Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus

Movement Disorders Clinical PracticeEarly View CASE REPORT Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus Ray Jen Neo MBBS, MRCP, Ray Jen Neo MBBS, MRCP Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Department of Neurology, Hospital Kuala Lumpur, Kuala Lumpur, MalaysiaSearch for more papers by this authorArpan R. Mehta BM BCh, MA, MRCP, PhD, Arpan R. Mehta BM BCh, MA, MRCP, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorMikail Weston MB BCh BaO, MRCP, Mikail Weston MB BCh BaO, MRCP Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorFrancesca Magrinelli MD, PhD, Francesca Magrinelli MD, PhD orcid.org/0000-0003-4706-6245 Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorAndrea Quattrone MD, PhD, Andrea Quattrone MD, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Department of Medical and Surgical Sciences, Institute of Neurology, Magna Graecia University of Catanzaro, Catanzaro, ItalySearch for more papers by this authorSonia Gandhi MD, PhD, Sonia Gandhi MD, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorEileen M. Joyce MA, PhD, MRCP, FRCPsych, Eileen M. Joyce MA, PhD, MRCP, FRCPsych Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorKailash P. Bhatia MD, DM, FRCP, Corresponding Author Kailash P. Bhatia MD, DM, FRCP [email protected] orcid.org/0000-0001-8185-286X Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Correspondence to: Professor Kailash P. Bhatia, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK; E-mail: [email protected]Search for more papers by this author Ray Jen Neo MBBS, MRCP, Ray Jen Neo MBBS, MRCP Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Department of Neurology, Hospital Kuala Lumpur, Kuala Lumpur, MalaysiaSearch for more papers by this authorArpan R. Mehta BM BCh, MA, MRCP, PhD, Arpan R. Mehta BM BCh, MA, MRCP, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorMikail Weston MB BCh BaO, MRCP, Mikail Weston MB BCh BaO, MRCP Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorFrancesca Magrinelli MD, PhD, Francesca Magrinelli MD, PhD orcid.org/0000-0003-4706-6245 Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorAndrea Quattrone MD, PhD, Andrea Quattrone MD, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Department of Medical and Surgical Sciences, Institute of Neurology, Magna Graecia University of Catanzaro, Catanzaro, ItalySearch for more papers by this authorSonia Gandhi MD, PhD, Sonia Gandhi MD, PhD Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorEileen M. Joyce MA, PhD, MRCP, FRCPsych, Eileen M. Joyce MA, PhD, MRCP, FRCPsych Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSearch for more papers by this authorKailash P. Bhatia MD, DM, FRCP, Corresponding Author Kailash P. Bhatia MD, DM, FRCP [email protected] orcid.org/0000-0001-8185-286X Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom Correspondence to: Professor Kailash P. Bhatia, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK; E-mail: [email protected]Search for more papers by this author First published: 15 November 2023 https://doi.org/10.1002/mdc3.13928 Relevant disclosures and conflict of interest are listed at the end of this article. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat References 1Meinck HM, Thompson PD. Stiff man syndrome and related conditions. Mov Disord 2002; 17: 853–866. 10.1002/mds.10279 CASPubMedWeb of Science®Google Scholar 2Chang A, Lin KY, Chuang KJ, et al. Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature. Clin Neurol Neurosurg 2021; 208:106807. 10.1016/j.clineuro.2021.106807 PubMedWeb of Science®Google Scholar 3Balint B, Jarius S, Nagel S, et al. Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies. Neurology 2014; 82: 1521–1528. 10.1212/WNL.0000000000000372 CASPubMedWeb of Science®Google Scholar 4Wada K, Yokotani N, Hunter C, Doi K, Wenthold RJ, Shimasaki S. Differential expression of two distinct forms of mRNA encoding members of a dipeptidyl aminopeptidase family. Proc Natl Acad Sci U S A 1992; 89: 197–201. 10.1073/pnas.89.1.197 CASPubMedWeb of Science®Google Scholar 5Nadal MS, Ozaita A, Amarillo Y, et al. The CD26-related dipeptidyl aminopeptidase-like protein DPPX is a critical component of neuronal A-type K1 channels. Neuron 2003; 37: 449–461. 10.1016/S0896-6273(02)01185-6 CASPubMedWeb of Science®Google Scholar 6Clark BD, Kwon E, Maffie J, et al. DPP6 localization in brain supports function as a Kv4 channel associated protein. Front Mol Neurosci 2008; 1: 8. 10.3389/neuro.02.008.2008 CASPubMedWeb of Science®Google Scholar 7Boronat A, Gelfand JM, Gresa-Arribas N, et al. Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6, a subunit of Kv4.2 potassium channels. Ann Neurol 2013; 73: 120–128. 10.1002/ana.23756 CASPubMedWeb of Science®Google Scholar 8Xiao J, Pei-cai F, Li Z-J. Clinical and imaging analysis to evaluate the response of patients with anti-DPPX encephalitis to immunotherapy. BMC Neurol 2022; 22: 129. 10.1186/s12883-022-02649-7 CASPubMedWeb of Science®Google Scholar Early ViewOnline Version of Record before inclusion in an issue ReferencesRelatedInformation