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The Prevalence and Clinical Phenotype of Dual Positive Neuromyelitis Optica Spectrum Disorders (NMOSD) at a National Reference Centre.
Lalwani, Chirag; Faisal, Fida; Yadav, Anjali; Kannoth, Sudheeran; Nambiar, Vivek; Gopinath, Sibi; Kumar, Anand; Saraf, Udit; Vincent, Jino; Anoop, Sruthi; Mathai, Annamma; Panicker, Suprabha
doi : 10.1212/01.wnl.0000903752.47975.59
To assess the prevalence of dual positive NMOSD and outline its clinical phenotype. Background: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune syndrome that is frequently positive for Aquaporin 4 (AQP4) IgG or Myelin Oligodendrocyte Glycoproteins (MOG) IgG. However, dual positivity to both is rare.
Upregulated Complement Receptors Correlate With Fc Gamma Receptor 3A-Positive Natural Killer Cells (NK) and Natural Killer-T Cells (NKT) in Neuromyelitis Optica Spectrum Disorder.
Nishiyama, Shuhei; Wright, Amy; Lotan, Itay; Paul, Friedemann; Levy, Michael
doi : 10.1212/01.wnl.0000903056.59789.7a
To clarify if NK and NKT cells are activated via complement in NMOSD. Background: Inhibition of terminal complement in NMOSD using eculizumab has been shown to be helpful in preventing relapses but exactly how the drug is working is not clear.
FDA Approved Treatments for Neuromyelitis Optica Spectrum Disorder in Clinical Practice: A Survey of Academic Neuroimmunologists.
Thon, Jesse; Sharkus, Robert; Thakkar, Richa; Hunter, Krystal; Siegler, James; Thon, Olga
doi : 10.1212/01.wnl.0000903060.69682.6b
To assess utilization and barriers to use of three recently FDA-approved treatments for neuromyelitis optica spectrum disorder (NMOSD) by academic neuroimmunologists.
Safety and Effectiveness of Eculizumab in Japanese Patients With Aquaporin-4 Antibody-Positive Neuromyelitis Optica Spectrum Disorder: Interim Analysis of a Post-marketing Surveillance Study.
Nakashima, Ichiro; Hoshino, Yo; Okamura, Kazumi; Kikui, Hidekazu; Fujihara, Kazuo; Edwards, Yuriy
doi : 10.1212/01.wnl.0000903064.51564.88
NA. Background: The terminal complement C5 inhibitor eculizumab is approved in Japan for the prevention of aquaporin-4 antibody-positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD) relapse and undergoing mandatory post-marketing surveillance (PMS) of real-world use.
Long-Term Eculizumab in AQP4+ NMOSD: Relapse-Risk Reduction and Safety in PREVENT and its Completed Open-Label Extension.
Berthele, Achim; Wingerchuk, Dean; Fujihara, Kazuo; Palace, Jacqueline; Levy, Michael; Kim, Ho Jin; Nakashima, Ichiro; Oreja-Guevara, Celia; Wang, Kai-Chen; Shang, Shulian; Yountz, Marcus; Pittock, Sean
doi : 10.1212/01.wnl.0000903068.75712.b8
NA. Background: Eculizumab is well tolerated and significantly reduces relapse risk versus placebo in patients with aquaporin-4 immunoglobulin G-positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD).
A Phase 3 Efficacy and Safety Study of Ravulizumab in Adult Patients With Neuromyelitis Optica Spectrum Disorder: Study Design and Methodology.
Pittock, Sean; Allen, Kerstin; Mashhoon, Yasmin; Yountz, Marcus
doi : 10.1212/01.wnl.0000903072.14566.c2
To present the design and rationale for the phase 3 trial ALXN-1210-NMO-307 (NCT04201262). Background: Eculizumab is a complement component 5 (C5) inhibitor approved for adults with anti-aquaporin-4 antibody-positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD).
Disease Characteristics of Seropositive Neuromyelitis Optica Spectrum Disorder in a Turkish Cohort.
Cam, Samet; Gulec, Bade; Tutuncu, Melih; Saip, Sabahattin; Siva, Aksel; Uygunoglu, Ugur
doi : 10.1212/01.wnl.0000903076.79930.c1
To determine the clinical, demographic and imaging characteristics of a Turkish cohort with aquaporin-4-antibody positive (AQP4-IgG+) neuromyelitis optica spectrum disorder (NMOSD) from a single center.
Nipocalimab's Selective Targeting of FcRn and IgG Clearance Preserves Key Immune Functions.
Ling, Leona; Tyler, Steven; Beneduce, Christopher; Yu, Faye; Brown, Julia; Kumar, Sujatha; Xu, Rui; Duffner, Jay; Avery, William
doi : 10.1212/01.wnl.0000903080.38659.15
To characterize the effect of nipocalimab, a fully human, effectorless IgG1 anti-neonatal Fc receptor (FcRn) monoclonal antibody on immune function.
Validation of MATCH Score: A Predictive Tool for Identification of Patients With Kelch-Like Protein-11 Autoantibodies.
Hammami, M Bakri; Rezk, Mohamed; Dubey, Divyanshu
doi : 10.1212/01.wnl.0000903084.26865.8a
To validate performance of the MATCH score using an independent paraneoplastic neurological syndrome (PNS) cohort.
Caveolae-Associated Protein (cavin)-4 Autoantibodies in Immune Mediated Rippling Muscle Disease.
Hammami, M Bakri; Beecher, Grayson; Knight, Andrew; Liewluck, Teerin; Triplett, James; Datta, Abhigyan; Dasari, Surendra; Zhang, Youwen; Roforth, Matthew; Jerde, Calvin; Murphy, Stephen; Litchy, William; Amato, Anthony; Lennon, Vanda; McKeon, Andrew; Mills, John; Pittock, Sean; Milone, Margherita; Dubey, Divyanshu
doi : 10.1212/01.wnl.0000903088.98540.52
To describe a novel autoantibody biomarker of Immune mediated rippling muscle disease (iRMD).
Anti-Neuronal Nuclear Antibody 3 Autoimmunity Targets Dachshund Homolog 1.
Zekeridou, Anastasia; Yang, Binxia; Lennon, Vanda; Yong, Guo; Wu, Liang; Lucchinetti, Claudia; McKeon, Andrew; Pittock, Sean; Flanagan, Eoin
doi : 10.1212/01.wnl.0000903092.20137.01
To identify the autoantigen defined by Anti-Neuronal Nuclear Antibody-type 3 (ANNA3)-IgG and describe the clinical phenotype of seropositive patients.
Examining the Differences in Biomarkers of Neuronal and Glial Injury Between Autoimmune Neurologic Disease Patients and Healthy Controls.
Borko, Tyler L.; Barrera, Britney; Mizenko, Christopher; Ledreux, Aurelie; Kammeyer, Ryan; Ritchie, Alanna; Selva, Sean; Sillau, Stefan; Engebretson, Eric; Seale, Rebecca; Valdez, Brooke; Bennett, Jeffrey L.; Vollmer, Timothy L.; Nair, Kavita; Piquet, Amanda
doi : 10.1212/01.wnl.0000903096.36040.1d
To evaluate differences in concentrations of serum-based biomarkers obtained from a screened healthy control (HC) population compared to age and sex matched autoimmune and inflammatory neurologic disease (AIND) patients.
A Severe Case of Lupus Cerebritis.
Szewczyk, Christopher; Gonzalez, Hemil
doi : 10.1212/01.wnl.0000903100.95793.8a
Lupus cerebritis is a rare but potentially fatal complication of SLE. Prompt diagnosis and rapid initiation of therapy can prove lifesaving.
A Rapidly Fatal Case of Anti-GFAP Receptor Encephalitis Due to Acute Brain Edema and Herniation.
Kahila, Roua; Kaleem, Zafar
doi : 10.1212/01.wnl.0000903104.67881.57
Glial fibrillary acidic protein antibody (GFAP) is a newly recognized biomarker for an immunotherapy responsive autoimmune meningo-encephalomyelitis with a wide variety of clinical presentations.
Characterization of Retinal Nerve Fiber Layer Thickness in a Cohort with Glutamic Acid Decarboxylase 65 and Glycine Receptor Autoimmunity.
Hoshina, Yoji; Wong, Ka-Ho; Galli, Jonathan; Greenlee, John; Klein, Julia; Soldan, M. Paz; Clardy, Stacey; Fjeldstad, Anette; Rose, John; Kadish, Robert
doi : 10.1212/01.wnl.0000903108.88950.46
To describe the retinal nerve fiber layer (RNFL) with the demographic and clinical profile in patients with glutamic acid decarboxylase 65 (GAD65) and glycine receptor (GlyR) neurological autoimmunity.
Differential Diagnosis in the Management of CPI Immunotoxicity: Case Series of Etiologies not to Miss.
Gregory, Timothy; Tummala, Sudhakar
doi : 10.1212/01.wnl.0000903112.22250.16
To present on treatable conditions arising with exposure to checkpoint inhibiting immunotherapy for malignancy. Each case was diagnostically obscured by presumed immunotoxicity.
Giant Cell Arteritis of the Superior Mesenteric Artery Presenting With Wernicke Encephalopathy From Thiamine Deficiency.
Shapiro, Sarah; Renner, David; Farese, Ludovica
doi : 10.1212/01.wnl.0000903116.61112.46
N/A. Background: Giant cell arteritis (GCA) is one of the most common systemic vasculitides in adults over the age of 50 with incidence ranging from 15 to 35 per 100,000 individuals.
Stiff Person Syndrome Misdiagnosis: Clinical and Ancillary Testing Characteristics.
Chia, Nicholas; McKeon, Andrew; Flanagan, Eoin; Dubey, Divyanshu; Zalewski, Nicholas; Pittock, Sean; Zekeridou, Anastasia
doi : 10.1212/01.wnl.0000903120.29940.d6
To assess stiff person syndrome (SPS) misdiagnosis and identify factors differentiating SPS from non-SPS. Background: SPS is a heterogeneous immune-mediated central hyperexcitability disorder that is challenging to differentiate from alternative diagnoses.
Characterization of Cardiac Bradyarrhythmia Associated With LGI1-IgG Autoimmune Encephalitis.
Zhao-Fleming, Hannah; Zahid, Anza; Lu, Tong; Sun, Xiaojing; Pittock, Sean; Lee, Hon-Chi; Dubey, Divyanshu
doi : 10.1212/01.wnl.0000903124.01060.58
To evaluate and characterize cardiac arrhythmias associated with LGI1-IgG (Leucine-rich glioma inactivated 1-IgG) autoimmune encephalitis (AE).
Are Insulin Mimetics Protective Against Comorbidity in Patients With Neuro-Autoimmune Disease?.
Ahmed, Mohsen; Ahmed, Afaaq; Trivedi, Ronak; Ors, Muhammed; Ahmed, Nabeel; Jaffry, Mustafa; Souayah, Nizar
doi : 10.1212/01.wnl.0000903128.39153.dc
To investigate the neuroprotective potential of insulin mimetics (IM) in patients with neuro autoimmune disease (NAD) and high risk comorbidities.
