Clinical and Imaging Findings in Children with Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease (MOGAD): From Presentation to Relapse

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FIG 1.
Thirteen-year-old adolescent boy who presented with encephalopathy after a viral prodrome, found to have papilledema and opening pressure >39, subsequently found to have serum positivity for anti-MOG antibody. Brain MR imaging demonstrates features of ADEM-like pattern of pediatric MOGAD. A-C, FLAIR hyperintensity involving the left frontal subcortical white matter with ill-defined borders and beginning confluence (white arrowheads), bilateral thalami (black arrows), pons and brachium pontis (white arrow). D, Solid nodular enhancement associated with the left frontal white matter lesions.
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FIG 2.
Ten-year-old boy with rapid-onset altered mental status and seizures. A, Cortical FLAIR hyperintensity (white arrow) involving the left parietal lobe. B, Associated leptomeningeal enhancement (black arrow). C, Concurrently acquired arterial spine labeling image demonstrates hyperperfusion of the left parietal cortex (white arrowhead), reflective of associated seizure activity.
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FIG 3.
Imaging patterns at presentation and relapses.

Table 1:

Demographics and clinical data at time of diagnosis in cohort of pediatric patients with MOGAD

	n = 61
Age, median (IQR), years	7 (4–11)
Female (%)	34 (55.7)
Race (%)
African American	6 (9.8)
Hispanic	13 (21.3)
White	22 (36.1)
Other	14 (23.0)
Unknown	6 (9.8)
Initial clinical presentation
Syndrome
ADEM	14 (23.0)
ON	31 (50.8)
Myelitis	12 (19.7)
Symptom
Headache	19 (31.1)
Altered mental status	12 (19.7)
Ataxia	7 (11.5)
Fever	11 (18.0)
Seizure	2 (3.3)
Anti-MOG titer, median (IQR)^a	1:100 (1:80–1:1000)
Relapsing disease	45 (73.8)
Total episodes up to 10, median (IQR)	3 (1–4)
Time to first relapse, median (IQR), months	5 (2–13)

^aTiter while acutely symptomatic during initial presentation was not available for 45 patients.

Table 2:

Brain MR imaging features at presentation for serum antibody-positive MOGAD

Brain Involvement	n = 44		n = 44
Supratentorial	41/44 (93.2)	Margins^a
Cortex	9/44 (20.5)	Well-defined	3/42 (7.1)
Deep gray^b	26/44 (59.1)	Ill-defined	32/42 (76.2)
Caudate	9/44 (20.5)	Mixed	7/42 (16.7)
Putamen	9/44 (20.5)	Confluence^a
Globus pallidus	6/44 (13.6)	Discrete	16/42 (38.1)
Thalamus	21/44 (47.7)	Beginning confluence	18/42 (42.9)
White matter^b		Confluent	8/42 (19)
Frontal lobe	31/44 (70.5)	Mass effect	19/44 (43.2)
Parietal lobe	28/44 (63.6)	Enhancement^b
Temporal lobe	22/44 (50)	Solid/nodular	17/44 (38.6)
Occipital lobe	19/44 (43.2)	Peripheral ring	0
Insula	21/44 (47.7)	Incomplete ring	1/44 (2.3)
White matter^b		Ill-defined	9/44 (20.5)
Subcortical	33/44 (75)	Linear	10/44 (22.7)
Deep	17/44 (38.6)	Leptomeningeal	4/44 (9.1)
Periventricular	14/44 (31.8)	Cortical	0/44
Internal capsule	9/44 (20.5)	None	17/44 (2.3)
Corpus callosum	8/44 (18.2)	>1 enhancement characteristic	10/44 (22.7)
Infratentorial^b	31/44 (70.5)	Low diffusivity^c	2/43 (4.7)
Midbrain	15/44 (34.1)	Susceptibility^d	0/36
Pons	21/44 (47.7)
Medulla	9/44 (20.5)
Brachium pontis	12/44 (27.3)
Cerebellum	15/44 (34.1)
Other	5/44 (11.4)

^aNot applicable in 2 cases of cortical only involvement.
^bPercentages do not add up to 100% as many cases had multiple combinations of features.
^cUnknown in 1 patient due to lack of DWI sequence.
^dUnknown in 8 patients due to lack of SWI/gradient echo sequence.

Table 3:

Orbit and spine MR imaging features at presentation for serum antibody-positive MOGAD

Orbital Involvement	n =31	Spine Involvement	n = 11
Unilateral	6/31 (19.4)	Long segment	4/11 (36.4)
Bilateral	25/31 (80.6)	Short segment	6/11 (54.5)
Segments involved		Cauda equina enhancement	1/11 (9.1)
Orbital	24/31 (77.4)	Focality
Intracanalicular	20/31 (64.5)	Unifocal	3/11 (27.3)
Prechiasmatic	17/31 (54.8)	Multifocal	8/11 (72.7)
Optic chiasm	5/31 (16.1)	Location
Optic tract	4/31 (12.9)	Cervical	8/11 (72.7)
Optic nerve enhancement^a	25/29 (86.2)	Thoracic	6/11 (54.5)
Perineural enhancement^b	17/25 (68)	Conus	5/11 (45.4)
Optic nerve low diffusivity^c	10/15 (66.7)	Cauda equina	1/11 (9)
		Pattern
		Central	6/11 (54.5)
		Peripheral	4/11 (36.4)
		Entire cross-section	3/11 (27.3)
		Central gray only	4/11 (36.4)
		Enhancement
		None	7/11 (63.6)
		Intramedullary	3/11 (27.3)
		Leptomeningeal	1/11 (9)
		Cord expansion	5/11 (45.4)

Table 4:

Variation of imaging patterns at relapse stratified by age greater or less than 9 years