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Case Reports
. 2025 Apr 2:12:100320.
doi: 10.1016/j.prdoa.2025.100320. eCollection 2025.

Anti-dipeptidyl-peptidase-like protein-6 (DPPX) autoimmune encephalitis associated with severe multifocal dystonia

Eric Roddy  1 , Erik Gentry  1 , Victoria Holiday  1 , David Robertson  2 , Peter Hedera  1
Affiliations
Case Reports

Anti-dipeptidyl-peptidase-like protein-6 (DPPX) autoimmune encephalitis associated with severe multifocal dystonia

Eric Roddy et al. Clin Park Relat Disord. .

Abstract

Anti-DPPX encephalitis is a rare form of autoimmune encephalitis characterized antibodies against a subunit of Kv4.2 potassium channels. Characteristic clinical features include cognitive dysfunction, parasomnias, psychosis, and seizures. Motor symptoms typically include myoclonus, tremor, and midline ataxia. DPPX encephalitis presenting as new-onset focal dystonia has not been previously described.

Keywords: Autoimmune encephalitis; DPPX; Dystonia; Immunotherapy; Toxin therapy.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

References

    1. Hsien P.-C., Wu Y.-R. Diagnosis and Clinical Features in Autoimmune-Mediated Movement Disorders. J Mov Disord. 2022;26(15):95–105. - PMC - PubMed
    1. Balint B., Vincent A., Meinck H.M., et al. Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology. Brain. 2018;141:13–36. - PMC - PubMed
    1. Lin J., Zhu M., Mao X., et al. Anti-dipeptidyl-peptidase-like protein 6 encephalitis with pure cerebellar ataxia: a case report. BMC Neurol. 2022;22:242. - PMC - PubMed
    1. Gövert F., Leypoldt F., Junker R., et al. Antibody-Related Movement Disorders – a Comprehensive Review of Phenotype-Autoantibody Correlations and a Guide to Testing. Neurol Res Pract. 2020;20(2):6. - PMC - PubMed
    1. Boronat A., Gelfand J.M., 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. - PMC - PubMed

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