Sex differences in brain atrophy and cognitive impairment in Parkinson ’s disease patients with and without probable rapid eye movement sleep behavior disorder
ConclusionMale sex is related to increased cognitive impairment and subcortical atrophy in de novo PD-pRBD. Accordingly, we suggest that sex differences are relevant and should be considered in future clinical and translational research. (Source: Journal of Neurology)
Source: Journal of Neurology - August 3, 2021 Category: Neurology Source Type: research

Prognostic factors in adult brainstem glioma: a tertiary care center analysis and review of the literature
AbstractIntroductionAdult brainstem gliomas (BSGs) are rare central nervous system tumours characterized by a highly heterogeneous clinical course. Median survival times range from 11 to 84  months. Beyond surgery, no treatment standard has been established. We investigated clinical and radiological data to assess prognostic features providing support for treatment decisions.Methods34 BSG patients treated between 2000 and 2019 and aged  ≥ 18 years at the time of diagnosis were retrospectively identified from the databases of the two largest Austrian Neuro-Oncology centres. Clinical data including...
Source: Journal of Neurology - August 3, 2021 Category: Neurology Source Type: research

Disentangling the PIGD classification for the prediction of cognitive impairment in de novo Parkinson ’s disease
ConclusionsOur findings suggest that postural instability, as assessed by MDS-UPDRS III, may serve as a possible indicator of the risk of developing cognitive impairment in de novo PD patients rather than the PIGD phenotype. (Source: Journal of Neurology)
Source: Journal of Neurology - August 2, 2021 Category: Neurology Source Type: research

Outcome measures assisting treatment optimization in multiple sclerosis
ConclusionsMuch progress has been made in recent decades for the management of MS and for the evaluation of disease progression. New technology, such as wearable sensors, will provide new opportunities to better understand changes in function, dexterity, and cognition. Essential work over the decades since EDSS was introduced continues to improve our ability to treat this debilitating disease. (Source: Journal of Neurology)
Source: Journal of Neurology - August 2, 2021 Category: Neurology Source Type: research

Neurological manifestations of patients infected with the SARS-CoV-2: a systematic review of the literature
AbstractObjectiveTo perform an updated review of the literature on the neurological manifestations of COVID-19-infected patientsMethodsA PRISMA-guideline-based systematic review was conducted on PubMed, EMBASE, and SCOPUS. Series reporting neurological manifestations of COVID-19 patients were studied.Results39 studies and 68,361 laboratory-confirmed COVID-19 patients were included. Up to 21.3% of COVID-19 patients presented neurological symptoms. Headache (5.4%), skeletal muscle injury (5.1%), psychiatric disorders (4.6%), impaired consciousness (2.8%), gustatory/olfactory dysfunction (2.3%), acute cerebrovascular events (...
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Critical illness-associated cerebral microbleeds for patients with severe COVID-19: etiologic hypotheses
ConclusionsBlood –brain barrier dysfunction secondary to hypoxemia and high concentration of uremic toxins seems to be the main mechanism leading to critical illness-associated cerebral microbleeds, and this complication remains to be frequently described in severe COVID-19 patients. (Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Correction to: Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome
A correction to this paper has been published: https://doi.org/10.1007/s00415-021-10634-2 (Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Myasthenic crisis following SARS-CoV-2 infection and delayed virus clearance in a patient treated with rituximab: clinical course and 6-month follow-up
(Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Adolph Seeligm üller (1837–1912)
(Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Mononeuritis multiplex: an unexpectedly frequent feature of severe COVID-19
AbstractThe prolonged mechanical ventilation that is often required by patients with severe COVID-19 is expected to result in significant intensive care unit-acquired weakness (ICUAW) in many of the survivors. However, in our post-COVID-19 follow-up clinic we have found that, as well as the anticipated global weakness related to loss of muscle mass, a significant proportion of these patients also have disabling focal neurological deficits relating to multiple axonal mononeuropathies. Amongst the 69 patients with severe COVID-19 that have been discharged from the intensive care units in our hospital, we have seen 11 individ...
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

COVID-19 in cladribine-treated relapsing-remitting multiple sclerosis patients: a monocentric experience
(Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Correction to: DRPLA: understanding the natural history and developing biomarkers to accelerate therapeutic trials in a globally rare repeat expansion disorder
A correction to this paper has been published: https://doi.org/10.1007/s00415-021-10644-0 (Source: Journal of Neurology)
Source: Journal of Neurology - July 19, 2021 Category: Neurology Source Type: research