Do Those With Neuro-Autoimmune Disease Carry a Higher Burden of Disease?.
Ahmed, Mohsen; Ahmed, Afaaq; Trivedi, Ronak; Ors, Muhammed; Ahmed, Nabeel; Souayah, Nizar
doi : 10.1212/01.wnl.0000903132.43114.6b
To investigate the burden of disease and their prognosis amongst patients with neuro-autoimmune disease (NAD). Background: NAD has been shown to increase overall mortality and early death among patients.
"Obvious" Indications for Neural Antibody Testing in Epilepsy or Seizures: The ONES Checklist.
Chang, Yiu-Chia; Nouri, Maryam; Mirsattari, Seyed; Burneo, Jorge; Budhram, Adrian
doi : 10.1212/01.wnl.0000903136.62286.52
To develop a checklist that identifies patients who have "obvious" indications for neural antibody testing, and compare its diagnostic performance to predictive scores.
Application of APE2 and RITE2 Scores in a Stanford Cohort of Autoimmune Encephalitis Patients.
Rafferty, Trevor; Koeppen-Babcock, Anna; Muppidi, Srikanth; Le, Scheherazade
doi : 10.1212/01.wnl.0000903140.61364.7c
The goal of our study was to apply the APE2 and RITE2 scores in a cohort of autoimmune encephalitis (AE) patients at Stanford with immune-mediated seizures.
Treatment Outcome of Autoimmune Associated Epilepsy (AAE) vs Acute Symptomatic Seizures in Autoimmune Encephalitis (ASSAE)-A Single Center Experience.
Zhong, Ning; Waheed, Mark
doi : 10.1212/01.wnl.0000903144.80173.4c
To assess the seizure treatment outcome in autoimmune encephalitis (AE). Background: Seizures due to AE etiology are increasingly recognized.
Spectrum of Brain MRI Features in CASPR2 Associated Autoimmune Encephalitis: A Case Report With Parietal Lobe Involvement.
Manrique-Trujillo, Luis; Franada, Tiffani
doi : 10.1212/01.wnl.0000903148.02345.a9
Contactin-associated-protein-like 2 (CASPR2)-antibody-mediated autoimmune encephalitis (AE) is characterized by diverse clinical manifestations with involvement of both central and peripheral nerve systems.
Do Anti-Epileptic Drugs Increase High Risk Comorbidity Amongst Patients With Neuro-Autoimmune Disease?.
Ahmed, Mohsen; Ahmed, Afaaq; Trivedi, Ronak; Ors, Muhammed; Ahmed, Nabeel; Mandava, Kranthi; Souayah, Nizar
doi : 10.1212/01.wnl.0000903152.06982.75
To investigate the effects of anti-epileptic drugs (AED) on the overall burden of disease in patients with neuro-autoimmune disease (NAD).
Neuroinflammatory Disease Responsive to MEK-Inhibitor.
Chen, Vicky; Yadav, Vijayshree; Soldatos, Ariane; Cho, Yoon-Jae; Nath, Avindra; Brown, Desmond; Diamond, Eli; Solit, David; Woltjer, Randall; Sayama, Christina; Winer, Jesse; Garavatti, Emily; Garrett, McKinnon; Angappan, Dhanalakshmi; Nicholson, Eugene
doi : 10.1212/01.wnl.0000903156.10274.d4
Illustrate that some neuroinflammatory diseases may respond best to antiproliferative therapies rather than immunomodulatory therapies.
Protective Association of HLA-DRB1*04 Subtypes in Neurodegenerative Diseases Implicates Acetylated Tau PHF6 Sequences.
Luo, Guo; Le Guen, Yann; Ambati, Adityasai; Yogeshwar, Selina; Peris-Sempere, Vicente; Lambert, Jean-Charles; Greicius, Michael; Mignot, Emmanuel; Collaborators, AD/PD
doi : 10.1212/01.wnl.0000903160.72715.2d
To explore genetic association between human leukocyte antigen (HLA) and neurodegenerative diseases and investigate mechanisms behind the association.
Real-World Resource Utilization and Productivity Loss Among Patients With Myasthenia Gravis in Sweden: A Nationwide Population-Based Study.
Zhang, Qiaoyi; Cai, Qian; Batyrbekova, Nurgul; Di Scala, Lilla; Kavanagh, Shane
doi : 10.1212/01.wnl.0000903164.78899.f9
To assess annual healthcare resource utilization including inpatient admission and outpatient visits, employment status, and sickness absence associated with myasthenia gravis (MG).
Anti-myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder (MOGAD) in a Pediatric Patient with Rare Presentation of a Cerebellar Tumefactive Lesion.
Sanghi, Avni; Gombolay, Grace; Khan, Tuba
doi : 10.1212/01.wnl.0000903168.54950.6a
NA. Background: Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been commonly associated with optic neuritis, myelitis, and acute disseminated encephalomyelitis but rarely with tumefactive lesions, especially in children.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) as a novel presentation of CNS autoimmunity in a pediatric patient with Wiskott-Aldrich syndrome (WAS).
Xie, Vivien; Kornbluh, Alexandra
doi : 10.1212/01.wnl.0000903172.52024.08
Report a novel case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) presenting as relapsing bilateral optic neuritis in a pediatric patient with Wiskott-Aldrich syndrome (WAS).
MOGAD in the Mountain West: Epidemiology and Outcomes in Pediatric and Adult Patients at Two Large Academic Referral Centers.
Wright, Melissa; Liu, Suzanne; Wong, Ka-Ho; Mizenko, Christopher; Kammeyer, Ryan; Schreiner, Teri; Kadish, Robert; Smith, Tammy; Galli, Jonathan; Klein, Julia; Greenlee, John; Rose, John; Soldan, M. Paz; Bennett, Jeffrey; Bonkowsky, Joshua; Peterson, Lisa; Piquet, Amanda; Clardy, Stacey
doi : 10.1212/01.wnl.0000903176.51636.cf
To describe the characteristics and outcomes in adult and pediatric patients diagnosed with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at the two major referral centers in the Mountain West of the United States, a geographic area encompassing roughly 15% of the land mass of the continental US.
A Case of Recurrent Cerebral Cortical Encephalitis in MOG Antibody-Associated Disease.
Cacciaguerra, Laura; Chen, John J.; Flanagan, Eoin P.
doi : 10.1212/01.wnl.0000903180.06286.ba
NA. Background: Cerebral cortical encephalitis is a recently recognized syndrome of myelin-oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD), yet most descriptions report single episodes without recurrence.
Development of New or Enlarging MRI Lesions Outside of Clinical Attacks in MOG-Antibody-Associated Disease.
Syc-Mazurek, Stephanie; Chen, John; Morris, Padraig; Sechi, Elia; Mandrekar, Jayawant; Tillema, Jan-Mendelt; Lopez, Alfonso; Lucchinetti, Claudia; Zalewski, Nicholas; Cacciaguerra, Laura; Buciuc, Marina; Krecke, Karl; Messina, Steven; Bhatti, M. Tariq; Pittock, Sean; Flanagan, Eoin
doi : 10.1212/01.wnl.0000903184.42775.d4
To determine the frequency of new/enlarging T2 or enhancing asymptomatic lesions in myelin-oligodendrocyte-glycoprotein-antibody-associated-disease (MOGAD) and compare to multiple sclerosis (MS) and aquaporin-4 antibody-positive-neuromyelitis-optica-spectrum-disorder (AQP4+NMOSD). Background: Data on new asymptomatic lesions in MOGAD is limited.
Recurrent Brainstem Lesions as a Manifestation of MOGAD.
Ardakani, Rumyar; Blackburn, Kyle
doi : 10.1212/01.wnl.0000903188.99072.4d
NA. Background: MOG antibody-associated disease (MOGAD) is an inflammatory and demyelinating disease of the central nervous system.
Spinal Central Canal Dilation in MOG Antibody-Associated Disease Versus Other CNS Demyelinating Disorders.
Webb, Lauren; Cacciaguerra, Laura; Chen, John; Sechi, Elia; Redenbaugh, Vyanka; Dubey, Divyanshu; Pittock, Sean; Flanagan, Eoin
doi : 10.1212/01.wnl.0000903192.58738.71
To assess the frequency of spinal cord central canal dilation on magnetic resonance imaging (MRI) in patients with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) myelitis compared to myelitis patients with aquaporin-4-positive-neuromyelitis optic spectrum disorder (AQP4+NMOSD) and multiple sclerosis (MS).
Exposure to TNF Inhibitors is Rare at MOGAD Diagnosis.
Redenbaugh, Vyanka; Flanagan, Eoin; Floris, Valentina MD; Zara, Pietro; Bhatti, M. Tariq; Moreno, Francisco Sanchez; Koster, Matthew; Mariotto, Sara; Pittock, Sean; Chen, John; Cauli, Alberto; Solla, Paolo; Sechi, Elia
doi : 10.1212/01.wnl.0000903196.96505.e8
To assess the potential association between TNF-inhibitors and MOGAD Background: The association of tumor necrosis factor-a (TNF)-inhibitors with MS has previously been suggested, whereas little is known about MOG-IgG-associated disease (MOGAD) in the context of these drugs.
Neuroinvasive West Nile Virus Disease Presenting as Opsoclonus-Myoclonus-Ataxia Syndrome.
Sharma, Aditi; Otarula, Karina Gonzalez; Wahed, Lama Abdel; Rodriguez, Adriana; Gill, Christine
doi : 10.1212/01.wnl.0000903200.89236.1e
Describe a case of probable Neuroinvasive West Nile Virus (WNV) disease presenting as opsoclonus-myoclonus-ataxia syndrome (OMS).
Etiology and Factors Related Outcomes of Longitudinally Extensive Transverse Myelitis in Thailand.
Vorasoot, Nisa; Saichua, Pilantana; Sirikarn, Prapassara; Boonrod, Arunnit; Kasemsap, Narongrit; Kongbunkiat, Kannikar; Tiamkao, Somsak
doi : 10.1212/01.wnl.0000903204.16114.2a
This study aimed to evaluate the clinical features, etiology, and prognosis of longitudinally extensive transverse myelitis (LETM) patients in Thailand.
Characterization of Neurosarcoid Myelitis and Assessment of Treatment Response: A Multicenter Retrospective Study.
Balaban, Denis; Manzano, Giovanna; Ali, Ahya; Flanagan, Eoin; Aksamit, Allen; Redenbaugh, Vyanka; Clardy, Stacey; Samudralwar, Rohini; Agyei, Paunel; Zabeti, Aram; Baughman, Robert; Chwalisz, Bart; Levy, Michael; Bhattacharyya, Shamik
doi : 10.1212/01.wnl.0000903208.61287.7f
To determine if initial treatment with corticosteroids plus steroid-sparing immunosuppressive therapy (IST) results in faster gadolinium contrast-enhancement resolution in neurosarcoid myelitis (NSM) than corticosteroid monotherapy (CSM).
