Emma Johnson1, Antoniya Marinova Doynova2, Natalie Moreo2, Sagarika Nallu2
1USF MCOM, 2University of South Florida
Background:
A twelve-year-old girl reported an acute transient two-minute headache and stiff neck followed by transient two hour left facial droop, dysarthria, and left extremities paresthesia that prompted negative stroke work up and discharge. Headaches, and intermittent abnormal movements of left hand such as piano playing fingers persisted for three days that evolved in new onset generalized seizures.
Results:
Repeat scan was unremarkable however upon revision previous MRI showed abnormal T2 and FLAIR intense signals in right cerebellum with enhancement. Repeat MRI revealed edema and non-enhancing right brachium pontis suspicious for infiltrating glioma, and later right cortical leptomeningeal enhancement. EEG showed right temporal slowing, and CSF studies were remarkable only for pleocytosis. An extensive oncology work up was unremarkable, and the exam improved except for residual left side numbness, headache, and intermittent low-grade fevers. The infectious workup was unremarkable that prompted discharge on Keppra and Motrin with pending MOG and NMO antibodies. A day later the patient was readmitted for intractable headache, nausea, and vomiting, and started on empirical three-day high dose steroids with full resolution of symptoms and discharge on taper. MOG antibodies came back positive with a 1:80 titer, followed by a five-day course of high dose steroids for recurrent headaches and repeat MOG Ab titer increase to 1:160.
Conclusions:
This MOGAD case was challenging for the uncommon presentation for this age with polyfocal cerebral deficits and cerebral cortical encephalitis. Given the clinical phenotype, and a positive MOG antibody test, it fits the international MOGAD Panel proposed criteria. Children aged ten and over have a higher risk of relapse. Since there are no randomized controlled clinical trials on acute or chronic immunotherapy in MOGAD, a decision about prolonged glucocorticoid taper over several months to a year must be made because of the significant risk of relapse upon corticosteroid cessation.