MOG antibody associated disease (MOGAD) presenting with extensive brain stem encephalitis: A case report

Elisabeth Olbert; Cornelia Brunner; Naela Alhani; Christian Našel; Walter Struhal

doi:10.1016/j.ensci.2022.100432

MOG antibody associated disease (MOGAD) presenting with extensive brain stem encephalitis: A case report

eNeurologicalSci. 2022 Oct 27:29:100432. doi: 10.1016/j.ensci.2022.100432. eCollection 2022 Dec.

Authors

Elisabeth Olbert^{1

2}, Cornelia Brunner^{1

2}, Naela Alhani^{1

2}, Christian Našel^{1

3

4}, Walter Struhal^{1

2}

Affiliations

¹ Karl Landsteiner University of Health Sciences, Dr. Karl-Dorrek-Straße 30, 3500 Krems, Austria.
² Department of Neurology, University Hospital Tulln, Alter Ziegelweg 10, 3430 Tulln, Austria.
³ Department of Radiology, University Hospital Tulln, Alter Ziegelweg 10, 3430 Tulln, Austria.
⁴ Center for Medical Physics and Biomedical Engineering, Medical University of Vienna, Vienna, Austria.

Abstract

Background: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a relatively new entity of demyelinating diseases, clinically presenting with optic neuritis, transverse myelitis, or encephalic symptoms. Typical radiological features include demyelinating cerebral and spinal lesions, cortical involvement, leptomeningeal enhancement, or tumefactive lesions. Here we present a rare case of a young patient with extensive brain stem lesion on the MRI while exhibiting nystagmus, singultus and somnolence.

Case presentation: A 30-year-old male patient presented initially with fever and impaired consciousness, but furthermore developed nystagmus, singultus and tetraparesis during the following week. Repeated MRI examinations revealed extensive brain stem edema with notable bilateral affection of the cerebellar peduncles and the pons. Antiviral and antibiotic treatment was changed to intravenous corticosteroids and immunoglobulins as soon as the diagnosis of MOGAD was established by testing serum and cerebrospinal fluid positive for MOG specific antibodies. MRI alterations vanished completely over time with a delayed, nearly complete clinical recovery of our patient.

Conclusion: Brain stem affection in MOGAD is rare. However, in patients presenting with an unclear brain stem encephalitis the possibility of MOGAD should be considered and tested using MOG antibodies. In case of a positive testing treatment with steroids and immunoglobulins seems recommendable.

Keywords: AB, antibody; ADC, apparent diffusion coefficient; ADEM, acute demyelinating encephalomyelitis; Brainstem encephalitis; CLIPPERS, Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; CNS, cerebral nervous system; CSF, cerebrospinal fluid; Case report; DW, diffusion weighted; IVIG, intravenous immunoglobulin; LETM, longitudinal extensive transverse myelitis; LP, lumbal puncture; MOG antibody encephalitis; MOG, myelin oligodendrocyte glycoprotein; MOG-AB, myelin oligodendrocyte glycoprotein antibody; MOGAD, myelin oligodendrocyte glycoprotein antibody disease; MRI, magnetic resonance imaging; MS, multiple sclerosis; NMOSD, neuromyelitis optica spectrum disorder; OCB, oligoclonal bands; T2w, T2 weighted; ceT1w, contrast enhanced T1 weighted.

Publication types

Case Reports