Intracerebral haemorrhage on the acute stroke unit
(Source: Journal of Neurology)
Source: Journal of Neurology - December 9, 2019 Category: Neurology Source Type: research

New awakenings: current understanding of sleep dysfunction and its treatment in Parkinson ’s disease
AbstractThe non-motor features of Parkinson ’s disease (PD) are increasingly being recognised. This review deals with the spectrum of sleep disorders associated with PD, which have a multifactorial aetiology and can significantly have an impact on the quality of life of patients and their carers. Some sleep disorders represent a prodromal p hase of PD, with REM sleep behaviour disorder (RBD) being of particular interest in this regard, whereas others become more common as the disease advances. Understanding the pathophysiology of these sleep disturbances will hopefully lead to new treatment opportunities in the futur...
Source: Journal of Neurology - December 5, 2019 Category: Neurology Source Type: research

Serial position effects rapidly distinguish Alzheimer ’s from frontotemporal dementia
ConclusionWe conclude that obtaining a simplerecency dominance index from a single, 15-item word list memory trial can help discriminate patients with AD from patients with bvFTD, even if they present with similarly severe memory impairment. (Source: Journal of Neurology)
Source: Journal of Neurology - December 5, 2019 Category: Neurology Source Type: research

The clinical and radiological profile of primary lateral sclerosis: an annotation on its pathological and clinical background
(Source: Journal of Neurology)
Source: Journal of Neurology - December 5, 2019 Category: Neurology Source Type: research

Novel NAXE variants as a cause for neurometabolic disorder: implications for treatment
AbstractNeurometabolic disorders are often inherited and complex disorders that result from abnormalities of enzymes important for development and function of the nervous system. Recently, biallelic mutations inNAXE (APOA1BP) were found in patients with an infantile, lethal, neurometabolic disease. Here, exome sequencing was performed in two affected sisters and their healthy parents. The best candidate,NAXE, was tested for replication in exome sequencing data from 4351 patients with neurodevelopmental disorders. Quantitative RT-PCR, western blot and form factor analysis were performed to assessNAXE expression, protein lev...
Source: Journal of Neurology - December 5, 2019 Category: Neurology Source Type: research

Comment on “Early presentation of urinary retention in multiple system atrophy: can the disease begin in the sacral spinal cord?”
(Source: Journal of Neurology)
Source: Journal of Neurology - December 5, 2019 Category: Neurology Source Type: research

The motor prodromes of parkinson ’s disease: from bedside observation to large-scale application
AbstractThere is sufficient evidence that the pathological process that causes Parkinson ’s disease begins years before the clinical diagnosis is made. Over the last 15 years, there has been much interest in the existence of a prodrome in some patients, with a particular focus on non-motor symptoms such as reduced sense of smell, REM-sleep disorder, depression, and constipation. Give n that the diagnostic criteria for Parkinson’s disease depends on the presence of bradykinesia, it is somewhat surprising that there has been much less research into the possibility of subtle motor dysfunction as a pre-diagnos...
Source: Journal of Neurology - December 4, 2019 Category: Neurology Source Type: research

Suicidal risk and demoralization in Parkinson disease
AbstractObjectiveWe aimed to determine suicide risk and lifetime suicidal ideation in Parkinson disease (PD) patients versus controls and how depression, demoralization, and insomnia are associated with suicidality.MethodsIn this case –control study, PD patients and matched controls were recruited from movement disorder clinics, Michael J. Fox Foundation, and Research Match websites. Suicide risk and suicidal ideation were assessed using the Suicidal Behavior Questionnaire-revised (SBQ-R) and Columbia-Suicide Severity Rating Sc ale. Lifetime depression was assessed using the Brief Lifetime Depression Scale, sleep usi...
Source: Journal of Neurology - December 4, 2019 Category: Neurology Source Type: research

Parkinson ’s Disease Multimodal Complex Treatment improves motor symptoms, depression and quality of life
In conclusion, Parkinson’s Disease Multimodal Complex Treatment improves motor symptoms, depression and quality of life. A more detailed selection of patients who will benefit best from this interve ntion should be examined in future studies. (Source: Journal of Neurology)
Source: Journal of Neurology - December 3, 2019 Category: Neurology Source Type: research

Global variation in prevalence and incidence of amyotrophic lateral sclerosis: a systematic review and meta-analysis
ConclusionsWith the aggravation of population aging and the rapid growth of economy, developing regions following the development pattern of the developed regions may suffer rising ALS prevalence and incidence which may increase their disease burden as well. These data highlight the need for research into underlying mechanism and innovations in health-care systems. (Source: Journal of Neurology)
Source: Journal of Neurology - December 3, 2019 Category: Neurology Source Type: research

