Adult-onset Rasmussen’s encephalitis with anti-AMPA receptor antibodies

A woman aged 49 years developed focal seizures, with right hemispheric focal slowing on electroencephalograph and right hemisphere focal cortical hyperintensities with subtle atrophy on MR brain scan. Cerebrospinal fluid identified anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor auto-antibodies, but her condition did not respond to immunotherapy for AMPA receptor encephalitis. Subsequent MR brain scan showed striking progressive unihemispheric atrophy, consistent with Rasmussen's encephalitis; she made a good functional recovery with cyclophosphamide. This case highlights the diagnostic and treatment challenges surrounding adult-onset Rasmussen's encephalitis. The unusual co-occurrence of anti-AMPA receptor autoantibody positivity emphasises the need for careful interpretation of such associations. It is important to think of Rasmussen's encephalitis as a spectrum of diseases with shared T-cell-mediated neuronal loss pathways but varying humoral components to understand this complex disease better.

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Publication Details

Journal
Practical Neurology
Published
2025-04-01
DOI
https://doi.org/10.1136/pn-2025-004520
Primary Topic
Neuroscience and Neuropharmacology Research
Type
article
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article

Adult-onset Rasmussen’s encephalitis with anti-AMPA receptor antibodies

Presaad Pillai, Yi Tang, Yuen Kang Chia, Sheila Ong et al.
Practical Neurology
Neuroscience and Neuropharmacology Research
article

Adult-onset Rasmussen’s encephalitis with anti-AMPA receptor antibodies

Presaad Pillai, Yi Tang, Yuen Kang Chia, Sheila Ong, Joshua Chin Ern Ooi, Yun Xiu Yong, Jack Son Wee, Shirley Lee, L Tan, Maizatul Idazarina Mahadi
article en

Abstract

A woman aged 49 years developed focal seizures, with right hemispheric focal slowing on electroencephalograph and right hemisphere focal cortical hyperintensities with subtle atrophy on MR brain scan. Cerebrospinal fluid identified anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor auto-antibodies, but her condition did not respond to immunotherapy for AMPA receptor encephalitis. Subsequent MR brain scan showed striking progressive unihemispheric atrophy, consistent with Rasmussen's encephalitis; she made a good functional recovery with cyclophosphamide. This case highlights the diagnostic and treatment challenges surrounding adult-onset Rasmussen's encephalitis. The unusual co-occurrence of anti-AMPA receptor autoantibody positivity emphasises the need for careful interpretation of such associations. It is important to think of Rasmussen's encephalitis as a spectrum of diseases with shared T-cell-mediated neuronal loss pathways but varying humoral components to understand this complex disease better.

Practical NeurologyVol. 25(5)
Queen Elizabeth Hospital (MY)
Good health and well-being
Openalex Percentile: Top 93%
Neuroscience and Neuropharmacology Research
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Adult-onset Rasmussen’s encephalitis with anti-AMPA receptor antibodies — Presaad Pillai, Yi Tang, et al. · Practical Neurology (2025) | TGRS Research Map | TGRS