Resolution of Recurrent VZV Myelitis With the Use of Intravenous (IV) Immunoglobulin (IG).
Paredes, Danelvis; Lackey, Elijah; Eckstein, Christopher
doi : 10.1212/01.wnl.0000903212.89120.b9
To present a case of a patient with recurrent VZV myelitis successfully treated with Intravenous Immunoglobulin (IVIG). Background: VZV myelitis is a rare complication of VZV reactivation that tends to be monophasic.
Sjogren's Sensory Neuropathy: A Potentially Treatable Condition with Early Intervention.
Naum, Ryan; Gwathmey, Kelly
doi : 10.1212/01.wnl.0000903216.87229.45
To describe 2 cases of Sjogren's syndrome sensory neuronopathy (SSSN) in which early intervention resulted in excellent clinical outcomes.
NMDA Receptor Encephalitis With Severe Orofacial Dyskinesias Treated With Tramadol and Clonazepam.
Fernandes, Falen; Clift, Fraser; Chu, Laura
doi : 10.1212/01.wnl.0000903220.64631.0e
N/A. Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a neuroinflammatory disease mediated by antibodies targeting the GluN1 subunit of the NMDAR.
Predictors for the Development of Neurological Immune-Related Adverse Events of Immune Checkpoint Inhibitors and Impact on Mortality.
Yan, Chen; Huang, Merry; Swetlik, Carol; Toljan, Karlo; Bena, James; Funchain, Pauline; McGinley, Marisa
doi : 10.1212/01.wnl.0000903224.28427.4c
To report the incidence, predictors for development, impact on mortality, and impact on pre-existing neurological conditions of neurological immune-related adverse events (irAEs) in a large clinical cohort.
Do Aspirin and Other Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) Have a Protective Effect Against Neuro-Autoimmune Disease and Comorbidity?.
Ahmed, Mohsen; Ahmed, Afaaq; Trivedi, Ronak; Ors, Muhammed; Ahmed, Nabeel; Souayah, Nizar
doi : 10.1212/01.wnl.0000903228.65517.bd
To investigate the neuroprotective potential of Aspirin (AS) and other non-steroidal anti-inflammatory drugs (NSAIDs) against neuro-autoimmune diseases (NAD) and additional comorbidity.
Therapeutic Plasma Exchange in a Patient With Acute Motor Axonal Neuropathy Subtype of Guillain-Barre Syndrome and Systemic Lupus Erythematosus.
Huberman, Melissa; Akgun, Yamac; Langlie, Jake; Wu, YanYun
doi : 10.1212/01.wnl.0000903232.01916.8a
To highlight treatment resistance of concurrent Guillain-Barre syndrome (GBS) and active systemic lupus erythematosus (SLE).
Neurochondrin Autoimmunity With Slow Saccades: A Case Report.
Tanke, Laurel; Eggenberger, Eric; Pless, Misha
doi : 10.1212/01.wnl.0000903236.27201.d1
N/A. Background: Neurochondrin is a cytoplasmic neuronal antigen that can be targeted by specific antibodies, resulting in an antigen-specific T-cell response and autoimmune cerebellar and brain stem degeneration.
Refractory Pediatric NMDA Receptor Encephalitis: A Case Series.
Kannan, Varun; Rospigliosi, Delia; Adeseye, Victoria; Lai, Yi-Chen; Lotze, Timothy; Muscal, Eyal; Shukla, Nikita
doi : 10.1212/01.wnl.0000903240.06447.09
To characterize clinical features of our institution's refractory pediatric NMDA receptor encephalitis (NMDARE) patients, in the hopes of identifying predictive risk factors and specific treatment escalation targets.
Clinical Improvement Following Delayed Initiation of Immunotherapy in a Case of LGI1 Antibody Encephalitis Presenting with Faciobrachial Dystonic Seizures Following COVID-19 Vaccination.
Martin, Kayla; Creech, Matthew; Lane, Michael; Bernard, Jacqueline
doi : 10.1212/01.wnl.0000903244.59854.3a
To demonstrate a case of suspected post-vaccine autoimmune encephalitis associated with leucine-rich glioma-inactivated protein (LGI1) antibodies with significant clinical improvement after initiation of immunotherapy nearly a year after symptom onset.
Therapeutic Plasma Exchange in the Management of Stiff Person Syndrome Spectrum Disorders: A Case Series and Review of the Literature.
Roy, Shuvro; Mercure-Corriveau, Nicolas; Obando, Danielle; Wang, Yujie; Daou, Laetitia; Tobian, Aaron; Bloch, Evan; Newsome, Scott
doi : 10.1212/01.wnl.0000903248.75997.4
To describe the safety, tolerability, and response to TPE in patients with SPSD. Background: Stiff person syndrome spectrum disorders (SPSD) are a rare group of disabling neuroimmunological disorders.
Clinical and Paraclinical Features of Non-Paraneoplastic NIF-Mediated Disease Associated With Concurrent SARS-CoV-2 Infection.
Schmidt, Lauren; Karel, Jon; Rodenbeck, Stefanie
doi : 10.1212/01.wnl.0000903252.71277.22
To describe clinical and paraclinical features of non-paraneoplastic NIF-mediated disease associated with concurrent SARS-CoV-2 infection.
Real-World Utilization Patterns of Intravenous Immunoglobulin in Adults With Generalized Myasthenia Gravis in the United States.
Qi, Cynthia; Hughes, Tom; Gelinas, Deborah; Li, Yuebing MD; Goyal, Amit; Brauer, Edward; Bhuwalka, Arpit; Sato, Mai; Jadhav, Sudhir; Phillips, Glenn
doi : 10.1212/01.wnl.0000903256.02960.8f
To evaluate real-world utilization patterns of intravenous immunoglobulin (IVIg) among patients with generalized myasthenia gravis (gMG) over 3 years post-IVIg initiation.
Neurological Immune-Related Adverse Events After COVID-19 Vaccination: A Systematic Review.
Shafiq, Ameena; Salameh, Mohammad; Laswi, Ibrahim; Mohammed, Ibrahim; Mhaimeed, Omar; Mhaimeed, Nada; Mhaimeed, Narjis; Paul, Pradipta; Mushannen, Malik; Elshafeey, Abdallah; Fares, Ahmed; Holroyd, Sean; Zakaria, Dalia
doi : 10.1212/01.wnl.0000903260.53081.8b
The goal of this study is to compile published data reporting neurological immune-related adverse events following COVID-19 vaccination, not including those relating to hematologic abnormalities such as thrombosis or hemorrhage.
Post COVID-19 Vaccination Associated Acquired Neuromyotonia.
Shivangi, Shivangi; Singh, Amit Shankar; Singhvi, Jeenendra Prakash; Bansal, Namit; Gupta, Rajat
doi : 10.1212/01.wnl.0000903264.64985.38
Acquired neuromyotonia or Isaacs' syndrome is an immune mediated inflammatory disorder characterized by involuntary continuous muscle fiber activity manifesting as twitching and stiffness along with autonomic dysfunctions like hyperhidrosis and/or tachycardia. Here we report a young male who developed acquired neuromyotonia following COVID-19 vaccination.
Medusa-head Antibodies-More than Cerebellar Ataxia: Report of Two Cases.
Liu, Lei; Zhang, Jingxiao
doi : 10.1212/01.wnl.0000903268.63910.3f
To report an anti-metabotropic glutamate receptor 1 (mGluR1) encephalitis case present with cerebellar ataxia and a paraneoplastic retinopathy and optic neuropathy case with anti-inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) antibodies.
Testing for N-type Voltage Gated Calcium Channel Antibody has Limited Utility in Evaluating Patients With Suspected Lambert-Eaton Myasthenic Syndrome.
Majed, Masoud; Zekeridou, Anastasia; Lennon, Vanda; Pittock, Sean; McKeon, Andrew; Klein, Christopher; Dubey, Divyanshu; Mills, John
doi : 10.1212/01.wnl.0000903272.62514.70
To determine if testing for N-type voltage-gated calcium channel antibody improves diagnostic performance of serological testing for Lambert-Eaton Myasthenic Syndrome.
CAR-T Cell-Mediated B Cell Depletion in Central Nervous System Autoimmunity.
Gupta, Sasha; Simic, Milos; Sagan, Sharon; Duecker, Jason; Shepherd, Chanelle; Sobel, Raymond; Hauser, Stephen; Lim, Wendell; Wilson, Michael; Zamvil, Scott
doi : 10.1212/01.wnl.0000903276.26170.3f
Evaluate chimeric antigen receptor (CAR)-T cell mediated B cell depletion in experimental autoimmune encephalomyelitis (EAE). Background: CAR-T cells are autologous T cells expressing a non-MHC target antigen specific receptor.
A Rare Neuromyelitis Optica Mimic: Primary CNS Histiocytic Sarcoma.
Rogawski, David; Nirschl, Jeffrey; McDonald, Jamie; Nie, Esther; Schwartz, Nicholas; Vogel, Hannes; Scott, Brian; Gold, Carl; Kipp, Lucas
doi : 10.1212/01.wnl.0000903280.55740.ae
NA. Background: NA. Design/Methods: NA. Results: Primary CNS histiocytic sarcoma is a rare hematolymphoid malignancy with features of mature histiocytes and carries a poor prognosis.
Recurrent Foreign Body Reactions to Neuroendovascular Polymers-A Clinicopathologic Case Study.
Lou, William; Nguyen, Thuhien; Latimer, Caitlin
doi : 10.1212/01.wnl.0000903284.95480.eb
We provide histopathologic and neuroimaging evidence of recurrent foreign body reactions in a patient following separate stent-assisted coiling of two contralateral intracranial aneurysms a decade apart.
Neuro-Behcet's Disease Presenting as a Psuedotumoral Brainstem Mass: A Case Report.
Yong, Heather; Camara-Lemarroy, Carlos; Alikhani, Katayoun
doi : 10.1212/01.wnl.0000903288.32799.e2
Herein, we present a rare and diagnostically challenging case of neuro-Behcet disease (NBD) manifesting as a psuedotumoral brainstem mass.
Epidemiology and Burden of NMOSD, MS, and MOGAD in Thailand: a Population-Based Study.
Tisavipat, Nanthaya; Jitpratoom, Pornpong; Siritho, Sasitorn; Prayoonwiwat, Naraporn; Apiwattanakul, Metha; Rattanathamsakul, Natthapon; Jitprapaikulsan, Jiraporn
doi : 10.1212/01.wnl.0000903292.80558.d3
To determine cumulative incidence and point prevalence of neuromyelitis optica spectrum disorder (NMOSD), multiple sclerosis (MS), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in Thailand using population-based data of Chumphon province.
Acute Central Nervous System Demyelination Following COVID-19 Vaccination.
