Long term outcomes in patients with anti-DPPX autoimmunity

Area of Science:

• Neurology
• Immunology
• Neuroimmunology

Background:

• DDPX antibody-associated encephalitis presents with cognitive, neuropsychiatric, and central nervous system hyperexcitability symptoms.
• Prodromal gastrointestinal issues like weight loss and diarrhea are common.
• Limited data exists on the long-term prognosis of this condition.

Purpose of the Study:

• To evaluate the long-term outcomes of patients diagnosed with DDPX antibody-associated encephalitis.

Main Methods:

• Retrospective analysis of six patients with positive anti-DPPX antibodies in serum and CSF.
• Inclusion criteria required a minimum follow-up of 36 months from symptom onset.
• Assays included cell-based and mouse tissue-based immunofluorescence.

Main Results:

• All patients achieved resolution of gastrointestinal symptoms.
• 4/6 (67%) experienced residual cognitive impairment.
• 50% achieved clinical stability with immunotherapy; 33% discontinued immunotherapy and remained stable.
• One patient died from an unclear cause.

Conclusions:

• Long-term outcomes for anti-DPPX encephalitis are favorable, with significant symptom improvement possible.
• While immunotherapy can aid recovery, complete return to baseline function is uncommon.
• Residual cognitive deficits are a frequent long-term consequence.