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Volume 8, Number 2, March 2021Neurology.org/NN
A peer-reviewed clinical and translational neurology open access journal
ARTICLE
Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Diff erences in the Immune Response Between the Cerebral Hemispheres e936
ARTICLE
Human Herpesvirus 6 Encephalitis in Immunocompetent and Immunocompromised Hosts e942
ARTICLE
Aryl Hydrocarbon Receptor Activation in Astrocytes by Laquinimod Ameliorates Autoimmune Infl ammation in the CNS e946
ARTICLE
Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies e947
Academy OfficersJames C. Stevens, MD, FAAN, PresidentOrly Avitzur, MD, MBA, FAAN, President ElectAnn H. Tilton, MD, FAAN, Vice PresidentCarlayne E. Jackson, MD, FAAN, SecretaryJanis M. Miyasaki, MD, MEd, FRCPC, FAAN, TreasurerRalph L. Sacco, MD, MS, FAAN, Past President
CEO, American Academy of NeurologyMary E. Post, MBA, CAEChief Executive Officer201 Chicago AveMinneapolis, MN 55415Tel: 612-928-6100
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Morgan S. Sorenson, Managing EditorNeurology® Neuroimmunology & NeuroinflammationNeurology® Genetics
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Neurology® Neuroimmunology & Neuroinflammation (eISSN 2332-7812) is anopen access journal published online for the American Academy of Neurology,201 Chicago Ave, Minneapolis, MN 55415, by Wolters Kluwer Health, Inc. at14700 Citicorp Drive, Bldg 3, Hagerstown, MD 21742. Business offices arelocated at Two Commerce Square, 2001 Market St., Philadelphia, PA 19103.Production offices are located at 351 West Camden Street, Baltimore, MD21201-2436. © 2021 American Academy of Neurology.
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Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
A peer-reviewed clinical and translational neurology open access journal Neurology.org/NN
Neurology® Neuroimmunology & Neuroinflammation
EditorJosep O. Dalmau, MD, PhD
Deputy EditorScott S. Zamvil, MD, PhD, FAAN
Associate EditorsMarinos Dalakas, MD, FAANMichael Heneka, MDDennis Kolson, MD, PhDFriedmann Paul, MDRaquel Sanchez-Valle, MD, PhD
Editorial BoardSergio E. Baranzini, PhDSusanne Benseler, MDJeffrey L. Bennett, MD, PhD, FAANSeverine Boillee, PhDRiley M. Bove, MD, MMSCChiara Briani, MDFabienne Brilot, PhDMatthijs C. Brouwer, MD, PhDWolfgang Bruck, MDDavid B. Clifford, MD, FAANAnne Haney Cross, MDRussell C. Dale, MBChB, MRCP, PhDThomas G. Forsthuber, MDKazuo Fujihara, MDChristian Geis, MDJeffrey M. Gelfand, MD, MAS, FAANFrancesc Graus, MD, PhDKerstin Hellwig, MDMatilde Inglese, MD, PhDTakahiro Iizuka, MDLuisa Klotz, MD
Kristen M. Krysko, MD, MASAnnette Langer-Gould, MD, PhDFrank Leypoldt, MDErin Longbrake, MD, PhDAndrew McKeon, MDAnne Louise Oaklander, MD PhD,
FAAN, FANAAnne-Katrin Probstel, MDRichard M. Ransohoff, MDAlbert Saiz, MD, PhDJens Schmidt, MD, FEAN, FAANChristine Stadelmann, MDIsrael Steiner, MDLawrence Steinman, MDSilvia N. Tenembaum, MDCharlotte E. Teunissen, PhDMaarten J. Titulaer, MD, PhDAri Waisman, PhDGregory F. Wu, MD, PhD
Neurology® Journals
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Section Editors
BiostatisticsRichard J. Kryscio, PhDSue Leurgans, PhDV. Shane Pankratz, PhD
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Vision Neurology® Neuroimmunology &Neuroinflammation will be the premierpeer-reviewed journal in the fields ofneuroimmunology and neuroinflammation.
