Annals of Neurology




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Annals of Neurology: Volume 93, Number 2, February 2023

doi : 10.1002/ana.26611

Volume 93, Issue 2

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NINDS Corner: The Helping to End Addiction Long-Term Initiative and Pain Neurology

doi : 10.1002/ana.26567

Buy The Package and View The Article Online


The AUPN: Past, Present, and Future

L. John Greenfield Jr MD, PhD

doi : 10.1002/ana.26564

Buy The Package and View The Article Online


In Memoriam: Daniel B. Drachman

Justin C. McArthur MBBS, MPH, Klaus Toyka Dr. Med, Ahmet Hoke MD, PhD

doi : 10.1002/ana.26565

Buy The Package and View The Article Online


Sports Concussion and Chronic Traumatic Encephalopathy: Finding a Path Forward

James P. Kelly MA, MD, David S. Priemer MD, Daniel P. Perl MD, Christopher M. Filley MD

doi : 10.1002/ana.26566

Sports concussion has recently assumed special importance because of the widely publicized entity of chronic traumatic encephalopathy (CTE).

Buy The Package and View The Article Online


Midazolam Prevents the Adverse Outcome of Neonatal Asphyxia

Midazolam Prevents the Adverse Outcome of Neonatal Asphyxia

doi : 10.1002/ana.26498

Birth asphyxia (BA) is the most frequent cause of neonatal death as well as central nervous system (CNS) injury. BA is often associated with neonatal seizures, which only poorly respond to anti-seizure medications and may contribute to the adverse neurodevelopmental outcome.

Buy The Package and View The Article Online


Heterozygous Seryl-tRNA Synthetase 1 Variants Cause Charcot–Marie–Tooth Disease

Jin He MD, Xiao-Xuan Liu MD, PhD, Ming-Ming Ma MD, Jing-Jing Lin MD, Jun Fu MD, Yi-Kun Chen MD, Guo-Rong Xu MD, Liu-Qing Xu MD, Zhi-Fei Fu PhD, Dan Xu PhD, Wen-Feng Chen PhD, Chun-Yan Cao MD, Yan Shi PhD, Yi-Heng Zeng MD, Jing Zhang PhD, Xiao-Chun Chen MD, PhD, Ru-Xu Zhang MD, Ning Wang MD, PhD, Marina Kennerson PhD, Dong-Sheng Fan MD, PhD, Wan-Jin Chen MD, PhD

doi : 10.1002/ana.26501

Despite the increasing number of genes associated with Charcot–Marie-Tooth (CMT) disease, many patients currently still lack appropriate genetic diagnosis for this disease. Autosomal dominant mutations in aminoacyl-tRNA synthetases (ARSs) have been implicated in CMT. Here, we describe causal missense mutations in the gene encoding seryl-tRNA synthetase 1 (SerRS) for 3 families affected with CMT.

Buy The Package and View The Article Online


Progressive Multifocal Leukoencephalopathy Treated by Immune Checkpoint Inhibitors

Xavier Boumaza MD, Baptiste Bonneau MSc, Damien Roos-Weil PhD, Carmela Pinnetti MD, Sebastian Rauer PhD, Louisa Nitsch MD, Arnaud Del Bello PhD, Ilijas Jelcic MD, Kurt-Wolfram Sühs MD, Jacques Gasnault MD, Yasemin Goreci MD, Oliver Grauer PhD, Sharmilee Gnanapavan MD, Rebecca Wicklein MD, Nicolas Lambert MD, Thomas Perpoint MD, Martijn Beudel PhD, David Clifford MD, Agnès Sommet PhD, Irene Cortese MD, Guillaume Martin-Blondel PhD, for the Immunotherapy for PML Study Group

doi : 10.1002/ana.26512

Our aim was to assess the real-world effectiveness of immune checkpoint inhibitors for treatment of patients with progressive multifocal leukoencephalopathy (PML).