Lee, Sydney; Muccilli, Alexandra; Schneider, Raphael; Selchen, Daniel; Krysko, Kristen
doi : 10.1212/01.wnl.0000903296.89024.e9
To describe features of central nervous system (CNS) demyelinating events following vaccination against coronavirus disease 19 (COVID-19). Background: Several reports suggest a potential association between COVID-19 vaccines and acute CNS inflammation.
Spinal Cord Neurosarcoidosis: A Clinical-Radiological Correlation of 39 Cases.
Al-Hader, Rami; Schultz, Lonni; Nofar, Justin; Rai, Vivek; Cerghet, Mirela
doi : 10.1212/01.wnl.0000903300.80412.bf
Present radiological and clinical data of spinal cord neurosarcoidosis and response to treatment. Background: The diagnosis of neurosarcoidosis is challenging. Stern et al.
Serum Autoantibody Lowering by the Anti-FcRn Monoclonal Antibody, Nipocalimab, Correlates With Clinical Improvement in Generalized Myasthenia Gravis Patients.
Ramchandren, Sindhu; Guptill, Jeff; Antozzi, Carlo; Bril, Vera; Gamez, Josep; Meuth, Sven; Nowak, Richard; Quan, Dianna; Sevilla Mantecon, Maria Teresa; Ling, Leona; Zhu, Yaowei; Karcher, Keith; Sun, Hong
doi : 10.1212/01.wnl.0000903304.73134.e3
To evaluate the relationship between clinical improvement in Myasthenia Gravis-Activities of Daily Living (MG-ADL) scores and the pharmacodynamic effects of IgG autoantibody lowering induced by nipocalimab in the Vivacity MG Phase 2 study.
Long-term Safety and Efficacy of Efgartigimod in Patients With Generalized Myasthenia Gravis: Interim Results of the ADAPT+ Study.
Howard, James; Bril, Vera; Vu, Tuan; Karam, Chafic; Peric, Stojan; De Bleecker, Jan; Murai, Hiroyuki; Meisel, Andreas; Beydoun, Said; Pasnoor, Mamatha; Guglietta, Antonio; Ulrichts, Peter; T'joen, Caroline; Brauer, Edward; Utsugisawa, Kimiaki; Verschuuren, Jan; Mantegazza, Renato
doi : 10.1212/01.wnl.0000903308.81107.e2
To evaluate the safety and efficacy of efgartigimod in patients with generalized myasthenia gravis (MG) enrolled in the ADAPT+ long-term extension study.
Numbers Needed to Treat and Costs Per Improved Outcome Among Treatments for Myasthenia Gravis.
Hughes, Tom; Qi, Cynthia; Wang, Jessie; Yang, Hongbo; Gelinas, Deborah; Brauer, Edward; Du, Mandy; Sun, Rochelle; Phillips, Glenn
doi : 10.1212/01.wnl.0000903312.05490.05
Assess number needed to treat (NNT) and costs required to achieve improvements in symptoms and functional activities with targeted therapies for myasthenia gravis (MG).
The ExTINGUISH Trial: A Phase-2B Randomized Placebo-Controlled Trial of Inebilizumab in Anti-NMDA Receptor Encephalitis.
Wong, Ka-Ho; Day, Gregory; Titulaer, Maarten; Torner, James; Cudkowicz, Merit; Coffey, Christopher; Lungu, Codrin; Klawiter, Eric; Singleton, J.; Mitchell, Dana; Fedler, Janel; Ecklund, Dixie; Klements, David; Costigan, Michele; Clardy, Stacey
doi : 10.1212/01.wnl.0000903316.50895.83
To assess the safety and efficacy of inebilizumab in patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Background: The lack of approved therapies for NMDAR encephalitis has led to substantial variability in treatment. High-quality data is needed to guide treatment and optimize long-term outcomes in recovering patients.
Safety, Efficacy, And Pharmacokinetics of Argx-117 in Adults With Multifocal Motor Neuropathy: A Global, Multicenter, Placebo Controlled Phase 2 Study (Arda).
Allen, Jeffrey; Nobile-Orazio, Eduardo; Peric, Stojan; Katzberg, Hans; Cadour, Stephanie; Van de Walle, Inge; Van Hoomissen, Iris; Jacquemyn, Bert; Van de Steen, Olivier; Ludo van der Pol, W.
doi : 10.1212/01.wnl.0000903320.23411.f9
To report the study design of a Phase 2, multicenter, randomized, placebo controlled, parallel-group study (ARDA, NCT05225675) will assess the safety, efficacy, PK, and PD of ARGX-117 in adults with MMN.
Vibrance-mg: Clinical Trial of Nipocalimab in Pediatric Myasthenia Gravis.
Ramchandren, Sindhu; Black, Shawn; Sun, Hong
doi : 10.1212/01.wnl.0000903324.87754.45
We describe an open-label study of nipocalimab to determine the effect of nipocalimab in pediatric participants with gMG. Background: Nipocalimab is a high affinity, fully human, aglycosylated, effectorless IgG1 anti FcRn monoclonal antibody that targets the neonatal Fc receptor (FcRn) with high affinity, thereby lowering IgG pathogenic antibodies in autoimmune disease.
Vivacity MG Phase 3 Study: Clinical Trial of Nipocalimab Administered to Adults With Generalized Myasthenia Gravis.
Ramchandren, Sindhu; Sanga, Panna; Burcklen, Michel; Sun, Hong
doi : 10.1212/01.wnl.0000903328.46907.49
We describe Vivacity-MG3. our pivotal Phase 3, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, Pharmacokinetics (PK), and Pharmacodynamics (PD) of Nipocalimab Administered to Adults With gMG (NCT04951622).
Response of Sjogren-related Small Fiber Neuropathy to rituximab.
Venkatesh, Anita; Muley, Suraj
doi : 10.1212/01.wnl.0000903332.92612.04
Small fiber neuropathy typically presents as loss of temperature and pain sensation and may be associated with other autonomic symptoms.
The First Randomized, Double-Blind, Placebo-Controlled Phase 2 Study to Evaluate the Efficacy and Safety of an FcRn Inhibitor, Rozanolixizumab, in Patients With Leucine-Rich Gliomainactivated 1 Autoimmune Encephalitis.
Dubey, Divyanshu; Titulaer, Maarten J; Koul, Anjana Dhar; Yates, Stephen; Irani, Sarosh R
doi : 10.1212/01.wnl.0000903336.22071.b3
To evaluate efficacy and safety of rozanolixizumab for treatment of leucine-rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AIE).
Do Statins Have a Protective Effect Against Neuro-Autoimmune Disease and Comorbidity?.
Ahmed, Mohsen; Ahmed, Afaaq; Ors, Muhammed; Trivedi, Ronak; Ahmed, Nabeel; Souayah, Nizar
doi : 10.1212/01.wnl.0000903340.03380.c8
To investigate the neuroprotective potential of statins (ST) against neuro-autoimmune disease (NAD) in patients, and risks of associated comorbidities.
Clinical Experience With Efgartigimod in Generalized Myasthenia Gravis: Results From a Case Series of US-Based Patients Participating in an Expanded Access Program.
Khella, Sami; Kuntz, Nancy; Ostrovskiy, Denis; Gelinas, Deborah; Rahman, Omar; Mahuwala, Zabeen
doi : 10.1212/01.wnl.0000903344.85938.3b
To describe the efgartigimod treatment regimens and response among 6 US-based patients with generalized myasthenia gravis (gMG) enrolled in an Expanded Access Program (EAP).
Steroid Un-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SUEAT) in Pediatric Patients.
Rathore, Geetanjali
doi : 10.1212/01.wnl.0000903348.35087.a1
We report a series of children with encephalopathy associated with thyroid antibodies who are refractory to steroid monotherapy
The Role of Plasmapheresis In Pediatric Antibody- Negative Autoimmune Encephalitis.
Rathore, Geetanjali
doi : 10.1212/01.wnl.0000903352.46732.01
We show the efficacy and relative safety of plasmapheresis as a treatment option for antibody negative AIE in children. Background: Plasmapheresis is well established therapy for antibody mediated autoimmune encephalitis (AIE).
First Case Report of AMPA Receptor Encephalitis Presenting With Features of Parkinsonism.
Lalwani, Chirag; Thushara, I.M; Kannoth, Sudheeran; Kumar, Anand; Nambiar, Vivek; Gopinath, Sibi; Saraf, Udit; Mathai, Annamma; Antony, Dayana
doi : 10.1212/01.wnl.0000903356.12164.0d
NA. Background: Autoimmune Encephalitis is an inflammatory condition of the brain due to antibodies against onconeuronal proteins. Paraneoplastic Parkinsonism is very rare.
Rapidly Progressive Dementia With Recurrent Seizures and Hyponatremia; A Case of LGI1 Limbic Encephalitis.
Luster, Joshua; Barasa, Ashley; Hoffman, William
doi : 10.1212/01.wnl.0000903360.38972.63
N/A. Background: Leucine-Rich Glioma Inactivated Protein-1 (LGI1) autoimmune encephalitis was first described in 2001 as one of the syndromes caused antibodies against the voltage-gated potassium channels (VGKC) until it was discovered in 2010 that antibodies were instead being directed towards the protein LGI1.
Co-Occurrence of Sj/ITPR1 and NMDA Antibodies: A Case Report.
Chapman, William; Jordan, Allison; Broderick, Joseph; Ferioli, Simona
doi : 10.1212/01.wnl.0000903364.06027.a6
To highlight a case of concurrent anti-SJ/ITPR1 and anti-NMDA encephalitis. Background: The anti-Sj/inositol 1,4,5-trisphosphate receptor (ITPR1) has been associated with autoimmune cerebellar ataxia and malignancy.
Expanding Frontiers in Autoimmune Encephalitis.
Barroso, Habib Moutran; Reyes, Saul; Orozco, Jaime Rodriguez; Rosembaun, Hellen Kreinter; Jimenez Monsalve, Claudio Alejandro; Cote, Juan Esteban; Toro, Jaime
doi : 10.1212/01.wnl.0000903368.99437.39
To characterize a case series of Colombian patients with autoimmune encephalitis (AE). Background: AE is often an under-recognized entity and antibody testing is not widely available in many developing countries.
Concurrent Autoimmune Encephalitis, Diabetes, and Thyroiditis After a Single Dose of Pembrolizumab.
AlMoamen, Ali; Eibl, Maria del Pilar Guillermo Prieto; Carbunar, Olimpia; Tornes, Luis; Detyniecki, Kamil
doi : 10.1212/01.wnl.0000903372.20783.7d
We describe the case of a patient with an extensive autoimmune response after one dose of pembrolizumab, emphasizing the importance of early recognition of the diverse presentation of autoimmune complications from checkpoint inhibitors.
An Atypical Case of GFAP Astrocytopathy.
Jean, Maxime Junior; Canissario, Ryan; Diep, Judy; Williams, Zoe; Samkoff, Lawrence
doi : 10.1212/01.wnl.0000903376.79652.ed
To describe a case of anti-GFAP astrocytopathy with atypical features. Background: Glial fibrillary acidic protein (GFAP) astrocytopathy is a steroid-responsive autoimmune meningoencephalitis that is commonly characterized by preceding viral illness followed by encephalopathy and papillitis without significant effect on visual acuity.