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Copyright © 2021 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
TABLE OF CONTENTS Volume 8, Number 2, March, 2021 Neurology.org/NN
Editorial
e948 Learning More About HHV-6 EncephalitisJ.R. Berger
Open Access
Articles
e933 Aryl Hydrocarbon Receptor Plasma Agonist ActivityCorrelatesWith Disease Activity in ProgressiveMST. Tsaktanis, T. Beyer, L. Nirschl, M. Linnerbauer, V. Grummel,M. Bussas, E. Tjon, M. Muhlau, T. Korn, B. Hemmer,F.J. Quintana, and V. Rothhammer
Open Access
e935 Biological Significance of Anti–SARS-CoV-2Antibodies: Lessons Learned From ProgressiveMultifocal LeukoencephalopathyN. Manouchehri, L. Steinman, and O. Stuve
Open Access
e937 TDP-43–specific Autoantibody Decline in PatientsWith Amyotrophic Lateral SclerosisA.K. Nielsen, J. Folke, S. Owczarek, K. Svenstrup, K. Winge,B. Pakkenberg, S. Aznar, and T. Brudek
Open Access
e941 CSF SERPINA3 Levels Are Elevated in Patients WithProgressive MSN. Fissolo, C. Matute-Blanch, M. Osman, C. Costa, R. Pinteac,B. Miro, A. Sanchez, V. Brito, I. Dujmovic, M. Voortman, M. Khalil,E. Borras, E. Sabido, S. Issazadeh-Navikas, X. Montalban, andM. Comabella Lopez
Open Access
e940 Effect of Ocrelizumab in Blood Leukocytes of PatientsWith Primary Progressive MSJ.I. Fernandez-Velasco, J. Kuhle, E. Monreal, V. Meca-Lallana,J. Meca-Lallana, G. Izquierdo, F. Gascon-Gimenez,S. Sainz de la Maza, P.E. Walo-Delgado, A. Maceski,E. Rodrıguez-Martın, E. Roldan, N. Villarrubia, A. Saiz, Y. Blanco,P. Sanchez, E. Carreon-Guarnizo, Y. Aladro, L. Brieva, C. Iñiguez,I. Gonzalez-Suarez, L.A. Rodrıguez de Antonio, J. Masjuan,L. Costa-Frossard, and L.M. Villar
Open Access
e942 Human Herpesvirus 6 Encephalitis inImmunocompetent and ImmunocompromisedHostsG. Berzero, G. Campanini, E. Vegezzi, M. Paoletti, A. Pichiecchio,A.M. Simoncelli, A.A. Colombo, P. Bernasconi, O. Borsani,A. Di Matteo, V. Rossi, T. Foiadelli, S. Savasta, F. Compagno,M. Zecca, F. Baldanti, and E. Marchioni
Open Access Editorial, p. 948
e943 Mass Cytometry of CSF Identifies an MS-AssociatedB-cell PopulationD. Johansson, C. Rauld, J. Roux, C. Regairaz, E. Galli, I. Callegari,L. Raad, A.Waldt, R. Cuttat, G. Roma,M. Diebold, B. Becher, J. Kuhle,T. Derfuss, J.M. Carballido, and N.S.R. Sanderson
Open Access
e944 CIDP Antibodies Target Junction Proteins andIdentify Patient Subgroups: An AutoantigenomicApproachC.P. Moritz, Y. Tholance, O. Stoevesandt, K. Ferraud,J.-P. Camdessanche, and J.-C. Antoine
Open Access
e945 Immune Skew of Circulating Follicular HelperT Cells Associates With MyastheniaGravis SeverityS. Ashida, H. Ochi, M. Hamatani, C. Fujii, K. Kimura, Y. Okada,Y. Hashi, K. Kawamura, H. Ueno, R. Takahashi, T. Mizuno, andT. Kondo
Open Access Class of Evidence
e947 Discontinuation of Immunosuppressive Therapy inPatients With Neuromyelitis Optica SpectrumDisorder With Aquaporin-4 AntibodiesS.-H. Kim, H. Jang, N.Y. Park, Y. Kim, S.-Y. Kim, M.Y. Lee,J.-W. Hyun, and H.J. Kim
Open Access
e946 Aryl Hydrocarbon Receptor Activation in Astrocytesby Laquinimod Ameliorates AutoimmuneInflammation in the CNSV. Rothhammer, J.E. Kenison, Z. Li, E. Tjon, M.C. Takenaka,C.-C. Chao, K. Alves de Lima, D.M. Borucki, J. Kaye, and F.J. Quintana
Open Access
Cover ImageTop panels show the choroid plexus, periventricular white matter, andsubcortical white matter from a patient with multiple sclerosis (MS) andCOVID-19. Lower panels show that SARS-CoV-2 transcripts (pinkarrows) by in situ hybridization inMSCOVID-19 are detected in choroidplexus and ependyma but are not present in an MS lesion. Expression ofACE2 is indicated with white arrows. Stylized by Kaitlyn Aman Ramm,Digital Multimedia/Graphics Coordinator.See page e957
Continued
e936 Unilateral Relapsing Primary Angiitis of theCNS: An Entity Suggesting Differences in theImmune Response Between the CerebralHemispheresM.A. AbdelRazek, J.M. Hillis, Y. Guo, M.Martinez-Lage, T. Gholipour,J. Sloane, T. Cho, and M. Matiello
