Journal of Neurology




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The impact of the SARS-CoV-2-pandemic on patients with chronic inflammatory neuropathies: results from the German INHIBIT register

Alina Hieke, Marie Spenner, Fynn Schmitz, Aurelian Schumacher, Maximilian Schröder, Rafael Klimas, Melissa Sgodzai, Jil Brünger, Thomas Grüter, Ralf Gold, Kalliopi Pitarokoili, Anna Lena Fisse & Jeremias Motte

doi : 10.1007/s00415-022-11527-8

Volume 270, issue 4, April 2023

SARS-CoV-2 pandemic is especially compromising for patients with autoimmune diseases with or without immunomodulatory treatment. This study aimed to investigate the longitudinal changes in the health care of patients with immune-mediated neuropathies during the COVID-19 pandemic.

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Brain positron emission tomography (PET) and cognitive abnormalities one year after COVID-19

Roberta Ferrucci, Luca Cuffaro, Antonella Capozza, Chiara Rosci, Natale Maiorana, Elisabetta Groppo, Maria Rita Reitano, Barbara Poletti, Nicola Ticozzi, Luca Tagliabue, Vincenzo Silani & Alberto Priori

doi : 10.1007/s00415-022-11543-8

Emerging evidence indicates that the etiologic agent responsible for coronavirus disease 2019 (COVID-19), can cause neurological complications. COVID-19 may induce cognitive impairment through multiple mechanisms.

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Modifiable risk factors of COVID-19 in patients with multiple sclerosis: a single-centre case–control study

Federico Montini, Agostino Nozzolillo, Paola M. V. Rancoita, Chiara Zanetta, Lucia Moiola, Federica Cugnata, Federica Esposito, Maria A. Rocca, Vittorio Martinelli & Massimo Filippi

doi : 10.1007/s00415-023-11618-0

Disease and treatment-associated immune system abnormalities may confer higher risk of Coronavirus disease 2019 (COVID-19) to people with multiple sclerosis (PwMS). We assessed modifiable risk factors associated with COVID-19 in PwMS.

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Common neural correlates of vestibular stimulation and fear learning: an fMRI meta-analysis

Nicola Neumann, Miquel A. Fullana, Joaquim Radua, Thomas Brandt, Marianne Dieterich & Martin Lotze

doi : 10.1007/s00415-023-11568-7

A bidirectional functional link between vestibular and fear-related disorders has been previously suggested.

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Cognition-oriented treatments and physical exercise on cognitive function in Huntington’s disease: a systematic review

Katharine Huynh, Leila Nategh, Sharna Jamadar, Julie Stout, Nellie Georgiou-Karistianis & Amit Lampit

doi : 10.1007/s00415-022-11516-x

Cognitive impairment is prevalent in Huntington’s disease (HD), with no treatments currently available. While cognition-oriented treatments and physical exercise have shown efficacy in improving cognition in other populations, they have not been systematically reviewed in HD.

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Awareness impairment in Alzheimer’s disease and frontotemporal dementia: a systematic MRI review

Michela Leocadi, Elisa Canu, Angela Paldino, Federica Agosta & Massimo Filippi

doi : 10.1007/s00415-022-11518-9

This review aims to define awareness impairment and related disturbances in neurodegenerative diseases, including Alzheimer’s disease (AD) and frontotemporal lobar degeneration (FTLD) spectrum of disorders.

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Validity of serum neurofilament light chain as a prognostic biomarker of disease activity in multiple sclerosis

Monokesh K. Sen, Md Jakir Hossain, David A. Mahns & Bruce J. Brew

doi : 10.1007/s00415-022-11507-y

Multiple sclerosis (MS) is a chronic demyelinating and neuroinflammatory disease of the human central nervous system with complex pathoetiology, heterogeneous presentations and an unpredictable course of disease progression.

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Cerebral hemorrhage after thrombolysis in stroke patients with unruptured intracranial aneurysms: a systemic review and meta-analysis

Dingke Wen, Yuqi Chen, Wei Zhu, Zongjun Peng & Lu Ma

doi : 10.1007/s00415-022-11533-w

For ischemic stroke patients with concomitant unruptured aneurysm, intravenous thrombolysis therapy (IVT) remains a disputable decision. We hence performed a meta-analysis to identify the related brain hemorrhage rate of unruptured aneurysms and the risk ratio for their rupture comparing to stroke patients who do not have aneurysms.

