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A case of Rasmussen encephalitis treated with rituximab

Nature Reviews Neurology volume 5, pages 458–462 (2009)Cite this article

Abstract

Background. A 20-year-old woman was referred to our intensive care unit with a high frequency (every 1–2 min) of focal motor seizures. She had been diagnosed as having biopsy-proven Rasmussen encephalitis (RE) of the right hemisphere 7 years previously, since when she had been treated with numerous antiepileptic drugs, as well as with continuous immunotherapies, such as tacrolimus, corticosteroids, mycophenolate mofetil, intravenous immunoglobulin and immunoadsorption. Although hemispherectomy had been avoided due to slow progression of RE, she had not been seizure-free for more than 7 weeks since diagnosis.

Investigations. EEG and MRI.

Diagnosis. Focal motor status epilepticus associated with right hemispheric RE, causing continuous epileptic activity and unilateral atrophy with edema in the right hemisphere.

Management. Immunoadsorption was used initially to treat the seizures. Once they had ceased, we used 375 mg/m2 intravenous rituximab—a monoclonal anti-CD20 antibody—once-weekly for 4 weeks to stabilize the condition, leading to complete depletion of CD19+ B cells. Rituximab infusions were used again when concentrations of CD19+ B cells rose and focal seizures re-emerged. The patient remained on antiepileptic therapy (levetiracetam, oxcarbazepine, zonisamide and phenobarbital) throughout treatment.

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Figure 1: Graph depicting development of hemispheric atrophy in a patient with Rasmussen encephalitis.
Figure 2: Therapeutic approaches and epileptic activity in a patient with Rasmussen encephalitis.

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Acknowledgements

We thank our patient and her family for their help and cooperation in preparing this report. Written consent for publication was obtained from the patient. We are grateful to Dr. Rainald Zeuner, from the 2nd Medical Department at the Christian-Albrechts-University in Kiel, for his practical advice regarding rituximab treatment in this particular case.

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Authors and Affiliations

  1. Department of Neurology, Christianalbrechts-University, Kiel, Germany

    Barbara Thilo, Robert Stingele, Karina Knudsen, Günther Deuschl & Nicolas Lang

  2. Department of Neuropediatrics, Christianalbrechts-University, Kiel, Germany

    Rainer Boor

  3. Department of Epileptology, University of Bonn, Bonn, Germany

    Christian G. Bien

Authors
  1. Barbara Thilo
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  2. Robert Stingele
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  3. Karina Knudsen
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  4. Rainer Boor
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  5. Christian G. Bien
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  6. Günther Deuschl
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  7. Nicolas Lang
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Correspondence to Barbara Thilo.

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Thilo, B., Stingele, R., Knudsen, K. et al. A case of Rasmussen encephalitis treated with rituximab. Nat Rev Neurol 5, 458–462 (2009). https://doi.org/10.1038/nrneurol.2009.98

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