Buy The Package and View The Article Online


MOG and AQP4 Antibodies among Children with Multiple Sclerosis and Controls

Cristina M. Gaudioso MD, Soe Mar MD, T. Charles Casper PhD, Rachel Codden MPH, Adam Nguyen MPH, Gregory Aaen MD, Leslie Benson MD, Tanuja Chitnis MD, Carla Francisco MD, Mark P. Gorman MD, Manu S. Goyal MD, Jennifer Graves MD, Benjamin M Greenberg MD, Janace Hart BA, Lauren Krupp MD, Timothy Lotze MD, Sona Narula MD, Sean J. Pittock MD, Mary Rensel MD, Moses Rodriguez MD, John Rose MD, Teri Schreiner MD, Jan-Mendelt Tillema MD, Amy Waldman MD, Bianca Weinstock-Guttman MD, Yolanda Wheeler PhD, Emmanuelle Waubant MD, Eoin P. Flanagan MBBCh, United States Network of Pediatric Multiple Sclerosis Centers

doi : 10.1002/ana.26502

The purpose of this study was to determine the frequency of myelin oligodendrocyte glycoprotein (MOG)-IgG and aquaporin-4 (AQP4)-IgG among patients with pediatric-onset multiple sclerosis (POMS) and healthy controls, to determine whether seropositive cases fulfilled their respective diagnostic criteria, to compare characteristics and outcomes in children with POMS versus MOG-IgG-associated disease (MOGAD), and identify clinical features associated with final diagnosis.

Buy The Package and View The Article Online


Phase 1 Evaluation of Elezanumab (Anti–Repulsive Guidance Molecule A Monoclonal Antibody) in Healthy and Multiple Sclerosis Participants

Hari V. Kalluri PharmD, PhD, Matthew R. Rosebraugh PhD, Thomas P. Misko PhD, Adam Ziemann MD, PhD, Wei Liu PhD, Bruce A. C. Cree MD, PhD, MAS

doi : 10.1002/ana.26503

This study was undertaken to describe the safety, tolerability, pharmacokinetics, and immunogenicity of elezanumab (ABT-555), a fully human monoclonal antibody (mAb) directed against repulsive guidance molecule A (RGMa), in healthy and multiple sclerosis (MS) study participants.

Buy The Package and View The Article Online


Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Cristina Valencia-Sanchez MD, PhD, Yong Guo MD, PhD, Karl N. Krecke MD, John J. Chen MD, PhD, Vyanka Redenbaugh MD, Mayra Montalvo MD, Paul M. Elsbernd MD, Jan-Mendelt Tillema MD, Sebastian Lopez-Chiriboga MD, Adrian Budhram MD, Elia Sechi MD, Amy Kunchok MD, Divyanshu Dubey MD, Sean J. Pittock MD, Claudia F. Lucchinetti MD, Eoin P. Flanagan MD

doi : 10.1002/ana.26549

Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort).

Buy The Package and View The Article Online


Systemic Metabolic Alteration Dependent on the Thyroid-Liver Axis in Early PD

Kengo Miyamoto MS, Shinji Saiki MD, PhD, Hirotaka Matsumoto PhD, Ayami Suzuki BS, Yuri Yamashita MD, PhD, Tatou Iseki MD, Shin-Ichi Ueno MD, PhD, Kenta Shiina MD, Tetsushi Kataura PhD, Koji Kamagata MD, PhD, Yoko Imamichi BS, Yukiko Sasazawa PhD, Motoki Fujimaki MD, PhD, Wado Akamatsu MD, PhD, Nobutaka Hattori MD, PhD

doi : 10.1002/ana.26510

Parkinson's disease (PD) is a common neurodegenerative disease characterized by initial involvement of the olfactory bulb/amygdala or autonomic nerves followed by nigral degeneration.