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Salivary Aβ1–42 may be a quick-tested biomarker for clinical use in Alzheimer’s disease: a meta-analysis

Ziqi Fan, Zheyu Li, Shuai Zhao, Yanxing Chen, Yujie Su, Guoping Peng & Benyan Luo

doi : 10.1007/s00415-022-11509-w

Alzheimer’s disease (AD) is the most prevalent form of dementia among the aging population. Cumulative studies aim to find non-invasive biomarkers in the early stages of AD. Saliva can be obtained easily, and salivary biomarkers have been proven effective in detecting neurodegenerative diseases.

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Vestibular migraine or Meniere's disease: a diagnostic dilemma

Jing-Yu Chen, Zhao-Qi Guo, Jun Wang, Dan Liu, E. Tian, Jia-qi Guo, Wei-Jia Kong & Su-Lin Zhang

doi : 10.1007/s00415-022-11532-x

Meniere’s disease (MD) represents one of the vertigo disorders characterized by triad symptoms (recurrent vertigo, fluctuating hearing loss, tinnitus or ear fullness). The diagnosis of MD relies on the accurate and detailed taking of medical history, and the differentiation between MD and vestibular migraine (VM) is of critical importance from the perspective of the treatment efficacy.

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A whole new world: embracing the systems-level to understand the indirect impact of pathology in neurodegenerative disorders

Natasha L. Taylor & James M. Shine

doi : 10.1007/s00415-022-11550-9

The direct link between neuropathology and the symptoms that emerge from damage to the brain is often difficult to discern. In this perspective, we argue that a satisfying account of neurodegenerative symptoms most naturally emerges from the consideration of the brain from the systems-level.

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Language impairment in the genetic forms of behavioural variant frontotemporal dementia

Kiran Samra, Amy M. MacDougall, Arabella Bouzigues, Martina Bocchetta, David M. Cash, Caroline V. Greaves, Rhian S. Convery, John C. van Swieten, Harro Seelaar, Lize Jiskoot, Fermin Moreno, Raquel Sanchez-Valle, Robert Laforce, Caroline Graff, Mario Masellis, Maria Carmela Tartaglia, James B. Rowe, Barbara Borroni, Elizabeth Finger, Matthis Synofzik, Daniela Galimberti, Rik Vandenberghe, Alexandre de Mendonça, Christopher R. Butler, Alexander Gerhard, Simon Ducharme, Isabelle Le Ber, Pietro Tiraboschi, Isabel Santana, Florence Pasquier, Johannes Levin, Markus Otto, Sandro Sorbi, Jonathan D. Rohrer & Lucy L. Russell On Behalf of the Genetic FTD Initiative (GENFI)

doi : 10.1007/s00415-022-11512-1

Behavioural variant fronto-temporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal lobes.

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Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices

Ahmad Nehme, Sylvain Lanthier, Marion Boulanger, Achille Aouba, Patrice Cacoub, David Jayne, Jean-Paul Makhzoum, Christian Pagnoux, Maxime Rhéaume, Benjamin Terrier, Emmanuel Touzé & Hubert de Boysson

doi : 10.1007/s00415-022-11528-7

Primary angiitis of the central nervous system (PACNS) is a rare disease, for which no validated guidelines exist. We report the findings of a survey on the clinical practice of physicians who manage adults with PACNS.

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Timed up-and-go performance is associated with objectively measured life space in patients 3 months after ischemic stroke: a cross-sectional observational study

Roland Rössler, Nikki Rommers, Eun-Kyeong Kim, Laura Iendra, Alexander Sofios, Eleftheria Giannouli, Erja Portegijs, Taina Rantanen, Denis Infanger, Stephanie Bridenbaugh, Stefan T. Engelter, Arno Schmidt-Trucksäss, Robert Weibel, Nils Peters & Timo Hinrichs

doi : 10.1007/s00415-022-11524-x

Stroke is a common cause of mobility limitation, including a reduction in life space. Life space is defined as the spatial extent in which a person moves within a specified period of time. We aimed to analyze patients’ objective and self-reported life space and clinical stroke characteristics.

