Encefalomielitis diseminada aguda, síndrome de Susac y astrocitopatía anti-PGFA

  1. Alberte Woodward, M. 1
  2. Pouso Diz, J.M. 1
  3. Prieto González, J.M.Ó 1
  1. 1 Servicio de Neurología, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, La Coruña, España
Revista:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Año de publicación: 2023

Título del ejemplar: Enfermedades del sistema nervioso (IX): Enfermedades desmielinizantes

Serie: 13

Número: 78

Páginas: 4641-4645

Tipo: Artículo

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Otras publicaciones en: Medicine: Programa de Formación Médica Continuada Acreditado

Resumen

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease more common in children than in adults, often preceded by a nonspecific infectious process. It is typically characterized by encephalopathy and multifocal symptoms and signs and, in some cases, may take a more aggressive form such as acute hemorrhagic leukoencephalitis. A substantial number of patients will be positive for IgG-MOG (especially in children), and to a lesser extent for IgG-AQP4. ADEM is usually monophasic, but recurrent and/or persistent seropositive forms will require chronic immunomodulation. Susac syndrome is an inflammatory endotheliopathy characterized by occlusion of retinal arterial branches, hearing loss and encephalopathy, with no known biomarker and with neuroimaging findings amenable to differential diagnosis with multiple sclerosis. Anti-glial fibrillary acidic protein astrocytopathy (PGFA) can present with meningitis, encephalitis, myelitis, papillitis and epilepsy, is paraneoplastic in a third of cases and has an exquisite response to corticosteroids.

Referencias bibliográficas

  • Chitnis T. Pediatric central nervous system demyelinating diseases. Continuum (Minneap Minn). 2019;25(3):793-814.
  • Brenton JN. Pediatric acquired demyelinating disorders. Continuum (Minneap Minn). 2022;28(4):1104-30.
  • Tenembaum SN. Acute disseminated encephalomyelitis. Handb Clin Neurol. 2013;112:1253-62.
  • Anilkumar AC, Foris LA, Tadi P. Acute disseminated encephalomyelitis. En: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022
  • Wang Y, Wang Y, Huo L, Li Q, Chen J, Wang H. SARS-CoV-2 associated acute disseminated encephalomyelitis: a systematic review of the literature. J Neurol. 2022;269(3):1071-92.
  • Noorbakhsh F, Johnson RT, Emery D, Power C. Acute disseminated encephalomyelitis: Clinical and pathogenesis features. Neurol Clin. 2008;26(3):759-80
  • Baxter R, Lewis E, Goddard K, Fireman B, Bakshi N, DeStefano F. Acute demyelinating events following vaccines: A case-cen- tered analysis. Clin Infect Dis. 2016;63(11):1456-62.
  • Karussis D. The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 2014;48-49:134-42.
  • Boski N, Gulati V, Raj R, Gulati P. Multiple sclerosis-minimizing errors in radiological diagnosis. Neurol India. 2021;69(6):1539-46.
  • Bruijstens AL, Lechner C, Flet Berliac L, Deiva K, Neuteboom RF, Hemingway C. E.U. paediatric MOG consortium consensus: Part 1 –Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders. Eur J Paediatr Neurol. 2020;29:2-13.
  • Ketelslegers IA, Visser IER, Neuteboom RF, Boon M, Catsman Berrevoets CE, Hintzen RQ. Disease course and outcome of acute disseminated encephalomyelitis is more severe in adults than in children. Mult Scler. 2011;17(4):441-8.
  • Flanagan EP. Neuromyelitis optica spectrum disorder and other non-multiple sclerosis central nervous system inflammatory diseases. Continuum (Minneap Minn). 2019;25(3):815-44.
  • Longbrake E. Myelin oligodendrocyte glycoprotein-associated disorders. Continuum (Minneap Minn). 2022;28(4):1171-93.
  • Marrodan M, Fiol MP, Correale J. Susac syndrome: challenges in the diagnosis and treatment. Brain. 2022;145(3):858-71.
  • David C, Sacré K, Henri Feugeas MC, Klein I, Doan S, Cohen FA. Susac syndrome: A scoping review. Autoimmun Rev. 2022;21(6):103097.
  • van Asperen JV, Robe PAJT, Hol EM. GFAP Alternative splicing and the relevance for disease – A focus on diffuse gliomas. ASN Neuro. 2022;14:17590914221102064.
  • Flanagan EP, Hinson SR, Lennon VA, Fang B, Aksamit AJ, Morris PP. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients. Ann Neurol. 2017;81(2):298-309.
  • Iorio R, Damato V, Evoli A, Gessi M, Gaudino S, Di Lazzaro V. Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients. J Neurol Neurosurg Psychiatry. 2018;89(2):138-46.
  • Chen JJ, Aksamit AJ, McKeon A, Pittock SJ, Weinshenker BG, Leavitt JA. Optic disc edema in glial fibrillary acidic protein autoantibody-positive meningoencephalitis. J Neuroophthalmol. 2018;38(3):276-81.
  • Issa N, Martin C, Dulau C, Camou F. Severe anti-GFAP meningo-encephalomyelitis following viral infection. Mult Scler Relat Disord. 2020;45:102448.
Fuente de los datos: Dialnet