
Neuroradiology
Case TypeClinical Case
Authors
Mohit Khandelwal
Patient6 years, female
A 6-year-old female child presented with acute onset (2-3 days) of seizures and altered sensorium. No pyrexia, recent vaccination, limb weakness, visual/bowel/bladder symptoms. Fever and cold history about 10–11 days back. No previous history of seizures. On clinical findings, no evidence of neurocutaneous markers or dysmorphism. EEG was normal.
Plain MRI brain revealed multiple large confluent fluffy and poorly demarcated T2 FLAIR hyperintensities in bilateral frontoparietal juxtacortical, subcortical, deep white matter, centrum semiovale (Figure 1), bilateral insular cortex (Figure 2), bilateral external capsules (Figure 3). Relatively symmetrical T2 FLAIR hyperintensities are also seen in bilateral thalamus (Figure 3) and dorsal pons (Figure 4). Few lesions show T1 hypointensity (Figure 5). Similar hyperintensities are also seen in left side of midbrain (Figure 6), left frontal cortex (Figure 7) and periventricular region (Figure 8). Subtle T2 FLAIR hyperintensities are also seen in bilateral basal ganglia (Figure 9). No evidence of diffusion restriction, significant mass effect or GRE blooming is seen.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disorder characterised by presence of IgG antibodies against myelin oligodendrocyte glycoprotein (MOG) present on outer lamellae of myelin sheaths and oligodendrocytes. Acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disease (NMOSD), and multiple sclerosis (MS) are clinically related, but MOGAD is a different entity that is becoming more and clearer in recent years.
Higher frequency of ADEM, seizure and encephalopathic presentations are seen in paediatric populations as compared to a higher prevalence of opticospinal presentations seen in adults [1].
MOGAD shows more common lesions involving the brainstem, deep grey nuclei in children with large, bilateral lesions in periventricular/deep white matter, juxtacortical region, cerebellar peduncles, and cortical grey matter [2,3] with typical lesions in cerebellar peduncles as well [4]. Adults have less brain abnormalities at the beginning (<50%) [6,7], showing ≤ 3 poorly delineated, fluffy, bilateral, T2-hyperintense lesions [3]. One-third of lesions are infratentorial, predominantly in the brainstem.
In contrast to AQP4-IgG-positive NMOSD, thalamic and pontine lesions are more common in MOGAD [7], whereas medulla oblongata and region postrema lesions are more common in AQP4-IgG-positive NMOSD. Contrast enhancement was seen in 12% of MOGAD brain lesions [7].
Complete eradication of brain lesions on follow-up suggests MOGAD rather than NMOSD or MS, which is more likely to have permanent lesions [8].
Oedematous, swollen, twisted bilateral optic nerve with short/long segment of T2 hyperintensity is seen on orbital MRI with MOGAD. It spares optic chiasm and retrochiasmatic pathways and predominantly involves front parts of optic nerves [9] as compared to NMOSD optic neuritis (ON), which shows mild swelling, rare tortuosity, and long segment involvement, with frequent involvement of the posterior segments [9]. MS-associated ON typically has unilateral short-segment involvement with less extensive contrast enhancement [9,10]. No orbital findings were present in this patient.
The axial “H” spinal cord sign (central cord grey matter T2 hyperintensity), conus medullaris involvement, and longitudinally significant transverse myelitis with a sagittal T2-hyperintense intramedullary spinal lines are indicative of spinal involvement.
Anti MOG Ig-G antibodies were positive in the serum. Lymphocyte-predominant CSF pleocytosis was seen. Intravenous methylprednisolone was given, and the patient completely recovered symptomatically. Follow-up was recommended. Serum AQP4 antibodies and CSF oligoclonal bands were negative.
Take Home Message
MOGAD should be considered in afebrile children presenting with new seizures or encephalopathy showing large multiple bilateral lesions with involvement of the brainstem and deep grey matter.
Written informed patient consent for publication has been obtained.