Buy The Package and View The Article Online


Actigraphy Enables Home Screening of Rapid Eye Movement Behavior Disorder in Parkinson's Disease

Flavio Raschellà PhD, Stefano Scafa MSc, Alessandro Puiatti MSc, Eduardo Martin Moraud PhD, Pietro-Luca Ratti MD, PhD

doi : 10.1002/ana.26517

Rapid eye movement sleep behavior disorder (RBD) is a potentially harmful, often overlooked sleep disorder affecting up to 70% of Parkinson's disease patients. Current diagnosis relies on nocturnal video-polysomnography, which is an expensive and cumbersome examination requiring specific clinical expertise. Here, we explored the use of wrist actigraphy to enable automatic RBD diagnoses in home settings.

Buy The Package and View The Article Online


Recessive NUP54 Variants Underlie Early-Onset Dystonia with Striatal Lesions

Philip Harrer MD, Audrey Schalk MD, Masaru Shimura MD, PhD, Sarah Baer MD, Nadège Calmels PharmD, PhD, Marie Aude Spitz MD, Marie-Thérèse Abi Warde MD, Elise Schaefer MD, PhD, Volker M.Sc Kittke MSc, Yasemin Dincer MSc, Matias Wagner MD, Ivana Dzinovic MSc, Riccardo Berutti PhD, Tatsuharu Sato MD, Toshihiko Shirakawa MD, PhD, Yasushi Okazaki MD, PhD, Kei Murayama MD, PhD, Konrad Oexle MD, Holger Prokisch PhD, Volker Mall MD, Ivo MelÄ�ák PhD, Juliane Winkelmann MD, Michael Zech MD

doi : 10.1002/ana.26544

Infantile striatonigral degeneration is caused by a homozygous variant of the nuclear-pore complex (NPC) gene NUP62, involved in nucleo-cytoplasmic trafficking. By querying sequencing-datasets of patients with dystonia and/or Leigh(-like) syndromes, we identified 3 unrelated individuals with biallelic variants in NUP54.

Buy The Package and View The Article Online


Neuroprotection of Low-Frequency Repetitive Transcranial Magnetic Stimulation after Ischemic Stroke in Rats

Cathrin M. Buetefisch MD, PhD, Ling Wei MD, Xiaohuan Gu BS, Charles M. Epstein MD, Shan P. Yu MD, PhD

doi : 10.1002/ana.26509

Stroke is a leading cause of human death and disability. Effective early treatments with reasonable therapeutic windows remain critically important to improve the outcomes of stroke.

Buy The Package and View The Article Online


Outcomes in the Year After First-Ever Ischemic Stroke in a Bi-Ethnic Population

Lynda D. Lisabeth PhD, Devin L. Brown MD, Liming Dong PhD, Darin B. Zahuranec MD, Madeline Kwicklis MS, Xu Shi PhD, Erin Case BA, Melinda A. Smith DrPh, Morgan Campbell MD, Joseph F. Carrera MD, Lewis B. Morgenstern MD

doi : 10.1002/ana.26513

To investigate stroke outcomes at 3, 6, and 12 months post-stroke overall and by ethnicity in a population-based, longitudinal study.

Buy The Package and View The Article Online


Association of Cortical Superficial Siderosis with Post-Stroke Epilepsy

Tomotaka Tanaka MD, Kazuki Fukuma MD, PhD, Soichiro Abe MD, Soichiro Matsubara MD, PhD, Shuhei Ikeda MD, Naruhiko Kamogawa MD, Hiroyuki Ishiyama MD, Satoshi Hosoki MD, PhD, Katsuya Kobayashi MD, PhD, Akihiro Shimotake MD, PhD, Yuriko Nakaoku MD, PhD, Soshiro Ogata PhD, Kunihiro Nishimura MD, PhD, Masatoshi Koga MD, PhD, Kazunori Toyoda MD, PhD, Riki Matsumoto MD, PhD, Ryosuke Takahashi MD, PhD, Akio Ikeda MD, PhD, Masafumi Ihara MD, PhD

doi : 10.1002/ana.26497

To assess whether post-stroke epilepsy (PSE) is associated with neuroimaging findings of hemosiderin in a case–control study, and whether the addition of hemosiderin markers improves the risk stratification models of PSE.

