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Abstract Details

DPPX autoimmune encephalitis presenting with chronic diarrhea, multidirectional nystagmus, opsoclonus and prominent hyperekplexia
Autoimmune Neurology
P11 - Poster Session 11 (8:00 AM-9:00 AM)
15-004
To highlight the classical clinical features and diagnostic challenges associated with Dipeptidyl peptidase like protein-6 (DPPX) encephalitis in a patient who was treated at Mayo Clinic Arizona.
Dipeptidyl-peptidase-like protein-6 (DPPX) autoimmune encephalitis (DPPX) is a rare autoimmune disorder. Diagnosis is often delayed due to its varied presenting features.

A case report
We present the case of a 48-year-old woman evaluated at our facility due to a 12 month history of multiple neurological complaints, protracted diarrhea, and 50-pound weight loss.  Neurological symptoms included diplopia, slowed mentation, brain fog, tremors and an exaggerated startle response. Evaluation prior to presentation at our facility included upper and lower gastrointestinal endoscopies, which were nonrevealing. Neurological evaluation at our facility revealed multidirectional nystagmus with a torsional component, upper and lower extremity tremulousness, and gait instability. Magnetic resonance imaging of the brain and spinal cord was normal. Cerebrospinal fluid (CSF) analysis showed a mild lymphocytic pleocytosis with normal glucose and protein. Murine tissue-based indirect immunofluorescence assay of serum and CSF demonstrated a synaptic pattern of IgG binding characteristic of DPPX antibody (end-point dilution 1:15360 and 1:16 respectively), with confirmation by DPPX-specific cell-binding assay. This established a diagnosis of DPPX encephalitis. The patient was treated with 5 days of methylprednisolone and 7 cycles of therapeutic plasma exchange. This was then transitioned to rituximab infusions, which she continues to receive in the outpatient setting. One month after initiation of treatment, the patient reported significant symptomatic improvement.
A diagnosis of DPPX autoimmune encephalitis should be considered in patients presenting with a chronic encephalitic syndrome of unclear etiology. Patients typically present with a triad of encephalitis, signs of CNS hyperexcitability and chronic diarrhea with or without weight loss.
Authors/Disclosures
Karissa Arca, MD (Mayo Clinic)
PRESENTER 		The institution of Dr. Arca has received research support from Miles for Migraine. Dr. Arca has received personal compensation in the range of $0-$499 for serving as a speaker with Miles for Migraine.
Amir A. Mbonde, MD Dr. Mbonde has nothing to disclose.
David Gritsch, MD, PhD Dr. Gritsch has nothing to disclose.
Benzion Blech, MD Dr. Blech has nothing to disclose.
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Jonathan L. Carter, MD (Mayo Clinic) Dr. Carter has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lundbeck Pharmaceuticals A/S. The institution of Dr. Carter has received research support from MedDay Pharmaceuticals . The institution of Dr. Carter has received research support from Roche Pharmaceuticals.