Journal of Neurology




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Risk and course of COVID-19 in immunosuppressed patients with myasthenia gravis

Frauke Stascheit, Ulrike Grittner, Sarah Hoffmann, Philipp Mergenthaler, Michael Schroeter, Tobias Ruck, Mark Pawlitzki, Franz Blaes, Julia Kaiser, Ulrike Schara, Adela Della-Marina, Andrea Thieme, Tim Hagenacker, Christian Jacobi, Benjamin Berger, Peter P. Urban, Karl Christian Knop, Berthold Schalke, De-Hyung Lee, Petra Kalischewski, Heinz Wiendl & Andreas Meisel 

doi : 10.1007/s00415-022-11389-0

Patients with myasthenia gravis (MG) are potentially prone for a severe COVID-19 course, but there are limited real-world data available on the risk associated with COVID-19 for patients with MG. Here, we investigate whether current immunosuppressive therapy (IST) influences the risk of SARS-CoV-2 infection and COVID-19 severity.

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Structural brain changes in patients with persistent headache after COVID-19 resolution

�lvaro Planchuelo-Gómez, David García-Azorín, �ngel L. Guerrero, Margarita Rodríguez, Santiago Aja-Fernández & Rodrigo de Luis-García 

doi : 10.1007/s00415-022-11398-z

Headache is among the most frequently reported symptoms after resolution of COVID-19. We assessed structural brain changes using T1- and diffusion-weighted MRI processed data from 167 subjects: 40 patients who recovered from COVID-19 but suffered from persistent headache without prior history of headache (COV), 41 healthy controls, 43 patients with episodic migraine and 43 patients with chronic migraine.

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Anti-SARS-CoV-2 humoral and cellular responses in multiple sclerosis patients treated with anti-CD20 monoclonal antibodies

Philippe Nicolas, Hugo Marion-Moffet, Morgane Gossez, Sandra Vukusic, Guillaume Monneret, Romain Marignier & Fabienne Venet 

doi : 10.1007/s00415-022-11353-y

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Association of admission serum levels of neurofilament light chain and in-hospital mortality in geriatric patients with COVID-19

Francesca Marchegiani, Rina Recchioni, Fiorella Marcheselli, Mirko Di Rosa, Jacopo Sabbatinelli, Giulia Matacchione, Angelica Giuliani, Deborah Ramini, Pierpaolo Stripoli, Leonardo Biscetti, Giuseppe Pelliccioni, Riccardo Sarzani, Francesco Spannella, Antonio Cherubini, Andrea Corsonello, Antonio Domenico Procopio, Anna Rita Bonfigli, Massimiliano Bonafè, Fabrizia Lattanzio & Fabiola Olivieri 

doi : 10.1007/s00415-022-11373-8

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Acetyl-dl-leucine in cerebellar ataxia ([18F]-FDG-PET study): how does a cerebellar disorder influence cortical sensorimotor networks?

Sandra Becker-Bense, Lena Kaiser, Regina Becker, Katharina Feil, Carolin Muth, Nathalie L. Albert, Marcus Unterrainer, Peter Bartenstein, Michael Strupp & Marianne Dieterich 

doi : 10.1007/s00415-022-11252-2

The aim of the study was to deepen our insights into central compensatory processes of brain networks in patients with cerebellar ataxia (CA) before and with treatment with acetyl-dl-leucine (AL) by means of resting-state [18F]-FDG-PET brain imaging.

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Homeostatic plasticity of eye movement performance in Xenopus tadpoles following prolonged visual image motion stimulation

Michael Forsthofer & Hans Straka 

doi : 10.1007/s00415-022-11311-8

Visual image motion-driven ocular motor behaviors such as the optokinetic reflex (OKR) provide sensory feedback for optimizing gaze stability during head/body motion.

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Endolymphatic space is age-dependent

Marianne Dieterich, Tatjana Hergenroeder, Rainer Boegle, Johannes Gerb, Emilie Kierig, Sophia Stöcklein & Valerie Kirsch 

doi : 10.1007/s00415-022-11400-8

Knowledge of the physiological endolymphatic space (ELS) is necessary to estimate endolymphatic hydrops (ELH) in patients with vestibulocochlear syndromes. Therefore, the current study investigated age-dependent changes in the ELS of participants with normal vestibulocochlear testing.

