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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory brainstem syndrome of uncertain etiology, with distinct radiologic features.1 Autoimmunity has been postulated, although specific CNS antibodies have not been reported. Our patient initially presented with classical clinicoradiologic features of CLIPPERS. Five months later, she developed a longitudinally extensive spinal cord inflammatory lesion affecting mainly the conus, and had antibodies to myelin-oligodendrocyte glycoprotein (MOG). Although neuromyelitis optica spectrum disorders (NMOSD) with brainstem involvement may feature in the broad differential diagnosis of CLIPPERS, this is the first report describing an overlap with the anti-MOG phenotype of NMOSD, and highlights that CLIPPERS may not be a distinct nosologic entity.
Footnotes
Author contributions: Mkael Symmonds: drafting/revising the manuscript, study concept or design, accepts responsibility for conduct of research and final approval. Patrick J. Waters: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, contribution of vital reagents/tools/patients, acquisition of data. Wilhelm Kuker: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, acquisition of data. Maria Isabel Leite: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, study supervision. Ursula G.R. Schulz: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval.
Study funding: No targeted funding reported.
Disclosure: M. Symmonds has received a travel grant from UCB Pharma. P. Waters and the University of Oxford hold patents and receive royalties and payments for antibody tests. Dr. Waters has received speaker honoraria from Biogen Idec and Euroimmun AG. He is supported by the NHS National Specialised Commissioning Group for Neuromyelitis Optica and by NIHR Oxford Biomedical Research Centre. W. Kuker reports no disclosures relevant to the manuscript. M. Leite is involved in AQP4 and MOG antibody testing, is supported by NHS National Specialised Commissioning Group for Neuromyelitis Optica and NIHR Oxford Biomedical Research Centre, and has received speaking honoraria from Biogen Idec, Japan, and travel and educational grants from Biogen Idec, UK. U. Schulz is supported by the Oxford Biomedical Research Centre (BRC). Go to Neurology.org for full disclosures.
- Received July 28, 2014.
- Accepted in final form November 18, 2014.
- © 2015 American Academy of Neurology
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Letters: Rapid online correspondence
- Re:CLIPPERS and MOG-opathies
- CLIPPERS and MOG-opathies
- CLIPPERS and MOG-opathies
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