Hypermetabolism associated with worse prognosis of amyotrophic lateral sclerosis
AbstractBackground and objectiveExploration of hypermetabolism in amyotrophic lateral sclerosis (ALS) with different ethnicities is needed to understand its metabolic implications for clinical management. We aimed to evaluate the features of hypermetabolism and investigate its association with clinical characteristics and prognosis of ALS in a prospective Chinese cohort.MethodsThis prospective study was conducted at Peking University Third Hospital, China from 2017 to 2020. 343 participants were enrolled initially. After strict screening, 147 matched health controls and 93 patients with ALS were eligible and underwent deta...
Source: Journal of Neurology - July 18, 2021 Category: Neurology Source Type: research

Outcome after surgical treatment of cerebrospinal fluid leaks in spontaneous intracranial hypotension —a matter of time
AbstractObjectiveSpinal cerebrospinal fluid (CSF) leaks cause spontaneous intracranial hypotension (SIH). Microsurgery can sufficiently seal spinal CSF leaks. Yet, some patients suffer from residual symptoms. Aim of the study was to assess predictors for favorable outcome after surgical treatment of SIH.MethodsWe included consecutive patients with SIH treated surgically from January 2013 to May 2020. Subjects were surveyed by a questionnaire. Primary outcome was resolution of symptoms as rated by the patient. Secondary outcome was postoperative headache intensity on the numeric rating scale (NRS). Association between varia...
Source: Journal of Neurology - July 18, 2021 Category: Neurology Source Type: research

Risk of intracranial haemorrhage and ischaemic stroke after convexity subarachnoid haemorrhage in cerebral amyloid angiopathy: international individual patient data pooled analysis
ConclusionsPatients with cSAH associated with probable or possible CAA have high risk of future ICH and recurrent cSAH. Convexity SAH associated with probable (vs possible) CAA is associated with increased risk of ICH, and cSAH but not ischemic stroke. Our data provide precise risk estimates for key vascular events after cSAH associated with CAA which can inform management decisions. (Source: Journal of Neurology)
Source: Journal of Neurology - July 17, 2021 Category: Neurology Source Type: research

Frequency of silent brain infarction in transient global amnesia
ConclusionsIn a relevant proportion of clinical TGA patients, MRI reveals acute ischemic cerebral lesions. Therefore, cerebral MRI should be performed in patients with TGA to identify a possible cardiac involvement and to detect stroke chameleons. (Source: Journal of Neurology)
Source: Journal of Neurology - July 17, 2021 Category: Neurology Source Type: research

Bilateral vestibulopathy decreases self-motion perception
ConclusionSelf-motion perception is significantly decreased in BV patients compared to control subjects regarding nearly all rotations and translations. Furthermore, decreased self-motion perception is generally associated with lower residual vestibular function in BV patients.Trial registrationTrial registration number NL52768.068.15/METC (Source: Journal of Neurology)
Source: Journal of Neurology - July 14, 2021 Category: Neurology Source Type: research

Contribution of multi-modal imaging to our understanding of dystonia pathogenesis
(Source: Journal of Neurology)
Source: Journal of Neurology - July 14, 2021 Category: Neurology Source Type: research

Adult-onset mitochondrial movement disorders: a national picture from the Italian Network
ConclusionMovement disorders, mainly ataxia and parkinsonism, are important findings in adult primary mitochondrial diseases. This study underlies the importance of looking for a mitochondrial etiology in the diagnostic flowchart of a movement disorder and may help direct genetic screening in daily practice. (Source: Journal of Neurology)
Source: Journal of Neurology - July 14, 2021 Category: Neurology Source Type: research

Irritable bowel syndrome and subsequent risk of Parkinson ’s disease: a nationwide population-based matched-cohort study
ConclusionsWe found temporal relationship between IBS and PD at aged  ≥ 65 years. There might be a possibility that IBS was an early manifestation of PD, and future studies for causal link between the two diseases to elucidate biomechanism are warranted. (Source: Journal of Neurology)
Source: Journal of Neurology - July 13, 2021 Category: Neurology Source Type: research