Long-term follow-up of a large cohort with focal epilepsy of unknown cause: deciphering their clinical and prognostic characteristics
AbstractBackground and purposeFocal epilepsy of unknown cause (FEUC) is an under-investigated topic despite its remarkable frequency. We aimed to report the long-term follow-up findings along with the drug-response, 5  year remission rates and diagnostic changes to give an insight about the heterogeneous characteristics of FEUC.MethodsDemographic, clinical, neurophysiological and imaging data of 196 patients diagnosed as FEUC according to ILAE criteria, with a minimum 5-year follow-up were evaluated in a tertiary epilepsy center. The drug resistance, 5  years of remission and relapse rates were investigated and t...
Source: Journal of Neurology - December 2, 2019 Category: Neurology Source Type: research

Changes of brain structural network connection in Parkinson ’s disease patients with mild cognitive dysfunction: a study based on diffusion tensor imaging
ConclusionsThis study provided new evidence that PD with cognitive dysfunction is associated with WM structural alterations. The nodal efficiency and sub-network analyses focusing on the orbitofrontal part might provide new ideas to explore the physiological mechanism of PD-MCI. (Source: Journal of Neurology)
Source: Journal of Neurology - December 2, 2019 Category: Neurology Source Type: research

Retinal changes in amyotrophic lateral sclerosis: looking at the disease through a new window
AbstractAmyotrophic lateral sclerosis (ALS) is the most frequent degenerative disease affecting motor neurons (MN). ALS has been traditionally considered as a pure motor system disease; however, there are currently sufficient evidences supporting the involvement of other non-motor systems. Recently, the development and the implementation of the optical coherence tomography (OCT) have provided new data regarding the ocular involvement in the disease. In this sense, alterations in retinal nerve fiber layer thickness (RNFL), other retinal layers thicknesses such as outer nuclear layer (ONL) and inner nuclear layer (INL) and c...
Source: Journal of Neurology - December 2, 2019 Category: Neurology Source Type: research

Post-stroke medication adherence and persistence rates: a meta-analysis of observational studies
ConclusionsMedication adherence and persistence rates are low in patients after suffering a stroke. Patient medication adherence or persistence and their influencing factors should be considered for the treatment of stroke patients. More detailed disease prevention and management strategies need to be developed for stroke patients with different comorbidities. (Source: Journal of Neurology)
Source: Journal of Neurology - December 2, 2019 Category: Neurology Source Type: research

Specific intranasal and central trigeminal electrophysiological responses in Parkinson ’s disease
AbstractOlfactory dysfunction is a frequent early non-motor symptom of Parkinson ’s disease (PD). There is evidence that with regard to trigeminal perception, PD-related olfactory dysfunction is different from other olfactory disorders. More specifically, trigeminal sensitivity, when measured behaviorally, was unimpaired in PD patients as opposed to patients with non-Parkinson ian olfactory dysfunction (NPOD). We sought to investigate the trigeminal pathway by measuring electrophysiological recordings from the nasal epithelium and EEG-derived event-related potentials in response to a specific trigeminal stimulus in 2...
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Elevated serum creatine kinase in the early stage of sporadic amyotrophic lateral sclerosis
AbstractObjectiveTo assess the changes of muscle-related biomarkers at the early stage of amyotrophic lateral sclerosis, and to confirm these findings in an experimental animal model.MethodsThirty-nine subjects with sporadic amyotrophic lateral sclerosis and 20 healthy controls were enrolled and longitudinally evaluated. We evaluated serum creatine kinase and creatinine levels and appendicular lean soft-tissue mass using dual X-ray absorptiometry. The levels of biomarkers at early ALS stages were estimated using linear mixed models with unstructured correlation and random intercepts. We also analyzed the longitudinal chang...
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Quality of life in idiopathic dystonia: a systematic review
ConclusionThe findings demonstrate that patients ’ QoL should routinely be assessed and monitored, as this may affect subsequent management. Further research will allow for more robust management of factors contributing to impaired QoL, aside from the physical defects found in dystonia. (Source: Journal of Neurology)
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Genetic and clinical analyses of spinocerebellar ataxia type 8 in mainland China
ConclusionFor the first time, we described the PKD phenotype in association with CTA/CTG expansion, suggesting that CTA/CTG expansion might play a role in the pathogenesis of paroxysmal dyskinesia symptoms. (Source: Journal of Neurology)
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

