(080) Autoimmune Encephalitis in Youth: The Importance of C-L Psychiatry Advocacy

Location: Griffin Hall

Trainee Involvement: Yes

Presenting Author(s)

Abram Estafanous, DO

Resident Physician
Staten Island University Hospital (Northwell Health)
Staten Island, New York, United States

Co-Author(s)

Elliot Melaney, MD

Resident Physician
Staten Island University Hospital (Northwell)
Staten Island, New York, United States
Jayinee Basu, DO

Resident Physician
Zucker SOM at Northwell - Staten Island University Hospital
Brooklyn, New York, United States
OS

Olusola Segun, MD

Director, Consultation and Liaison Psychiatry
Staten island University Hospital, Northwell
Staten Island, New York, United States
Xavier Jimenez, MD, MA, FACLP

Director, Consultation Liaison Psychiatry
Long Island Jewish Medical Center (Northwell)
New Hyde Park, New York, United States

Background:
Autoimmune encephalitis (AE) is an immune-mediated disorder that can present with neuropsychiatric symptoms along with unremarkable imaging, which may lead to inaccurate diagnoses of primary psychiatric disorders in youth. Consultation-liaison (CL) psychiatrists advocating for early testing of AE may improve outcomes and decrease the risk of adverse events in these young patients (Cellucci et al., 2020).

Cases:
A retrospective review of the medical records of four patients diagnosed with autoimmune encephalitis at a tertiary care center between 2019 and 2023 was conducted. All patients are noted to be under 21.

Case 1: 20-year-old female with no psychiatric history who presented after one week of sudden- onset psychosis and seizures. CT and MRI of the head were unremarkable. CSF studies demonstrated NMDA-receptor antibodies. Transvaginal ultrasound demonstrated an ovarian teratoma, which was surgically removed.

Case 2: 9-year-old male with autism spectrum disorder who presented with new-onset agitation, self-injurious behavior, and altered mental status. MRI of the head was unremarkable. Serum studies showed NMDA-receptor antibodies.

Case 3: 16-year-old female with a history of developmental delay who presented with new- onset behavioral outbursts, aggression, as well as motor and vocal tics. MRI of the head was unremarkable. Serum studies demonstrated anti-VGKC antibodies.

Case 4: 17-year-old female with a past medical history of acute lymphocytic leukemia and no past psychiatric history who presented with mood lability, suicidal ideation, and psychomotor retardation. MRI of the head was unremarkable. Serum studies revealed anti-CV-2 antibodies.

Discussion:

These cases demonstrate the importance of advocating for early immunological testing for young adults and children who present with new-onset psychiatric symptoms and negative head imaging. Effective immunomodulatory treatment for AE is typically started only after diagnostic antibody testing is completed. Delays in diagnosis and treatment in young patients may result in permanent neurocognitive deficits and adverse side-effects such as catatonia from inappropriate antipsychotic use (Sarkis et al., 2019). Thus, CL psychiatrists have an important role in advocating for immunological workup to prevent long-term complications in young patients.

Conclusion:

CL psychiatrists serve an important role in the evaluation of young patients presenting with new-onset neuropsychiatric symptoms. CL psychiatrists may play a vital role in the early diagnosis and treatment of AE in youth by advocating for early immunological testing even when initial head imaging is unremarkable. This would result in decreased long-term morbidity and improved patient outcomes.

References:
Cellucci T, Van Mater H, Graus F, Muscal E, Gallentine W, Klein-Gitelman MS, Benseler SM, Frankovich J, Gorman MP, Van Haren K, Dalmau J, Dale RC. Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. Neurol Neuroimmunol Neuroinflamm. 2020 Jan 17;7(2):e663. doi: 10.1212/NXI.0000000000000663. Erratum in: Neurol Neuroimmunol Neuroinflamm. 2020 Apr 15;7(4): PMID: 31953309; PMCID: PMC7051207.
Sarkis RA, Coffey MJ, Cooper JJ, Hassan I, Lennox B. Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Review of Psychiatric Phenotypes and Management Considerations: A Report of the American Neuropsychiatric Association Committee on Research. J Neuropsychiatry Clin Neurosci. 2019 Spring;31(2):137-142. doi: 10.1176/appi.neuropsych.18010005. Epub 2018 Dec 18. PMID: 30561283.