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 6, 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 4, 2019 Category: Neurology Source Type: research

RCVS and TGA: a common pathophysiology?
(Source: Journal of Neurology)
Source: Journal of Neurology - August 4, 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 1, 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 1, 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 1, 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 1, 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 - July 31, 2019 Category: Neurology Source Type: research

Alternatives to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy
(Source: Journal of Neurology)
Source: Journal of Neurology - July 31, 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 16, 2019 Category: Neurology Source Type: research

Wiktor Stein (1899 –1979)
(Source: Journal of Neurology)
Source: Journal of Neurology - July 16, 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 15, 2019 Category: Neurology Source Type: research

High openness and high extroversion are linked with better time-based prospective memory in multiple sclerosis
ConclusionsLow levels of openness and extroversion traits are associated with a greater risk of developing time-based PM deficit. Therefore, personality assessment and behavioural interventions should be encouraged in MS clinical practice. (Source: Journal of Neurology)
Source: Journal of Neurology - July 15, 2019 Category: Neurology Source Type: research

Brachial multisegmental amyotrophy caused by cervical anterior horn cell disorder associated with a spinal CSF leak: a report of five cases
ConclusionsA spinal dural defect may be the essential cause of brachial multisegmental amyotrophy. Surgical dural repair may alter the progressive course of this rare condition. (Source: Journal of Neurology)
Source: Journal of Neurology - July 15, 2019 Category: Neurology Source Type: research

Defining the clinical-genetic and neuroradiological features in SPG54: description of eight additional cases and nine novel DDHD2 variants
AbstractRecessive mutations inDDHD2 cause SPG54, a complex hereditary spastic paraplegia (HSP) with less than forty patients reported worldwide. In this retrospective, multicenter study we describe eight additional SPG54 cases harboring homozygous or compound heterozygousDDHD2 variants. Finally, we reviewed literature data on SPG54, with the aim to better define the phenotype and the brain magnetic resonance imaging (MRI) pattern as well as genotype –phenotype correlations. SPG54 is typically characterized by early-onset (i.e., congenital or, more frequently, infantile) delay in motor and cognitive milestones, couple...
Source: Journal of Neurology - July 12, 2019 Category: Neurology Source Type: research

Deep brain stimulation for Meige syndrome: a meta-analysis with individual patient data
AbstractBackgroundDeep brain stimulation (DBS) is an effective intervention for Meige syndrome, a type of dystonia characterized by blepharospasm, facial, and oromandibular dystonia. This individual patient-level data meta-analysis was to identify the potential outcome predictors, compare the stimulation targets and summarize the efficacy of DBS for Meige syndrome.MethodsThree electronic databases (PubMed, Web of Science and Embase) were searched with no publication data restriction to identify studies regarding DBS for Meige syndrome. The primary outcome was the improvement in BFMDRS-M score. Pearson ’s correlation ...
Source: Journal of Neurology - July 12, 2019 Category: Neurology Source Type: research

Pioneers in neurology: Leonardo da Vinci (1452 –1519)
(Source: Journal of Neurology)
Source: Journal of Neurology - July 12, 2019 Category: Neurology Source Type: research

Retraction Note to: Diagnostic value of atypical lymphocytes in cerebrospinal fluid from adults with enteroviral meningitis
The Joint Editors-in-Chief have retracted this article [1]. An investigation by Kurume University has not been able to establish what the appropriate authorship should be because one of the authors, Yoshihiro Sato, is now deceased. As the appropriate authorship cannot be determined the Editors-in-Chief no longer have confidence in this article. Kurume University has also stated that it has not been possible to verify the data presented as none of the research material is available for investigation. We have not been able to contact Yoshitaka Ohta, Yoshiaki Honda, Masahide Kaji and Kotaro Oizumi about this retraction. (Sour...
Source: Journal of Neurology - July 9, 2019 Category: Neurology Source Type: research