High-dose steroid therapy for CNS inflammatory diseases increases INR in patients taking oral vitamin K antagonist
(Source: Journal of Neurology)
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Gaetano Rummo (1853 –1917)
(Source: Journal of Neurology)
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Letter to the editors: comment on “Dissociation of caloric and head impulse tests: a marker of Meniere’s disease”
(Source: Journal of Neurology)
Source: Journal of Neurology - November 12, 2019 Category: Neurology Source Type: research

Survival in treated idiopathic normal pressure hydrocephalus
AbstractObjectiveTo describe survival and causes of death in 979 treated iNPH patients from the Swedish Hydrocephalus Quality Registry (SHQR), and to examine the influence of comorbidities, symptom severity and postoperative outcome.MethodsAll 979 patients operated for iNPH 2004 –2011 and registered in the SHQR were included. A matched control group of 4890 persons from the general population was selected by Statistics Sweden. Data from the Swedish Cause of Death Registry was obtained for patients and controls.ResultsAt a median 5.9 (IQR 4.2 –8.1) year follow-up, 37% of the iNPH patients and 23% of the controls...
Source: Journal of Neurology - November 11, 2019 Category: Neurology Source Type: research

Developing a smartphone application, triaxial accelerometer-based, to quantify static and dynamic balance deficits in patients with cerebellar ataxias
ConclusionsWe can state that the APP is an easy, reliable, and valid evaluating system to quantify the trunk sway in a static position and during the gait. (Source: Journal of Neurology)
Source: Journal of Neurology - November 11, 2019 Category: Neurology Source Type: research

Detailed phenotyping of posterior vs. anterior circulation ischemic stroke: a multi-center MRI study
AbstractObjectivePosterior circulation ischemic stroke (PCiS) constitutes 20 –30% of ischemic stroke cases. Detailed information about differences between PCiS and anterior circulation ischemic stroke (ACiS) remains scarce. Such information might guide clinical decision making and prevention strategies. We studied risk factors and ischemic stroke subtypes in PCiS vs. ACiS and lesion location on magnetic resonance imaging (MRI) in PCiS.MethodsOut of 3,301 MRIs from 12 sites in the National Institute of Neurological Disorders and Stroke (NINDS) Stroke Genetics Network (SiGN), we included 2,381 cases with acute DWI lesi...
Source: Journal of Neurology - November 11, 2019 Category: Neurology Source Type: research

Prognostic utility of serum 25-hydroxyvitamin D in patients with stroke: a meta-analysis
ConclusionsLower serum 25-hydroxyvitamin D level is significantly associated with poorer prognosis in stroke patients. Future prospective studies are warranted to verify the prognostic role and to examine the association in different subtypes of stroke. (Source: Journal of Neurology)
Source: Journal of Neurology - November 8, 2019 Category: Neurology Source Type: research

White matter burden does not influence the outcome of mechanical thrombectomy
AbstractBackground and purposeThe significance of white matter hyperintensities (WMHs) in the setting of mechanical thrombectomy (MT) remains poorly documented. We assessed whether pre-existing WMHs burden was associated with baseline clinical and imaging factors and neurological outcome in patients undergoing MT.MethodsThis retrospective single-center study included consecutive acute ischemic stroke (AIS) patients with stroke due to large vessel occlusion treated with MT. WMHs were assessed on baseline T2 fluid-attenuated inversion recovery magnetic resonance imaging. Neurological outcome was assessed at day 90 by the mod...
Source: Journal of Neurology - November 8, 2019 Category: Neurology Source Type: research

Striking loss of second language in bilingual patients with semantic dementia
DiscussionA striking contrast in performance between two languages in bilingual patients with SD indicates that a bilingual ’s L2 or less proficient language is more vulnerable to neurodegeneration. Our findings also support a common semantic network in the brain for the different languages of bilinguals. (Source: Journal of Neurology)
Source: Journal of Neurology - November 8, 2019 Category: Neurology Source Type: research

Prognostic factor of poor outcome in anti-MAG neuropathy: clinical and electrophysiological analysis of a French Cohort
ConclusionEven though disability seems to progress in most cases despite the treatments used, our results suggest that an early electrophysiological reduction in sensory nerves could be considered a ‘red flag’ for the prompt initiation of rituximab to try to delay long-term disability. (Source: Journal of Neurology)
Source: Journal of Neurology - November 8, 2019 Category: Neurology Source Type: research

