“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.

Fig. 1 Brain MRI at diagnosis: prominent extensive and bilateral hyperintense T2 signal in the supratentorial subcortical and juxtacortical white matter without grey matter involvement (A: sagittal, B and C: axial). Gadolinium enhancement with slow and progressive permeability pattern on dynamic contrast enhancement MRI perfusion (D: axial T1 with gadolinium). Brain MRI after 1 year of treatment with corticotherapy and rituximab: Evident reduction of bilateral T2 hyperintense territory in the supratentorial subcortical and juxtacortical white matter (E: coronal, F and G: axial) with absence of gadolinium enhancement (H: axial T1 with gadolinium). Grey matter is also preserved.

MOG-abs have emerged as a new biomarker in demyelinating central nervous system diseases.[1] [2] 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.[3] [4] [5]

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.

Publikationsverlauf

Eingereicht: 27. Oktober 2021

Angenommen: 13. Januar 2022

Accepted Manuscript online:
14. Januar 2022

Artikel online veröffentlicht:
02. März 2022

• References

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