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Vestibular paroxysmia entails vestibular nerve function, microstructure and endolymphatic space changes linked to root-entry zone neurovascular compression

Emilie Kierig, Johannes Gerb, Rainer Boegle, Birgit Ertl-Wagner, Marianne Dieterich & Valerie Kirsch 

doi : 10.1007/s00415-022-11399-y

Combining magnetic resonance imaging (MRI) sequences that permit the determination of vestibular nerve angulation (NA = change of nerve caliber or direction), structural nerve integrity via diffusion tensor imaging (DTI), and exclusion of endolymphatic hydrops (ELH) via delayed gadolinium-enhanced MRI of the inner ear (iMRI) could increase the diagnostic accuracy in patients with vestibular paroxysmia (VP).

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Monkeypox virus from neurological complications to neuroinvasive properties: current status and future perspectives

Ali Sepehrinezhad, Rezan Ashayeri Ahmadabad & Sajad Sahab-Negah 

doi : 10.1007/s00415-022-11339-w

Cases of monkeypox (MPV) are sharply rising around the world. While most efforts are being focused on the management of the first symptoms of monkeypox, such as cutaneous lesions and flu-like symptoms, the effect of the monkeypox virus (MPXV) on multiple organs still remains unclear.

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Autonomic dysfunction in progressive supranuclear palsy

Francesca Baschieri, Maria Vitiello, Pietro Cortelli, Giovanna Calandra-Buonaura & Francesca Morgante 

doi : 10.1007/s00415-022-11347-w

The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results.

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Nonconvulsive status epilepticus following cardiac arrest: overlooked, untreated and misjudged

Pia De Stefano, Peter W. Kaplan, Hervé Quintard, Margitta Seeck & Raoul Sutter 

doi : 10.1007/s00415-022-11368-5

Seizures and status epilepticus (SE) are detected in almost a third of the comatose cardiac arrest survivors. As the literature is quite exhaustive regarding SE with motor symptoms in those patients, little is known about nonconvulsive SE (NCSE).

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Prevalence of axial postural abnormalities and their subtypes in Parkinson’s disease: a systematic review and meta-analysis

Shuangshuang Cao, Yusha Cui, Jianing Jin, Fangfei Li, Xin Liu & Tao Feng 

doi : 10.1007/s00415-022-11354-x

Axial postural abnormalities, mainly involving the spinal deformities, are disabling symptoms of Parkinson’s disease (PD). However, the prevalence of axial postural abnormalities in PD and their clinical correlates remain unclear. The present study aimed to conduct a systematic review and meta-analysis of the prevalence of overall and subtypes of axial postural abnormalities in PD.

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A systematic review and meta-analysis of voxel-based morphometric studies of migraine

Xinyue Zhang, Jun Zhou, Mengyuan Guo, Shirui Cheng, Yilin Chen, Nannan Jiang, Xinling Li, Shengjie Hu, Zilei Tian, Zhengjie Li & Fang Zeng 

doi : 10.1007/s00415-022-11363-w

To comprehensively summarize and meta-analyze the concurrence across voxel-based morphometric (VBM) neuroimaging studies of migraine.

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The manifestation of affective symptoms in multiple sclerosis and discussion of the currently available diagnostic assessment tools

Melanie Filser, Axel Buchner, Gereon Rudolf Fink, Stefan M. Gold & Iris-Katharina Penner 

doi : 10.1007/s00415-022-11359-6

In addition to physical and cognitive symptoms, patients with multiple sclerosis (MS) have an increased risk of experiencing mental health problems.

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The inherited cerebellar ataxias: an update

Giulia Coarelli, Thomas Wirth, Christine Tranchant, Michel Koenig, Alexandra Durr & Mathieu Anheim 

doi : 10.1007/s00415-022-11383-6

This narrative review aims at providing an update on the management of inherited cerebellar ataxias (ICAs), describing main clinical entities, genetic analysis strategies and recent therapeutic developments.

