Sleep disturbances are common in patients with autoimmune encephalitis
ConclusionSleep disturbances are common in AE, warranting active surveillance in affected patients. Improved identification and treatment of sleep disorders may reduce morbidity associated with AE and improve long-term outcomes. (Source: Journal of Neurology)
Source: Journal of Neurology - February 11, 2019 Category: Neurology Source Type: research

Thymomatous myasthenia gravis: novel association with HLA DQB1*05:01 and strengthened evidence of high clinical and serological severity
AbstractBackgroundThe relative prevalence of myasthenia gravis (MG) subtypes is changing, and their differential features and association with HLA class II alleles are not completely understood.MethodsAge at onset, presence/absence of autoantibodies (Ab) and thymoma were retrospectively considered in 230 adult Italian patients. Clinical severity, assessed by MGFA scale, and the highest Ab titer were recorded. Furthermore, we performed low/high resolution typing of HLA-DRB1 and HLA-DQB1 alleles to detect associations of these loci with MG subtypes.ResultsThere were two peaks of incidence: under 41  years of age, with f...
Source: Journal of Neurology - February 11, 2019 Category: Neurology Source Type: research

Infliximab biosimilar for treating neurosarcoidosis: tolerance and efficacy in a retrospective study including switch from the originator and initiation of treatment
ConclusionsIn this retrospective study, the infliximab biosimilar was efficacious and safe for treating neurosarcoidosis. (Source: Journal of Neurology)
Source: Journal of Neurology - February 9, 2019 Category: Neurology Source Type: research

Effectiveness and feasibility of cilostazol in patients with aneurysmal subarachnoid hemorrhage: a systematic review and meta-analysis
AbstractBackgroundDelayed cerebral ischemia seriously affects the prognosis of patients surviving the initial aneurysmal subarachnoid hemorrhage. Application of cilostazol was reported to ameliorate vasospasm and improve outcomes in series and clinical trials. But the effectiveness and feasibility of cilostazol on aneurysmal subarachnoid hemorrhage remained controversial. We performed a systematic review to clarify this issue.MethodsPubMed, Ovid and Cochrane library database were systematically searched up to May 2018 for eligible publications in English. Quality assessment was conducted for included studies. Meta-analysis...
Source: Journal of Neurology - February 9, 2019 Category: Neurology Source Type: research

Early microvascular cerebral blood flow response to head-of-bed elevation is related to outcome in acute ischemic stroke
ConclusionsEarly CBF response to mild HOB elevation in the ipsilesional hemisphere is related to functional outcome. Further studies may enable optical monitoring at the bedside to individualize management strategies in the early phase of AIS. (Source: Journal of Neurology)
Source: Journal of Neurology - February 9, 2019 Category: Neurology Source Type: research

Intravenous immunoglobulins as first-line therapy for IgLON5 encephalopathy
(Source: Journal of Neurology)
Source: Journal of Neurology - February 8, 2019 Category: Neurology Source Type: research

Correction to: Usefulness of MOG-antibody titres at first episode to predict the future clinical course in adults
The original version of this article unfortunately contained a mistake. (Source: Journal of Neurology)
Source: Journal of Neurology - February 7, 2019 Category: Neurology Source Type: research

Five-year outcome in the copaxone observatory: a nationwide cohort of patients with multiple sclerosis starting treatment with glatiramer acetate in France
The objective of this prospective observational cohort study performed in France was to evaluate the effectiveness and safety of glatiramer acetate in patients with relapsing –remitting multiple sclerosis over a 5-year period. All neurologists in France were invited to participate and enroll adult patients starting a first treatment with brand glatiramer acetate 20 mg. Given the observational nature of the study, no fixed study visits were imposed; consultations took place according to the investigator’s normal practice. Occurrence of disease exacerbations and adverse events was documented and neurological...
Source: Journal of Neurology - February 7, 2019 Category: Neurology Source Type: research