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The natural history of terms describing functional (neurological) disorders in the medical literature of the last 60 years

Christo Bratanov, Pascal Hot & Laurent Vercueil

doi : 10.1007/s00415-022-11526-9

Functional neurological disorders (FND), a subtype of functional disorders (FD), are a frequent motive for neurology referrals. The various presentations and the unknown physiopathology of FD have led to the multiplication of terms describing these disorders over the years.

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Adipokines in multiple sclerosis patients are related to clinical and radiological measures

Floor C. Loonstra, Kim F. Falize, Lodewijk R. J. de Ruiter, Menno M. Schoonheim, Eva M. M. Strijbis, Joep Killestein, Helga E. de Vries, Bernard M. J. Uitdehaag & Merel Rijnsburger

doi : 10.1007/s00415-022-11519-8

An imbalance of adipokines, hormones secreted by white adipose tissue, is suggested to play a role in the immunopathology of multiple sclerosis (MS). In people with MS (PwMS) of the same age, we aimed to determine whether the adipokines adiponectin, leptin, and resistin are associated with MS disease severity. Furthermore, we aimed to investigate whether these adipokines mediate the association between body mass index (BMI) and MS disease severity.

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Modern vestibular tests can accurately separate stroke and vestibular neuritis

Benjamin Nham, Chao Wang, Nicole Reid, Zeljka Calic, Belinda Y. C. Kwok, Deborah A. Black, Andrew Bradshaw, GMichael Halmagyi & Miriam S. Welgampola

doi : 10.1007/s00415-022-11473-5

To separate posterior-circulation stroke (PCS) and vestibular-neuritis (VN) using quantitative vestibular tests.

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CSF lymphocytic pleocytosis does not predict a less favourable long-term prognosis in MS

Lauren Astbury, Seema Kalra, Radu Tanasescu & Cris S. Constantinescu

doi : 10.1007/s00415-022-11521-0

The role of CSF lymphocytic pleocytosis in predicting the clinical outcome of multiple sclerosis is unclear. We explored the impact of CSF pleocytosis at diagnosis on long-term disease progression in a large UK cohort.

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Clinical and epidemiological correlates of treatment change in patients with NMOSD: insights from the CIRCLES cohort

Shervin Gholizadeh, Alex Exuzides, Katelyn E. Lewis, Chella Palmer, Michael Waltz, John W. Rose, Anna Marie Jolley, Jacinta M. Behne, Megan K. Behne, Terrence F. Blaschke, Terry J. Smith, Jennifer Sinnott, Lawrence J. Cook, Michael R. Yeaman on behalf of The Guthy-Jackson Charitable Foundation CIRCLES Study Group

doi : 10.1007/s00415-022-11529-6

Neuromyelitis optica spectrum disorders (NMOSD) represent rare autoimmune diseases of the central nervous system largely targeting optic nerve(s) and spinal cord. The present analysis used real-world data to identify clinical and epidemiological correlates of treatment change in patients with NMOSD.

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Hippocampal subfield volumetry in corticobasal syndrome of diverse underlying pathologies

Vasilios C. Constantinides, Vasileios Tentolouris-Piperas, George P. Paraskevas, Efstratios-Stylianos Pyrgelis, Georgios Velonakis, Efstratios Karavasilis, Panagiotis Toulas, Fotini Boufidou, Leonidas Stefanis & Elisabeth Kapaki

doi : 10.1007/s00415-022-11538-5

Multiple pathologies may underlie corticobasal syndrome (CBS), including Alzheimer’s disease (AD). Typical amnestic AD is characterized by early selective hippocampal atrophy. The profile of hippocampal atrophy in AD patients presenting as CBS (CBS-AD), compared to CBS patients of non-AD pathologies (CBS-nAD) and amnestic AD patients, has not been studied.

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Associations between neuropsychiatric symptoms and incident Alzheimer’s dementia in men versus women

Ioannis Liampas, Vasileios Siokas, Constantine G. Lyketsos & Efthimios Dardiotis

doi : 10.1007/s00415-022-11541-w

To examine whether associations between individual neuropsychiatric symptoms (NPS) and incident Alzheimer’s dementia (AD) differ in men versus women.