Buy The Package and View The Article Online


Regional Precuneus Cortical Hyperexcitability in Alzheimer's Disease Patients

Elias P. Casula PhD, Ilaria Borghi MSc, Michele Maiella MSc, Maria C. Pellicciari PhD, Sonia Bonnì PhD, Lucia Mencarelli PhD, Martina Assogna MD, Alessia D'Acunto BSc, Francesco Di Lorenzo MD, Danny A. Spampinato PhD, Emiliano Santarnecchi PhD, Alessandro Martorana MD, PhD, Giacomo Koch MD, PhD

doi : 10.1002/ana.26514

Neuronal excitation/inhibition (E/I) imbalance is a potential cause of neuronal network malfunctioning in Alzheimer's disease (AD), contributing to cognitive dysfunction.

Buy The Package and View The Article Online


Allergic Diseases and Risk of Incident Dementia and Alzheimer's Disease

Hee-Kyung Joh MD, MPH, PhD, Hyuktae Kwon MD, MPH, PhD, Ki Young Son MD, MPH, PhD, Jae Moon Yun MD, MPH, Su Hwan Cho MD, Kyungdo Han PhD, Jin-Ho Park MD, MPH, PhD, Belong Cho MD, PhD

doi : 10.1002/ana.26506

To examine the associations between the allergic triad (asthma, allergic rhinitis, atopic dermatitis) and risk of dementia.

Buy The Package and View The Article Online


Staufen Impairs Autophagy in Neurodegeneration

Sharan Paul PhD, Warunee Dansithong PhD, Mandi Gandelman PhD, Karla P. Figueroa MS, Tao Zu PhD, Laura P. W. Ranum PhD, Daniel R. Scoles PhD, Stefan M. Pulst MD Dr med

doi : 10.1002/ana.26515

The mechanistic target of rapamycin (mTOR) kinase is one of the master coordinators of cellular stress responses, regulating metabolism, autophagy, and apoptosis. We recently reported that staufen1 (STAU1), a stress granule (SG) protein, was overabundant in fibroblast cell lines from patients with spinocerebellar ataxia type 2 (SCA2), amyotrophic lateral sclerosis, frontotemporal degeneration, Huntington's, Alzheimer's, and Parkinson's diseases as well as animal models, and patient tissues.

Buy The Package and View The Article Online


Preferential Perfusion to the Posterior Circulation in Hypoxia

Arsany Hakim MD, Franca Wagner MD

doi : 10.1002/ana.26539

Buy The Package and View The Article Online


Recording of Ictal Epileptic Activity Using on-Scalp Magnetoencephalography

Odile Feys MD, Pierre Corvilain PhD, Audrey Van Hecke MD, Claudine Sculier MD, Estelle Rikir MD, PhD, Benjamin Legros MD, Nicolas Gaspard MD, PhD, Gil Leurquin-Sterk MD, PhD, Niall Holmes PhD, Matthew Brookes PhD, Serge Goldman MD, PhD, Vincent Wens PhD, Xavier De Tiège MD, PhD

doi : 10.1002/ana.26562

Buy The Package and View The Article Online


Classifying Inherited Small Vessel Disease-Related Intracerebral Hemorrhage

Chih-Hao Chen MD, PhD, Sung-Chun Tang MD, PhD

doi : 10.1002/ana.26594

Buy The Package and View The Article Online


Response to Letter by Drs Chen and Tang, Titled “Classifying Inherited Small Vessel Disease-Related Intracerebral Hemorrhage�

Nicolas Raposo MD, PhD, David J. Werring MD, PhD, Anand Viswanathan MD, PhD

doi : 10.1002/ana.26592

Buy The Package and View The Article Online


Independent Confirmation

Christian Herweh MD, Simon Nagel MD

doi : 10.1002/ana.26578

Buy The Package and View The Article Online


Reply to “Independent Confirmation�

Grant Mair MD, Phil White MD, Philip Bath DSc, Keith W. Muir MD, Francesca M. Chappell PhD, Joanna M. Wardlaw MD, on behalf of the RITeS Collaborative

