Correction to: Switching from natalizumab to fingolimod treatment in multiple sclerosis: real life data from the Austrian MS Treatment Registry
The original version of this article unfortunately contained a mistake. First and last names of the authors were interchanged. The correct author names are given below. (Source: Journal of Neurology)
Source: Journal of Neurology - September 17, 2019 Category: Neurology Source Type: research

Frequency, aetiology, and impact of vestibular symptoms in the emergency department: a neglected red flag
ConclusionFrequency and impact of vestibular symptoms in patients visiting the ED were higher than previously reported, and life-threatening aetiologies such as strokes are common. Therefore, awareness among physicians regarding the importance of vestibular symptoms has to be improved. (Source: Journal of Neurology)
Source: Journal of Neurology - September 17, 2019 Category: Neurology Source Type: research

Predisposing and precipitating factors for delirium in neurology: a prospective cohort study of 1487 patients
AbstractIntroductionPredisposing and precipitating factors for delirium are well known; however, their interaction and impact on delirium in neurological patients remains largely unknown. Therefore, those factors were evaluated in hospitalized patients with neurological disorders.MethodsIn this prospective cohort study, 1487 neurological patients were included, 356 patients with delirium and 1131 without delirium. Relevant neurological- and medical-related clusters were assessed with multiple regression analyses, prediction models, and cluster analysis evaluating their association with delirium.ResultsThe 1-year incidence ...
Source: Journal of Neurology - September 13, 2019 Category: Neurology Source Type: research

MR-imaging pattern is not a predictor of occult atrial fibrillation in patients with cryptogenic stroke
ConclusionsIn this MRI-based analysis of patients with cryptogenic stroke who had received ICM implantation, the detection rate of AF in patients with ICM was not related to the imaging pattern. In addition, the lacunar infarct pattern should not be an exclusion criterion for ICM insertion in patients with cryptogenic stroke. ICM insertion in patients with cryptogenic stroke should not be evaluated solely on the basis of reference to infarct patterns. (Source: Journal of Neurology)
Source: Journal of Neurology - September 11, 2019 Category: Neurology Source Type: research

Zygmunt Bychowski (1865 –1934)
(Source: Journal of Neurology)
Source: Journal of Neurology - September 10, 2019 Category: Neurology Source Type: research

Interaction between the heart and the brain in transient global amnesia
We examined the incidence of high-sensitivity cardiac troponin I (hs-cTNI) level elevation, electrocardiogram (ECG) findings, and data on clinical management.ResultsAmong the TGA patients, 17 (8.4%) exhibited elevated levels of hs-cTNI. Although none of the patients had ST elevation, 12 (6.7%) showed QTc prolongation and 11 (6.1%) an inverted T wave on ECG. No typical clinical symptoms suggestive of myocardial infarction were present in any of the cases, however, 17 (8.4%) patients complained of mild somatic symptoms. Patients with hs-cTNI level elevation had a significantly greater likelihood of a history of coronary hear...
Source: Journal of Neurology - September 10, 2019 Category: Neurology Source Type: research

How satisfied are cervical dystonia patients after 3  years of botulinum toxin type A treatment? Results from a prospective, long-term observational study
ConclusionsDespite objective clinical improvements over 3  years, subject satisfaction with symptom control remained relatively constant, indicating that factors other than symptom control also play a role in patient satisfaction. (Source: Journal of Neurology)
Source: Journal of Neurology - September 9, 2019 Category: Neurology Source Type: research

Spinal cord lesions are frequently asymptomatic in relapsing –remitting multiple sclerosis: a retrospective MRI survey
AbstractBackgroundSpinal cord (SC) involvement correlates with poor prognosis in patients with multiple sclerosis (MS). Nevertheless, there is no consensus on the use of SC-MRI at follow-up, mainly because of the belief that SC lesions are nearly always symptomatic.ObjectivesThe aim of the present study was to investigate the frequency of asymptomatic SC combined unique activity (CUA, new/enlarging T2 or gadolinium-positive [Gd+] lesions) on MRI in a cohort of patients diagnosed with clinically isolated syndrome (CIS) or relapsing –remitting MS (RRMS).MethodsWe retrospectively investigated all scans showing SC-CUA in...
Source: Journal of Neurology - September 7, 2019 Category: Neurology Source Type: research