Non-alcoholic Wernicke encephalopathy presenting as bilateral hearing loss: a case report
(Source: Journal of Neurology)
Source: Journal of Neurology - February 7, 2019 Category: Neurology Source Type: research

Lack of accuracy of the international classification of disease, ninth (ICD-9) codes in identifying patients with encephalitis
(Source: Journal of Neurology)
Source: Journal of Neurology - February 7, 2019 Category: Neurology Source Type: research

Effect of thymectomy in elderly patients with non-thymomatous generalized myasthenia gravis
In conclusion, thymectomy may have a beneficial effect in elderly patients with non-thymomatous generalized MG. (Source: Journal of Neurology)
Source: Journal of Neurology - February 6, 2019 Category: Neurology Source Type: research

Six-minute walk test is reliable and sensitive in detecting response to therapy in CIDP
ConclusionsThe combination of the 6MWT with the other clinical measures increased the chance to detect the quote of responders. We propose to include the 6MWT in the routine assessment of CIDP patients and the MCID cutoff at 20 m could be set for identifying the responders and properly guiding the therapy management. (Source: Journal of Neurology)
Source: Journal of Neurology - February 5, 2019 Category: Neurology Source Type: research

A case of anaphylaxis to alemtuzumab
(Source: Journal of Neurology)
Source: Journal of Neurology - February 4, 2019 Category: Neurology Source Type: research

Muscle pain in mitochondrial diseases: a picture from the Italian network
AbstractMuscle pain may be part of many neuromuscular disorders including myopathies, peripheral neuropathies and lower motor neuron diseases. Although it has been reported also in mitochondrial diseases (MD), no extensive studies in this group of diseases have been performed so far. We reviewed clinical data from 1398 patients affected with mitochondrial diseases listed in the database of the “Nation-wide Italian Collaborative Network of Mitochondrial Diseases”, to assess muscle pain and its features. Muscle pain was present in 164 patients (11.7%). It was commonly observed in subjects with chronic progressive...
Source: Journal of Neurology - February 2, 2019 Category: Neurology Source Type: research

Brain tissue volumes and relaxation rates in multiple sclerosis: implications for cognitive impairment
AbstractObjectiveBoth normal gray matter atrophy and brain tissue relaxation rates, in addition to total lesion volume, have shown significant correlations with cognitive test scores in multiple sclerosis (MS). Aim of the study was to assess the relative contributions of macro- and microstructural changes of both normal and abnormal brain tissues, probed, respectively, by their volumes and relaxation rates, to the cognitive status and physical disability of MS patients.MethodsMRI studies from 241 patients with relapsing –remitting MS were retrospectively analyzed by fully automated multiparametric relaxometric segmen...
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

Incidence of Parkinson ’s disease in a large patient cohort with idiopathic smell and taste loss
ConclusionThis large patient cohort study extends the previous literature, indicating that risk stratification might be considerably improved by correct diagnostic allocation and emphasizes the need for an exhaustive olfactory and gustatory assessment in specialized centers. (Source: Journal of Neurology)
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

Association between the missense alcohol dehydrogenase rs1229984T variant with the risk for Parkinson ’s disease in women
AbstractBackground/objectiveSeveral meta-analyses including retrospective case –control studies have shown that the risk of developing Parkinson’s disease (PD) correlates inversely with alcohol consumption and (PD), although the results of prospective longitudinal studies are far from being conclusive. The reasons for this inverse association are not well-known. Because al cohol dehydrogenase is one of the most important alcohol-detoxification enzymes, we tried to replicate a putative association of the risk of developing PD with two missense gene variations affecting the alcohol dehydrogenase 1B (ADH1B) gene (...
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

Sez6l2-antibody-associated progressive cerebellar ataxia: a differential diagnosis of atypical parkinsonism
(Source: Journal of Neurology)
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

Clinical and neuroimaging investigations of language disturbance in frontotemporal dementia –motor neuron disease patients
In conclusion, language disturbances in FTD–MND are heterogeneous and more mixed than seen in FTD language phenotypes. Atrophy of the caudate and putamen was correlated with disproportionate impairment of syntactic comprehension. A pure semantic dementia l ike syndrome appears to be rare in FTD–MND. (Source: Journal of Neurology)
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

