Application of immunotherapy for neurological manifestations in hypermobile Ehlers-Danlos syndrome

Therapeutic Advances in Neurological Disorders
Manabu ArakiTakashi Yamamura

Abstract

Ehlers-Danlos syndrome (EDS) is a heterogeneous heritable connective tissue disorder with various neurological manifestations, including chronic pain. The neurological manifestations in EDS are often regarded as being caused by the associated musculoskeletal disorders or polyneuropathy. Here, we present two patients with hypermobile EDS (hEDS), presenting with relapsing central nervous system (CNS) manifestations. Although the two patients showed relapsing signs of CNS manifestations like multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD), they were unique in that they had widespread opioid-dependent chronic pain, which is not consistent with the symptoms of MS/NMOSD. Unexpectedly, the serious pain of unknown origin was remarkably mitigated by plasmapheresis, and magnetic resonance imaging (MRI) examinations conducted for one of the patients were negative. Collectively, we speculate that hEDS may be more susceptible to 'normal-appearing imaging, neuroimmunologically justified, autoimmune-mediated encephalomyelitis (NINJA).' Analysis of the presented cases and an additional three patients with EDS with chronic pain indicates that treatable immune-mediated mechanisms deserve considerations for neurological ...Continue Reading

References

Dec 20, 2002·Nature·Christophe Benoist, Diane Mathis
Jan 23, 2008·Multiple Sclerosis : Clinical and Laboratory Research·J VilisaarC S Constantinescu
Jun 27, 2009·Annals of Neurology·Nicol C VoermansBaiel G van Engelen
Jun 29, 2010·Journal of Pain and Symptom Management·Nicol C VoermansBaziel G van Engelen
Feb 16, 2011·Proceedings of the National Academy of Sciences of the United States of America·Norio ChiharaTakashi Yamamura
Jul 15, 2015·The New England Journal of Medicine·Theoharis C TheoharidesCem Akin
Nov 1, 2016·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Ruwan A WeerakkodyTimothy J Aitman
Feb 22, 2017·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Fraser C HendersonNicol C Voermans
Mar 7, 2017·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Suranjith L SeneviratneLawrence Afrin
Mar 18, 2017·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Fransiska MalfaitBrad Tinkle
Apr 5, 2018·Neurology. Neuroimmunology and Neuroinflammation·Daiki TakewakiTakashi Yamamura

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Citations

Jul 23, 2019·Haemophilia : the Official Journal of the World Federation of Hemophilia·Rohith JesudasChristina M Laukaitis

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Methods Mentioned

BETA
flow cytometry
biopsy

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