Deterioration of specific aspects of gait during the instrumented 6-min walk test among people with multiple sclerosis
AbstractProlonged walking is typically impaired among people with multiple sclerosis (pwMS), however, it is unclear what the contributing factors are or how to evaluate this deterioration. We aimed to determine which gait features become worse during sustained walking and to examine the clinical correlates of gait fatigability in pwMS. Fifty-eight pwMS performed the 6-min walk test while wearing body-fixed sensors. Multiple gait domains (e.g., pace, rhythm, variability, asymmetry and complexity) were compared across each minute of the test and between mild- and moderate-disability patient groups. Associations between the d...
Source: Journal of Neurology - September 6, 2019 Category: Neurology Source Type: research

Economic evaluation of deep-brain stimulation for Tourette ’s syndrome: an initial exploration
ConclusionsOur study indicates that DBS may be a cost-effective treatment for severe TS, based on the very limited clinical data available and under particular assumptions. While the limited availability of data presents a challenge, we also conduct sensitivity analyses to test the robustness of the results to the assumptions used in the analysis. We nevertheless recommend the implementation of randomised controlled trials that collect a comprehensive range of costs and the use of a widely accepted health-related quality of life instrument to enable more definitive statements about the cost-effectiveness of DBS for TS. (So...
Source: Journal of Neurology - September 4, 2019 Category: Neurology Source Type: research

Clinical features, outcomes and prognostic factors of tuberculous meningitis in adults worldwide: systematic review and meta-analysis
ConclusionsAdults with TBM have high rates of mortality. Clinicians should maintain a high clinical suspicion for patients who present with certain clinical features, and should pay more attention to prognostic factors. (Source: Journal of Neurology)
Source: Journal of Neurology - September 4, 2019 Category: Neurology Source Type: research

Role of complement and potential of complement inhibitors in myasthenia gravis and neuromyelitis optica spectrum disorders: a brief review
AbstractThe complement system is a powerful member of the innate immune system. It is highly adept at protecting against pathogens, but exists in a delicate balance between its protective functions and overactivity, which can result in autoimmune disease. A cascade of complement proteins that requires sequential activation, and numerous complement regulators, exists to regulate a proportionate response to pathogens. In spite of these mechanisms there is significant evidence for involvement of the complement system in driving the pathogenesis of variety of diseases including neuromyelitis optica spectrum disorders (NMOSD) a...
Source: Journal of Neurology - September 3, 2019 Category: Neurology Source Type: research

DIZZYNET 2019: approaching the future of vestibular research
(Source: Journal of Neurology)
Source: Journal of Neurology - September 1, 2019 Category: Neurology Source Type: research

Vestibular rehabilitation therapy in Europe: chances and challenges
(Source: Journal of Neurology)
Source: Journal of Neurology - September 1, 2019 Category: Neurology Source Type: research

Can verapamil be effective in controlling vertigo and headache attacks in vestibular migraine accompanied with Meniere ’s disease? A preliminary study
(Source: Journal of Neurology)
Source: Journal of Neurology - September 1, 2019 Category: Neurology Source Type: research

Mutation pattern in 606 Duchenne muscular dystrophy children with a comparison between familial and non-familial forms: a study in an Indian large single-center cohort
ConclusionMLPA could identify mutations in a high percentage of our DMD children. The preponderance of small mutations was noted to be distinctly higher in the familial group. Intriguingly, the familial form of DMD formed a small percentage of the entire cohort. The reasons could be increasing awareness among parents and physicians with early identification of DMD cases, genetic counseling and prenatal testing. (Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Cumulative alcohol consumption and stroke risk in men
ConclusionsCumulative alcohol consumption is an independent risk factor of total stroke and IS in men in a community-based cohort. Even light alcohol intake increases the risk of total stroke and IS. (Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Prognostic factors of postoperative seizure outcome in patients with temporal lobe epilepsy and normal magnetic resonance imaging
ConclusionCarefully selected patients with MRI-negative TLE can be good candidates for surgery. Surgery should be considered with caution in patients with clinical features of neocortical seizure onset and contralateral propagation of the discharge. Use of18F-FDG PET may be helpful to improve SEEG and surgical strategies. The presented data help in optimising the selection of patients with MRI-negative TLE with good chances to benefit from surgery. (Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