Safety of alemtuzumab in a nationwide cohort of Finnish multiple sclerosis patients
ConclusionsSAEs were more frequent in the present cohort than in previous studies. Even though alemtuzumab is a highly effective therapy for MS, vigorous monitoring with a long enough follow-up time is advised. (Source: Journal of Neurology)
Source: Journal of Neurology - July 13, 2021 Category: Neurology Source Type: research

Vestibular function testing in patients with RFC1 mutations
(Source: Journal of Neurology)
Source: Journal of Neurology - July 13, 2021 Category: Neurology Source Type: research

A rise in cases of nitrous oxide abuse: neurological complications and biological findings
ConclusionWe report an elevated incidence of neurological complications of nitrous oxide abuse occurring during the recent COVID-19 lockdown. Nitrous oxide abuse should be tracked down in patients presenting with compatible neurological symptoms and elevated homocysteinemia. Vitamin B-12 should be supplemented as soon as the diagnosis is made. (Source: Journal of Neurology)
Source: Journal of Neurology - July 10, 2021 Category: Neurology Source Type: research

Retina thickness in atypical parkinsonism: a systematic review and meta-analysis
ConclusionsThe retina thickness was significantly thinner in PSP and MSA than those in HC and PD. The specific patterns of retina thinning in MSA could be clinical importance for differentiation among atypical parkinsonism. (Source: Journal of Neurology)
Source: Journal of Neurology - July 10, 2021 Category: Neurology Source Type: research

Acute vestibular syndrome: is skew deviation a central sign?
ConclusionsContrary to prior knowledge, skew deviation proved to be more prevalent in patients with AVS and occurred in every forth patient with AUVP. Large skew deviations (>  3.3 deg), were pointing toward a central lesion. Clinical and video test of skew offered little additional diagnostic value compared to other diagnostic tests such as the head impulse test and nystagmus test. Video test of skew could aid to quantify skew in the ED setting in which neurotological expertise is not always readily available. (Source: Journal of Neurology)
Source: Journal of Neurology - July 9, 2021 Category: Neurology Source Type: research

Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings
AbstractObjectiveTo approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.MethodsStenoocclusive lesions, vessel-wall contrast enhancement (VW-CE) and diffusion-restricted lesions were analyzed in patients with a diagnosis of CNSV. On available VWI follow-up, progression, regression or stability of VW-CE were evaluated and correlated with the clinical status.ResultsOf the 45 patients included, 28 exhibited stenoses visible on MR angiography (MRA-positive) w...
Source: Journal of Neurology - July 8, 2021 Category: Neurology Source Type: research

Cerebral mucormycosis: neuroimaging findings and histopathological correlation
ConclusionsMRI is the critical exam for cerebral mucormycosis. Weak ring enhancement and reduced halo diffusion suggest the diagnosis of fungal infections. Involvement of the frontal lobes should raise suspicion of mucormycosis (along with aspergillosis). The perineural spread can be considered a more specific extension pathway of mucormycosis. (Source: Journal of Neurology)
Source: Journal of Neurology - July 8, 2021 Category: Neurology Source Type: research

The angiographic presentation of European Moyamoya angiopathy
AbstractBackground and purposeLittle is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.MethodsConventional cerebral angiograms from 155 Caucasian patients diagnosed as MMA were analyzed with respect to extracranial champagne bottle neck sign, Suzuki stages, collateral status, as well as presence of aneurysms and posterior cerebral artery stenosis.ResultsIn 84 of 155 angiograms, the extracranial carotid artery was visualized, in 65 of them (77.4%), a champagne bottle neck sign was noted. Of the 278 analyzable hemispheres, 13.7%,11.2%, 37.8%, 27.3%, 8.6%, and 1.4% were classified...
Source: Journal of Neurology - July 8, 2021 Category: Neurology Source Type: research

Prevalence of freezing of gait in Parkinson ’s disease: a systematic review and meta-analysis
ConclusionThe result from this systematic review confirms that FOG is very common in PD and its prevalence is usually underestimated in hospital settings. Importantly, a more accurate assessment of FOG in future clinical researches would involve the use of special FOG scale rather than a single item on a scale or a general clinical inquiry. (Source: Journal of Neurology)
Source: Journal of Neurology - July 8, 2021 Category: Neurology Source Type: research