doi : 10.1002/ana.26570

Buy The Package and View The Article Online


Annals of Neurology: Volume 93, Number 2, February 2023

doi : 10.1002/ana.26611

Volume 93, Issue 2

Buy The Package and View The Article Online



NINDS Corner: The Helping to End Addiction Long-Term Initiative and Pain Neurology

doi : 10.1002/ana.26567

Buy The Package and View The Article Online


The AUPN: Past, Present, and Future

L. John Greenfield Jr MD, PhD

doi : 10.1002/ana.26564

Buy The Package and View The Article Online


In Memoriam: Daniel B. Drachman

Justin C. McArthur MBBS, MPH, Klaus Toyka Dr. Med, Ahmet Hoke MD, PhD

doi : 10.1002/ana.26565

Buy The Package and View The Article Online


Sports Concussion and Chronic Traumatic Encephalopathy: Finding a Path Forward

James P. Kelly MA, MD, David S. Priemer MD, Daniel P. Perl MD, Christopher M. Filley MD

doi : 10.1002/ana.26566

Sports concussion has recently assumed special importance because of the widely publicized entity of chronic traumatic encephalopathy (CTE).

Buy The Package and View The Article Online


Midazolam Prevents the Adverse Outcome of Neonatal Asphyxia

Midazolam Prevents the Adverse Outcome of Neonatal Asphyxia

doi : 10.1002/ana.26498

Birth asphyxia (BA) is the most frequent cause of neonatal death as well as central nervous system (CNS) injury. BA is often associated with neonatal seizures, which only poorly respond to anti-seizure medications and may contribute to the adverse neurodevelopmental outcome.

Buy The Package and View The Article Online


Heterozygous Seryl-tRNA Synthetase 1 Variants Cause Charcot–Marie–Tooth Disease

Jin He MD, Xiao-Xuan Liu MD, PhD, Ming-Ming Ma MD, Jing-Jing Lin MD, Jun Fu MD, Yi-Kun Chen MD, Guo-Rong Xu MD, Liu-Qing Xu MD, Zhi-Fei Fu PhD, Dan Xu PhD, Wen-Feng Chen PhD, Chun-Yan Cao MD, Yan Shi PhD, Yi-Heng Zeng MD, Jing Zhang PhD, Xiao-Chun Chen MD, PhD, Ru-Xu Zhang MD, Ning Wang MD, PhD, Marina Kennerson PhD, Dong-Sheng Fan MD, PhD, Wan-Jin Chen MD, PhD

doi : 10.1002/ana.26501

Despite the increasing number of genes associated with Charcot–Marie-Tooth (CMT) disease, many patients currently still lack appropriate genetic diagnosis for this disease. Autosomal dominant mutations in aminoacyl-tRNA synthetases (ARSs) have been implicated in CMT. Here, we describe causal missense mutations in the gene encoding seryl-tRNA synthetase 1 (SerRS) for 3 families affected with CMT.

Buy The Package and View The Article Online


Progressive Multifocal Leukoencephalopathy Treated by Immune Checkpoint Inhibitors

Xavier Boumaza MD, Baptiste Bonneau MSc, Damien Roos-Weil PhD, Carmela Pinnetti MD, Sebastian Rauer PhD, Louisa Nitsch MD, Arnaud Del Bello PhD, Ilijas Jelcic MD, Kurt-Wolfram Sühs MD, Jacques Gasnault MD, Yasemin Goreci MD, Oliver Grauer PhD, Sharmilee Gnanapavan MD, Rebecca Wicklein MD, Nicolas Lambert MD, Thomas Perpoint MD, Martijn Beudel PhD, David Clifford MD, Agnès Sommet PhD, Irene Cortese MD, Guillaume Martin-Blondel PhD, for the Immunotherapy for PML Study Group

doi : 10.1002/ana.26512

Our aim was to assess the real-world effectiveness of immune checkpoint inhibitors for treatment of patients with progressive multifocal leukoencephalopathy (PML).

