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Case Reports: Rare disease
Inflammatory epilepsy (FIRES) and haemophagocytic lymphohistiocytosis (HLH): an adult case
  1. Ian Kam1,
  2. David Prentice2,
  3. Lay Kun Kho1 and
  4. Ferry Dharsono3
  1. 1Neurology, Royal Perth Hospital, Perth, Western Australia, Australia
  2. 2Neurology, Perron Institute for Neurological and Translational Science, Nedlands, Western Australia, Australia
  3. 3The Neurological Intervention & Imaging Service of Western Australia, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia
  1. Correspondence to Dr Ian Kam; iantingxiang.kam{at}gmail.com

Abstract

We describe a man in his 30s with haemophagocytic lymphohistiocytosis (HLH), secondary to an upper respiratory tract infection, with subsequent febrile infection-related epilepsy syndrome. He had a prolonged hospital admission, during which he was treated with chemotherapy for HLH and antiepileptic medications for refractory seizures. He was discharged fully dependent to a care facility and died from aspiration pneumonia 11 months later. This case report highlights his management and discusses these conditions’ pathophysiology and future management.

  • Epilepsy and seizures
  • Immunology

https://doi.org/10.1136/bcr-2022-252637

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    Footnotes

    • Contributors LKK, DP and IK conceived and planned the case report. IK gathered the information and wrote the manuscript with support from DP. LKK supervised and proof read the case report. FD provided the imaging highlighting the patient’s disease and recovery. All authors discussed and commented on the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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