[1] Reindl M, Waters P (2019) Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol 15(2):89-102. doi: 10.1038/s41582-018-0112-x. (PMID: 30559466)
[2] Cobo-Calvo Á, Ruiz A, D'Indy H, Poulat AL, Carneiro M, Philippe N, Durand-Dubief F, Deiva K, Vukusic S, Desportes V, Marignier R (2017) MOG antibody-related disorders: common features and uncommon presentations. J Neurol 264(9):1945-1955. doi: 10.1007/s00415-017-8583-z. (PMID: 28770374)
[3] Jurynczyk M, Geraldes R, Probert F, Woodhall MR, Waters P, Tackley G, DeLuca G, Chandratre S, Leite MI, Vincent A, Palace J (2017) Distinct brain imaging characteristics of autoantibody-mediated CNS conditions and multiple sclerosis. Brain 140(3):617-627. doi: 10.1093/brain/aww350. (PMID: 28364548)
[4] Hacohen Y, Mankad K, Chong WK, Barkhof F, Vincent A, Lim M, Wassmer E, Ciccarelli O, Hemingway C (2017) Diagnostic algorithm for relapsing acquired demyelinating syndromes in children. Neurology 89(3):269-278. doi: 10.1212/WNL.0000000000004117. (PMID: 28615429)
[5] Hamid SHM, Whittam D, Saviour M, Alorainy A, Mutch K, Linaker S, Solomon T, Bhojak M, Woodhall M, Waters P, Appleton R, Duddy M, Jacob A (2018) Seizures and Encephalitis in Myelin Oligodendrocyte Glycoprotein IgG Disease vs Aquaporin 4 IgG Disease. JAMA Neurol 75(1):65-71. doi: 10.1001/jamaneurol.2017.3196. (PMID: 29131884)
[6] Sato DK, Callegaro D, Lana-Peixoto MA, Waters PJ, de Haidar Jorge FM, Takahashi T, Nakashima I, Apostolos-Pereira SL, Talim N, Simm RF, Lino AM, Misu T, Leite MI, Aoki M, Fujihara K (2014) Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology 82(6):474-81. doi: 10.1212/WNL.0000000000000101. (PMID: 24415568)
[7] Cobo-Calvo A, Ruiz A, Maillart E, Audoin B, Zephir H, Bourre B, Ciron J, Collongues N, Brassat D, Cotton F, Papeix C, Durand-Dubief F, Laplaud D, Deschamps R, Cohen M, Biotti D, Ayrignac X, Tilikete C, Thouvenot E, Brochet B, Dulau C, Moreau T, Tourbah A, Lebranchu P, Michel L, Lebrun-Frenay C, Montcuquet A, Mathey G, Debouverie M, Pelletier J, Labauge P, Derache N, Coustans M, Rollot F, De Seze J, Vukusic S, Marignier R; OFSEP and NOMADMUS Study Group (2018) Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study. Neurology 90(21):e1858-e1869. doi: 10.1212/WNL.0000000000005560. (PMID: 29695592)
[8] Kitley J, Waters P, Woodhall M, Leite MI, Murchison A, George J, Küker W, Chandratre S, Vincent A, Palace J (2014) Neuromyelitis optica spectrum disorders with aquaporin-4 and myelin-oligodendrocyte glycoprotein antibodies: a comparative study. JAMA Neurol 71(3):276-83. doi: 10.1001/jamaneurol.2013.5857. (PMID: 24425068)
[9] Ramanathan S, Prelog K, Barnes EH, Tantsis EM, Reddel SW, Henderson AP, Vucic S, Gorman MP, Benson LA, Alper G, Riney CJ, Barnett M, Parratt JD, Hardy TA, Leventer RJ, Merheb V, Nosadini M, Fung VS, Brilot F, Dale RC (2016) Radiological differentiation of optic neuritis with myelin oligodendrocyte glycoprotein antibodies, aquaporin-4 antibodies, and multiple sclerosis. Mult Scler 22(4):470-82. doi: 10.1177/1352458515593406. (26163068)
[10] Zabad RK, Stewart R, Healey KM (2017) Pattern Recognition of the Multiple Sclerosis Syndrome. Brain Sci 7(10):138. doi: 10.3390/brainsci7100138. (PMID: 29064441)
URL: | https://eurorad.org/case/18443 |
DOI: | 10.35100/eurorad/case.18443 |
ISSN: | 1563-4086 |
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