Buy The Package and View The Article Online


MOG and AQP4 Antibodies among Children with Multiple Sclerosis and Controls

Cristina M. Gaudioso MD, Soe Mar MD, T. Charles Casper PhD, Rachel Codden MPH, Adam Nguyen MPH, Gregory Aaen MD, Leslie Benson MD, Tanuja Chitnis MD, Carla Francisco MD, Mark P. Gorman MD, Manu S. Goyal MD, Jennifer Graves MD, Benjamin M Greenberg MD, Janace Hart BA, Lauren Krupp MD, Timothy Lotze MD, Sona Narula MD, Sean J. Pittock MD, Mary Rensel MD, Moses Rodriguez MD, John Rose MD, Teri Schreiner MD, Jan-Mendelt Tillema MD, Amy Waldman MD, Bianca Weinstock-Guttman MD, Yolanda Wheeler PhD, Emmanuelle Waubant MD, Eoin P. Flanagan MBBCh, United States Network of Pediatric Multiple Sclerosis Centers

doi : 10.1002/ana.26502

The purpose of this study was to determine the frequency of myelin oligodendrocyte glycoprotein (MOG)-IgG and aquaporin-4 (AQP4)-IgG among patients with pediatric-onset multiple sclerosis (POMS) and healthy controls, to determine whether seropositive cases fulfilled their respective diagnostic criteria, to compare characteristics and outcomes in children with POMS versus MOG-IgG-associated disease (MOGAD), and identify clinical features associated with final diagnosis.

Buy The Package and View The Article Online


Phase 1 Evaluation of Elezanumab (Anti–Repulsive Guidance Molecule A Monoclonal Antibody) in Healthy and Multiple Sclerosis Participants

Hari V. Kalluri PharmD, PhD, Matthew R. Rosebraugh PhD, Thomas P. Misko PhD, Adam Ziemann MD, PhD, Wei Liu PhD, Bruce A. C. Cree MD, PhD, MAS

doi : 10.1002/ana.26503

This study was undertaken to describe the safety, tolerability, pharmacokinetics, and immunogenicity of elezanumab (ABT-555), a fully human monoclonal antibody (mAb) directed against repulsive guidance molecule A (RGMa), in healthy and multiple sclerosis (MS) study participants.

Buy The Package and View The Article Online


Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Cristina Valencia-Sanchez MD, PhD, Yong Guo MD, PhD, Karl N. Krecke MD, John J. Chen MD, PhD, Vyanka Redenbaugh MD, Mayra Montalvo MD, Paul M. Elsbernd MD, Jan-Mendelt Tillema MD, Sebastian Lopez-Chiriboga MD, Adrian Budhram MD, Elia Sechi MD, Amy Kunchok MD, Divyanshu Dubey MD, Sean J. Pittock MD, Claudia F. Lucchinetti MD, Eoin P. Flanagan MD

doi : 10.1002/ana.26549

Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort).

Buy The Package and View The Article Online


Systemic Metabolic Alteration Dependent on the Thyroid-Liver Axis in Early PD

Kengo Miyamoto MS, Shinji Saiki MD, PhD, Hirotaka Matsumoto PhD, Ayami Suzuki BS, Yuri Yamashita MD, PhD, Tatou Iseki MD, Shin-Ichi Ueno MD, PhD, Kenta Shiina MD, Tetsushi Kataura PhD, Koji Kamagata MD, PhD, Yoko Imamichi BS, Yukiko Sasazawa PhD, Motoki Fujimaki MD, PhD, Wado Akamatsu MD, PhD, Nobutaka Hattori MD, PhD

doi : 10.1002/ana.26510

Parkinson's disease (PD) is a common neurodegenerative disease characterized by initial involvement of the olfactory bulb/amygdala or autonomic nerves followed by nigral degeneration.