High performance of cerebrospinal fluid immunoglobulin G analysis for diagnosis of multiple sclerosis
ConclusionsIn the absence of a gold standard, latent class analysis indicates good diagnostic properties of CSF IgG analysis for MS. This test could thus be useful, especially for patients who tested negative for the 2005 and 2010 McDonald criteria. These findings deserve to be confirmed prospectively. (Source: Journal of Neurology)
Source: Journal of Neurology - February 1, 2019 Category: Neurology Source Type: research

Bethlem myopathy: a series of 16 patients and description of seven new associated mutations
ConclusionsManifestations of Bethlem myopathy are quite variable, so either contractures or weakness may be lacking, and no phenotype-genotype associations can be brought. (Source: Journal of Neurology)
Source: Journal of Neurology - January 31, 2019 Category: Neurology Source Type: research

Prospective association between adherence to the MIND diet and subjective memory complaints in the French NutriNet-Sant é cohort
ConclusionsThese findings suggest that the MIND diet could help to prevent or delay SMC among older adults without depressive symptoms. (Source: Journal of Neurology)
Source: Journal of Neurology - January 31, 2019 Category: Neurology Source Type: research

Outcome after individualized stereoelectroencephalography (sEEG) implantation and navigated resection in patients with lesional and non-lesional focal epilepsy
ConclusionFavourable post-surgical outcome can be achieved in patients with resistant focal epilepsy, using individualized sEEG evaluation and tailored navigated resection, even in patients with non-lesional or extratemporal focal epilepsy. (Source: Journal of Neurology)
Source: Journal of Neurology - January 30, 2019 Category: Neurology Source Type: research

Magnetic resonance imaging in immune-mediated myelopathies
AbstractImmune-mediated myelopathies are a heterogeneous group of inflammatory spinal cord disorders including autoimmune disorders with known antibodies, e.g. aquaporin-4 IgG channelopathy or anti-myelin oligodendrocyte glycoprotein-associated myelitis, myelopathies in the context of multiple sclerosis and systemic autoimmune disorders with myelopathy, as well as post-infectious and paraneoplastic myelopathies. Although magnetic resonance imaging of the spinal cord is still challenging due to the small dimension of the cord cross-section and frequent movement and susceptibility artifacts, recent methodological advances ha...
Source: Journal of Neurology - January 29, 2019 Category: Neurology Source Type: research

Individual parkinsonian motor signs and striatal dopamine transporter deficiency: a study with [I-123]FP-CIT SPECT
AbstractIntroductionTotal 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 associated with the highest likelihood of striatal dopamin e deficiency could aid the differential diagnostics of parkinsonian and tremor syndromes.MethodsIn this cross-sectional clinical and imaging study, detailed motor examinations were ...
Source: Journal of Neurology - January 28, 2019 Category: Neurology Source Type: research

Pre-treatment cerebral microbleeds and intracranial hemorrhage in patients with ischemic stroke receiving endovascular therapy: a systematic review and meta-analysis
ConclusionsThere is no evidence that pre-treatment CMBs were associated with an increased incidence of ICH in patients with ischemic stroke receiving EVT. (Source: Journal of Neurology)
Source: Journal of Neurology - January 28, 2019 Category: Neurology Source Type: research

Cognitive changes of older adults with an equivocal amyloid load
ConclusionsThis original work pointed out a population that may be of great interest for interventional studies, raising the question of how amyloid status should be defined and integrated in such studies. These findings should be replicated in future studies on larger datasets, to confirm what methodological approach would be the most suitable to highlight this specific neuroimaging entity. (Source: Journal of Neurology)
Source: Journal of Neurology - January 28, 2019 Category: Neurology Source Type: research

Charles Bonnet syndrome in Leber ’s hereditary optic neuropathy
(Source: Journal of Neurology)
Source: Journal of Neurology - January 28, 2019 Category: Neurology Source Type: research

