MOG Antibody-associated Hemorrhagic Acute Demyelinating Encephalo-myelitis (ADEM) with Elevated CSF IL-6 After Tdap Vaccination
Carson Moseley1, Akash Virupakshaiah1, Steven Elicegui2, Lee Gerwitz3, Elizabeth George1, Maulik Shah1, Michael Wilson1, Emmanuelle Waubant1, Scott Zamvil1
1Neurology, University of California, San Francisco, 2University of Nevado Reno School of Medicine, 3Renown Regional Medical Center
Objective:

To highlight the importance of CSF cytokine testing in management of MOGAD


Background:

It has been documented that infections can trigger myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We present a unique case of hemorrhagic acute demyelinating encephalo-myelitis that emerged after a recent Tdap vaccination.


Design/Methods:

A 35-year-old man, who had received a Tdap vaccination 14 days prior, presented to urgent care with headaches and retro-orbital pain. Initially diagnosed with migraines and prescribed medication, he returned three days later with persistent symptoms, including new-onset urinary retention, visual disturbance, and vertigo. MRI revealed specific lesions, and initial CSF analysis indicated elevated protein and leukocytosis without oligoclonal bands. Treatment with IV methylprednisolone was followed by a discharge, but symptoms worsened, requiring additional IVMP and oral prednisone. As his neurological status deteriorated, he was intubated and transferred to a tertiary care center for further management.

Results:

Repeat MRI showed multifocal T2 hyperintensities, associated hemorrhage, thrombosis of internal cerebral veins and venous sinuses.  CSF showed WBC of 87 cells/µl (85% polys), 89 mg/dl protein. MOG-IgG by CBA returned positive with a titer level of 1:1000, and CSF interleukin-6 (IL-6) was 17,904.6 pg/ml. As the patient did not respond to IV steroids, he underwent plasma exchange, and improved. The cerebral sinus venous thrombosis was presumed secondary to inflammation due to MOGAD. To prevent further relapses, he was started on tocilizumab. He has not experienced relapses and has sustained to improvement. 

Conclusions:

This case showcases a remarkable instance of MOG IgG-associated hemorrhagic ADEM with significantly elevated CSF IL-6 following Tdap vaccination. The association of Tdap vaccination with ADEM and TM prompts consideration of its role in precipitating this patient's condition. Emphasizing the importance of testing CSF cytokines, including IL-6, in new onset MOGAD, our findings endorse the use of anti-IL-6R monoclonal antibody therapy in MOGAD.


10.1212/WNL.0000000000208329