Journal of Neurology

http://link.springer.com/journal/415

List of Papers (Total 733)

Classification of degenerative parkinsonism subtypes by support-vector-machine analysis and striatal 123I-FP-CIT indices

ObjectivesTo provide an automated classification method for degenerative parkinsonian syndromes (PS) based on semiquantitative 123I-FP-CIT SPECT striatal indices and support-vector-machine (SVM) analysis.Methods123I-FP-CIT SPECT was performed at a single-center level on 370 individuals with PS, including 280 patients with Parkinson’s disease (PD), 21 with multiple system atrophy...

Structural correlates of attention dysfunction in Lewy body dementia and Alzheimer’s disease: an ex-Gaussian analysis

BackgroundLewy body dementia (LBD) and Alzheimer’s disease (AD) are common forms of degenerative dementia. While they are characterized by different clinical profiles, attentional deficits are a common feature. The objective of this study was to investigate how attentional problems in LBD and AD differentially affect different aspects of reaction time performance and to identify...

Prevalence and risk factors of stroke in the elderly in Northern China: data from the National Stroke Screening Survey

BackgroundThe overall global burden of stroke is considerable and increasing. In China, stroke is the leading cause of death and disability.MethodsFor this study, we used data from the National Stroke Screening Survey in 2012 and the 2010 Chinese population from sixth National Census of Populations to calculate a standardized (by age, gender, and education) stroke prevalence...

A novel COL6A2 mutation causing late-onset limb-girdle muscular dystrophy

Limb-girdle muscular dystrophies (LGMD) are genetic disorders characterized by weakness of predominantly proximal limb and trunk muscles due to progressive loss of muscle tissue. Collagen VI-related muscular dystrophies usually display more generalized muscle involvement combined with contractures and/or hyperlaxity of distal finger joints. LGMD-like phenotype of collagenopathy...

The etiology of Bell’s palsy: a review

Bell’s palsy is the most common condition involving a rapid and unilateral onset of peripheral paresis/paralysis of the seventh cranial nerve. It affects 11.5–53.3 per 100,000 individuals a year across different populations. Bell’s palsy is a health issue causing concern and has an extremely negative effect on both patients and their families. Therefore, diagnosis and prompt...

The etiology of Bell’s palsy: a review

Bell’s palsy is the most common condition involving a rapid and unilateral onset of peripheral paresis/paralysis of the seventh cranial nerve. It affects 11.5–53.3 per 100,000 individuals a year across different populations. Bell’s palsy is a health issue causing concern and has an extremely negative effect on both patients and their families. Therefore, diagnosis and prompt...

Triple and quadruple cervical artery dissections: a systematic review of individual patient data

Background and purposeSimultaneous dissection of three or four cervical arteries rarely occurs. As a result, limited information is available on clinical characteristics, underlying causes, treatment, and outcome of these patients.MethodsWe performed a systematic review of individual patient data on triple and quadruple cervical artery dissection (CeAD). We included all cases for...

Effect of small-vessel disease on cognitive trajectory after atrial fibrillation-related ischaemic stroke or TIA

Post-stroke dementia is common but has heterogenous mechanisms that are not fully understood, particularly in patients with atrial fibrillation (AF)-related ischaemic stroke or TIA. We investigated the relationship between MRI small-vessel disease markers (including a composite cerebral amyloid angiopathy, CAA, score) and cognitive trajectory over 12 months. We included patients...

Ocrelizumab efficacy in subgroups of patients with relapsing multiple sclerosis

ObjectiveThe efficacy and safety of ocrelizumab, versus interferon (IFN) β-1a, for the treatment of relapsing multiple sclerosis (RMS) from the identically designed OPERA I (NCT01247324) and OPERA II (NCT01412333) phase III studies has been reported; here we present subgroup analyses of efficacy endpoints from the pooled OPERA I and OPERA II populations.MethodsPatients with RMS...

