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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.

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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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