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Endovascular thrombectomy with or without intravenous alteplase in acute stroke: a systematic review and meta-analysis of randomized clinical trials

Xuan Bai, Jianting Qiu & Yujie Wang 

doi : 10.1007/s00415-022-11413-3

This study investigated clinical outcomes after direct endovascular thrombectomy (EVT) compared to bridging therapy (EVT with prior intravenous alteplase) in acute stroke within 4.5 h after onset.

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Immunotherapy with subcutaneous immunoglobulin or plasmapheresis in patients with postural orthostatic tachycardia syndrome (POTS)

Katrina Kesterson, Jill Schofield & Svetlana Blitshteyn 

doi : 10.1007/s00415-022-11344-z

Postural orthostatic tachycardia syndrome (POTS), one of the most common autonomic disorders, is associated with significant morbidity and functional impairment. Although several possible etiologies have been proposed, autoimmunity has emerged as one of the leading causes with various specific and non-specific antibodies identified in patients with POTS. Treatment with intravenous immunoglobulin has been previously described.

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Relation between retina, cognition and brain volumes in MS: a consequence of asymptomatic optic nerve lesions

Jean-Baptiste Davion, Caroline Jougleux, Renaud Lopes, Xavier Leclerc & Olivier Outteryck 

doi : 10.1007/s00415-022-11348-9

Asymptomatic optic nerve lesions are frequent in multiple sclerosis (MS) and their impact on cognition and/or brain volume has never been taken into account.

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Nociceptive pain in adult patients with 5q-spinal muscular atrophy type 3: a cross-sectional clinical study

Elena Sagerer, Corinna Wirner, Benedikt Schoser & Stephan Wenninger 

doi : 10.1007/s00415-022-11351-0

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by mutations in the SMN gene, leading to progressive muscular weakness, atrophy and so far neglected musculoskeletal pain.

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Reliability and acceptance of dreaMS, a software application for people with multiple sclerosis: a feasibility study

Tim Woelfle, Silvan Pless, Oscar Reyes, Andrea Wiencierz, Anthony Feinstein, Pasquale Calabrese, Konstantin Gugleta, Ludwig Kappos, Johannes Lorscheider & Yvonne Naegelin 

doi : 10.1007/s00415-022-11306-5

There is an unmet need for reliable and sensitive measures for better monitoring people with multiple sclerosis (PwMS) to detect disease progression early and adapt therapeutic measures accordingly.

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Ocrelizumab effect on humoral and cellular immunity in multiple sclerosis and its clinical correlates: a 3-year observational study

Nicola Capasso, Raffaele Palladino, Vincenza Cerbone, Antonio Luca Spiezia, Bianca Covelli, Antonia Fiore, Roberta Lanzillo, Antonio Carotenuto, Maria Petracca, Lucia Stanziola, Giulia Scalia, Vincenzo Brescia Morra & Marcello Moccia 

doi : 10.1007/s00415-022-11350-1

We aim to evaluate 3-year effects of ocrelizumab (humanized anti-CD20 monoclonal antibody for the treatment of multiple sclerosis (MS)) on lymphocytes, neutrophils and immunoglobulins: (1) when compared with pre-infusion assessment; (2) over the course of treatment; and (3) possible clinical correlates of the observed immunological modifications.

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Paraneoplastic neurological syndromes associated with renal or bladder cancer: case series and PRISMA-IPD systematic review

Macarena Villagrán-García, Sergio Muñiz-Castrillo, Nicolás Lundahl Ciano-Petersen, Alberto Vogrig, Antonio Farina, Marine Villard, Dimitri Psimaras, Agusti Alentorn, David Gonçalves, Nicole Fabien, Véronique Rogemond, Bastien Joubert & Jérôme Honnorat 

doi : 10.1007/s00415-022-11356-9

The link between paraneoplastic neurological syndromes (PNS) and renal cell and bladder cancer (RCC/BC) is rare and uncertain. Our aim was to clinically evaluate, in light of the updated PNS criteria, these uncommon associations.