Autoimmune Encephalitis With Ganglionic a3-AChR Antibodies.
Zaid, Ghaida Khalaf; Saini, Chetan; Gachechiladzi, Leila; Salhab, Mahmoud
doi : 10.1212/01.wnl.0000903380.14508.43
To describe a case of autoimmune encephalitis presented as new onset refractory nonconvulsive status epilepticus and autonomic failure caused by alpha-3 ganglionic acetylcholine receptor autoantibodies (a3-AChR Ab).
Autistic Regression Sequelae or Relapse for NMDA Receptor Antibody Encephalitis.
Zaid, Ghaida Khalaf; Saini, Chetan; Awais, Khadija; Shah, Namrata S
doi : 10.1212/01.wnl.0000903384.12380.b2
To highlight a pediatric presentation of autistic regression secondary to relapsing NMDA receptor antibody encephalitis (NMDARAE) post-HSV infection. Background: It is reported that 30% of patients develop NMDA receptor antibodies (NMDARA) after HSV Encephalitis.
CSF Indices in Autoimmune Encephalitis: Promising Predictors of Treatment Response.
Popova, Ekaterina; Nair, Pranav; Mathai, Annamma; Sasikumar, Sruthi; Gopinath, Siby; Nambiar, Vivek; Kumar, Anand; Umesh, Saraf Udit; Leelamaniamma, Jyothi; Kannoth, Sudheeran
doi : 10.1212/01.wnl.0000903388.67855.1f
To study CSF Indices and their association with prognosis of autoimmune encephalitis. Background: Till date, there is no published data on use of CSF indices in diagnosing or prognosticating of autoimmune encephalitis.
Recurrent Acute Necrotizing Encephalopathy with underlying RANBP2 mutation.
Angappan, Dhanalakshmi; Hollen, Christopher
doi : 10.1212/01.wnl.0000903392.44757.af
N/A. Background: Acute necrotizing encephalopathy (ANE) is a rapidly progressive encephalopathy that can occur in otherwise healthy children after common viral infections such as influenza and parainfluenza.
Primary Immune Dysregulation in Subacute Sclerosing Panencephalitis: A Case-Control Study.
Varman, Vijay; Suresh, Vinay; Malhotra, Hardeep; Kumar, Neeraj; Garg, Ravindra
doi : 10.1212/01.wnl.0000903396.99709.aa
The primary objective was to study the pattern of immune dysregulation in cases with subacute sclerosing panencephalitis (SSPE). The secondary objective was to assess the correlation between the measured immunological variables and disability/death at 6 months, Background: SSPE is a chronic progressive neurological condition caused by a defective measles virus.
Rank Wise Effect of HLA-DQ5 Explains Risk for the Development of Anti-IgLON5 Disease.
Yogeshwar, Selina; Peris-Sempere, Vicente; Muniz-Castrillo, Sergio; Luo, Guo; Finke, Carsten; Honnorat, Jerome; Mignot, Emmanuel; Anti-IgLON5 Disease Collaborators
doi : 10.1212/01.wnl.0000903400.88243.d4
To better characterize the genetic association between human leukocyte antigen (HLA) and anti-IgLON5 disease and to explore auto-antigen binding to associated HLA molecules and their functional involvement in pathophysiology.
Cohort Study of Autoimmune Encephalitis (AIE) in Pediatric and Adult Population from India-A Single Tertiary Centre Experience.
Sharma, Nagashree; Nemani, Tarishi; Manohar, Radhika; Kekatpure V, Minal; Acharya, Harirama; Huded, Vikram; Dash, Gopal; Shetty S, Kuldeep; Kulkarni V, Anirudh; Shah, Mudasir Mushtaq; Philip, Vivek J
doi : 10.1212/01.wnl.0000903404.40371.c0
Study and compare clinico-epidemiological data and long-term outcomes in pediatric (<18 yrs) and adult AIE patients based on serostatus.
Long-Term Psychiatric Symptoms in Autoimmune Encephalitis Remission.
Gabarin, Ramy; Hebert, Julien; Climans, Seth; Muccilli, Alexandra; Lee, Sydney; Day, Gregory; Wennberg, Richard; Tang-Wai, David
doi : 10.1212/01.wnl.0000903408.52359.84
To identify the prevalence of self-reported symptoms of depression and anxiety among patients in remission from autoimmune encephalitis (AE).
The Clinical Response of California Serogroup Virus Neuroinvasive Disease in a Pediatric Patient to Intravenous Immunoglobulin (IVIG) Therapy.
Sanghi, Avni; Gombolay, Grace; Khan, Tuba
doi : 10.1212/01.wnl.0000903412.50488.45
NA. Background: California serogroup (CSG) viruses are commonly associated with neurologic disease. There are few cases of CSG viruses where IVIG has been proven to help.
Expanding Our Knowledge of the Immunogenetic Characteristics of Anti-LGI1 Encephalitis-A Study of an Israeli Cohort Suggests Additional Significant HLA Associations With DQ Alleles.
Segal, Yahel; Nisnboym, Michal; Regev, Keren; Karni, Arnon; Kolb, Hadar; Fahoum, Firas; Aizenstein, Orna; Paran, Yael; Louzoun, Yoram; Israeli, Sapir; Loewenthal, Ron; Svetlicky, Nina; Alcalay, Yifat; Gadoth, Avi
doi : 10.1212/01.wnl.0000903416.71951.5a
Exploring the clinical characteristics and HLA associations of patients with anti-leucine-rich glioma-inactivated 1 encephalitis (LGI1E) from a large single center in Israel Background: Anti-LGI1E is one of the most commonly diagnosed antibody-associated encephalitic syndromes in adults.
Broadening the Differential of Autoimmune Encephalitis: Diagnostic and Therapeutic Considerations in Down Syndrome Disintegrative Disorder.
Elfasi, Aisha; Rempe, Torge
doi : 10.1212/01.wnl.0000903420.38917.c0
To assess clinical features of Down Syndrome Disintegrative Disorder (DSDD) in trisomy 21 (T21) patients with a presumed diagnosis of autoimmune encephalitis (AE) and analyze immunotherapy regimens and timing.
Understanding Correlation of Electroclinical Findings With Functional and Neuropsychiatric Outcomes in Patients with LGI1-Encephalitis.
Swetlik, Carol; Kunchok, Amy; Lapin, Brittany; Li, Yadi; Punia, Vineet
doi : 10.1212/01.wnl.0000903424.19914.85
To evaluate the interplay between seizures, quality of life (QoL), and neuropsychiatric outcomes in patients with leucine-rich glioma-inactived 1 antibody encephalitis (LGI1-Ab-E).
An Observational Study on the Humoral and Cellular Immune Response to SARS-CoV-2 mRNA Vaccination in Multiple Sclerosis and Other Autoimmune Neurological Disorders Treated With Anti-CD20 Therapies.
Borko, Tyler L.; Selva, Sean; Baxter, Ryan; Cabrera-Martinez, Berenice; Rester, Cody; Sillau, Stefan; Pastula, Daniel M.; Sabalza, Maite; Venkataraman, Iswariya; Thiruppathi, Eagappanath; Bennett, Jeffrey L.; Alvarez, Enrique; Gross, Robert; Shah, Anna; Kammeyer, Ryan; Vollmer, Timothy L.; Kedl, Ross; Corboy, John R.; Hsieh, Elena; Piquet, Amanda L.
doi : 10.1212/01.wnl.0000903428.98962.0a
To assess adaptive immunity to SARS-CoV-2 in anti-CD20 treated individuals with mRNA vaccination. Background: Anti-CD20 therapies attenuate humoral responses to vaccines.
Creation and Implementation of a Multi-Disciplinary Clinical Workflow Aimed at Earlier Diagnostic Evaluation for Autoimmune Encephalitis for Patients Presenting With Atypical Psychosis: A Pilot Study.
Russo, Grace; Noy, Gad; Stojanovic, Konstantin; Thakur, Kiran
doi : 10.1212/01.wnl.0000903432.06615.ff
To implement a clinical workflow that facilitates earlier diagnostic evaluation for autoimmune encephalitis (AE) among patients presenting with atypical psychosis (AP).
Two Cases of Isolated Neurofilament Heavy Chain Antibody Syndrome.
Mirzoev, Alexander
doi : 10.1212/01.wnl.0000903436.03488.75
Novel clinical and laboratory findings in anti-neurofilament heavy chain encephalitis Background: Antibodies to mature components of neuronal intermediate filament (NIF) have been implicated in several neurological disorders, including multiple sclerosis, amyotrophic lateral sclerosis, and more recently, various autoimmune encephalitides.
False Positive Cerebrospinal Fluid NMDA Receptor Antibodies: A Single Center Case Series.
Ardakani, Rumyar; Vernino, Steven; Blackburn, Kyle
doi : 10.1212/01.wnl.0000903440.65833.71
To report the presence of CSF NMDA receptor antibodies in four patients without NMDA receptor encephalitis encountered at a single tertiary care center.
Treatment-Refractory Autoimmune Glial Fibrillary Acidic Protein Meningoencephalomyelitis in a Young Adult Female.
Arellano, Janetta; Sy, Michael
doi : 10.1212/01.wnl.0000903444.16135.69
: To describe a case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy refractory to immunotherapy without evidence of malignancy or coexisting autoimmunity.
Autoimmune Encephalitis Misdiagnosis in Adults; A Multicenter Observational Study of Outpatient Subspecialty Clinics.
Geschwind, Michael; Lopez-Chiriboga, A. Sebastian; Blackburn, Kyle; Turaga, Sanchit; Binks, Sophie; Zitser, Jennifer; Gelfand, Jeffrey; Day, Gregory; Dunham, Steven; Rodenbeck, Stefanie; Clardy, Stacey; Solomon, Andrew; Pittock, Sean; McKeon, Andrew; Dubey, Divyanshu; Zekeridou, Anastasia; Toledano, Michel; Turner, Lindsey; Vernino, Steven; Irani, Sarosh; Flanagan, Eoin
doi : 10.1212/01.wnl.0000903448.98416.0e
To determine the diseases misdiagnosed as AE and potential reasons for misdiagnosis. Background: Misdiagnosis of autoimmune encephalitis (AE) may harm patients.
Glial Fibrillary Acidic Protein (GFAP) Antibody-Associated Astrocytopathy in Systemic Sarcoidosis.
Matthews, Elizabeth; Smets, Ide; Kammeyer, Ryan; Titulaer, Maarten; Piquet, Amanda
doi : 10.1212/01.wnl.0000903452.01952.40
To report two cases of glial fibrillary acidic protein (GFAP) antibody-associated meningoencephalitis in patients with biopsy-proven systemic sarcoidosis.
Case of Anti-NMDA Receptor Encephalitis Presenting in a Toddler With Hemorrhagic Cavernomas.
