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Acute cerebral atrophy in autoimmune encephalitis complicated by haemophagocytic lymphohistiocytosis
  1. Qian Wu1,
  2. Shujuan Dai1,
  3. Lin Zhu1 and
  4. Charlie Weige Zhao2
  1. 1Neurology, Kunming Medical University, Kunming, Yunnan, China
  2. 2Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA
  1. Correspondence to Charlie Weige Zhao; weige.zhao{at}


Autoimmune encephalitis is a disease characterised by neural-specific antibodies. This case report presents a 20-year-old young man with a recent history of suspected viral encephalitis who presented with recurrent fevers and episodes of confusion. He was found to have anti-N-methyl-D-aspartate receptor (NMDAR) and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 receptor (AMPAR1) positive autoantibodies and was diagnosed with autoimmune encephalitis. He subsequently developed global cerebral atrophy and was found to meet diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH). This patient’s presentation was consistent with existing literature showing that autoimmune encephalitis may develop after an initial viral meningoencephalitis. However, concurrent anti-NMDAR and anti-AMPAR1 positive autoimmune encephalitis has not been reported in literature to date, and this case report represents one instance of its presentation. We speculate that multiple antibodies against neural surface antigens may increase the risk for systemic immune activation leading to HLH and acute cerebral atrophy.

  • haematology (incl blood transfusion)
  • immunology
  • neurology
  • epilepsy and seizures
  • neuroimaging

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  • Contributors Conception and design: QW, CWZ; data acquisition and analysis: SD, LZ; draft: CWZ; final approval: QW, SD, LZ, CWZ. All authors agree to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved.

  • Funding This study was funded by National Natural Science Foundation of China (81601134).

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.