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Adenosine A2A receptor availability in patients with early- and moderate-stage Parkinson’s disease

Imran Waggan, Eero Rissanen, Jouni Tuisku, Juho Joutsa, Semi Helin, Riitta Parkkola, Juha O. Rinne & Laura Airas 

doi : 10.1007/s00415-022-11342-1

Adenosine 2A (A2A) receptors co-localize with dopamine D2 receptors in striatopallidal medium spiny neurons of the indirect pathway. A2A receptor activation in the striatum or pallidum decreases D2 signaling.

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Most degenerative cervical myelopathy remains undiagnosed, particularly amongst the elderly: modelling the prevalence of degenerative cervical myelopathy in the United Kingdom

Ben Grodzinski, Daniel J. Stubbs & Benjamin M. Davies 

doi : 10.1007/s00415-022-11349-8

Degenerative cervical myelopathy (DCM) is a poorly recognised form of spinal cord injury which arises when degenerative changes in the cervical spine injure the spinal cord. Timely surgical intervention is critical to preventing disability.

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Serum glial fibrillary acidic protein is sensitive to acute but not chronic tissue damage in cerebral small vessel disease

Thomas Gattringer, Christian Enzinger, Daniela Pinter, Simon Fandler-Höfler, Markus Kneihsl, Melanie Haidegger, Sebastian Eppinger, Rina Demjaha, Arabella Buchmann, Andrea Jerkovic, Reinhold Schmidt & Michael Khalil 

doi : 10.1007/s00415-022-11358-7

Serum glial fibrillary acidic protein (sGFAP) has been proposed as a biomarker in various neurological diseases but has not yet been systematically investigated in patients with cerebral small vessel disease (CSVD).

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Muscle quantitative MRI as a novel biomarker in hereditary transthyretin amyloidosis with polyneuropathy: a cross-sectional study

Elisa Vegezzi, Andrea Cortese, Niels Bergsland, Roberta Mussinelli, Matteo Paoletti, Francesca Solazzo, Riccardo Currò, Lucia Ascagni, Ilaria Callegari, Ilaria Quartesan, Alessandro Lozza, Xeni Deligianni, Francesco Santini, Enrico Marchioni, Giuseppe Cosentino, Enrico Alfonsi, Cristina Tassorelli, Stefano Bastianello, Giampaolo Merlini, Giovanni Palladini, Laura Obici & Anna Pichiecchio 

doi : 10.1007/s00415-022-11336-z

The development of reproducible and sensitive outcome measures has been challenging in hereditary transthyretin (ATTRv) amyloidosis. Recently, quantification of intramuscular fat by magnetic resonance imaging (MRI) has proven as a sensitive marker in patients with other genetic neuropathies. The aim of this study was to investigate the role of muscle quantitative MRI (qMRI) as an outcome measure in ATTRv.

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Efficacy and safety of long-term botulinum toxin treatment for acquired cervical dystonia: a 25-year follow-up

Martina Petracca, Maria Rita Lo Monaco, Tamara Ialongo, Enrico Di Stasio, Maria Luana Cerbarano, Loredana Maggi, Alessandro De Biase, Giulia Di Lazzaro, Paolo Calabresi & Anna Rita Bentivoglio 

doi : 10.1007/s00415-022-11343-0

Botulinum toxin A (BoNT/A) is the first-line treatment for idiopathic cervical dystonia (ICD) and is widely used in the clinical setting. To date, scanty data are available on the effectiveness of BoNT in treating acquired cervical dystonia (ACD).

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A real-world study of interleukin-6 receptor blockade in patients with neuromyelitis optica spectrum disorder

Shu Yang, Chen Zhang, Tian-Xiang Zhang, Bin Feng, Dongmei Jia, Shasha Han, Ting Li, Yi Shen, Guangxun Yan & Chao Zhang 

doi : 10.1007/s00415-022-11364-9

Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing autoimmune disease that can cause permanent neurological disabilities. However, the interleukin-6 (IL-6) signaling pathway is a promising therapeutic target for relapse prevention.

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Rehabilitation effects in idiopathic normal pressure hydrocephalus: a randomized controlled trial

Yasutaka Nikaido, Hideyuki Urakami, Yohei Okada, Toshihiro Akisue, Yuki Kawami, Naoya Ishida, Yoshinaga Kajimoto & Ryuichi Saura 

doi : 10.1007/s00415-022-11362-x

Gait–balance disturbances are core symptoms of idiopathic normal pressure hydrocephalus (iNPH). However, the rehabilitation effects of cerebrospinal fluid (CSF) shunting along with other treatment or no further treatment (natural course [NC]) for iNPH are unknown.