Early MRI predictors of prognosis in multiple sclerosis
(Source: Journal of Neurology)
Source: Journal of Neurology - November 8, 2019 Category: Neurology Source Type: research

Cerebrospinal fluid non-phosphorylated tau in the differential diagnosis of Creutzfeldt –Jakob disease: a comparative prospective study with 14-3-3
AbstractCerebrospinal fluid (CSF) non-phosphorylated tau (non-p-tau) is increased in sporadic Creutzfeldt –Jakob disease (CJD), but its accuracy in the differential diagnosis has not been previously established. Here, we first used a retrospective cohort of non-CJD (n = 135) and CJD (n = 137) cases to determine the optimal cutoff point for the discrimination of CJD cases. Next, we prospectively quantified non-p-tau and 14-3-3 protein in a cohort of 1427 cases received for CSF testing at the German National Reference Center for transmissible spongiform encephalopathies. Among them, 36 were ...
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Do pain, anxiety and depression influence quality of life for people with amyotrophic lateral sclerosis/motor neuron disease? A national study reconciling previous conflicting literature
ConclusionsThese results show the importance of expressing quality of life in a conceptually appropriate way, as failing to take account of the multidimensional nature of QoL can result in important nuances being overlooked. Clinicians must be aware that pain, depression and anxiety all worsen QoL across their ranges, and not just when pain is severe or when anxiety or depression reach case level. (Source: Journal of Neurology)
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Neurological manifestations in adults with phenylketonuria: new cases and review of the literature
ConclusionNeurological manifestations can complicate PKU in adult patients with elevated phenylalanine levels, after long or short period of diet discontinuation. Neurologists should be aware of this diagnosis, and measure phenylalaninemia in case of neurological symptoms associated with non-specific leucopathy on brain MRI. PKU patients should be systematically encouraged to continue their diet and their medical follow-up to avoid neurological complications. (Source: Journal of Neurology)
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Symptomatic carotid near-occlusion causes a high risk of recurrent ipsilateral ischemic stroke
ConclusionsSymptomatic carotid near-occlusion has a high short-term risk of recurrent ipsilateral ischemic stroke, especially near-occlusion with full collapse. (Source: Journal of Neurology)
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Do heterozygous mutations of Niemann –Pick type C predispose to late-onset neurodegeneration: a review of the literature
Discussion/conclusionThis finding has wider implications in so far as NPC-related forms of Parkinsonian syndromes, dementia, motor neuron disease and other neurodegenerative disorders may benefit from NPC-mechanistic therapies, in particular related to lysosomal dysfunction. Further research is warranted to generate systematic data of heterozygous mutation carriers, including longitudinal data. (Source: Journal of Neurology)
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Susac ’s syndrome as an immune-related adverse event after pembrolizumab: a case report
(Source: Journal of Neurology)
Source: Journal of Neurology - November 7, 2019 Category: Neurology Source Type: research

Risk of Parkinson ’s disease after colectomy: longitudinal follow-up study using a national sample cohort
ConclusionsColectomy was associated with the development of PD, suggesting that colon issues play an important role in the pathophysiological mechanisms of PD. (Source: Journal of Neurology)
Source: Journal of Neurology - November 5, 2019 Category: Neurology Source Type: research

Silent brain infarctions and cognition decline: systematic review and meta-analysis
AbstractBackgroundSilent brain infarction (SBI) may be associated with cognitive decline in the general population. We systematically reviewed prior literature on: (1) SBI and cognition cross-sectionally; (2) baseline SBI and future cognitive decline and risk for cognitive disorders including dementia, and (3) incident SBI and the emergence of cognitive decline or cognitive disorders.MethodsThe MEDLINE and EMBASE databases were searched for relevant studies. Data were independently extracted by two reviewers. Quality was assessed using the Newcastle Ottawa Scale. Data were pooled using a random effects model when more than...
Source: Journal of Neurology - November 5, 2019 Category: Neurology Source Type: research

Eighty years of Medication-Overuse Headache: what about Medication-Overuse Backpain?
ConclusionsConsidering our results, the described withdrawal method seems to be a simple and safe method to achieve pain reduction while simultaneously preventing organ damage. Despite the preliminary character of our results, our hypothesis might stimulate a new understanding of CLBP ’s pathophysiology. (Source: Journal of Neurology)
Source: Journal of Neurology - November 4, 2019 Category: Neurology Source Type: research

Alternating monocular adducting saccadic pulses and dissociated adducting nystagmus during lateral gazes in spinocerebellar ataxia type 3
(Source: Journal of Neurology)
Source: Journal of Neurology - November 4, 2019 Category: Neurology Source Type: research

