Abstract Details

Title Treatment Resistant DPPX Antibody Associated Encephalitis With an Excellent Response to Combination Therapy of Rituximab and Monthly IVIG

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 025

Objective N/A

Background Dipeptidyl-peptidase-like protein 6 (DPPX) is a subunit of voltage-gated potassium channels that are found on gut and brain neurons. Anti-DPPX encephalitis is a rare disease in which autoantibodies are directed against DPPX. First-line therapy is typically immunotherapy (a course of corticosteroids and plasmapheresis or IVIG) and second-line therapy includes Rituximab or cyclophosphamide. Most patients show substantial improvement with immunotherapy, with a relapse rate of 23%.

Design/Methods N/A

Results

A 29-year-old female presented with loss of appetite, unintentional weight loss (over 100 pounds), profound cognitive impairment, personality changes, easy startle, bradykinesia, apathy, hyperreflexia, cerebellar and extrapyramidal exam findings including a wide-based, unsteady gait, and nystagmus. Prodromal symptoms included episodes of generalized shooting pain, brain fog, vertigo, and bouncy eyes. DPPX titers of 1:7680 and 1:7680 on two separate occasions supported the diagnosis. MRI Brain, EEG, CT chest/abdomen/pelvis, and pelvic ultrasound were normal.

The patient was initially treated with high dose steroids, five sessions of plasmapheresis, and IVIG. She experienced minimal to no improvement. Subsequently, Rituximab infusions were started and also ineffective. She was then started on monthly IVIG alongside Rituximab infusions. The patient is now back to her baseline with monthly IVIG and Rituximab infusions.

Conclusions We present a case of a 29-year-old female diagnosed with DPPX antibody associated encephalitis who had an excellent response to treatment with monthly IVIG and Rituximab infusions 1000 g every 6 months. This report discusses a treatment plan option for patients with DPPX antibody associated encephalitis.

Authors/Disclosures
Gowri Warikoo PRESENTER	Ms. Warikoo has nothing to disclose.
Afeerah Malik, MD (University of MIssouri Kansas City)	Dr. Malik has nothing to disclose.
Rola Mahmoud, MD (Kansas City Physician Partners)	Dr. Mahmoud has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeutics, EMD Serono, Amgen and Sanofi . Dr. Mahmoud has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for EMD sorono.

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