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Clinical features of Guillain–Barré syndrome and factors associated with mortality during the 2019 outbreak in Peru

Mary F. Reyes-Vega, M. Gabriela Soto-Cabezas, Anderson N. Soriano-Moreno, Andree Valle-Campos, Francisco Aquino-Peña, Noemi Flores-Jaime, Luis A. Ordóñez-Ibargüen, Kevin S. Martel & Cesar V. Munayco 

doi : 10.1007/s00415-022-11331-4

Peru has suffered an increase of Guillain Barre Syndrome incidence since 2015, being the biggest outbreak during 2019. We aimed to describe the clinical features, outcomes, and factors associated with mortality among cases reported in the 2019 outbreak.

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Etiologic reclassification of cryptogenic stroke after implantable cardiac monitoring and computed tomography angiography re-assessment

Francesco Mele, Giuseppe Scopelliti, Arianna Manini, Carola Ferrari Aggradi, Matteo Baiardo, Marco Schiavone, Maurizio Viecca, Andrea Ianniello, Pierluigi Bertora, Giovanni B. Forleo & Leonardo Pantoni 

doi : 10.1007/s00415-022-11370-x

Different mechanisms may underlie cryptogenic stroke, including subclinical atrial fibrillation (AF), nonstenotic carotid plaques (NCP), and aortic arch atherosclerosis (AAA). In a cohort of cryptogenic stroke patients, we aimed to: (1) evaluate the prevalence of subclinical AF, NCP, and AAA, and reclassify the etiology accordingly; (2) compare the clinical features of patients with reclassified etiology with those with confirmed cryptogenic stroke.

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Postural instability in Parkinson’s disease assessed with clinical “pull test� and standardized postural perturbations: effect of medication and body weight support

Chiahao Lu, Kenneth H. Louie, Amber M. Stutz, Colum D. MacKinnon & Scott E. Cooper 

doi : 10.1007/s00415-022-11375-6

This experiment tested if balance performance differed between a standardized treadmill surface perturbation task and a clinical pull test and was affected by medication or the presence of body weight support in people with Parkinson’s disease (PD).

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Anxiety and depression in Charcot-Marie-Tooth disease: data from the Italian CMT national registry

Marta Bellofatto, Alessandro Bertini, Irene Tramacere, Fiore Manganelli, Gian Maria Fabrizi, Angelo Schenone, Lucio Santoro, Tiziana Cavallaro, Marina Grandis, Stefano C. Previtali, Isabella Allegri, Luca Padua, Costanza Pazzaglia, Daniela Calabrese, Paola Saveri, Aldo Quattrone, Paola Valentino, Stefano Tozza, Luca Gentile, Massimo Russo, Anna Mazzeo, Giuseppe Vita, Sylvie Piacentini, Chiara Pisciotta & for the Italian CMT Network

doi : 10.1007/s00415-022-11365-8

There is little information about neuropsychiatric comorbidities in Charcot-Marie-Tooth disease (CMT). We assessed frequency of anxiety, depression, and general distress in CMT.

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Neuropsychological functioning in children and adolescents with anti-NMDA receptor encephalitis (anti-NMDARE)

Karine Hageboutros, Nina Hattiangadi Thomas, Melissa Hutchinson, Brenda Banwell & Katherine T. Baum 

doi : 10.1007/s00415-022-11372-9

The objective of this study was to describe neuropsychological functioning and associated medical features in pediatric patients with anti-NMDA receptor encephalitis (anti-NMDARE).

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Discontinuation of second- versus first-line disease-modifying treatment in middle-aged patients with multiple sclerosis

Maëlle Chappuis, Chloé Rousseau, Emma Bajeux, Sandrine Wiertlewski, David Laplaud, Emmanuelle Le Page, Laure Michel, Gilles Edan & Anne Kerbrat 

doi : 10.1007/s00415-022-11341-2

There has been scant research on the consequences of discontinuing second-line disease-modifying treatment (DMT) in middle-aged patients with multiple sclerosis (MS). The objective was therefore to examine the occurrence of focal inflammatory activity after the discontinuation of second versus first-line DMT in patients over 45 years.