Jacques, Kayla; Marcus, Lydia
doi : 10.1212/01.wnl.0000903456.42128.6d
N/A. Background: N/A. Design/Methods: Introduction: Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis signifies an autoimmune antibody-mediated neuropsychiatric disease that often presents with a set of well-described clinical characteristics and other times manifests with more rare features.
Anti- DPPX Antibody Encephalitis With a Pan-Positive Review of Systems.
Donmez, Mustafa; Mazzola, Maria; Deeb, Wissam; Ionete, Carolina
doi : 10.1212/01.wnl.0000903460.17054.3e
NA. Background: Subacute encephalitides like anti-DDPX encephalitis are challenging diagnoses due to their unusual presentations. Anti-DPPX encephalitis usually involves gastrointestinal, nervous, and respiratory systems.
Acute Autoimmune Encephalitis With Features of Bickerstaff Brainstem Encephalitis (BBE) and Two Abnormal Autoantibodies Presenting With Prominent Cerebellar Abnormality on MRI-A Case Report.
Ozel, Osman; Lai, Eugene; Ramy, Maya; Masdeu, Joseph; Rizk, Christine; Poursheykhi, Meryim; Eid, Abdulmunaim; Karim, Sanaa; Benitez, Sara; Pascual, Belen; Hodics, Timea
doi : 10.1212/01.wnl.0000903464.94171.d5
To present an unusual cerebellar imaging finding of a patient with clinical features of BBE Background: BBE is characterized by progressive ataxia, ophthalmoplegia and impaired consciousness.
Active Immunization Against NMDA NR1 Subunit as a Model of Autoimmune Encephalitis.
Jachimiec, Grace; Motlagh, Negin Jalali; Lin, Chih-Chung; Kuellenberg, Enrico; Planaguma, Jesus; Wojtkiewicz, Gregory; Chen, John; Linnoila, Jenny
doi : 10.1212/01.wnl.0000903468.46642.e8
To identify and develop the optimal active immunization induction method for NMDAR encephalitis in rodents. Background: Encephalitis is a devastating neurologic disorder with high morbidity and mortality. Many cases are autoimmune.
The Texas Children's Hospital Experience With Pediatric Anti-NMDAR Encephalitis: 2010-2021.
Sandweiss, Alexander; Jiang, Yike; Erickson, Tim; Lotze, Timothy; Muscal, Eyal; Murray, Kristy
doi : 10.1212/01.wnl.0000903472.11556.dd
Our objective was to characterize the patients with anti-NMDAR encephalitis and identify the most common presenting symptoms and etiologies.
A Case of Bilateral Sequential Optic Neuropathies With Pachymeningitis and Aortitis: Difficulty in Differentiating Erdheim-Chester Disease From IgG4-Related Disease.
Perera, Tefani; McKenzie, Erica; Alikhani, Katayoun
doi : 10.1212/01.wnl.0000903476.18885.bc
We describe a case of bilateral sequential optic neuropathies with pachymeningitis and aortitis, with findings that raised suspicion of Erdheim-Chester disease versus IgG-4 related disease.
Clinical Characteristics of Double SeroPositive Myasthenia Gravis.
Lalwani, Chirag; Movva, Hamsini; Kannoth, Sudheeran; Nambiar, Vivek; Gopinath, Sibi; Kumar, Anand; Saraf, Udit; N, Sreelakshmi; Jose, Bijoy; Mathai, Annamma; Leelamani, Jyothi V.
doi : 10.1212/01.wnl.0000903480.75544.09
This study aims to delineate the clinical phenotype of DSP-MG and assess the histopathological correlation with thymoma. Background: Double SeroPositive Myasthenia Gravis (DSP-MG), a rare autoimmune disease that affects neuromuscular transmission, is characterized by antibodies against both AChR and MuSK receptors.
The Eye as a Window to the Brain: Prominent Retinal Vasculopathy Points to Neuro-Behcet Diagnosis for an Undifferentiated Solitary Brain Lesion.
Dilwali, Sonam; Harroud, Adil; Rasool, Nailyn; Green, Ari
doi : 10.1212/01.wnl.0000903484.13341.29
To report a perplexing case of Behcet disease (BD) presenting as a focal parenchymal lesion that reached a diagnosis after noting a prominent retinal vasculopathy, highlighting the importance of ophthalmologic evaluation in undifferentiated CNS disease.
A Case of Recurrent Idiopathic Hypertrophic Pachymeningitis After Years of Quiescence.
Bird, Benjamin; Sheikh, Zahir; Zachariah, Jikku Jose
doi : 10.1212/01.wnl.0000903488.03225.fc
To report a case of idiopathic hypertrophic pachymeningitis with recurrence in a new region of the brain after years of quiescence. Background: Idiopathic hypertrophic pachymeningitis (IHP) is a rare condition defined by thickening of the dural layer secondary to inflammation without discernible cause.
Unmasking of a Relapsing Encephalomyelitis After SARS-CoV-2 Infection and COVID-19 Vaccination.
Roy, Shuvro; Barreras, Paula; Pardo-Villamizar, Carlos; Newsome, Scott
doi : 10.1212/01.wnl.0000903492.27780.7c
NA. Background: Prior case studies suggest that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its vaccines may unmask neuroinflammatory conditions.
Characteristics and Evolution of Cerebral Aneurysms Among Adults Living With HIV: A Retrospective, Longitudinal Case Series.
White, Emily I; Anand, Pria; Cervantes-Arslanian, Anna Marisa
doi : 10.1212/01.wnl.0000903496.30639.a5
To describe the characteristics and evolution of cerebral aneurysms in a large cohort of adults living with human immunodeficiency virus (ALWH).
Headache as Initial Presentation of Human Chronic Necrotizing Granulomatous Meningoencephalitis.
Lateef, Sohaib; Gharaibeh, Khaled; Zamir, Danya; Sheikh, Ajaz
doi : 10.1212/01.wnl.0000903500.99408.20
Chronic necrotizing and granulomatous meningoencephalitis is an idiopathic inflammatory disease with possible autoimmune mediated delayed type hypersensitivity response.
Ischemic Stroke in Neurosarcoidosis: A Retrospective Cohort Analysis.
Hutto, Spencer; Kyle, Kevin; Balaban, Denis; Venna, Nagagopal
doi : 10.1212/01.wnl.0000903504.95803.1c
To provide a detailed analysis of the clinical features and course of ischemic stroke secondary to sarcoidosis of the CNS and to observe the effects of immunosuppression in the prevention of recurrent cerebrovascular disease.
Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-Term Outcomes.
Chakales, Pressley; Herman, Max; Chien, Ling Chen; Hutto, Spencer
doi : 10.1212/01.wnl.0000903508.38348.16
To study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment.
Case Review of Alternative Treatment in Patient With Immunotherapy Induced Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).
Huberman, Melissa; Larinde, Oluwagbemiga
doi : 10.1212/01.wnl.0000903512.40716.bd
To emphasize recognition of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) due to Immune checkpoint inhibitor (ICI) therapy Atezolizumab and efficacy of steroids in treatment.
Opsoclonus Myoclonus Syndrome and Supraventricular Tachycardia in a Pediatric Patient: A Case Report and Literature Review.
Giakas, Alec; Garner, Sydney; Korin, John; Edwards, Hollie
doi : 10.1212/01.wnl.0000903516.29674.61
NA. Background: Opsoclonus myoclonus syndrome (OMS) is a rare movement disorder in children often associated with an underlying neuroblastoma.
GABABR IgG Associated Encephalitis: Clinical Presentations and Measures to Improve Diagnostic Assay Specificity.
McCombe, Jennifer; Mills, John; Datta, Abhigyan; Rezk, Mohamed; Chia, Nicholas; Knight, Andrew; Zekeridou, Anastasia; Flanagan, Eoin; McKeon, Andrew; Pittock, Sean; Dubey, Divyanshu
doi : 10.1212/01.wnl.0000903520.84465.3b
To review the clinical/oncological presentations of gamma aminobutyric acid-B receptor (GABABR)-IgG and evaluate the clinical specificity of antibody testing methodologies.
A Case of Paraneoplastic NMOSD With Sarcoma and Unusual Cauda Equina Enhancement.
Hussein, Mohammed; Samkutty, Danny; Durica, Sarah; Anadani, Nidhiben
doi : 10.1212/01.wnl.0000903524.66021.04
To describe a case of sarcoma associated with NMOSD presenting with myeloradiculitis. Background: Neuromyelitis optica is a demyelinating disease of the CNS that predominantly affects the spinal cord and optic nerves.
Missed Opportunities to Prevent N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis in a DREAMer.
Crane, Paul; Jensen, Matthew; Liu, Suzanne; Abbatemarco, Justin; Wold, Jana; Leydard, Holly; Swami, Umang; Miranda, Michelle; Clardy, Stacey
doi : 10.1212/01.wnl.0000903528.36976.47
Describe a case of NMDAR encephalitis in a young Latino male patient, additionally the factors resulting in delayed preventative and diagnostic medical care, which contributed to the development of a preventable case of NMDAR encephalitis.
Neuronal Uptake of Paraneoplastic and Other IgGs is Mediated by the Fc Portion of the IgG Molecule and Involves Previously Uncharacterized Neuronal Fc[gamma]RI Receptors: Implications for Antibody-Mediated Neuronal Injury.
Smith, Tammy; Liu, Suzanne; Carlson, Noel; Clardy, Stacey; Greenlee, John
doi : 10.1212/01.wnl.0000903532.23948.76
To investigate the mechanisms by which neurons take up paraneoplastic and other antibodies. Background: Our laboratory has previously demonstrated that neurons can take up both normal and paraneoplastic IgGs and that paraneoplastic autoantibodies such as anti-Yo and anti-Hu can bind to their intracellular target antigens to produce neuronal death.
Anti-Tr/DNER Paraneoplastic Cerebellar Degeneration with Marked Cerebellar and Psychological Symptoms Responsive to Plasma Exchange.
Crane, Paul; Wynn, DonRaphael Pratt; DeWitt, Dana; Greenlee, John
doi : 10.1212/01.wnl.0000903536.25426.aa
We present a patient who developed cerebellar degeneration and severe psychological symptoms leading to the diagnosis of Hodgkin's disease and detection of anti-Tr/DNER antibodies.
CASPR-2 Antibody Associated Autoimmunity in the Setting of COVID-19 (Infection, Vaccination, or Both?) and Chronic Lymphocytic Leukemia: Case Report and Review of the Literature.
Sattarnezhad, Neda; McDonald, Jamie; Tomczak, Anna; Sumera, Julia; Loeffler, Jacob; Han, May
doi : 10.1212/01.wnl.0000903540.76175.dc
To report a case of Anti-Contactin-Associated Protein-like2 (CASPR-2) autoimmunity in a patient with low-grade Chronic Lymphocytic Leukemia (CLL) following COVID-19 vaccination and infection.
A Woman With Kelch-like Protein-11 Encephalitis and Unmasked Metastatic Carcinoma.
