MOG Antibody Disease Presenting as Multiphasic Disseminated Encephalomyelitis

A 3-year-old girl with a history of hand–foot–mouth disease 2 months prior presented to the emergency department with abnormal gait and hand incoordination for 2 days. Magnetic resonance imaging (MRI) showed brain and spinal cord lesions in a distribution suggestive of Myelin Oligodendrocyte Glycoprotein (MOG-IgG1) antibody disease ([Figs. 1] and [2]). The patient was treated with high-dose steroids and showed partial improvement. Three months after this first episode, the patient experienced recurrent waxing and waning motor deficits. Her brain MRI showed new lesions ([Fig. 3]). MOG-IgG1 fluorescence-activated cell sorting (FACS) assay showed high MOG antibody titers. Overall, imaging findings and clinical picture were consistent with multiphasic disseminated encephalomyelitis associated with MOG antibody.

Fig. 1 (A, B) Axial T2-FLAIR images of the initial MRI head show multiple T2-FLAIR hyperintense lesions in the middle cerebellar peduncles and periventricular white matter. Middle cerebellar peduncle involvement and large supratentorial white matter lesions are suggestive of anti-MOG disorder. (C, D). Axial T1-weighted post contrast images show associated incomplete ring enhancement, a finding suggestive of demyelination. FLAIR, fluid-attenuated inversion recovery; MRI, magnetic resonance imaging.

Fig. 2 (A, B) Sagittal T1- and T2-weighted precontrast images of the MRI cervical spine 2 weeks after the initial episode show an expansile T1 isointense, T2 hyperintense intramedullary lesion extending from C3–C5 levels. (C) Sagittal T1-weighted postcontrast image of the MRI cervical spine shows incomplete ring enhancement. (D) Sagittal T2-weighted image of the MRI lumbar spine shows a T2-hyperintense lesion in the conus medullaris. MRI, magnetic resonance imaging.

Fig. 3 (A, B) Axial T2-FLAIR images of MRI head 3 months after the initial episode show new T2-FLAIR hyperintense lesions in the right middle cerebellar peduncle and in the subcortical white matter of the right temporal lobe and left insula. (C, D) Axial T1-weighted postcontrast images show new enhancing lesions in the right middle cerebellar peduncle and left frontal lobe. FLAIR, fluid-attenuated inversion recovery; MRI, magnetic resonance imaging.

Pediatric MOG antibody disorders (MOGADs) consist of a broad range of inflammatory conditions. While younger children typically present with acute disseminated encephalomyelitis (ADEM)-like episodes, older children tend to have neuromyelitis optica (NMO)-like presentation such as optic neuritis or transverse myelitis.[1] [2] [3] The demyelinating cerebral lesions are often large with ill-defined margins and typically demonstrate incomplete ring enhancement. Spinal cord lesions are typically extensive longitudinally with a predilection for the conus medullaris, unlike the smaller lesions seen in multiple sclerosis. Optic neuritis associated with MOG antibody is often longitudinally extensive and bilateral, involving the optic discs and sparing the optic chiasm.[1] [2] [3] The presence of MOG antibody, particularly persistent high titers, in children with an ADEM-like presentation increases the risk of a recurrent/relapsing course, as illustrated by this case.[2] [3] [4]

Publication History

Received: 04 November 2021

Accepted: 23 February 2022

Accepted Manuscript online:
01 March 2022

Article published online:
24 June 2022

• References

• 1 Chhabda S, Malik P, Reddy N. et al. Relapsing demyelinating syndromes in children: a practical review of neuroradiological mimics. Front Neurol 2020; 11: 627
• 2 Server Alonso A, Sakinis T, Pfeiffer HCV, Sandvig I, Barlinn J, Marthinsen PB. Understanding pediatric neuroimmune disorder conflicts: a neuroradiologic approach in the molecular era. Radiographics 2020; 40 (05) 1395-1411
• 3 Hennes EM, Baumann M, Lechner C, Rostásy K. MOG spectrum disorders and role of MOG-antibodies in clinical practice. Neuropediatrics 2018; 49 (01) 3-11
• 4 Bruijstens AL, Lechner C, Flet-Berliac L. et al; E.U. paediatric MOG consortium. E.U. paediatric MOG consortium consensus: part 1 - classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders. Eur J Paediatr Neurol 2020; 29: 2-13