Open Access
e950 Different Fumaric Acid Esters Elicit DistinctPharmacologic ResponsesB.T. Wipke, R. Hoepner, K. Strassburger-Krogias, A.M. Thomas,D. Gianni, S. Szak, M.S. Brennan, M. Pistor, R. Gold, A. Chan, andR.H. Scannevin
Open Access
e953 Amyotrophic Lateral Sclerosis SurvivalAssociates With Neutrophils in a Sex-specificMannerB.J. Murdock, S.A. Goutman, J. Boss, S. Kim, and E.L. Feldman
Open Access Class of Evidence
e952 Guillain-Barre Syndrome Outbreak in Peru 2019Associated With Campylobacter jejuni InfectionA.P. Ramos, S.E. Leonhard, S.K. Halstead, M.A. Cuba,C.C. Castañeda, J.A. Dioses, M.A. Tipismana, J.T. Abanto, A. Llanos,D. Gourlay, M. Grogl, M. Ramos, J.D. Rojas, R. Meza, D. Puiu,R.M. Sherman, S.L. Salzberg, P.J. Simner, H.J. Willison, B.C. Jacobs,D.R. Cornblath, H.F. Umeres, and C.A. Pardo
Open Access
e954 Incidence and Impact of COVID-19 in MS: A SurveyFrom a Barcelona MS UnitM. Sepulveda, S. Llufriu, E. Martınez-Hernandez, M. Catala,M. Artola, A. Hernando, C. Montejo, I. Pulido-Valdeolivas,E. Martınez-Heras, M. Guasp, E. Solana, L. Llanso, D. Escudero,M. Aldea, C. Prats, F. Graus, Y. Blanco, and A. Saiz
Open Access
e955 Absence of B Cells in Brainstem and White MatterLesions Associates With Less Severe Disease andAbsence of Oligoclonal Bands in MSN.L. Fransen, B.A. de Jong, K. Heß, T. Kuhlmann, M.C.J. Vincenten,J. Hamann, I. Huitinga, and J. Smolders
Open Access
e960 Defining Benign/Burnt-Out MS and DiscontinuingDisease-Modifying TherapiesD.McFaul, N.N.Hakopian, J.B. Smith, A.S.Nielsen, andA. Langer-Gould
Open Access Class of Evidence
e961 Improvement of Comorbid Psoriasis in Patients WithMS Treated With NatalizumabR. Berkovich, A. Yakupova, J. Eskenazi, N.G. Carlson, and L. Steinman
Open Access
e951 Altered CSF Albumin Quotient Links PeripheralInflammation and Brain Damage in MSM. Puthenparampil, P. Tomas-Ojer, T. Hornemann, A. Lutterotti,I. Jelcic, M. Ziegler, A.J. Hulsmeier, C. Cruciani, W. Faigle,R. Martin, and M. Sospedra
Open Access
e958 Long-term Functional Outcomes and Relapse ofAnti-NMDA Receptor Encephalitis: A CohortStudy in Western ChinaX. Gong, C. Chen, X. Liu, J. Lin, A. Li, K. Guo, D. Zhou, and Z. Hong
Open Access
e962 Anti-IgLON5 Disease: A New Bulbar-Onset MotorNeuron Mimic SyndromeJ. Werner, I. Jelcic, E.I. Schwarz, E. Probst-Muller, J. Nilsson,B. Schwizer, K.E. Bloch, A. Lutterotti, H.-H. Jung, and B. Schreiner
Open Access
e963 Paraneoplastic Cerebellar Degeneration: TheImportance of Including CDR2L as a DiagnosticMarkerI. Herdlevær, M. Haugen, K. Mazengia, C. Totland, andC. Vedeler
Open Access Class of Evidence
e959 Clinical and Radiologic Disease Activity in Pregnancyand Postpartum in MSA. Anderson, K.M. Krysko, A. Rutatangwa, T. Krishnakumar, C. Chen,W. Rowles, Z. Zhao, M.K. Houtchens, and R. Bove
Open Access
Clinical/Scientific Notes
e939 Evidence for Independent Hepatitis E VirusReplication in the BrainE.P.M. den Drijver, A.E. Brouwer, N.E. Synhaeve, J.P. Keijer,J.J. Verweij, J.-L. Murk, and S.D. Pas
Open Access
e956 Reversible Cerebral Vasculopathy, TransverseMyelitis, andActive Systemic LupusErythematosusin an Aquaporin-4 Antibody–Positive PatientD. Gritsch, A.A. Mbonde, E.Y. Harahsheh, B.W. Chong, andD.M. Wingerchuk
Open Access
e957 Presence of SARS-CoV-2 Transcripts in theChoroid Plexus of MS and Non-MS PatientsWith COVID-19V. Fuchs, M. Kutza, S. Wischnewski, N. Deigendesch, L. Lutz,L. Kulsvehagen, G. Ricken, L. Kappos, A. Tzankov, S. Hametner,S. Frank, L. Schirmer, and A.-K. Probstel
Open Access
Corrections
e968 Treating MS After Surviving PML: DiscreteStrategies for Rescue, Remission, andRecovery Patient 2: From the NationalMultiple Sclerosis Society Case ConferenceProceedings
e969 Tumefactive Inflammatory Lesions in JuvenileMetachromatic Leukodystrophy
TABLE OF CONTENTS Volume 8, Number 2, March, 2021 Neurology.org/NN
2021;8; Neurol Neuroimmunol Neuroinflamm 8 (2)
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