Agyei, Paunel; Gupta, Rajesh
doi : 10.1212/01.wnl.0000903544.99209.7e
To illustrate a case of a woman with rhombencephalitis with antibodies to Kelch-like protein-11 (KLHL11) and a metastatic carcinoma. Background: KLHL11 encephalitis is an autoimmune paraneoplastic syndrome first described in 2019.
Immunotherapy With Subcutaneous Immunoglobulin or Plasmapheresis in Patients With Postural Orthostatic Tachycardia Syndrome (POTS).
Nelson, Renee; Kesterson, Katrina; Schofield, Jill; Blitshteyn, Svetlana
doi : 10.1212/01.wnl.0000903548.26150.37
To assess improvement in autonomic symptoms and functional impairment following immunotherapy with subcutaneous immunoglobulin (SCIG) or plasmapheresis (PLEX) in patients with postural orthostatic tachycardia syndrome (POTS).
Role of Immunotherapy in Down Syndrome Disintegrative Disorder (DSDD).
Anadani, Nidhiben; Chrusciel, Deepti
doi : 10.1212/01.wnl.0000903552.74099.b4
To describe case series of patients with DSDD, successfully treated with immunotherapy including Intravenous Immunoglobulin (IVIG) at a single academic center.
EEG Characteristics in Hospitalized Patients With Acute COVID-19 Symptoms.
Murthy, Ganesh; Fayard, Daniel; Chung, Ryan; Chung, Steve
doi : 10.1212/01.wnl.0000903556.46715.bb
Our objective was to evaluate the incidence of seizures, pattern of EEG abnormalities, and localization of abnormal discharges in hospitalized patients with COVID-19.
Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis: A Multi-Center Case Series.
McEntire, Caleb R.S. MD; Fletcher, Anita MD; Toledano, Michel MD; Epstein, Samantha MD; Tan, Sabrina MD; Mao-Draayer, Yang MD, PhD; Banks, Samantha MD; Aksamit, Allen MD; Gelfand, Jeffrey M. MD, MAS; Thakur, Kiran MD; Cortese, Irene MD; Bhattacharyya, Shamik MD
doi : 10.1212/01.wnl.0000903560.32562.13
We aim to describe the clinical, laboratory, and radiographic features that characterize patients with progressive multifocal leukoencephalopathy (PML) in the context of sarcoidosis (S-PML).
"Trigeminal Tract Sign" in Patients With Herpes Zoster Ophthalmicus: A Case Series of a Novel Imaging Finding.
Yong, Heather; Wallace, Carla; Kapadia, Ronak
doi : 10.1212/01.wnl.0000903564.62886.c1
Herein, we report three patients presenting with zoster ophthalmicus caused by varicella zoster virus (VZV) with unexpected and novel cervico-medullary findings on magnetic resonance imaging (MRI).
Characterization of Clinical and Paraclinical Features Associated With TS-HDS Autoantibody Seropositivity.
Rezk, Mohamed; Mirman, Igal; Berini, Sarah; Chompoopong, Pitcha; Klein, Christopher; Mills, John R.; Dubey, Divyanshu
doi : 10.1212/01.wnl.0000903568.79179.7c
To evaluate neuropathy phenotypes and clinical outcomes associated with trisulfated heparin disaccharide (TS-HDS) autoantibodies. Background: TS-HDS autoantibody has been reported as a biomarker of immune-mediated neuropathy.
BNT162b2 mRNA COVID-19 Vaccine Three-Dose Safety and Risk of COVID-19 in Patients With Myasthenia Gravis.
Doron, Alon; Piura, Yoav; Vigiser, Ifat; Kolb, Hadar; Regev, Keren; Nesher, Nahum; Karni, Arnon
doi : 10.1212/01.wnl.0000903572.81447.33
To study the COVID-19 vaccine three-dose safety and risk of COVID-19 in patients with myasthenia gravis Background: Various vaccines, including those against SARS-CoV-2, were reported to trigger or exacerbate myasthenia gravis (MG).
Comparison of Fixed Cell-based Assay to Radioimmunoprecipitation Assay for Acetylcholine Receptor Antibody Detection in Myasthenia Gravis.
Mirian, Ario; Nicolle, Michael; Budhram, Adrian
doi : 10.1212/01.wnl.0000903576.06122.e0
To compare specificity and sensitivity of a commercially available fixed cell-based assay (F-CBA) to radioimmunoprecipitation assay (RIPA) for acetylcholine receptor antibody (anti-AChR) detection in myasthenia gravis (MG).
Piloting an Advanced Neuroimmunology Elective for Neurology Residents.
Singh, Sonia Kaur; Samudralwar, Rohini
doi : 10.1212/01.wnl.0000903580.67771.39
To describe the creation of an Advanced Neuroimmunology elective for residents with a special interest in clinical neuroimmunology.
A Case of Pembrolizumab (Anti-PD-1) Induced Encephalitis.
Zahid, Anza; Poursheykhi, Meryim; Saeed, Mujtaba; Tremont, Ivo
doi : 10.1212/01.wnl.0000903584.28612.37
N/A. Background: PD-1 Immune checkpoint inhibitors (ICI) have been associated with neurologic immune-related adverse events including meningoencephalitis and limbic encephalitis that can manifest as paraneoplastic syndromes.
Neuroblastoma Presentation With Multiple Cranial Nerve Involvement.
Arshad, Aysha; Arellano, Janetta; Chumakova, Anastasia; Taraman, Sharief
doi : 10.1212/01.wnl.0000903588.17297.bf
NA. Background: We report a case of neuroblastoma, a pediatric neuroendocrine tumor of the sympathetic nervous system, in a 3-year-old female with multiple cranial nerve involvement.
Co-Occurring NMDA-Receptor and Anti-GAD65 Antibodies in the CSF of a Patient With Encephalitis: Case Report.
McEntire, Caleb; Manzano, Giovanna; Linnoila, Jenny
doi : 10.1212/01.wnl.0000903592.90530.d1
We describe a 58-year-old woman who presented with rapid cognitive changes and was found to have concurrent CSF NMDA and GAD65 receptor antibodies.
Evidence for and Against Subclinical Disease Activity and Progressive Disease in MOG Antibody Disease and Neuromyelitis Optica Spectrum Disorder.
Molazadeh, Negar; Filippatou, Angeliki G.; Vasileiou, Eleni S.; Levy, Michael; Sotirchos, Elias S.
doi : 10.1212/01.wnl.0000903596.72722.d2
To investigate the evidence for and against relapse-independent clinical progression and/or subclinical disease activity in patients with Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD) and Aquaporin-4 IgG Seropositive Neuromyelitis Optica Spectrum Disorder (AQP4-IgG+ NMOSD).
Coexistance of Probable Neurosarcoidosis and Generalized Myasthenia Gravis: Case Report.
Abad, Itziar; Pujol, Mariona Hervas; Estela, Jordi; Viguera, Maria Luisa; Barrachina-Esteve, Oriol; Ribera, Gisela; Feijoo, Carlos; Vazquez, Olalla; Lozano, Carme; Caresia, Ana Paula
doi : 10.1212/01.wnl.0000903600.28886.23
NA. Background: Sarcoidosis is an immune mediated disorder characterized by the formation of non-necrotizing granulomas in multiple organs.
Autoimmune Encephalitis Misdiagnosis: A Review of Reported Cases.
Orellana Zambrano, Maria Daniela; Day, Gregory; Sechi, Elia; Lopez, Alfonso
doi : 10.1212/01.wnl.0000903604.96861.fa
To identify autoimmune encephalitis (AE) mimics and clinical features reported in the literature. Background: Recent evidence suggesting that AE is as frequent as infectious encephalitis has increased awareness and testing for immune-mediated causes of neurological impairment.
The Complicated Course of a Patient With Faciobrachial Dystonic Seizures Associated With LGI1-Antibody Limbic Encephalitis.
Sadeghpour, Shirin; Neupane, Dilasha; Mouti, Mariam; Clark, Jeffrey
doi : 10.1212/01.wnl.0000903608.11110.d4
Highlighting diagnostic and treatment challenges of Faciobrachial Dystonic Seizures (FBDS) associated with LGI1-antibody limbic encephalitis (LE) Background: Anti-LGI1 LE presents with FDBS as its hallmark: brief, recurrent, contractions of facial and upper limb muscles.
Autoimmune Encephalitis-A Diagnostic Challenge for the Neurologist.
Khanna, Laxmi; Gourie-Devi, Mandaville; Verma, Ritu
doi : 10.1212/01.wnl.0000903612.47691.5f
Autoimmune encephalitis, is a clinically challenging entity with varied neurological presentations. As autoimmune serology is negative in over 50% cases, our objective was to prioritise the use of electroencephalography supported by MRI Brain or PET-CT imaging to make a definitive diagnosis of autoimmune encephalitis.
Brains on Fire: Patient Outcomes and Quality of Life Following Autoimmune Encephalitis.
Easton, Ava
doi : 10.1212/01.wnl.0000903616.08384.e6
To present and improve understanding of patient outcomes and quality of life post-autoimmune encephalitis. Background: Patient outcome following encephalitis and in particular following autoimmune encephalitis is not well understood.
Spotlight on the December 13 Issue.
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology(R)
doi : 10.1212/WNL.0000000000201512
Volume 99(24) pgs. 1073-1133,e2637-e2727 December 13, 2022
Improving Our Understanding of Cognitive Aging in American Indian Peoples.
Hamilton, Roy H. MD, MS, FAAN; Goins, R. Turner PhD
Foreseeing Before Disease Onset: Brain Atrophy Progression in Genetic Frontotemporal Dementia.
Agosta, Federica MD, PhD; Spinelli, Edoardo Gioele MD; Filippi, Massimo MD
National Patterns of Interfacility Transfers for Seizure-Related Emergencies: Could the Utilization of Transfer Networks for Seizures Be Optimized?.
Taraschenko, Olga MD, PhD
Strategic Planning at NINDS: Translating Plans Into Action and Outcomes.
Jones, Lyell K. Jr MD; George, Paul MD, PhD
Educational and Clinical Associations With Longitudinal Cognitive Function and Brain Imaging in American Indians: The Strong Heart Study.
Suchy-Dicey, Astrid M. PhD; Oziel, Kyra BS; Sawyer, Charles BS; Olufadi, Yunusa PhD; Ali, Tauqeer PhD; Fretts, Amanda M. PhD; Umans, Jason G. MD; Shibata, Dean K. MD; Longstreth, W.T. Jr M.D.; Rhoads, Kristoffer PhD; Buchwald, Dedra S. MD; Grabowski, Thomas J. MD
doi : 10.1212/WNL.0000000000201261
Little is known about incidence of vascular and Alzheimer dementias in American Indians. Methods: We conducted a large, heterogeneous, population-based, longitudinal cohort study of brain aging in community-dwelling American Indians aged 64-95 years from 11 tribes across 3 states, with neurologic examinations, 1.5T MRI, and extensive cognitive testing. Visit 1 in 2010-2013 (n = 817) and visit 2 in 2017-2019 (n = 403) included all willing, surviving participants.