NFL is a marker of treatment response in children with SMA treated with nusinersen
AbstractBackgroundRecently, the anti-sense oligonucleotide drug nusinersen was approved for spinal muscular atrophy (SMA) and our aim was to find a response marker for this treatment.MethodsTwelve children with SMA type 1 and two copies of theSMN2 gene were included in a consecutive single-center study. The children were sampled for CSF at baseline and every time nusinersen was given intrathecally. The neuronal biomarkers NFL and tau and the glial biomarker GFAP were measured. Motor function was assessed using CHOP INTEND. Eleven similarly aged children, who were investigated to rule out neurological or infectious disease,...
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Motor and non-motor outcomes in patients with advanced Parkinson ’s disease treated with levodopa/carbidopa intestinal gel: final results of the GREENFIELD observational study
AbstractIntroductionThe GREENFIELD observational study assessed the effect of levodopa/carbidopa intestinal gel (LCIG) on motor and non-motor symptoms, and the related impact on patient quality of life and caregiver burden up to 8  years.MethodsFinal results of a large Italian cohort of patients who started LCIG in routine care between 2007 and 2014 are presented. Comparison between baseline (before LCIG) and follow-up visits on yearly basis (visit 2/3) is reported. Primary endpoint was Unified Parkinson ’s Disease Rating Scale (UPDRS-IV) Item 39; secondary endpoints were UPDRS I and II, dyskinesia items, PD Qua...
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Prevalence of pain in atypical parkinsonism: a systematic review and meta-analysis
In this study, a systematic review and meta-analysis was conducted and peer-reviewed literature was searched to determine the prevalence and types of pain in four atypical parkinsonism syndromes: MSA, PSP, CBD and LBD. The results show that pain was prevalent mainly in MSA patients in comparison to PSP and CBD patients. Pain was reported at an early stage and was found in females, with limb pain being the most common, followed by neck and back pain. In comparison to PSP, pain locations were similar to MSA patients. CBD patients experienced pain the least in comparison to MSA, PSP and LBD patients. (Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

HSV-2-encephalitis in a patient with multiple sclerosis treated with ocrelizumab
(Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Jean Baptiste Octave Landry (1866 –1940)
(Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Cochleovestibular artery syndrome: consideration based on VHIT, VEMP, and inner ear MRI
(Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Management of intravenous thrombolysis in case of mechanical thrombectomy: global real-life data from SITS centers
(Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

CADASIL with spinal cord involvement: a case report and literature review
(Source: Journal of Neurology)
Source: Journal of Neurology - August 8, 2019 Category: Neurology Source Type: research

Reduced gene expression of netrin family members in skin and sural nerve specimens of patients with painful peripheral neuropathies
AbstractObjectiveTo investigate the expression of axon guidance cues in skin and sural nerve biopsies of patients with polyneuropathies (PNP) as potential markers of nerve de- and regeneration and inflammation.MethodsWe prospectively recruited 88 patients with PNP and compared data between patient subgroups and healthy controls. All patients underwent skin punch and/or sural nerve biopsy at the lower leg and proximal thigh. We characterized gene expression profiles of netrin family members as target genes involved in neuronal de- and regeneration [netrin 1, deleted in colorectal cancer (DCC), uncoordinated5H2, neogenin 1 (...
Source: Journal of Neurology - August 7, 2019 Category: Neurology Source Type: research

Gaze stabilisation exercises in vestibular rehabilitation: review of the evidence and recent clinical advances
AbstractGaze stabilisation exercises are a mainstay of vestibular rehabilitation (VR). They are frequently prescribed to patients with vestibular hypofunction with the aim of improving the gain of the vestibular ocular reflex, improving visual acuity during head movement, and reducing the symptoms of dizziness and vertigo. This review will examine current evidence that has evaluated the efficacy of gaze stabilisation exercises on VOR gain, dynamic visual acuity and oscillopsia. In addition, recent experimental studies that have employed digital technologies and newer forms of gaze stabilisation exercises will be reviewed. ...
Source: Journal of Neurology - August 5, 2019 Category: Neurology Source Type: research

RCVS and TGA: a common pathophysiology?
(Source: Journal of Neurology)
Source: Journal of Neurology - August 5, 2019 Category: Neurology Source Type: research

