Autoimmune encephalitis and immune therapy: lessons from Argentina

Acta Neurol Belg. 2020 Jun;120(3):565-572. doi: 10.1007/s13760-018-1013-x. Epub 2018 Sep 4.

Abstract

Autoimmune encephalitis (AE) is a common cause of noninfectious acute encephalitis. We aimed to provide the first review of immune therapy regimens used for patients with AE in Latin America, as well as the safety and efficacy associated with them, by reviewing the medical records of Argentine patients with AE treated between 2013 and 2018. Data included clinical symptoms, laboratory tests, electroencephalography, magnetic resonance imaging, cerebral spinal fluid, and neoplasm screenings. We examined ten AE patients who received first-line immunotherapy at a median of 2.5 months following symptom onset. Among these patients, five required second-line treatment: three received therapy at a median of 4 months (2-112) after symptom onset and were treated with rituximab, while two received therapy at a median of 4.5 (4-5) months after onset and received methylprednisolone for 6 months and initiated chronic treatment with azathioprine. By the last follow-up, their respective outcomes improved significantly. On the modified Rankin Scale, the median score decreased from 5 to 1 (p ≤ 0.05). Only two of the ten patients in our series experienced relapses; both had been treated with a combination of methylprednisolone and IVIG. The regimen after recurrence included rituximab and corticoids plus azathioprine. Neither patient had experienced another relapse by their last follow-up. Our findings demonstrate the importance of early and aggressive immunosuppressive therapy to achieve a good clinical outcome and a fast recovery without relapses.

Keywords: Autoimmune encephalitis; Encephalitis; Immunotherapy; Prognosis.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Argentina
  • Autoimmune Diseases / drug therapy*
  • Azathioprine / therapeutic use
  • Child
  • Cyclophosphamide / therapeutic use
  • Encephalitis / drug therapy*
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use*
  • Immunotherapy / methods*
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Plasma Exchange / methods
  • Rituximab / therapeutic use
  • Treatment Outcome
  • Young Adult

Substances

  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Rituximab
  • Cyclophosphamide
  • Azathioprine
  • Methylprednisolone