Disease burden of myotonic dystrophy type 1

ObjectiveThe objective of this cross-sectional, observational study was to investigate the disease burden of myotonic dystrophy type 1 (DM1), a disabling muscle disorder.MethodsAdults with DM1 were recruited as part of the PhenoDM1 study from Newcastle University (Newcastle upon Tyne, UK). Disease burden data were recorded through the Individualized Neuromuscular Quality of Life...

Frequent central nervous system, pachymeningeal and plexus MRI changes in POEMS syndrome

ObjectivePolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.MethodsWe retrospectively evaluated CNS MRI in...

Five-year outcome in the copaxone observatory: a nationwide cohort of patients with multiple sclerosis starting treatment with glatiramer acetate in France

The benefits provided by disease-modifying treatments in multiple sclerosis have been demonstrated in clinical trials, but the extent to which they can be extrapolated to everyday care is less clear, as are the long-term benefits of treatment. The objective of this prospective observational cohort study performed in France was to evaluate the effectiveness and safety of...

Individual parkinsonian motor signs and striatal dopamine transporter deficiency: a study with [I-123]FP-CIT SPECT

IntroductionTotal parkinsonian motor symptom severity correlates with presynaptic striatal dopamine function in patients with Parkinson’s disease. There is a lack of studies that have investigated the associations between parkinsonian motor signs and striatal dopaminergic deficiency in patients with parkinsonism of an unknown origin. Identification of specific motor signs...

Expanding the importance of HMERF titinopathy: new mutations and clinical aspects

ObjectiveHereditary myopathy with early respiratory failure (HMERF) is caused by titin A-band mutations in exon 344 and considered quite rare. Respiratory insufficiency is an early symptom. A collection of families and patients with muscle disease suggestive of HMERF was clinically and genetically studied.MethodsAltogether 12 new families with 19 affected patients and diverse...

Deep brain stimulation for monogenic Parkinson’s disease: a systematic review

Deep brain stimulation (DBS) is an effective treatment for Parkinson’s disease (PD) patients with motor fluctuations and dyskinesias. The key DBS efficacy studies were performed in PD patients with unknown genotypes; however, given the estimated monogenic mutation prevalence of approximately 5–10%, most commonly LRRK2, PRKN, PINK1 and SNCA, and risk-increasing genetic factors...

Sensor-based algorithmic dosing suggestions for oral administration of levodopa/carbidopa microtablets for Parkinson’s disease: a first experience

ObjectiveDosing schedules for oral levodopa in advanced stages of Parkinson’s disease (PD) require careful tailoring to fit the needs of each patient. This study proposes a dosing algorithm for oral administration of levodopa and evaluates its integration into a sensor-based dosing system (SBDS).Materials and methodsIn collaboration with two movement disorder experts a knowledge...

Mills’ syndrome revisited

Mills’ syndrome is an idiopathic, slowly progressive, spastic hemiparesis. We describe three cases that have been under review for a minimum of 11 years (range 11–19). In all patients, symptoms started in a leg, with a mean age of onset of 59 years (range 53–63). The only abnormality on laboratory investigations was a mildly elevated CSF protein in one case. MRI demonstrated...

Clinical effect of successful reperfusion in patients presenting with NIHSS < 8: data from the BEYOND-SWIFT registry

Background and purposeIf patients presenting with large vessel occlusions (LVO) and mild symptoms should be treated with endvoascular treatment (EVT) remains unclear. Aims of this study were (1) assessing the safety and technical efficacy of EVT in patients with NIHSS < 8 as opposed to a comparison group of patients presenting with NIHSS ≥ 8 and (2) evaluation of the clinical...

Progressive multifocal leukoencephalopathy in Finland: a cross-sectional registry study

ObjectiveTo investigate if progressive multifocal leucoencephalopathy (PML) incidence has increased in Finland like in the neighbouring Sweden.MethodsNational administrative registries were searched for all PML admissions aged 16 years or more in 2004–2014 on all neurological and internal medicine wards in Finland. The mortality data of the patients was extracted from the...