Towards computerized diagnosis of neurological stance disorders: data mining and machine learning of posturography and sway
AbstractWe perform classification, ranking and mapping of body sway parameters from static posturography data of patients using recent machine-learning and data-mining techniques. Body sway is measured in 293 individuals with the clinical diagnoses of acute unilateral vestibulopathy (AVS,n = 49), distal sensory polyneuropathy (PNP,n = 12), anterior lobe cerebellar atrophy (CA,n = 48), downbeat nystagmus syndrome (DN,n = 16), primary orthostatic tremor (OT,n = 25), Parkinson’s disease (PD,n = 27), phobic postural vertigo (PPVn =...
Source: Journal of Neurology - July 7, 2019 Category: Neurology Source Type: research

Mutation update for myelin protein zero-related neuropathies and the increasing role of variants causing a late-onset phenotype
AbstractMutations of myelin protein zero gene (MPZ) are found in 5% of Charcot –Marie–Tooth patients. In 2004, Shy et al. identified two main phenotypes associated with them: an early-onset subtype with mainly demyelinating features and a late-onset subgroup with prominent axonal impairment. We evaluated whether novelMPZ mutations described in literature during the last 14  years could still fit with this classification. We collected and revised reports of 69 novelMPZ mutations. Almost 90% of them could be alternatively classified as responsible for: (a) an early-onset phenotype, with first limitations sta...
Source: Journal of Neurology - July 4, 2019 Category: Neurology Source Type: research

Selection of anterior circulation acute stroke patients for mechanical thrombectomy
ConclusionsTVSS is a reliable tool for selection of AIS candidates for MT and optimization of transfer to comprehensive stroke centers. (Source: Journal of Neurology)
Source: Journal of Neurology - July 2, 2019 Category: Neurology Source Type: research

Influence of procedure time on outcome and hemorrhagic transformation in stroke patients undergoing thrombectomy
ConclusionsThe PT is an independent predictor for 90-day outcomes in stent-retriever thrombectomy patients. Postprocedural HT was partially responsible for the worse outcome in patients who experienced a longer PT. (Source: Journal of Neurology)
Source: Journal of Neurology - July 2, 2019 Category: Neurology Source Type: research

Impact of 4-aminopyridine on vestibulo –ocular reflex performance
AbstractVestibulo –ocular reflexes (VOR) are mediated by frequency-tuned pathways that separately transform the different dynamic and static aspects of head motion/position-related sensory signals into extraocular motor commands. Voltage-dependent potassium conductances such as those formed by Kv1.1 are important f or the ability of VOR circuit elements to encode highly transient motion components. Here we describe the impact of the Kv1.1 channel blocker 4-aminopyridine (4-AP) on spontaneous and motion-evoked discharge of superior oblique motoneurons. Spike activity was recorded from the motor nerve in isolate d prep...
Source: Journal of Neurology - July 2, 2019 Category: Neurology Source Type: research

Discontinuing antiepileptic drugs in long-standing idiopathic generalised epilepsy
AbstractBackgroundOnce adults with long-standing idiopathic generalised epilepsy have achieved stable seizure remission, patients or physicians may attempt to discontinue their antiepileptic drug treatment. To date, risk of subsequent seizure relapse across the four idiopathic generalised epilepsy syndromes is largely unknown, and so are the clinical variables associated.MethodsFor this retrospective observational study, 256 adult outpatients with idiopathic generalised epilepsy were evaluated. Data were obtained from outpatient charts and, if possible, from additional telephone or mail interviews.ResultsIn 84 patients (33...
Source: Journal of Neurology - July 1, 2019 Category: Neurology Source Type: research

Test Your Memory (TYM test): diagnostic evaluation of patients with non-Alzheimer dementias
ConclusionsThe TYM test is a useful test in the detection of non-Alzheimer dementia. The TYM test performs much better than the MMSE at detecting non-Alzheimer dementias. (Source: Journal of Neurology)
Source: Journal of Neurology - July 1, 2019 Category: Neurology Source Type: research