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The association of serum immunoglobulins with cognition and dementia: the Rotterdam Study

Samer R. Khan, Amber Yaqub, M. Kamran Ikram, P. Martin van Hagen, Robin P. Peeters, Virgil A. S. H. Dalm, Layal Chaker & M. Arfan Ikram 

doi : 10.1007/s00415-022-11374-7

Chronic inflammation is involved in the pathophysiology of dementia, but the association of serum immunoglobulins with dementia has been understudied and longitudinal data are currently lacking. We investigated the association of serum immunoglobulin (Ig) A, G, and M with cognition and dementia in a population-based cohort.

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In vivo quantification of brain soma and neurite density abnormalities in multiple sclerosis

Monica Margoni, Elisabetta Pagani, Paolo Preziosa, Marco Palombo, Mor Gueye, Matteo Azzimonti, Massimo Filippi & Maria Assunta Rocca 

doi : 10.1007/s00415-022-11386-3

Soma and neurite density imaging (SANDI) is a new biophysical model that incorporates soma in addition to neurite density, thus possibly providing more specific information about the complex pathological processes of multiple sclerosis (MS).

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Multicenter data harmonization for regional brain atrophy and application in multiple sclerosis

Elisabetta Pagani, Loredana Storelli, Patrizia Pantano, Nikolaos Petsas, Gioacchino Tedeschi, Antonio Gallo, Nicola De Stefano, Marco Battaglini, Maria A. Rocca & Massimo Filippi for the INNI Network

doi : 10.1007/s00415-022-11387-2

In multiple sclerosis (MS), determination of regional brain atrophy is clinically relevant. However, analysis of large datasets is rare because of the increased variability in multicenter data.

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Sjögren syndrome and RFC1-CANVAS sensory ganglionopathy: co-occurrence or misdiagnosis?

Gorka Fernández-Eulate, Rabab Debs, Thierry Maisonobe, Philippe Latour, Fleur Cohen-Aubart, David Saadoun, Olivier Benveniste & Tanya Stojkovic 

doi : 10.1007/s00415-022-11382-7

RFC1-CANVAS and primary Sjögren syndrome (pSS) are among the most frequent causes of sensory ganglionopathy (SG) and can present simultaneously in a given patient, sharing confounding signs and symptoms.

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Freezing of gait is a risk factor for cognitive decline in Parkinson’s disease

Yi Qu, Jiangting Li, Yupeng Chen, Jingyi Li, Qixiong Qin, Danlei Wang, Jingwei Zhao, Qingmei Yang, Zhijuan Mao, Yongjie Xiong, Zhe Min & Zheng Xue 

doi : 10.1007/s00415-022-11371-w

Freezing of gait (FOG) and cognitive impairment are serious symptoms of Parkinson’s disease (PD). Understanding the association between FOG and cognition may help formulate specific interventions for PD individuals.

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Homocysteine levels, genetic background, and cognitive impairment in Parkinson’s disease

María Teresa Periñán, Daniel Macías-García, Silvia Jesús, Juan Francisco Martín-Rodríguez, Laura Muñoz-Delgado, Maria Valle Jimenez-Jaraba, Dolores Buiza-Rueda, Marta Bonilla-Toribio, Astrid Daniela Adarmes-Gómez, Pilar Gómez-Garre & Pablo Mir 

doi : 10.1007/s00415-022-11361-y

Hyperhomocysteinemia is considered an independent risk factor for cognitive impairment.

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Acute comitant strabismus in anti-GQ1b antibody syndrome

Sun-Uk Lee, Seol-Hee Baek, Hyo-Jung Kim, Jeong-Yoon Choi, Byung-Jo Kim & Ji-Soo Kim 

doi : 10.1007/s00415-022-11394-3

Ophthalmoplegia is the diagnostic hallmark of anti-GQ1b antibody syndrome. This study aimed to define the patterns of acute comitant strabismus in patients with anti-GQ1b antibody syndromes.

