Interdisciplinary Neurosurgery (Mar 2021)

ANTI-MA2 encephalitis mimicking diencephalic demyelinating syndrome

  • Agábio Diógenes Pessoa Neto,
  • Bárbara Cristina Vieira de Aquino,
  • Paulo Santiago de Morais Brito,
  • Rodrigo de Alencar e Silva,
  • Manuel Moreira Neto,
  • Orlando Graziani Povoas Barsottini,
  • Lívia Almeida Dutra,
  • Clécio de Oliveira Godeiro Junior

Journal volume & issue
Vol. 23
p. 100980

Abstract

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Anti Ma 2 encephalitis is an autoimmune encephalitis, usually paraneoplastic, characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. Limbic encephalitis (LE) is the most common manifestation. Anti-Ma2 LE can also be manifested as a pure psychiatric disturbance associated or not with hypokinesia. Other patients exhibit excessive daytime sleepiness, vertical ophthalmoparesis, or both associated with LE, due to diencephalic and/or upper brainstem involvement. These cases are challenging and demand extensive work-up for differential diagnosis.

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