MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein

Abstract

Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution. Younger children also tended to have poorly defined and widespread lesions. The majority of patients with an isolated optic neuritis (n = 16) only presented small non-specific brain lesions or none at all. A longitudinally extensive transverse myelitis mainly affecting the cervical, and less often so the thoracic, lumbar, and conus regions, was detected in 31 children. The three children of our cohort who were then finally diagnosed with multiple sclerosis had at onset already demarcated white matter lesions as well as transverse myelitis. In conclusion, children with MOG seropositive ADS present disparate, yet characteristic imaging patterns. These patterns have been seen to correlate to the disease entity as well as to age of symptom onset.

Bright Spotty Lesions in the Myelon: A Hallmark of AQP-4 Positive Neuromyelitis Optica Spectrum Disorders

“…[1][2][3][4] Furthermore, a similar MRI pattern in particular in the myelon can be seen in the ADEM or LETM associated with oligodendrocyte glycoprotein antibodies. 5 In conclusion, this is a typical AQP4-IgG positive case of neuromyelitis optica spectrum disorders (NMOSD) 6 in which peculiar bright spotty lesions (►Fig. 1E), which probably reflect intense damage of the cervical spinal cord, 2 are shown.…”

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorders: Toward a New Spectrum of Inflammatory Demyelinating CNS Disorders?

“…In MOG antibody-associated ON, typical imaging characteristics are a contrast enhancement of the optic nerve, a perineural enhancement in a proportion of the patients, and in 80%, more than half of the pre-chiasmic optic nerve length being affected ( 79 , 65 ). Lesion distribution in children seems to be age-dependent, with poorly demarcated, widespread lesions in younger children, in contrast with a normal brain MRI in older children ( 80 ). It has been possible to distinguish MOG antibody-associated NMOSD from MS with a specificity of 95% and a sensitivity of 91% by employing predefined MRI criteria for lesion distribution, including Dawson's fingers, subcortical U fiber lesions, and lesions adjacent to the lateral ventricles, as typical for MS ( 81 ).…”

Neuromyelitis optica spectrum and myelin oligodendrocyte glycoprotein antibody‐related disseminated encephalomyelitis

“…It was reported that ADEM cases with MOG antibody had a certain MRI feature (large, hazy or bilateral brain lesions), which was significantly different from MOG antibody negative cases. 32 In a pediatric cohort study of demyelinating disease patients (n = 210), MOG antibodies were positive in ADEM (57%) and less frequent in NMOSD (25%), clinically isolated syndrome (25%) and MS (8%). 33 In this cohort, ADEM had higher MOG antibody titers in patients with younger onset (aged <10 years, median 7 years), whereas pediatric MS occurred at older ages (median 14 years) and MOG antibody was detected in just a few such cases with low titers.…”

“…In MOG antibody‐associated diseases, common the clinical phenotypes are bilateral or isolated ON, ADEM and myelitis. It was reported that ADEM cases with MOG antibody had a certain MRI feature (large, hazy or bilateral brain lesions), which was significantly different from MOG antibody negative cases . In a pediatric cohort study of demyelinating disease patients ( n = 210), MOG antibodies were positive in ADEM (57%) and less frequent in NMOSD (25%), clinically isolated syndrome (25%) and MS (8%) .…”

“…MDEM actually belongs to the same disease entity as ADEM. In the follow up in another pediatric cohort, eight out of 36 cases initially diagnosed with ADEM had relapses (5 were MDEM), and thus we should pay attention to the disease course and MOG antibody serostatus in such cases …”