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The optimum anticoagulation time after endovascular thrombectomy for atrial fibrillation-related large vessel occlusion stroke: a real-world study

Hongrui Ma, Ruiwen Che, Qihan Zhang, Wantong Yu, Longfei Wu, Wenbo Zhao, Ming Li, Di Wu, Chuanjie Wu & Xunming Ji

doi : 10.1007/s00415-022-11515-y

To investigate the relationship between the initiation time of anticoagulation after endovascular treatment (EVT) and the outcomes in atrial fibrillation (AF)-related acute ischemic stroke (AIS) patients.

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Efgartigimod improved health-related quality of life in generalized myasthenia gravis: results from a randomized, double-blind, placebo-controlled, phase 3 study (ADAPT)

Francesco Saccà, Carolina Barnett, Tuan Vu, Stojan Peric, Glenn A. Phillips, Sihui Zhao, Cynthia Z. Qi, Deborah Gelinas, Silvia Chiroli & Jan J. G. M. Verschuuren

doi : 10.1007/s00415-022-11517-w

There are substantial disease and health-related quality-of-life (HRQoL) burdens for many patients with myasthenia gravis (MG), especially for those whose disease symptoms are not well controlled.

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Brain microstructural changes in essential tremor patients and correlations with clinical characteristics: a diffusion kurtosis imaging study

Jinyu Lu, Cheng Zhou, Jiali Pu, Jun Tian, Xinzhen Yin, Dayao Lv, Xiaojun Guan, Tao Guo, Minming Zhang, Baorong Zhang, Yaping Yan & Guohua Zhao

doi : 10.1007/s00415-023-11557-w

Essential tremor (ET) is the second most common movement disorder; however, the pathophysiological mechanism of ET is unclear. We aimed to investigate the microstructural degeneration of gray matter (GM) and white matter (WM) and their correlations with cognition and tremor in patients with ET.

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A blood mRNA panel that differentiates Alzheimer’s disease from other dementia types

Xiaofeng Fu, Changbiao Chu, Yana Pang, Huimin Cai, Ziye Ren & Longfei Jia

doi : 10.1007/s00415-023-11558-9

Messenger RNAs (mRNAs) have been reported to be associated with Alzheimer’s disease (AD). In this study, we investigated whether plasma-based mRNAs could distinguish AD from cognitively normal controls and other types of dementia, including vascular dementia (VaD), Parkinson’s disease dementia (PDD), behavioral variant frontotemporal dementia (bvFTD), and dementia with Lewy body (DLB).

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Neurofilament light (NfL) as biomarker in serum and CSF in status epilepticus

Nils G. Margraf, Justina Dargvainiene, Emily Theel, Frank Leypoldt, Wolfgang Lieb, Andre Franke, Klaus Berger, Jens Kuhle & Gregor Kuhlenbaeumer

doi : 10.1007/s00415-022-11547-4

We explored the potential of neurofilament light chain (NfL) in serum and cerebrospinal fluid as a biomarker for neurodestruction in status epilepticus.

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Optical coherence tomography reflects clinically relevant gray matter damage in patients with multiple sclerosis

Alessandro Cagol, Nuria Cerdá Fuertes, Marc Stoessel, Muhamed Barakovic, Sabine Schaedelin, Marcus D’Souza, Jens Würfel, Alexander U. Brandt, Ludwig Kappos, Till Sprenger, Yvonne Naegelin, Jens Kuhle, Cristina Granziera & Athina Papadopoulou

doi : 10.1007/s00415-022-11535-8

Retinal degeneration leading to optical coherence tomography (OCT) changes is frequent in patients with multiple sclerosis (PwMS).

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Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance

Peter Hermann, Matthias Schmitz, Maria Cramm, Stefan Goebel, Timothy Bunck, Julia Schütte-Schmidt, Walter Schulz-Schaeffer, Christine Stadelmann, Jakob Matschke, Markus Glatzel & Inga Zerr

doi : 10.1007/s00415-022-11549-2

Evaluation of the application of CSF real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance to investigate test accuracy, influencing factors, and associations with disease incidence.