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CIDP-like autoimmune nodopathy complicated with focal segmental glomerulosclerosis: a case study and literature review

Shufan Zhang, Shilin Yang, Jiahong Lu, Shaojun Liu, Weicheng Wu, Mingshi Gao, Jie Lin, Xiangjun Chen, Dongqing Zhu, Shuang Ye, Chun Yu, Shufen Chen, Qiang Dong, Bing Zhu & Xiang Han 

doi : 10.1007/s00415-022-11369-4

This study aimed to investigate the role of neurofascin186 (NF186) in the pathogenesis of the concurrent focal segmental glomerulosclerosis (FSGS) in CIDP-like autoimmune nodopathy patients.

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MRI correlates of motoneuron loss in SMA

Alina Sprenger-Sva�ina, Johannes Haensch, Kilian Weiss, Nils Große Hokamp, David Maintz, Marc Schlamann, Gereon R. Fink, Natalie Schloss, Kai Laukamp, Gilbert Wunderlich, Helmar C. Lehmann & Thorsten Lichtenstein 

doi : 10.1007/s00415-022-11326-1

Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders.

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Correlation between clinical phenotype and electromyographic parameters in amyotrophic lateral sclerosis

Eleonora Colombo, Alberto Doretti, Francesco Scheveger, Alessio Maranzano, Giulia Pata, Delia Gagliardi, Megi Meneri, Stefano Messina, Federico Verde, Claudia Morelli, Stefania Corti, Luca Maderna, Vincenzo Silani & Nicola Ticozzi 

doi : 10.1007/s00415-022-11404-4

Even if electromyography (EMG) is routinely used to confirm the diagnosis of amyotrophic lateral sclerosis (ALS), few studies have analysed the correlation between electrophysiological parameters and clinical characteristics of ALS.

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Multimodal visual system analysis as a biomarker of visual hallucinations in Parkinson’s disease

Maria Diez-Cirarda, Alberto Cabrera-Zubizarreta, Ane Murueta-Goyena, Antonio P. Strafella, Rocio Del Pino, Marian Acera, Olaia Lucas-Jiménez, Naroa Ibarretxe-Bilbao, Beatriz Tijero, Juan Carlos Gómez-Esteban & Iñigo Gabilondo 

doi : 10.1007/s00415-022-11427-x

Visual hallucinations (VH) are present in up to 75% of Parkinson’s disease (PD) patients. However, their neural bases and participation of the visual system in VH are not well-understood in PD.

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Correction to: Multimodal visual system analysis as a biomarker of visual hallucinations in Parkinson’s disease

Maria Diez-Cirarda, Alberto Cabrera-Zubizarreta, Ane Murueta-Goyena, Antonio P. Strafella, Rocio Del Pino, Marian Acera, Olaia Lucas-Jiménez, Naroa Ibarretxe-Bilbao, Beatriz Tijero, Juan Carlos Gómez-Esteban & Iñigo Gabilondo 

doi : 10.1007/s00415-022-11492-2

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Serum neurofilament levels correlate with electrodiagnostic evidence of axonal loss in paclitaxel-induced peripheral neurotoxicity

R. Velasco, A. A. Argyriou, C. Marco, S. Mariotto, A. Stradella, J. Hernández, S. Pernas, S. Ferrari & J. Bruna 

doi : 10.1007/s00415-022-11377-4

Paclitaxel-induced peripheral neurotoxicity (PIPN) typically manifests as a predominantly sensory axonopathy. Nerve conduction studies (NCS) represent the gold standard method to quantify axonal impairment in PIPN.

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Neural compensation in manifest neurodegeneration: systems neuroscience evidence from social cognition in frontotemporal dementia

Jiaze Sun, François-Laurent De Winter, Fiona Kumfor, Daphne Stam, Kristof Vansteelandt, Ron Peeters, Stefan Sunaert, Rik Vandenberghe, Mathieu Vandenbulcke & Jan Van den Stock 

doi : 10.1007/s00415-022-11393-4

It has been argued that symptom onset in neurodegeneration reflects the overload of compensatory mechanisms. The present study aimed to investigate whether neural functional compensation can be observed in the manifest neurodegenerative disease stage, by focusing on a core deficit in frontotemporal dementia, i.e. social cognition, and by combining psychophysical assessment, structural MRI and functional MRI with multidimensional neural markers that allow quantification of neural computations.