Cystatin C predicts futile recanalization in patients with acute ischemic stroke after endovascular treatment
This study aimed to evaluate the association between cystatin C and futile recanalization in AIS patients who underwent endovascular thrombectomy.MethodsWe carried out a retrospective study of acute ischaemic stroke patients with anterior circulation proximal arterial occlusion who achieved complete arterial recanalization after mechanical thrombectomy from May 2017 to April 2020. The patients with complete recanalization were divided into a useful recanalization group and a futile recanalization group according to their 3-month modified Rankin scale score. FR was defined as a modified mRS score of 3 –6 at 3 mon...
Source: Journal of Neurology - July 5, 2021 Category: Neurology Source Type: research

MRI radiomic features-based machine learning approach to classify ischemic stroke onset time
ConclusionsA unique deep learning model based on DWI/ADC radiomic features was constructed for TSS classification, which could aid in decision making for thrombolysis in patients with unknown stroke onset. (Source: Journal of Neurology)
Source: Journal of Neurology - July 4, 2021 Category: Neurology Source Type: research

The natural history of primary progressive aphasia: beyond aphasia
DiscussionDespite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration. (Source: Journal of Neurology)
Source: Journal of Neurology - July 3, 2021 Category: Neurology Source Type: research

COVID-19 and Parkinson ’s disease: a casual association or a possible second hit in neurodegeneration?
(Source: Journal of Neurology)
Source: Journal of Neurology - July 3, 2021 Category: Neurology Source Type: research

Neuropsychological deficits have only limited impact on psychological well-being in amyotrophic lateral sclerosis
ConclusionThe study provides evidence that minor neuropsychological deficits do not interfere with psychological well-being of ALS in contrast to alterations on behavioural level. Thus, abnormalities in individual cognitive domains have limited relevance for the patients ’ everyday life in comparison to the impact of behavioural alterations. (Source: Journal of Neurology)
Source: Journal of Neurology - July 2, 2021 Category: Neurology Source Type: research

NMOSD and MS prevalence in the Indigenous populations of Australia and New Zealand
ConclusionThe prevalence of NMOSD in the M āori population is similar to South East Asian countries, reflecting their historical origins. The prevalence of MS in this group is intermediate between those with South East Asian and European ancestry living in New Zealand. Both NMOSD and particularly MS appear to be uncommon in the Indigenous p opulations of Australia. (Source: Journal of Neurology)
Source: Journal of Neurology - July 2, 2021 Category: Neurology Source Type: research

Blink reflex recovery cycle distinguishes patients with idiopathic normal pressure hydrocephalus from elderly subjects
ConclusionsThis study represents the first evidence of brainstem hyperexcitability in iNPH patients. Given its low cost and wide availability, R2BRrc could be a useful tool for selecting elderly subjects with mild gait and urinary dysfunction who should undergo an extensive diagnostic workup for the diagnosis of NPH. (Source: Journal of Neurology)
Source: Journal of Neurology - July 2, 2021 Category: Neurology Source Type: research

Stroke care in Italy at the time of the COVID-19 pandemic: a lesson to learn
AbstractFrom March to May 2020, the Italian health care system, as many others, was almost entirely devoted to the fight against the COVID-19 pandemic. In this context, a number of questions arose, from the increased stroke risk due to COVID-19 infection to the quality of stroke patient care. The overwhelming need of COVID-19 patient management made mandatory a complete re-organization of the stroke pathways: many health professionals were reallocated and a number of stroke units was turned into COVID-19 wards. As a result, acute stroke care suffered from a shortage of services and delays in time-dependent treatments and d...
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

Vicente Dimitri (1885 –1955)
AbstractThe pioneer neuropathologist Vicente Dimitri (1885 –1955) was professor and chairman of neurology at the Medical School of the University of Buenos Aires. He founded the Revista Neurol ógica de Buenos Aires, the first Spanish-language journal of neurology (currently, Neurolog ía Argentina), and is eponymously remembered in ‘Sturge–Weber–Dimitri syndrome’ or encephalotrigeminal angiomatosis. (Source: Journal of Neurology)
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

Aseptic meningitis after glatiramer acetate
(Source: Journal of Neurology)
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

Pioneers in neurology: Felix Plaut (1877 –1940)
(Source: Journal of Neurology)
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