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Impact of advanced Parkinson’s disease on caregivers: an international real-world study

Pablo Martinez-Martin, Matej Skorvanek, Tove Henriksen, Susanna Lindvall, Josefa Domingos, Ali Alobaidi, Prasanna L. Kandukuri, Vivek S. Chaudhari, Apeksha B. Patel, Juan Carlos Parra, James Pike & Angelo Antonini

doi : 10.1007/s00415-022-11546-5

Caring for a partner or family member with Parkinson’s disease (PD) negatively affects the caregiver’s own physical and emotional well-being, especially those caring for people with advanced PD (APD). This study was designed to examine the impact of APD on caregiver perceived burden, quality of life (QoL), and health status.

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Incidence, characteristics, and neuroanatomical substrates of vestibular symptoms in supratentorial stroke

Ji-Yun Park, Jae-Hwan Choi, Jee-Hyun Kwon, Young Cheol Weon, Suk-Min Lee, Hyo Jung Kim, Seo Young Choi, Eun Hye Oh, Hyun Ah Kim, Hyung Lee, Ji-Soo Kim & Kwang-Dong Choi

doi : 10.1007/s00415-023-11566-9

The incidence and characteristics of acute vestibular symptoms, responsible structures, and lateralization of the causative lesions in supratentorial stroke remain unknown. This study aimed to determine the incidence, clinical features, and anatomical correlation of acute vestibular symptoms in supratentorial stroke.

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Sensory processing in the auditory and olfactory domains is normal in laryngeal dystonia

Saul A. Frankford, Lena C. O’Flynn & Kristina Simonyan

doi : 10.1007/s00415-023-11562-z

Abnormal sensory discriminatory processing has been implicated as an endophenotypic marker of isolated dystonia. However, the extent of alterations across the different sensory domains and their commonality in different forms of dystonia are unclear.

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Characteristics of Guillain-Barré syndrome in super-elderly individuals

Mai Minamino, Katsuichi Miyamoto, Motoi Kuwahara, Keisuke Yoshikawa, Jinsoo Koh, Susumu Kusunoki, Yoshitaka Nagai & Hidefumi Ito

doi : 10.1007/s00415-023-11567-8

Japan has the world’s largest super-aging population, and the number of elderly patients with various diseases is increasing. Herein, we reported the characteristics of super-elderly patients, aged over 80 years, with Guillain-Barré syndrome (GBS), a typical neuroimmune disease.

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Rare variants in IMPDH2 cause autosomal dominant dystonia in Chinese population

Junyu Lin, Chunyu Li, Yiyuan Cui, Yanbing Hou, Lingyu Zhang, Ruwei Ou, Qianqian Wei, Kuncheng Liu, Tianmi Yang, Yi Xiao, Qirui Jiang, Bi Zhao, Jing Yang, Xueping Chen & Huifang Shang

doi : 10.1007/s00415-023-11564-x

Recently, IMPDH2 has been linked to dystonia. However, no replication study from other cohorts has been conducted to confirm the association. We aimed to systematically evaluate the genetic associations of IMPDH2 with dystonia in a large dystonia cohort.

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Impaired glymphatic drainage underlying obstructive sleep apnea is associated with cognitive dysfunction

Jiuqi Wang, Yiming Tian, Chi Qin, Lin Meng, Renyi Feng, Shuqin Xu, Yanping Zhai, Dongxiao Liang, Rui Zhang, Haiyan Tian, Han Liu, Yongkang Chen, Yu Fu, Pei Chen, Qingyong Zhu, Junfang Teng & Xuejing Wang

doi : 10.1007/s00415-022-11530-z

Obstructive sleep apnea (OSA) is highly prevalent but easily undiagnosed and is an independent risk factor for cognitive impairment. However, it remains unclear how OSA is linked to cognitive impairment.

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DBS in tremor with dystonia: VIM, GPi or both? A review of the literature and considerations from a single-center experience

Davide Paoli, Russell Mills, Una Brechany, Nicola Pavese & Claire Nicholson

doi : 10.1007/s00415-023-11569-6

Deep brain stimulation (DBS) is an established treatment for dystonia and tremor. However, there is no consensus about the best surgical targeting strategy in patients with concomitant tremor and dystonia. Both the thalamic ventral intermediate nucleus (VIM) and the globus pallidus pars interna (GPi) have been proposed as targets. Few cases using them together in a double-target approach have also been reported.