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Hemihypomimia in Parkinson’s disease: an under-recognized clinical sign?

Juan Diego Guerra-Hiraldo, Alejandro López-Jiménez, Carmen Gasca-Salas, Teresa Maycas-Cepeda, Petra Gómez-Sanchez, Lydia López-Manzanares, Marina Mata Guerra-Hiraldo, Cristina Prieto-Jurczynska, Miriam Eimil, Lydia Vela-Desojo, Isabel Pareés, Adolfo Jiménez-Huete & Mónica M. Kurtis 

doi : 10.1007/s00415-022-11292-8

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Pseudo-pupil sparing oculomotor nerve palsy in cavernous-carotid fistula

Jun-Pyo Hong, Sun-Uk Lee, Sungwook Yu & Ji-Soo Kim 

doi : 10.1007/s00415-022-11332-3

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Slow stepping rate in the Unterberger test in persistent postural-perceptual dizziness

G. Armenis, A. Zachou & E. Anagnostou 

doi : 10.1007/s00415-022-11352-z

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Co-occurrence of amyotrophic lateral sclerosis and Leber’s hereditary optic neuropathy: is mitochondrial dysfunction a modifier?

Giulia Amore, Veria Vacchiano, Chiara La Morgia, Maria L. Valentino, Leonardo Caporali, Claudio Fiorini, Danara Ormanbekova, Fabrizio Salvi, Anna Bartoletti-Stella, Sabina Capellari, Rocco Liguori & Valerio Carelli 

doi : 10.1007/s00415-022-11355-w

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Stroke-like lesions confined to the cerebellum in MELAS and a possible association with neuronal hyperexcitability

Taisuke Kitamura, Masahiro Shijo, Mio Yokoi, Takako Maruyama, Masato Osaki, Udai Nakamura & Shuji Arakawa 

doi : 10.1007/s00415-022-11397-0

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Aperiodic alternating nystagmus in adult-onset Alexander disease with a novel mutation

Min Hye Kim, Jin Soo Lee, Ji Man Hong, Young Bae Sohn & Seong-Joon Lee 

doi : 10.1007/s00415-022-11390-7

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Antiseizure medications (antiepileptic drugs) in adults: starting, monitoring and stopping

Heather Angus-Leppan, Michael R. Sperling & Vicente Villanueva 

doi : 10.1007/s00415-022-11378-3

Up to 10% of people living to 80 years of age have one or more seizures; and many will not require anti-seizure medication (ASMs). In 85% of patients, the diagnosis comes from the history of the index event. One-third of patients with an apparent “first seizure� have previous events, changing their diagnosis to epilepsy.

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Can novel CT-and MR-based neuroimaging biomarkers further improve the etiological diagnosis of lobar intra-cerebral hemorrhage?

Jean-Claude Baron, Ulf Jensen-Kondering, Simona Sacco, Sacha Posener, Joseph Benzakoun, Johan Pallud, Catherine Oppenheim, Pascale Varlet & Guillaume Turc 

doi : 10.1007/s00415-022-11434-y

Lobar hematomas represent around half of all supratentorial hemorrhages and have high mortality and morbidity.

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Correction to: Can novel CT-and MR-based neuroimaging biomarkers further improve the etiological diagnosis of lobar intra-cerebral hemorrhage?

Jean-Claude Baron, Ulf Jensen-Kondering, Simona Sacco, Sacha Posener, Joseph Benzakoun, Johan Pallud, Catherine Oppenheim, Pascale Varlet & Guillaume Turc 

doi : 10.1007/s00415-022-11475-3

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Advances in the genetics of stroke risk and recovery

Sabbiha Nadia Majumder, James Hrastelj & Neil P. Robertson 

doi : 10.1007/s00415-022-11525-w

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Jacob Augustus Lockhart Clarke (1817–1880)

Ibrahim Demircubuk, Esra Candar & Gulgun Sengul 

doi : 10.1007/s00415-022-11384-5

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