Buy The Package and View The Article Online


Actigraphy Enables Home Screening of Rapid Eye Movement Behavior Disorder in Parkinson's Disease

Flavio Raschellà PhD, Stefano Scafa MSc, Alessandro Puiatti MSc, Eduardo Martin Moraud PhD, Pietro-Luca Ratti MD, PhD

doi : 10.1002/ana.26517

Rapid eye movement sleep behavior disorder (RBD) is a potentially harmful, often overlooked sleep disorder affecting up to 70% of Parkinson's disease patients. Current diagnosis relies on nocturnal video-polysomnography, which is an expensive and cumbersome examination requiring specific clinical expertise. Here, we explored the use of wrist actigraphy to enable automatic RBD diagnoses in home settings.

Buy The Package and View The Article Online


Recessive NUP54 Variants Underlie Early-Onset Dystonia with Striatal Lesions

Philip Harrer MD, Audrey Schalk MD, Masaru Shimura MD, PhD, Sarah Baer MD, Nadège Calmels PharmD, PhD, Marie Aude Spitz MD, Marie-Thérèse Abi Warde MD, Elise Schaefer MD, PhD, Volker M.Sc Kittke MSc, Yasemin Dincer MSc, Matias Wagner MD, Ivana Dzinovic MSc, Riccardo Berutti PhD, Tatsuharu Sato MD, Toshihiko Shirakawa MD, PhD, Yasushi Okazaki MD, PhD, Kei Murayama MD, PhD, Konrad Oexle MD, Holger Prokisch PhD, Volker Mall MD, Ivo MelÄ�ák PhD, Juliane Winkelmann MD, Michael Zech MD

doi : 10.1002/ana.26544

Infantile striatonigral degeneration is caused by a homozygous variant of the nuclear-pore complex (NPC) gene NUP62, involved in nucleo-cytoplasmic trafficking. By querying sequencing-datasets of patients with dystonia and/or Leigh(-like) syndromes, we identified 3 unrelated individuals with biallelic variants in NUP54.

Buy The Package and View The Article Online


Neuroprotection of Low-Frequency Repetitive Transcranial Magnetic Stimulation after Ischemic Stroke in Rats

Cathrin M. Buetefisch MD, PhD, Ling Wei MD, Xiaohuan Gu BS, Charles M. Epstein MD, Shan P. Yu MD, PhD

doi : 10.1002/ana.26509

Stroke is a leading cause of human death and disability. Effective early treatments with reasonable therapeutic windows remain critically important to improve the outcomes of stroke.

Buy The Package and View The Article Online


Outcomes in the Year After First-Ever Ischemic Stroke in a Bi-Ethnic Population

Lynda D. Lisabeth PhD, Devin L. Brown MD, Liming Dong PhD, Darin B. Zahuranec MD, Madeline Kwicklis MS, Xu Shi PhD, Erin Case BA, Melinda A. Smith DrPh, Morgan Campbell MD, Joseph F. Carrera MD, Lewis B. Morgenstern MD

doi : 10.1002/ana.26513

To investigate stroke outcomes at 3, 6, and 12 months post-stroke overall and by ethnicity in a population-based, longitudinal study.

Buy The Package and View The Article Online


Association of Cortical Superficial Siderosis with Post-Stroke Epilepsy

Tomotaka Tanaka MD, Kazuki Fukuma MD, PhD, Soichiro Abe MD, Soichiro Matsubara MD, PhD, Shuhei Ikeda MD, Naruhiko Kamogawa MD, Hiroyuki Ishiyama MD, Satoshi Hosoki MD, PhD, Katsuya Kobayashi MD, PhD, Akihiro Shimotake MD, PhD, Yuriko Nakaoku MD, PhD, Soshiro Ogata PhD, Kunihiro Nishimura MD, PhD, Masatoshi Koga MD, PhD, Kazunori Toyoda MD, PhD, Riki Matsumoto MD, PhD, Ryosuke Takahashi MD, PhD, Akio Ikeda MD, PhD, Masafumi Ihara MD, PhD

doi : 10.1002/ana.26497

To assess whether post-stroke epilepsy (PSE) is associated with neuroimaging findings of hemosiderin in a case–control study, and whether the addition of hemosiderin markers improves the risk stratification models of PSE.

