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

**Authors:** Eric Roddy, Erik Gentry, Victoria Holiday, David Robertson, Peter Hedera

PMC · DOI: 10.1016/j.prdoa.2025.100320 · Clinical Parkinsonism & Related Disorders · 2025-04-02

## TL;DR

This paper reports a rare case of anti-DPPX encephalitis presenting with severe dystonia, a symptom not previously associated with this condition.

## Contribution

The study identifies dystonia as a novel clinical manifestation of anti-DPPX encephalitis.

## Key findings

- Dystonia can be a presenting feature of anti-DPPX encephalitis.
- Immunosuppressive therapy is effective in treating anti-DPPX encephalitis.
- Botulinum toxin may help alleviate dystonia symptoms in this condition.

## Abstract

•Anti-DPPX encephalitis is characterized by complex symptoms, including movement disorder.•Dystonia has not been described as a phenomenon of anti-DPPX encephalitis.•Immunosuppressive therapy is the mainstay of treatment for anti-DPPX encephalitis.•Botulinum toxin administration may improve the symptoms of dystonia in anti-DPPX encephalitis.

Anti-DPPX encephalitis is characterized by complex symptoms, including movement disorder.

Dystonia has not been described as a phenomenon of anti-DPPX encephalitis.

Immunosuppressive therapy is the mainstay of treatment for anti-DPPX encephalitis.

Botulinum toxin administration may improve the symptoms of dystonia in anti-DPPX encephalitis.

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.

## Linked entities

- **Proteins:** DPP6 (dipeptidyl peptidase like 6), KCND2 (potassium voltage-gated channel subfamily D member 2)
- **Diseases:** dystonia (MONDO:0003441), psychosis (MONDO:0005485)

## Full-text entities

- **Genes:** DPP6 (dipeptidyl peptidase like 6) [NCBI Gene 1804] {aka DPL1, DPPX, MRD33, VF2}, KCND2 (potassium voltage-gated channel subfamily D member 2) [NCBI Gene 3751] {aka KV4.2, RK5}
- **Diseases:** tremor (MESH:D014202), psychosis (MESH:D011618), autoimmune encephalitis (MESH:D020274), dystonia (MESH:D004421), seizures (MESH:D012640), myoclonus (MESH:D009207), ataxia (MESH:D001259), cognitive dysfunction (MESH:D003072), DPPX encephalitis (MESH:D004660), Anti- (MESH:D006679), parasomnias (MESH:D020447)

## Full text

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## References

7 references — full list in the complete paper: https://tomesphere.com/paper/PMC12002181/full.md

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Source: https://tomesphere.com/paper/PMC12002181