Association of MRI Indices of Glymphatic System With Amyloid Deposition and Cognition in Mild Cognitive Impairment and Alzheimer Disease.
Kamagata, Koji MD, PhD *; Andica, Christina MD, PhD *; Takabayashi, Kaito MS; Saito, Yuya MS; Taoka, Toshiaki MD, PhD; Nozaki, Hayato MS; Kikuta, Junko MD, PhD; Fujita, Shohei MD; Hagiwara, Akifumi MD, PhD; Kamiya, Kouhei MD, PhD; Wada, Akihiko MD, PhD; Akashi, Toshiaki MD, PhD; Sano, Katsuhiro MD, PhD; Nishizawa, Mitsuo MD, PhD; Hori, Masaaki MD, PhD; Naganawa, Shinji MD, PhD; Aoki, Shigeki MD, PhD; for the Alzheimer's Disease Neuroimaging Initiative
doi : 10.1212/WNL.0000000000201300
The glymphatic system is a whole-brain perivascular network, which promotes CSF/interstitial fluid exchange. Alterations to this system may play a pivotal role in amyloid [beta] (A[beta]) accumulation.
Longitudinal Brain Atrophy Rates in Presymptomatic Carriers of Genetic Frontotemporal Dementia.
Poos, Jackie M. MSc; Grandpierre, Leonie D. M. MSc; van der Ende, Emma L. PhD; Panman, Jessica L. PhD; Papma, Janne M. PhD; Seelaar, Harro MD, PhD; van den Berg, Esther PhD; van 't Klooster, Ronald PhD; Bron, Esther PhD; Steketee, Rebecca PhD; Vernooij, Meike W. PhD, MD; Pijnenburg, Yolande A. L. MD, PhD; Rombouts, Serge A. R. B. PhD, MD; van Swieten, John MD, PhD; Jiskoot, Lize C. PhD
doi : 10.1212/WNL.0000000000201292
It is important to identify at what age brain atrophy rates in genetic frontotemporal dementia (FTD) start to accelerate and deviate from normal aging effects to find the optimal starting point for treatment.
Association Between White Matter Networks and the Pattern of Striatal Dopamine Depletion in Patients With Parkinson Disease.
Chung, Seok Jong MD, PhD *; Kim, Yae Ji BS *; Kim, Yun Joong MD, PhD; Lee, Hye Sun PhD; Jeong, Seong Ho MD; Hong, Ji-Man MD, PhD; Sohn, Young H. MD, PhD; Yun, Mijin MD, PhD; Jeong, Yong MD, PhD +; Lee, Phil Hyu MD, PhD +
doi : 10.1212/WNL.0000000000201269
Individual variability in nigrostriatal dopaminergic denervation is an important factor underlying clinical heterogeneity in Parkinson disease (PD). This study aimed to explore whether the pattern of striatal dopamine depletion was associated with white matter (WM) networks in PD.
Assessment of Bioenergetic Deficits in Patients With Parkinson Disease and Progressive Supranuclear Palsy Using 31P-MRSI.
Prasuhn, Jannik MD *; Gottlich, Martin PhD *; Ebeling, Britt MS; Kourou, Sofia MS; Gerkan, Friederike MS; Bodemann, Christina MS; Gro[latin sharp s]er, Sinja S. MD; Reuther, Katharina MD; Hanssen, Henrike MD; Bruggemann, Norbert MD
doi : 10.1212/WNL.0000000000201288
Bioenergetic disturbance, mainly caused by mitochondrial dysfunction, is an established pathophysiologic phenomenon in neurodegenerative movement disorders.
Genetically Predicted Smoking and Alcohol Consumption and Functional Outcome After Ischemic Stroke.
Zhang, Zhizhong PhD; Wang, Mengmeng MD; Gill, Dipender MD, PhD; Liu, Xinfeng PhD
doi : 10.1212/WNL.0000000000201291
Smoking and alcohol consumption have been adversely associated with poststroke outcome in traditional epidemiologic studies. The present study explored the association of genetically predicted smoking and alcohol consumption on poststroke outcomes using the mendelian randomization (MR) framework.
Association of Carotid Plaque and Flow Velocity With White Matter Integrity in a Middle-aged to Elderly Population.
Rimmele, David Leander MD; Petersen, Elina Larissa MSc; Schlemm, Eckhard MD, PhD; Kessner, Simon S. MD, PhD; Petersen, Marvin MD; Mayer, Carola MSc; Cheng, Bastian MD; Zeller, Tanja PhD; Waldeyer, Christoph MD; Behrendt, Christian-Alexander MD; Gerloff, Christian MD; Thomalla, Gotz MD
doi : 10.1212/WNL.0000000000201297
It is uncertain whether there is an association of carotid plaques (CPs) and flow velocities with peak width mean diffusivity (PSMD) and white matter hyperintensities (WMH) independent of shared risk factors.
Association of Plaque Morphology With Stroke Mechanism in Patients With Symptomatic Posterior Circulation ICAD.
Song, Xiaoyan MD *; Li, Shuang MD *; Du, Heng MD; Hu, Qimin MD; Zhou, Li MD, PhD; Zhao, Jinglong MD, PhD; Gu, Yue MD; Hu, Yiming MD; Lu, Haiyan MD, PhD; Wang, Guodong MD; Chen, Xiangyan MD, PhD +; Wang, Qiaoshu MD, PhD +
doi : 10.1212/WNL.0000000000201299
Although the main mechanisms of stroke in patients with intracranial atherosclerotic disease (ICAD)-perforating artery occlusion (PAO) and artery-to-artery embolism (AAE)-have been identified and described, relatively little is known about the morphology of the symptomatic plaques and how they differ between these 2 mechanisms.
Interfacility Transfers for Seizure-Related Emergencies in the United States.
Acton, Emily K. MSCE *; Blank, Leah J. MD, MPH *; Willis, Allison W. MD, MS; Hamedani, Ali G. MD, MHS
doi : 10.1212/WNL.0000000000201319
Interfacility transfer protocols are important for seizure-related emergencies, the cause of approximately 1% of all emergency department (ED) visits in the United States, but data on current practices are lacking.
Progressive Supranuclear Palsy Syndrome Associated With a Novel Tauopathy: Case Study.
Forrest, Shelley L. PhD; Tartaglia, Maria Carmela MD; Kim, Ain; Alcaide-Leon, Paula MD; Rogaeva, Ekaterina PhD; Lang, Anthony MD; Kovacs, Gabor G. MD, PhD
doi : 10.1212/WNL.0000000000201485
To report a novel tauopathy in a patient with protracted course progressive supranuclear palsy (PC-PSP). Methods: This was a clinical follow-up, gene analysis, neuropathologic study.
Planning and Implementing Strategically: Year 1 of the NINDS 2021-2026 Strategic Plan.
Schor, Nina F. MD, PhD; Scott, Paul PhD; Litvina, Elizabeth Y. PhD; Torborg, Christine PhD; Kim, Jenny L. PhD; Zalutsky, Robert PhD; Adams, Amy Bany PhD; on behalf of Taskforces on Science, Scientific Training and Workforce Diversity, Communications and Dissemination, and Workforce Culture
doi : 10.1212/WNL.0000000000201380
AB At the end of 2020, the National Institute of Neurological Disorders and Stroke, an institute of the NIH, completed an 18-month-long strategic planning process that involved and engaged diverse internal and external biomedical and general stakeholders.
Precision Terminology: Why to Avoid the Term "Atypical Optic Neuritis".
Eggenberger, Eric R. DO; Costello, Fiona MD; Frohman, Elliot MD, PhD; Pless, Misha MD; Van Stavern, Greg MD
Novel Application of 7T MRI in CSF1R-Related Leukoencephalopathy.
Dulski, Jaroslaw MD, PhD; Middlebrooks, Erik H. MD; Wszolek, Zbigniew K. MD
Child Neurology: Maternal Transmission of Congenital Myotonic Dystrophy Type 2: Case Report.
Tieleman, Alide A. MD, PhD; Damen, Manon J. MD; Verrips, Aad MD, PhD; Roelofs, Monique MD; Kamsteeg, Erik-Jan MSc; Voermans, Nicol C. MD, PhD
doi : 10.1212/WNL.0000000000201427
AB Congenital manifestations in Myotonic Dystrophy type 2 (DM2) point to anticipation and have only rarely been described. We report a three-generation family with genetically confirmed DM2.
Clinical Reasoning: A 59-Year-Old Man With Thymoma and Constitutional Symptoms, Seizures, and Multifocal CNS Lesions.
Stopschinski, Barbara E. MD; Fredrich, Sarah MD; Vernino, Steven MD, PhD; Phillips, Lauren MD; Blackburn, Kyle M. MD
doi : 10.1212/WNL.0000000000201381
AB A 59-year-old man first presented for an episode of left arm numbness. During workup, a thymoma was incidentally discovered and resected. The symptoms in his left arm were attributed to a cardiac pathology.
Clinical Reasoning: Patient With Prior Spinal Cord Injury Who Developed Altered Mental Status After a Fall.
Dessy, Alexa MD; Bhagat, Dhristie MD; Czeisler, Barry M. MD
doi : 10.1212/WNL.0000000000201383
AB An 18-year-old man with a history of complete traumatic spinal cord injury (SCI) at C5-C7 3 years before presented with unresponsiveness and hypoxia after a fall. There were no overt signs of bruising or swelling.
Teaching NeuroImage: Partially Reversible Widespread Leukoencephalopathy Associated With Atypical Hemolytic Uremic Syndrome.
Cani, Ilaria MD; Righini, Matteo MD; Cenni, Patrizia MD; Foschi, Matteo MD
Editors' Note: One-Stage, Limited-Resection Epilepsy Surgery for Bottom-of-Sulcus Dysplasia.
Lewis, Ariane MD; Galetta, Steven MD, FAAN
Reader Response: One-Stage, Limited-Resection Epilepsy Surgery for Bottom-of-Sulcus Dysplasia.
Hu, Wenhan; Zhao, Baotian; Zhang, Jianguo; Zhang, Kai
Author Response: One-Stage, Limited-Resection Epilepsy Surgery for Bottom-of-Sulcus Dysplasia.
Harvey, A. Simon; Macdonald-Laurs, Emma
Editors' Note: Race-Ethnic Disparities in Rates of Declination of Thrombolysis for Stroke.
Lewis, Ariane MD; Galetta, Steven MD, FAAN
Reader Response: Race-Ethnic Disparities in Rates of Declination of Thrombolysis for Stroke.
Patel, Karan; Wolfe, Jared; Siegler, James E.
Reader Response: Race-Ethnic Disparities in Rates of Declination of Thrombolysis for Stroke.
Ortega-Gutierrez, Santiago; Garg, Aayushi; Farooqui, Mudassir; Vivanco-Suarez, Juan; Galecio-Castillo, Milagros
