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
Journal:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Year of publication: 2023

Issue Title: Enfermedades del sistema nervioso (IX): Enfermedades desmielinizantes

Series: 13

Issue: 78

Pages: 4641-4645

Type: Article

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More publications in: Medicine: Programa de Formación Médica Continuada Acreditado

Abstract

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.

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