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Loss of torsional quick eye movements during head roll in progressive supranuclear palsy: a new diagnostic marker

Xia Ling, Hyo-Jung Kim, Jong-Hee Lee, Seonkyung Lee, Jeong-Yoon Choi, Li-Qun Zhong, Xu Yang & Ji-Soo Kim

doi : 10.1007/s00415-023-11578-5

Even though impaired horizontal and vertical saccades are well-known features of progressive supranuclear palsy (PSP), abnormalities of torsional quick phases of eye movements have not been defined in PSP and other Parkinsonian syndromes. This study aims to determine the diagnostic value of decreased torsional quick phases during head oscillations in the roll plane in patients with PSP.

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Comparison of biomarker for diagnosis of nitrous oxide abuse: challenge of cobalamin metabolic parameters, a retrospective study

Guillaume Grzych, Sylvie Deheul, Emeline Gernez, Jean-Baptiste Davion, Dries Dobbelaere, Louise Carton, Isabelle Kim, Jean Claude Guichard, Marie Girot, Linda Humbert, Anas Bennis, Marie Joncquel, Vincent Chieux, Audrey Joly, Phénicia Nasserdine, Nathalie Trillot, Claire Douillard, Pascal Pigny & Céline Tard

doi : 10.1007/s00415-023-11570-z

Recreational use of nitrous oxide (N2O) leads to neurological disorders including combined subacute degeneration of spinal cord, psychological disorders, and thrombosis. Serum or urine N2O assays could not be routinely performed. Hence, it is necessary to investigate other biological markers such as metabolic markers. We aimed here to challenge the three main biological markers used for the diagnosis of nitrous oxide abuse as total vitamin B12, homocysteine, and methylmalonic acid.

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Association between intravenous tirofiban and intracranial hemorrhage in acute large vessel occlusion stroke: insight from the RESCUE BT randomized placebo-controlled trial

Hongfei Sang, Jiacheng Huang, Bingwu Jiang, Qifeng Guo, Thanh N. Nguyen, Mohamad Abdalkader, Qin Han, Simin Zhou, Zhaojun Tao, An Mao, Zhizhong Yan, Jie Du, Ying Jin, Chuming Huang, Tianzhu Liu, Wenlong Zhao, Gangfeng Gu, Li Wang, Shugai Liu, Shiwei Luo, Jie Pu, Jinrong Hu, Jie Yang, Fengli Li, Wenjie Zi, Xiaogang Hu & Zhongming Qiu

doi : 10.1007/s00415-023-11579-4

The aim of this study is to investigate the association between intravenous tirofiban and symptomatic intracranial hemorrhage (SICH) in patients with acute ischemic stroke (AIS) secondary to large vessel occlusion (LVO) receiving endovascular thrombectomy (EVT) within 24 h of time last known well (LKW).

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Transient epileptic amnesia: a retrospective cohort study of 127 cases, including CSF amyloid and tau features

Benjamin Cretin, Nathalie Philippi, Olivier Bousiges & Frédéric Blanc

doi : 10.1007/s00415-023-11576-7

Transient epileptic amnesia (TEA) is a late-onset epilepsy syndrome encompassing transient iterative amnesias and interictal cognitive impairment, two features that overlap with incipient neurodegenerative dementias. We, therefore, examined the yield of CSF amyloid and tau biomarkers in TEA.

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Temporal evolution of new T1-weighted hypo-intense lesions and central brain atrophy in patients with a first clinical demyelinating event treated with subcutaneous interferon β-1a

H. Vrenken, M. Battaglini, M. L. de Vos, G. J. Nagtegaal, B. C. A. Teixeira, A. Seitzinger, D. Jack, M. P. Sormani, B. M. J. Uitdehaag, A. Versteeg, G. Comi, L. Kappos, N. De Stefano & F. Barkhof

doi : 10.1007/s00415-022-11554-5

Evaluate the effect of subcutaneous interferon β-1a (sc IFN β-1a) versus placebo on the evolution of T1-weighted MRI lesions and central brain atrophy in in patients with a first clinical demyelinating event (FCDE).

