Anti DPPX autoimmune encephalitis: neurological features

Evidence-based neurology checklist on anti dppx autoimmune encephalitis: neurological features: 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…

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

Sleep disturbances

Ophthalmic features

Other neurological features

Differential diagnosis

Prognosis

Acronym

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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