Idarucizumab administration in emergency situations: the Munich Registry of Reversal of Pradaxa ® in clinical routine (MR REPAIR)
ConclusionIn our study, we have shown that the administration of Idarucizumab is a rare intervention and restricted to patients with severe bleeding complications or ischemic stroke. The clinical outcome of patients who received Idarucizumab in emergency situations was poor. (Source: Journal of Neurology)
Source: Journal of Neurology - August 2, 2019 Category: Neurology Source Type: research

Circulating neurofilament light in ischemic stroke: temporal profile and outcome prediction
AbstractBackground and purposeNeurofilament light chain (NfL) is a marker of neuroaxonal damage. We aimed to study associations between serum NfL (sNfL) concentrations at different time points after ischemic stroke and outcomes.MethodsWe prospectively included ischemic stroke cases (n = 595, mean age 59 years, 64% males) and assessed outcomes by both the modified Rankin Scale (mRS) and the NIH stroke scale (NIHSS) at 3 months and by mRS at 2 years. In a subsample, long-term (7-year) outcomes were also assessed by both mRS and NIHSS. We used the ultrasensitive single-molec ule array assay to mea...
Source: Journal of Neurology - August 2, 2019 Category: Neurology Source Type: research

Cerebral blood flow in a case of typical aura without headache
(Source: Journal of Neurology)
Source: Journal of Neurology - August 2, 2019 Category: Neurology Source Type: research

Zona incerta as a therapeutic target in Parkinson ’s disease
AbstractThe zona incerta has recently become an important target for deep-brain stimulation (DBS) in Parkinson ’s disease (PD). The present review summarizes clinical, animal and anatomical data which have indicated an important role of this structure in PD, and discusses potential mechanisms involved in therapeutic effects of DBS. Animal studies have suggested initially some role of neurons as well as GAB Aergic and glutamatergic receptors of the zona incerta in locomotion and generation of PD signs. Anatomical data have indicated that thanks to its multiple interconnections with the basal ganglia, thalamus, cerebra...
Source: Journal of Neurology - August 2, 2019 Category: Neurology Source Type: research

Optimizing 3D FLAIR to detect MS lesions: pushing past factory settings for precise results
ConclusionAn optimized 3D FLAIR with a long TR significantly improved both overall lesion detection and CNR in MS patients as compared to a 3D FLAIR with factory settings. (Source: Journal of Neurology)
Source: Journal of Neurology - August 1, 2019 Category: Neurology Source Type: research

Alternatives to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy
(Source: Journal of Neurology)
Source: Journal of Neurology - August 1, 2019 Category: Neurology Source Type: research

Amyotrophic lateral sclerosis type 8 is not a pure motor disease: evidence from a neuropsychological and behavioural study
ConclusionsALS8 patients present mild executive dysfunction and behavioural changes such as mood disorders, apathy and stereotypic behaviour. Our findings suggest that ALS8 is not a pure motor disorder and it is associated with subtle cognitive and behavioural impairments. (Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Alcohol consumption and risk for Parkinson ’s disease: a systematic review and meta-analysis
AbstractThe possibility that alcohol consumption should be considered as a “protective factor” for Parkinson’s disease (PD) has been suggested by several case–control studies. However, other case–control studies and data from prospective longitudinal cohort studies have been inconclusive. We carried out a systematic review which included all the eligible studies published on PD risk related with alcohol consumption, and conducted a meta-analysis according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The systematic review was performed using two...
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Vestibular symptoms in acute hemispheric strokes
In conclusion, cerebral hemispheric infarction limited to the PIVC or TPSVC does not necessarily cause vertigo. However, unilateral hemispheric infarctions, restricted to the areas belonging to the vestibular cortical network may cause vestibul ar symptoms. The lesions responsible for vestibular symptoms are located more often in the right hemisphere. (Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Comparison of recent pivotal recommendations for the diagnosis and treatment of late-onset Pompe disease using diagnostic nodes —the Pompe disease burden scale
AbstractPompe disease is a rare autosomal-recessive disorder characterised by limb-girdle myopathy and respiratory weakness in the late-onset form (LOPD). Various mutations in the acid alpha-glucosidase gene lead to toxic lysosomal and extra-lysosomal glycogen accumulation in all organs due to ineffective glycogen clearance by the encoded enzyme. Only one randomized trial demonstrated beneficial effects of respiratory function and meters walked in the 6-min walking test with enzyme replacement therapy (ERT). These results were confirmed in several retrospective and prospective observations and in meta-analyses. Due to a po...
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