Buy The Package and View The Article Online


Regional Precuneus Cortical Hyperexcitability in Alzheimer's Disease Patients

Elias P. Casula PhD, Ilaria Borghi MSc, Michele Maiella MSc, Maria C. Pellicciari PhD, Sonia Bonnì PhD, Lucia Mencarelli PhD, Martina Assogna MD, Alessia D'Acunto BSc, Francesco Di Lorenzo MD, Danny A. Spampinato PhD, Emiliano Santarnecchi PhD, Alessandro Martorana MD, PhD, Giacomo Koch MD, PhD

doi : 10.1002/ana.26514

Neuronal excitation/inhibition (E/I) imbalance is a potential cause of neuronal network malfunctioning in Alzheimer's disease (AD), contributing to cognitive dysfunction.

Buy The Package and View The Article Online


Allergic Diseases and Risk of Incident Dementia and Alzheimer's Disease

Hee-Kyung Joh MD, MPH, PhD, Hyuktae Kwon MD, MPH, PhD, Ki Young Son MD, MPH, PhD, Jae Moon Yun MD, MPH, Su Hwan Cho MD, Kyungdo Han PhD, Jin-Ho Park MD, MPH, PhD, Belong Cho MD, PhD

doi : 10.1002/ana.26506

To examine the associations between the allergic triad (asthma, allergic rhinitis, atopic dermatitis) and risk of dementia.

Buy The Package and View The Article Online


Staufen Impairs Autophagy in Neurodegeneration

Sharan Paul PhD, Warunee Dansithong PhD, Mandi Gandelman PhD, Karla P. Figueroa MS, Tao Zu PhD, Laura P. W. Ranum PhD, Daniel R. Scoles PhD, Stefan M. Pulst MD Dr med

doi : 10.1002/ana.26515

The mechanistic target of rapamycin (mTOR) kinase is one of the master coordinators of cellular stress responses, regulating metabolism, autophagy, and apoptosis. We recently reported that staufen1 (STAU1), a stress granule (SG) protein, was overabundant in fibroblast cell lines from patients with spinocerebellar ataxia type 2 (SCA2), amyotrophic lateral sclerosis, frontotemporal degeneration, Huntington's, Alzheimer's, and Parkinson's diseases as well as animal models, and patient tissues.

Buy The Package and View The Article Online


Preferential Perfusion to the Posterior Circulation in Hypoxia

Arsany Hakim MD, Franca Wagner MD

doi : 10.1002/ana.26539

Buy The Package and View The Article Online


Recording of Ictal Epileptic Activity Using on-Scalp Magnetoencephalography

Odile Feys MD, Pierre Corvilain PhD, Audrey Van Hecke MD, Claudine Sculier MD, Estelle Rikir MD, PhD, Benjamin Legros MD, Nicolas Gaspard MD, PhD, Gil Leurquin-Sterk MD, PhD, Niall Holmes PhD, Matthew Brookes PhD, Serge Goldman MD, PhD, Vincent Wens PhD, Xavier De Tiège MD, PhD

doi : 10.1002/ana.26562

Buy The Package and View The Article Online


Classifying Inherited Small Vessel Disease-Related Intracerebral Hemorrhage

Chih-Hao Chen MD, PhD, Sung-Chun Tang MD, PhD

doi : 10.1002/ana.26594

Buy The Package and View The Article Online


Response to Letter by Drs Chen and Tang, Titled “Classifying Inherited Small Vessel Disease-Related Intracerebral Hemorrhage�

Nicolas Raposo MD, PhD, David J. Werring MD, PhD, Anand Viswanathan MD, PhD

doi : 10.1002/ana.26592

Buy The Package and View The Article Online


Independent Confirmation

Christian Herweh MD, Simon Nagel MD

doi : 10.1002/ana.26578

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Reply to “Independent Confirmation�

Grant Mair MD, Phil White MD, Philip Bath DSc, Keith W. Muir MD, Francesca M. Chappell PhD, Joanna M. Wardlaw MD, on behalf of the RITeS Collaborative

doi : 10.1002/ana.26570

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