Tollgate-based progression pathways of ALS patients
ConclusionUsing TASS, clinicians can inform ALS patients about their individualized likelihood of having critical disabilities and assistive-device needs (e.g., being dependent on wheelchair/ventilation, needing walker/wheelchair or communication devices), and help them better prepare for future. (Source: Journal of Neurology)
Source: Journal of Neurology - January 25, 2019 Category: Neurology Source Type: research

Transient seizure onset network for localization of epileptogenic zone: effective connectivity and graph theory-based analyses of ECoG data in temporal lobe epilepsy
ConclusionThe high temporal resolution ffADTF effective connectivity analysis combined with the graph theoretical analysis helps us to understand how epileptic activity is generated and propagated during the seizure period. The newly discovered seizure-specific transient seizure onset network could be an important biomarker and a promising tool for more precise localization of the SOZ and EZ in preoperative evaluations. (Source: Journal of Neurology)
Source: Journal of Neurology - January 25, 2019 Category: Neurology Source Type: research

Evolution of acute lacunar lesions in terms of size and shape: a PICASSO sub-study
AbstractThe imaging definition of lacunar infarcts is variable, particularly regarding their size and the presence of cavitation. We investigated the changes of diameter and evolution pattern of acute lacunar infarcts, and the factors associated with the evolution pattern. Patients with acute single subcortical hemispheric or brainstem ischemic lesions of penetrating arterial territories were included. Maximal diameters on initial diffusion-weighted image (DWI) and follow-up fluid-attenuated inversion recovery image (FLAIR), which performed  >  12 months after initial DWI, were semi-automatically measured....
Source: Journal of Neurology - January 23, 2019 Category: Neurology Source Type: research

Expanding the importance of HMERF titinopathy: new mutations and clinical aspects
ConclusionsOur collection of 12 new families expands mutational spectrum with two new mutations identified. HMERF is not that rare and can be found worldwide, but maybe underdiagnosed. Diagnostic process seems to be complex as this study shows with mostly single patients without clear dominant family history. (Source: Journal of Neurology)
Source: Journal of Neurology - January 21, 2019 Category: Neurology Source Type: research

Comparative efficacy and safety of therapy for the behavioral and psychological symptoms of dementia: a systemic review and Bayesian network meta-analysis
ConclusionPharmacological therapies should be the first choice for BPSD. Aripiprazole, haloperidol, quetiapine, and risperidone of antipsychotics showed the significant efficacy, while memantine, galantine, and donepezil may provide the modest effectiveness. The safety of all was thought to be acceptable. (Source: Journal of Neurology)
Source: Journal of Neurology - January 21, 2019 Category: Neurology Source Type: research

Treatment with alemtuzumab or rituximab after fingolimod withdrawal in relapsing –remitting multiple sclerosis is effective and safe
AbstractBackgroundIt has been described that treating relapsing –remitting multiple sclerosis (RRMS) patients with alemtuzumab following fingolimod could be less effective due to the different dynamics of lymphocyte repopulation. Effectiveness and safety of alemtuzumab compared to rituximab after fingolimod withdrawal were analyzed.Patients and methodsA follow-up of a cohort of RRMS patients treated with alemtuzumab or rituximab after fingolimod withdrawal was accomplished. Effectiveness, measured by the percentage of patients with no evidence of disease activity (NEDA), and the presence of side effects (SE) were reg...
Source: Journal of Neurology - January 19, 2019 Category: Neurology Source Type: research

Sensor-based algorithmic dosing suggestions for oral administration of levodopa/carbidopa microtablets for Parkinson ’s disease: a first experience
ConclusionThis study shows that it is possible to use algorithmic sensor-based dosing adjustments to optimize treatment with oral medication for  PD patients. (Source: Journal of Neurology)
Source: Journal of Neurology - January 18, 2019 Category: Neurology Source Type: research

