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Volume 8, Number 2, March 2021 Neurology.org/NN A peer-reviewed clinical and translational neurology open access journal ARTICLE Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences 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 Inflammation in the CNS e946 ARTICLE Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies e947

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Page 1: Unilateral Relapsing Primary Angiitis of the CNS: An

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

Page 2: Unilateral Relapsing Primary Angiitis of the CNS: An

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

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A peer-reviewed clinical and translational neurology open access journal Neurology.org/NN

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Page 4: Unilateral Relapsing Primary Angiitis of the CNS: An

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

Page 5: Unilateral Relapsing Primary Angiitis of the CNS: An

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

Page 6: Unilateral Relapsing Primary Angiitis of the CNS: An

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