Anti DPPX autoimmune encephalitis: neurological features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Anti DPPX autoimmune encephalitis: neurological features. This is caused by antibodies to DPPX. Fully referenced neurology checklist.

Pathology

  • This is caused by antibodies to DPPX
  • DPPX is a neuronal surface autoantigen
  • It is a regulatory subunit of the voltage gated potassium channel (VGKC) complex

Features of central hyperexcitability

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Sleep disturbances

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Ophthalmic features

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Other neurological features

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Differential diagnosis

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Prognosis

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Acronym

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References

  1. Doherty L, Gold D, Solnes L, Probasco J, Venkatesan A. Anti-DPPX encephalitis: prominent nystagmus reflected by extraocular muscle FDG-PET avidity. Neurol Neuroimmunol Neuroinflamm 2017; 4:e361. 
  2. Hara M, Ariño H, Petit-Pedrol M, et al. DPPX antibody-associated encephalitis: Main syndrome and antibody effects. Neurology 2017; 88:1340-1348. 
  3. Tobin WO, Lennon VA, Komorowski L, et al. DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients. Neurology 2014; 83:1797-1803. 
  4. Micieli JA, Newman NJ, Kase CS, Biousse V. Teaching Video NeuroImages: Opsoclonus in anti-DPPX encephalitis. Neurology 2019; 92:e2298. 
  5. Gadoth A, Devine MF, Pittock SJ, et al. Sleep disturbances associated with DPPX autoantibodies: a case series. J Neurol 2023; 270:3543-3552.
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