Joshua Chin Ern Ooi, Lip Sheng Tan, Yun Xiu Yong, Maizatul Idazarina Mahadi, Yi Lin Tang, Jack Son Wee, Sheila Ai Mei Ong, Shirley Lee, Presaad Pillai, Yuen Kang Chia
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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.
期刊介绍:
The essential point of Practical Neurology is that it is practical in the sense of being useful for everyone who sees neurological patients and who wants to keep up to date, and safe, in managing them. In other words this is a journal for jobbing neurologists - which most of us are for at least part of our time - who plough through the tension headaches and funny turns week in and week out. Primary research literature potentially relevant to routine clinical practice is far too much for any neurologist to read, let alone understand, critically appraise and assimilate. Therefore, if research is to influence clinical practice appropriately and quickly it has to be digested and provided to neurologists in an informative and convenient way.