2010.01452.x
更新日期:2011-06-01 00:00:00
abstract:OBJECTIVE:To describe successful treatment of tardive dyskinesia with levetiracetam. BACKGROUND:Tardive dyskinesia is a late-onset movement disorder caused by exposure to dopamine receptor blocking agents, most commonly neuroleptics. Metoclopramide is frequently used to treat gastrointestinal dysmotility. It has antid...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2007.00950.x
更新日期:2008-05-01 00:00:00
abstract:OBJECTIVES:Clinical research has documented a range of evidence-based treatment approaches for spatial neglect (SN), but there is a lack of research on the implementation of treatment into clinical practice. The purpose of this study is to describe the current clinical practice of SN treatment across healthcare sectors...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13179
更新日期:2020-01-01 00:00:00
abstract::Although intravenous (i.v.) heparin is widely used as the first line treatment for cerebral venous and sinus thrombosis (CVST), the most appropriate therapy for this disease is still controversial. We report 2 cases of CVST who were successfully treated by means of loco-regional thrombolysis with urokinase. In the fir...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.00058.x
更新日期:2001-01-01 00:00:00
abstract:OBJECTIVES:To describe the influence of the site of the bleeding on clinical spectrum and early outcome of patients with acute spontaneous intracerebral hemorrhage (ICH). MATERIALS AND METHODS:ICH was diagnosed in 229 (11%) of 2000 consecutive stroke patients included in a prospective stroke registry over a 10-year pe...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2002.1o170.x
更新日期:2002-04-01 00:00:00
abstract:OBJECTIVES:To examine the factors that influence acute and total length of stay (LOS) for stroke patients. MATERIALS AND METHODS:The basis of this investigation was a population-based cohort of first-ever stroke patients (n = 388). Subjects were survivors of the initial hospitalization (n = 295). Age, sex, social fact...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2006.00756.x
更新日期:2007-07-01 00:00:00
abstract::There has been considerable interest in the possible association between impaired glucose tolerance (IGT) and neuropathy. A systematic literature search (Medline) and review was here performed. Twenty-three studies were evaluated. Fourteen investigated for the presence of neuropathy in patients with IGT. Nine studied ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0404.2010.01425.x
更新日期:2011-07-01 00:00:00
abstract::A headache disorder with shortlasting, frequently occurring (6-18/24 hours) head pain attacks is reported. The pain is excruciatingly severe, unilateral (always on the same side), unaccompanied by visual phenomena, nausea/vomiting, but accompanied by nasal congestion and lacrimation on the symptomatic side. The maximu...
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0404.1976.tb04788.x
更新日期:1976-08-01 00:00:00
abstract:OBJECTIVES:The aim of this pilot study was to evaluate a psychological therapy program used in the treatment of multiple sclerosis (MS) and including cognitive/behavioral strategies, relaxation training and physical exercise. MATERIAL AND METHODS:The participants were 29 patients with MS recruited from an outpatient u...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2003.00077.x
更新日期:2003-06-01 00:00:00
abstract:OBJECTIVES:We examined whether the apolipoprotein E (ApoE) allele epsilon4 influences imaging findings in stroke as assessed by diffusion- (DWI) and perfusion-weighted (PWI) magnetic resonance imaging, and MR angiography (MRA). METHODS:Eight ApoE epsilon4 carriers and 15 non-carriers with acute ischemic stroke in the ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2006.00654.x
更新日期:2006-11-01 00:00:00
abstract:BACKGROUND:It has been suggested that polymorphisms in the WT1 gene modulate the effect of IFN-β treatment in multiple sclerosis (MS) through regulation of the relationship between IFN-β and vitamin D. OBJECTIVE:To examine whether WT1 modulates the relationship between IFN-β and vitamin D in a longitudinal study with ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12448
更新日期:2016-04-01 00:00:00
abstract::This is the first epidemiological study of myasthenia gravis (MG) in the area of Belgrade. During the survey period (1983 1992), 124 incidental cases of MG were observed, producing an average annual incidence rate of 7.1 per million population (women, 8.3; men, 5.8). Age and sex specific incidence rates for females de...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1999.tb00733.x
更新日期:1999-09-01 00:00:00
abstract::IgG and IgM concentrations in tears of multiple sclerosis patients (n = 38) are increased compared to normal controls (n = 23). The occurrence of oligoclonal tear IgG bands (7.9%)--as determined by immunoblotting--did not differ between groups. Our findings suggest an altered reactivity of the secretory immune system ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb04034.x
更新日期:1992-03-01 00:00:00
abstract::Taiwan is geographically, culturally, and socially close to southern Japan, which is an endemic area of human T-lymphotropic virus type 1-associated myelopathy (HAM). Taiwan, however, is not an endemic area of HAM. We report the first case of HAM in a native Taiwanese, a 61-year-old woman, without known HTLV-1 risk fa...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1991.tb04947.x
更新日期:1991-09-01 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::Focal Dystonia (FD) is a chronic neurological disorder, which causes twisting and repetitive movements and abnormal postures induced by involuntary sustained contractions of agonist and antagonist muscles. Based on the hypothesis that several dystonia-related brain regions, including cerebellum, are implicated in ocul...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13224
更新日期:2020-05-01 00:00:00
abstract::In a double-blind cross-over trial with placebo, FPL 57787, a chromone derivate, was tested on 15 patients as a prophylactic against migraine. There was found to be no effect. ...
journal_title:Acta neurologica Scandinavica
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0404.1979.tb02923.x
更新日期:1979-03-01 00:00:00