Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) and Anti-N-Methyl-D-Aspartate Receptor Encephalitis (NMDARE) Overlapping Syndrome Presenting as Tics and Cognitive Symptoms
Thomas Flagiello1, Benjamin Fuchs2
1Neurology, NYU Langone Health, 2NYU Langone Health
Objective:
To discuss a case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) overlapping syndrome.
Background:
A 22-year-old woman with attention deficit hyperactivity disorder presented with one week of tics in the setting of one month of difficulty focusing, insomnia, depressed mood, anxiety and affective lability. Exam was notable for vocal and motor tics (mouth popping, whistling, forehead tapping) and a Montreal Cognitive Assessment score of 22/30 with deficits in attention, calculations, language, abstraction, and recall. She had a witnessed seizure characterized by rightward head and eye deviation followed by bilateral upper extremity extension lasting 30 seconds. Brain MRI revealed an enhancing 9x7mm left temporal FLAIR hyperintensity. Cerebrospinal fluid studies were notable for pleocytosis of 22 cells/mm3 (97% lymphocytes), 13 CSF-specific oligoclonal bands, NMDA-R Ab titer 1:8, and elevated CSF MOG-IgG (26.93, normal <2.5). Serum studies revealed MOG IgG titer 1:40 and negative NMDA-R Ab. Pelvic ultrasound and CT chest, abdomen and pelvis were negative for malignancy. She was treated with 1g IV methylprednisolone (IVMP) daily for five days and 2g/kg IVIG with clinical improvement. She re-presented one month later with worsening cognitive symptoms and re-emergence of tics and was given another five-day course of IVMP with concurrent plasmapheresis. She received one dose of rituximab as an outpatient and has recovered to her premorbid functioning.
Conclusions:
The coexistence of anti-MOG and anti-NMDAR antibodies has become increasingly recognized, referred to as anti-MOGAD/anti-NMDARE overlapping syndrome (MNOS). This case highlights the importance of exploring alternative causes when evaluating patients with atypical features of either disease (e.g. a patient with MOGAD exhibiting neuropsychiatric symptoms). This is the first case reported in the literature of MNOS presenting with new-onset tics. As tic disorders typically onset prior to adulthood, presentations such as this should prompt further investigation for an underlying autoimmune etiology.
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