Deep brain stimulation for monogenic Parkinson ’s disease: a systematic review
AbstractDeep 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 commonlyLRRK2, PRKN, PINK1 andSNCA, and risk-increasing genetic factors such asGBA, proper characterization is becoming increasingly relevant. We performed a systematic review of 46 studies that reported DBS effects in 221 genetic PD patients. The results suggest that monogenic PD patients have variable...
Source: Journal of Neurology - January 18, 2019 Category: Neurology Source Type: research

Factors interfering with parenthood decision-making in an Italian sample of people with multiple sclerosis: an exploratory online survey
ConclusionMS diagnosis impacted dramatically on the life project of 7% of pwMS that decided not to have children because of the disease and in pro-parenthood pwMS impacted especially on having the second child. Only a minority was counseled to plan pregnancy. A worse disease course driving to a progressive phenotype at survey time might have negatively impacted on parenthood desire. (Source: Journal of Neurology)
Source: Journal of Neurology - January 16, 2019 Category: Neurology Source Type: research

High efficacy of rituximab for myasthenia gravis: a comprehensive nationwide study in Austria
ConclusionIn this retrospective study on RTX for MG, the largest to date, RTX appeared safe, efficacious and fast acting. Benefit from RTX was greatest in MuSK ab  + MG. (Source: Journal of Neurology)
Source: Journal of Neurology - January 16, 2019 Category: Neurology Source Type: research

Painful legs and moving toes syndrome evaluated through brain single photon emission computed tomography: a case series
AbstractPainful legs and moving toes (PLMT) syndrome is a clinical entity characterized by persistent pain in the feet or legs and involuntary movements of one or more toes. The precise patho-mechanisms of PLMT still remain unknown. Herein, we examined ten patients clinically identified with PLMT syndrome. All patients first presented persistent pain prior to the onset of motor symptoms. Each patient was examined by neurological investigation, neuro-imaging methods including brain magnetic resonance imaging (MRI) and electrophysiological methods. The brain single photon emission computed tomography (SPECT) images of eight ...
Source: Journal of Neurology - January 14, 2019 Category: Neurology Source Type: research

Peripheral neuropathy in hereditary spastic paraplegia caused by REEP1 variants
The objective of this study was to characterize patients with pathogenicREEP1 variants and neurophysiologically assess the extent of peripheral nerve involvement in this patient group. Thirty-eight index cases were molecular-genetically tested, yielding two previously reported pathogenicREEP1 variants and a novel missense variant, in a total of four index patients. Three of four probands and five additional family members underwent nerve conduction studies, electromyography, quantitative sensory testing, and examination of the autonomic nervous system. None of the examined patients had completely unremarkable results of pe...
Source: Journal of Neurology - January 12, 2019 Category: Neurology Source Type: research

Long-term tolerability, safety and efficacy of rituximab in neuromyelitis optica spectrum disorder: a prospective study
AbstractBackgroundNeuromyelitis optica spectrum disorder (NMOSD) is a B-cell-mediated disease with autoimmunity towards the astrocyte water channel aquaporin-4 (AQP-4) in the central nervous system.ObjectiveTo assess the long-term safety and efficacy in NMOSD patients receiving maintenance therapy with B-cell-depleting agent rituximab for more than 2  years.MethodNMOSD patients were included prospectively from 2014 to 2018 and received continuous cycles of rituximab infusions biannually. Incidence of adverse events (AE), serious AEs (SAE), and infusion-related AEs were evaluated through monthly phone calls and neurolo...
Source: Journal of Neurology - January 11, 2019 Category: Neurology Source Type: research

Subjective cognitive decline and progression to dementia in Parkinson ’s disease: a long-term follow-up study
ConclusionsThe present investigation is the first to conduct a long-term follow-up study of PD-SCD and its relationship with the development of dementia. The results provide relevant data about the characterization of SCD in PD patients and show that PD-SCD is a risk factor for progression to dementia. (Source: Journal of Neurology)
Source: Journal of Neurology - January 11, 2019 Category: Neurology Source Type: research

