Subscribe to RSS
“Leukodystrophy-Like” Phenotype in Anti-MOG Antibody-Associated Disorders
A 7-year-old girl developed sudden-onset right-faciobrachial hemiparesis after 3-month period of progressive abnormal behavior and academic impairment without encephalopathy. Brain MRI at diagnosis showed an extensive and confluent bilateral white matter involvement and gadolinium enhancement ([Fig. 1]). Metabolic and infectious studies were negative. The CSF profile showed mild pleocytosis and oligoclonal-bands were negative. Serum autoantibodies against myelin-oligodendrocyte-glycoprotein (MOG-abs) were 1/1,280 at onset. After early treatment with intravenous methylprednisolone and rituximab, her motor function fully recovered. After 12-months follow-up, mild cognitive and visuospatial deficits persist and MOG-abs were 1/640. No relapses have occurred.
MOG-abs have emerged as a new biomarker in demyelinating central nervous system diseases.  Acute disseminated encephalomyelitis, optic neuritis, and less often transverse myelitis or neuromyelitis-optica spectrum disorders represent the highest incidence of pediatric-acquired demyelinating syndromes. However, the spectrum of MOG-ab-associated-disorders (MOGAD) is broader than previously expected, including atypical phenotypes such as leukodystrophy-like.  
Our case is a paradigm of this uncommon phenotype of MOGAD featured by atypical clinical presentation and by the widespread alteration of white matter overlapping with genetic/metabolic leukodystrophies. Although infrequent, leukodystrophy-like MOGAD is a treatable disorder and cases with extensive and confluent white matter lesions should prompt early testing of MOG-abs to ensure early therapy.
Received: 27 October 2021
Accepted: 13 January 2022
14 January 2022 (online)
© 2022. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
- 1 Reindl M, Di Pauli F, Rostásy K, Berger T. The spectrum of MOG autoantibody-associated demyelinating diseases. Nat Rev Neurol 2013; 9 (08) 455-461
- 2 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
- 3 Hacohen Y, Rossor T, Mankad K. et al. ‘Leukodystrophy-like’ phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disease. Dev Med Child Neurol 2018; 60 (04) 417-423
- 4 Armangue T, Olivé-Cirera G, Martínez-Hernandez E. et al; Spanish Pediatric anti-MOG Study Group. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol 2020; 19 (03) 234-246
- 5 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