HNRNPDL -related muscular dystrophy: expanding the clinical, morphological and MRI phenotypes
We describe two new unrelated Argentinean families, carrying the previously reported c.1132G>C p.(Asp378His)HNRNPDL mutation. There was a wide phenotypic spectrum including oligo-symptomatic cases, pure limb girdle muscle involvement or distal lower limb muscle weakness. Scapular winging was the most common finding, observed in all patients. Muscle MRIs of the thigh, at different stages of the disease, showed particular involvement of adductor magnus and vastus besides a constant preservation of the rectus femoris and the adductor longus muscles, defining a novel MRI pattern. Muscle biopsy findings were characterized by...
Source: Journal of Neurology - July 1, 2019 Category: Neurology Source Type: research

Prognostic value of Alzheimer ’s biomarkers in mild cognitive impairment: the effect of age at onset
AbstractObjectiveThe aim of this study is to assess the impact of age at onset on the prognostic value of Alzheimer ’s biomarkers in a large sample of patients with mild cognitive impairment (MCI).MethodsWe measured A β42,t-tau, hippocampal volume on magnetic resonance imaging (MRI) and cortical metabolism on fluorodeoxyglucose –positron emission tomography (FDG-PET) in 188 MCI patients followed for at least 1 year. We categorised patients into earlier and later onset (EO/LO). Receiver operating characteristic curves and corresponding areas under the curve (AUCs) were performed to assess and compar th...
Source: Journal of Neurology - July 1, 2019 Category: Neurology Source Type: research

Labyrinthine infarction caused by vertebral artery dissection: consideration based on MRI
(Source: Journal of Neurology)
Source: Journal of Neurology - July 1, 2019 Category: Neurology Source Type: research

Rudolf Altschul (1901 –1963)
(Source: Journal of Neurology)
Source: Journal of Neurology - June 30, 2019 Category: Neurology Source Type: research

Financial capacity in frontotemporal dementia and related presentations
ConclusionsThese initial findings suggest that accurate clinical assessment of financial skills and judgement in patients with possible neurodegenerative dementias requires performance-based assessment. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research

Gluten sensitivity and epilepsy: a systematic review
ConclusionPatients with epilepsy of unknown aetiology should be investigated for serological markers of gluten sensitivity as such patients may benefit from a GFD. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research

Classification of degenerative parkinsonism subtypes by support-vector-machine analysis and striatal 123 I-FP-CIT indices
ConclusionSemiquantitative123I-FP-CIT SPECT striatal evaluation combined with SVM represents a promising approach to disentangle PD from non-degenerative conditions and from atypical PS at the early stage. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research

Thrombocytopenia and declines in platelet counts: predictors of mortality and outcome after mechanical thrombectomy
ConclusionIn AIS patients treated with MT an initial TP was independently associated with higher mortality rates and a marked DPC with higher mortality rates as well as poorer clinical outcomes. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research

Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions
DiscussionAlthough imaging findings in patients with atypical inflammatory demyelinating lesions are heterogeneous, some common features such as peripheral DWI hyperintense rim with open-rim enhancement and absence of oedema argue in favour of a demyelinating lesion and should preclude a brain biopsy. In this context, AQP4 staining is systematically preserved and argues against an AQP4-positive NMOSD. Moreover, long-term follow-up is characterized by low recurrence rate. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research

Structural correlates of attention dysfunction in Lewy body dementia and Alzheimer ’s disease: an ex-Gaussian analysis
ConclusionsThis study shows that different aspects of reaction time performance are differentially affected by AD and LBD, with a difference in structural neural correlates underlying the observed behavioural deficits. While impaired attentional performance is linked to brain atrophy in AD, in LBD it might be related to functional or microstructural rather than macrostructural changes. (Source: Journal of Neurology)
Source: Journal of Neurology - June 20, 2019 Category: Neurology Source Type: research