A word of hope for ataxia trials in COVID-19 time and beyond
AbstractThe coronavirus disease 2019 (COVID-19) crisis confronted us, like many researchers worldwide, with an unforeseen challenge during the final stages of a randomized controlled trial involving ataxia patients. Institutional guidelines suddenly no longer allowed regular follow-up visits to take place, impeding the clinical evaluation of long-term outcomes. Here, we discuss the various scenarios that we considered in response to these imposed restrictions and share our experience of home video recording by dedicated, extensively instructed family members. Albeit somewhat unconventional at first glance, this last resort...
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

How important are the team dynamics in the battle against COVID-19?
(Source: Journal of Neurology)
Source: Journal of Neurology - June 21, 2021 Category: Neurology Source Type: research

Evaluation of a new body-focused group therapy versus a guided self-help group program for adults with psychogenic non-epileptic seizures (PNES): a pilot randomized controlled feasibility study
AbstractObjectivePsychogenic non-epileptic seizures (PNES), a common phenomenon in neurological settings, are regarded as a paroxysmal type of functional neurological disorder (FND). In a substantial proportion, PNES are disabling with poor long-term outcomes and high economic costs. Despite the clinical and financial consequences of PNES, there is still a lack of controlled clinical trials on the treatment of this challenging disorder. The study aims to evaluate the feasibility and collect first evidence of the efficacy of a group based-intervention in PNES-patients.MethodsA pilot randomized controlled feasibility study w...
Source: Journal of Neurology - June 18, 2021 Category: Neurology Source Type: research

Gabriel Anton (1858 –1933)
(Source: Journal of Neurology)
Source: Journal of Neurology - June 18, 2021 Category: Neurology Source Type: research

Long COVID: cognitive complaints (brain fog) and dysfunction of the cingulate cortex
AbstractMany patients who have suffered from acute COVID infections have long-lasting symptoms affecting several organs including the brain. This long COVID status can include “brain fog” and cognitive deficits that can disturb activities of daily living and can delay complete recovery. Here, we report two cases of neurological long COVID with abnormal FDG PET findings marked by hypometabolic regions of the cingulate cortex. (Source: Journal of Neurology)
Source: Journal of Neurology - June 18, 2021 Category: Neurology Source Type: research

Late-onset Huntington ’s disease associated with CAG repeat lengths of 30 and 31
(Source: Journal of Neurology)
Source: Journal of Neurology - June 17, 2021 Category: Neurology Source Type: research

What happens after fingolimod discontinuation? A multicentre real-life experience
ConclusionsThe main reason for the discontinuation of fingolimod was inefficacy. One-third of the patients had a relapse during the year after discontinuation, 15% experienced a severe reactivation, and approximately 50% of patients with available MRI scan had new/enhancing lesions. The risk factors for disease activity after discontinuation were low lymphocyte count and younger age. (Source: Journal of Neurology)
Source: Journal of Neurology - June 16, 2021 Category: Neurology Source Type: research

Mitochondrial genome variations are associated with amyotrophic lateral sclerosis in patients from mainland China
ConclusionsThis is the first study to profile mtDNA variants in ALS patients from mainland China. Our results suggest that an increase in the number of nonsynonymous variants is linked to the pathogenesis of ALS. Moreover, haplogroup Y and M7c may modulate the clinical expression of ALS. Our findings provide independent, albeit limited, evidence for the role of mtDNA in the pathogenesis of ALS. (Source: Journal of Neurology)
Source: Journal of Neurology - June 15, 2021 Category: Neurology Source Type: research

Acute bilateral optic/chiasm neuritis with longitudinal extensive transverse myelitis in longstanding stable multiple sclerosis following vector-based vaccination against the SARS-CoV-2
(Source: Journal of Neurology)
Source: Journal of Neurology - June 15, 2021 Category: Neurology Source Type: research

The impact of device-assisted therapies on the gut microbiome in Parkinson ’s disease
ConclusionsWe report on initial short-term GM changes in response to the initiation of PD DATs. Prior to the introduction of the DAT, a PD-associated GM was observed. Following initiation of DAT, several DAT-specific changes in GM composition were identified, suggesting DATs can influence the GM in PD. (Source: Journal of Neurology)
Source: Journal of Neurology - June 15, 2021 Category: Neurology Source Type: research