Cardiorespiratory fitness as a quantitative predictor of the risk of stroke: a dose –response meta-analysis
ConclusionThis meta-analysis provides the evidence that better CRF was with a lower risk of stroke incidence. (Source: Journal of Neurology)
Source: Journal of Neurology - November 4, 2019 Category: Neurology Source Type: research

Teleneurology clinics for polyneuropathy: a pilot study
ConclusionIn this pilot, we were able to efficiently see and examine patients remotely, promote guideline-concordant PN care, and provide a high-satisfaction encounter. (Source: Journal of Neurology)
Source: Journal of Neurology - November 3, 2019 Category: Neurology Source Type: research

Machine-learning-derived rules set excludes risk of Parkinson ’s disease in patients with olfactory or gustatory symptoms with high accuracy
ConclusionsApplying machine-learning techniques, a classifier was developed that took the shape of a set of six hierarchical rules with binary decisions about olfaction-related features or a familial burden of Parkinson ’s disease. Its main clinical strength lies in the exclusion of the possibility of developing Parkinson’s disease in a patient with olfactory or gustatory loss. (Source: Journal of Neurology)
Source: Journal of Neurology - November 1, 2019 Category: Neurology Source Type: research

Self-management practices associated with quality of life for adults with epilepsy
AbstractEpilepsy self-management practices enhance a patient ’s competence and confidence in managing their chronic condition, which is assumed to lead to an improved quality of life (QoL). We analyzed the relationship between the Epilepsy Self-Management Scale (ESMS) responses and the Quality of Life in Epilepsy (QOLIE-31) scores. Baseline questionnaires f rom HOBSCOTCH, a self-management program for cognitive problems, were administered in four New England epilepsy centers on adults (n = 100) with epilepsy and subjective memory complaints. There was no correlation between overall self-management score...
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Amyloid- β and Parkinson’s disease
AbstractParkinson ’s disease (PD) is the second commonest neurodegenerative disorder in the world with a rising prevalence. The pathophysiology is multifactorial but aggregation of misfolded α-synuclein is considered to be a key underpinning mechanism. Amyloid-β (Aβ) and tau deposition are also comorbid associat ions and especially Aβ deposition is associated with cognitive decline in PD. Some existing evidence suggests that low cerebrospinal fluid (CSF) Aβ42 is predictive of future cognitive impairment in PD. Recent studies also show that CSF A β is associated with the postural instabi...
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Zona incerta deep-brain stimulation in orthostatic tremor: efficacy and mechanism of improvement
ConclusionsBilateral caudal zona incerta deep-brain stimulation is effective in refractory orthostatic tremor. Two independent central oscillations were found at tremor and double tremor frequency. Zona incerta DBS produces improvement in OT patients possibly by modifying the abnormal oscillatory proprioceptive input from leg muscles. Frequent changes in deep-brain stimulation settings were required for maintaining the clinical benefit. (Source: Journal of Neurology)
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Motor and non-motor symptoms in blepharospasm: clinical and pathophysiological implications
ConclusionsNon-motor symptoms are independent of motor features and likely belong to the clinical spectrum of blepharospasm. The presence of non-motor symptoms possibly reflects a complex network disorder of blepharospasm. (Source: Journal of Neurology)
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Development and validation of the Heidelberg Neurological Triage System (HEINTS)
ConclusionsHEINTS predicted hospital admissions and resource utilization. Agreement between nurses and physicians was moderate. HEINTS, applied by physicians and by nurses after training, reliably detected neurological emergencies. (Source: Journal of Neurology)
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Imaging respiratory muscle quality and function in Duchenne muscular dystrophy
The objective of this study was to assess respiratory muscle quality and function in DMD using magnetic resonance imaging and to determine the relationship to clinical respiratory function.MethodsIndividuals with DMD (n = 36) and unaffected controls (n = 12) participated in this cross sectional magnetic resonance imaging study. Participants underwent dynamic imaging of the thorax to assess diaphragm and chest wall mobility and chemical shift-encoded imaging of the chest and abdomen to determine fatty infiltration of the accessory respiratory muscles. Additionally, clinical pulmonary function mea...
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Aperiodic alternating nystagmus in isolated vestibular nucleus infarction
(Source: Journal of Neurology)
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research

Legionella infection associated with dimethyl fumarate used for treatment of multiple sclerosis
(Source: Journal of Neurology)
Source: Journal of Neurology - October 21, 2019 Category: Neurology Source Type: research