Mills ’ syndrome revisited
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 laborator y investigations was a mildly elevated CSF protein in one case. MRI demonstrated focal T2 hyper-intensity located eccentrically in the cervical cord ipsilateral to the symptomatic side. No cerebral abnormality was demonstrated. Whilst visual and somatosensory evoked potentials were unremarkable, mot or evoked potentials were abnormal in all patients: central motor conduction times w...
Source: Journal of Neurology - January 10, 2019 Category: Neurology Source Type: research

Long-lasting diagonistic dyspraxia suppressed by rTMS applied to the right motor cortex
We describe the case of a 58-year-old patient with complete callosal agenesis, who developed after a stroke a long lasting and distressing diagonistic dyspraxia. We found two original treatments to relieve the patient from his left limb conflicting movements. Reinforcing left arm sensory input minimized dyspraxic movements but was difficult to apply daily and was found unsatisfactory by the patient. As left anarchic, unwished movements of diagonistic dyspraxia have been attributed to the lack of inhibition exerted by supplementary motor area on right motor cortex, we applied sham-controlled rTMS to the right motor cortex. ...
Source: Journal of Neurology - January 10, 2019 Category: Neurology Source Type: research

Incidence and mechanism of early neurological deterioration after endovascular thrombectomy
ConclusionNeurological deterioration frequently occurs after endovascular thrombectomy, and the risk factors of END differ according to the mechanism of END. (Source: Journal of Neurology)
Source: Journal of Neurology - January 10, 2019 Category: Neurology Source Type: research

Cerebral small vessel disease in patients with spontaneous cerebellar hemorrhage
ConclusionsCerebellar-ICH patients frequently show supratentorial CMBs. The mixed-CMBs pattern appears to be the most common. Our radiological and pathological results suggest that the majority of cerebellar-ICH patients harbor HTN-SVD as dominant microangiopathy. (Source: Journal of Neurology)
Source: Journal of Neurology - January 8, 2019 Category: Neurology Source Type: research

Clinical effect of successful reperfusion in patients presenting with NIHSS   & lt;  8: data from the BEYOND-SWIFT registry
AbstractBackground 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  
Source: Journal of Neurology - January 8, 2019 Category: Neurology Source Type: research

Adult CSF total protein upper reference limits should be age-partitioned and significantly higher than 0.45  g/L: a systematic review
ConclusionsThere is concordance in available literature to recommend increasing CSF total protein upper reference limits, and to consider implementing age-adjusted values above 0.60  g/L starting at age 50. This information merits worldwide dissemination, to reduce the risk of over-diagnosis. (Source: Journal of Neurology)
Source: Journal of Neurology - January 8, 2019 Category: Neurology Source Type: research

Patient MW: transient visual hemi-agnosia
AbstractThe concept of functional modularity in human visual processing was proposed 25 years ago with the distinction between a ventral pathway for object recognition and a dorsal pathway for action processing. Lesions along these pathways yield selective deficits. A 15-year-old patient (MW) presented with a seizure due to a lesion in the left occipitotemporal cortex. Surgical resection of the lesion was performed with sparing of the classic language areas and visual fields. Postoperatively MW had great difficulty reading and had a specific agnosia for more complex visual stimuli in the right hemifield. No deficit was see...
Source: Journal of Neurology - January 7, 2019 Category: Neurology Source Type: research

Continuous subcutaneous apomorphine in advanced Parkinson ’s disease patients treated with deep brain stimulation
We describe our experience with continuous subcutaneous apomorphine infusion (APO) in patients with DBS.MethodsWe undertook a retrospective analysis of all patients treated with DBS and APO at our centre over 12  years. Subjects were allocated to four groups: (1) APO temporarily before DBS, (2) APO after DBS complications before a new DBS, (3) APO after definitive DBS removal, and (4) APO in patients with DBS and declining response. Motor state and other parameters were analysed and compared for the differ ent treatments.ResultsData for 71 patients were evaluated. Group 1: (n = 18) patients improved thei...
Source: Journal of Neurology - January 7, 2019 Category: Neurology Source Type: research