A randomized controlled trial on the efficacy, safety, and pharmacokinetics of metformin in severe traumatic brain injury
AbstractObjectiveTraumatic brain injury (TBI) is a leading cause of morbidity and mortality worldwide. Metformin is reported to have pleiotropic neuroprotective effects through anti-inflammatory, antioxidative, and anti-ischemic activity, and improvements in vascular hemodynamics and endothelial function. The aim of this study is to examine the efficacy and safety of metformin therapy in severe TBI patients.MethodsThis single-blind, parallel-group, randomized controlled trial enrolled adult TBI patients. Of 158 trauma patients assessed, 30 met the eligibility criteria and were randomly allocated in a one-to-one ratio to re...
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

An update on genetic frontotemporal dementia
AbstractFrontotemporal dementia (FTD) is a highly heritable group of neurodegenerative disorders, with around 30% of patients having a strong family history. The majority of that heritability is accounted for by autosomal dominant mutations in the chromosome 9 open reading frame 72 (C9orf72), progranulin (GRN), and microtubule-associated protein tau (MAPT) genes, with mutations more rarely seen in a number of other genes. This review will discuss the recent updates in the field of genetic FTD. Age at symptom onset in genetic FTD is variable with recently identified genetic modifiers includingTMEM106B (inGRN carriers partic...
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Misjudgment of pre-stroke functional status contradicts beneficial outcomes after endovascular therapy for large vessel occlusion
AbstractEndovascular therapy (EVT) trials enrolled ischemic stroke patients with good pre-stroke functional status. However, this information needed for rapid decision-making is commonly lacking in clinical practice. We hypothesized that initial misjudgment of pre-stroke functional status attenuates clinical outcomes of EVT. Data were derived from our prospective registry of ischemic stroke patients undergoing EVT for anterior circulation large vessel occlusion (01/2016 –12/2017). Considering all information accumulated during hospital course, pre-stroke modified Rankin scale (mRS) was independently re-assessed and c...
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

The misdiagnosis of functional disorders as other neurological conditions
ConclusionsPatients diagnosed with neurological disease sometimes have a functional diagnosis at follow-up which, with hindsight, better explains the original symptoms. This occurs at a frequency similar to the misdiagnosis of ‘organic’ neurological disease as functional disorder. Misdiagnosis can harm patients in either direction, especially as we enter an era of evidence-based treatment for functional neurological disorders. (Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Jean Jacques Lhermitte (1877 –1959)
(Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Neurotoxicity of novel cancer immunotherapies
(Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

How often are patients with progressive supranuclear palsy really falling?
(Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Correction to: Telemedicine in general neurology: use of audiovisual consultation for on call back-up service in an acute care hospital
The original version of this article unfortunately contained a mistake in the acknowledgement section. (Source: Journal of Neurology)
Source: Journal of Neurology - July 18, 2019 Category: Neurology Source Type: research

Isolated tonsilar infarction presenting with positional vertigo and nystagmus
(Source: Journal of Neurology)
Source: Journal of Neurology - July 17, 2019 Category: Neurology Source Type: research

Wiktor Stein (1899 –1979)
(Source: Journal of Neurology)
Source: Journal of Neurology - July 17, 2019 Category: Neurology Source Type: research

Switching from natalizumab to fingolimod treatment in multiple sclerosis: real life data from the Austrian MS Treatment Registry
AbstractObjectivesTo compare the efficacy of natalizumab (NTZ) and fingolimod (FTY) in the treatment of relapsing –remitting multiple sclerosis (MS) in sequential use in common and as a function of transition periods in a nationwide observational cohort using prospectively collected data from a real-life setting.Materials and methodsWe included 195 patients from the Austrian MS Treatment Registry, who had started treatment with NTZ at any time since 2006 and stayed on NTZ for at least 24  months, switched afterwards within 1 year to FTY and stayed on FTY for at least another 12 months. Transition periods be...
Source: Journal of Neurology - July 16, 2019 Category: Neurology Source Type: research