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Wearable sensor-based gait analysis to discriminate early Parkinson’s disease from essential tremor

Shinuan Lin, Chao Gao, Hongxia Li, Pei Huang, Yun Ling, Zhonglue Chen, Kang Ren & Shengdi Chen

doi : 10.1007/s00415-023-11577-6

Differentiating early-stage Parkinson's disease (PD) from essential tremor (ET) is challenging since they have some overlapping clinical features. Since early-stage PD may present with slight gait impairment and ET generally does not, gait analysis could be used to differentiate PD from ET using machine learning.

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Predictors and reasons for epilepsy patients to decline surgery: a prospective study

Mirja Steinbrenner, Tabea Tito, Christoph Dehnicke & Martin Holtkamp

doi : 10.1007/s00415-022-11510-3

In patients with drug-resistant focal epilepsy, resective surgery is the most successful treatment option to achieve seizure freedom. However, a surprisingly high rate of patients declines their physicians’ recommendation to undergo removal of the seizure focus or—if necessary—further video-EEG monitoring (VEM).

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Sequential alterations in diffusion metrics as correlates of disease severity in amyotrophic lateral sclerosis

Hans-Peter Müller, Anna Behler, Maximilian Münch, Johannes Dorst, Albert C. Ludolph & Jan Kassubek

doi : 10.1007/s00415-023-11582-9

The neuropathology of amyotrophic lateral sclerosis (ALS) follows a regional distribution pattern in the brain with four stages. Using diffusion tensor imaging (DTI), this pattern can be translated into a tract-based staging scheme to assess cerebral progression in vivo. This study investigates the association between the sequential alteration pattern and disease severity in patients with ALS.

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Hypocortisolism as an unusual but treatable cause of dystonia-parkinsonism

C. Desjardins, M. Mongin, A. de Liège & B. Degos

doi : 10.1007/s00415-022-11522-z

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Beneficial effect of N-acetyl-DL-leucine on cognitive function, emotional well-being and QoL in a mentally healthy elderly person

Stefan A. Kolb 

doi : 10.1007/s00415-022-11534-9

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Varicella zoster vasculopathy associated with deep intracerebral haemorrhage

Philip S. Nash, F. Humphries, L. Benjamin & D. J. Werring

doi : 10.1007/s00415-022-11537-6

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Phosphodiesterase 10A autoimmunity presenting as cerebellar ataxia responsive to plasma exchange: a case report

Patrick Tierney, Anastasia Zekeridou & Octavian Adam

doi : 10.1007/s00415-022-11542-9

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PET-measurable innate immune cell activation reduction in chronic active lesions in PPMS brain after rituximab treatment: a case report

Jussi Lehto, Marcus Sucksdorff, Marjo Nylund, Roope Raitanen, Markus Matilainen & Laura Airas

doi : 10.1007/s00415-022-11539-4

To evaluate the effects of rituximab treatment on innate immune cell activation in primary progressive multiple sclerosis (PPMS).

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Neurological update: the palliative care landscape for atypical parkinsonian syndromes

Noreen O’Shea, Shane Lyons, Stephen Higgins & Sean O’Dowd

doi : 10.1007/s00415-023-11574-9

Atypical parkinsonian syndromes are neurodegenerative conditions, characterised by rapid disease progression and shorter life expectancy compared to idiopathic Parkinson’s disease.

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Disease-modifying treatments in Alzheimer’s disease

10.1007/s00415-023-11602-8

doi : 10.1007/s00415-023-11602-8

Marc Edwards & Robin Corkill

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Correction to: Monoallelic KIF1A-related disorders: a multicenter cross sectional study and systematic literature review

doi : 10.1007/s00415-023-11589-2

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Correction to: Italian version of the Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS): validation and longitudinal performance

Andrea Fortuna, Daniele Sabbatini, Annachiara Frigo, Luca Bello, Francesca Calvi, Lorenzo Blasi, Giulia Gianferrari, Ilaria Martinelli, Giacomo Minicuci, Elena Pegoraro, Jessica Mandrioli & Gianni Sorarù

doi : 10.1007/s00415-023-11620-6

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