How to differentiate inflammatory demyelinating diseases in children by imaging tests?

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“Children are not miniature adults”. This frequently repeated phrase in the pediatrics course does not happen by chance. And we must remember this when evaluating demyelinating diseases in pediatrics. It is necessary to consider that although the diseases have the same nomenclature as in adults, the frequency is different and the imaging aspect in magnetic resonance imaging can be crucial to define the diagnosis.

A common affection in childhood is acute disseminated encephalomyelitis, better known as ADEM, being a demyelinating syndrome that courses with encephalopathy associated with some other neurological deficit. It usually affects the cortex, as well as forming lesions with a tumefactive and cotton-wool aspect in the deep portions of the brain, including the region of the basal ganglia and the region of the brainstem and cerebellum. The classic process is preceded by an infectious process or vaccine picture.

desmielinizantes Learn more: Multiple myeloma diagnosis: the role of imaging exams

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The diagnoses

A common point of confusion is the differential diagnosis between ADEM and multiple sclerosis (MS). A neurological syndrome with signs and symptoms that is not accompanied by encephalitis, with altered mental status and possible seizures, should not be called ADEM, and the possibility of MS should be investigated. The diagnostic criteria for MS in childhood also use the spread in time and space as well as the presence of oligoclonal bands in the CSF. However, in childhood, a first episode of ADEM followed by an episode of neurological involvement without encephalopathy, with the appearance of new and characteristic lesions in the skull resonance can define the diagnosis of MS.

What has been recently described is the association of the clinical and radiological representation of ADEM with the presence of autoantibody against myelin glycoprotein (anti- MOG), configuring the MOGAD. Thus, multiphase ADEM (new episode 3 months after the first one) may be associated with this persistence of the antibody or its reappearance after another infectious trigger, which happens in up to 30% of initial cases. And we must consider that if a second episode of outbreak with neurological symptoms is accompanied by encephalopathy after 3 months, the first event may configure multiphase ADEM, and therefore, retesting of anti-MOG may be necessary due to its reappearance or non-disappearance.

MOGAD is associated with a pattern of extensive myelitis that must be differentiated from another immune-mediated inflammatory disease, neuromyelitis optical (NMO), the latter being quite rare in children. However, the diagnosis of NMO can be confirmed with the presence of the autoantibody against the water channel Aquaporin-4 (anti-AQP4). MOGAD usually affects the medullary cone and has the H signal, where the medullary H is brighter than usual on T2 sequences, whereas in NMO myelitis is extensive and predominantly located around the central canal.

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MOGAD can also cause extensive intra-orbital optic neuritis (ON), and may be difficult to differentiate from NO caused by NMO, the latter being generally more severe and affecting extensive proximal portions of the optic nerves, including the chiasm optical.

Considerations

Therefore, the main demyelinating diseases of childhood appear frequently. different than in adulthood and we should use clinical, radiological and immunological criteria to establish this differential diagnosis.

In this video you will learn how to differentiate inflammatory demyelinating diseases in children by imaging tests

Author:

Radiologist with a master’s and doctorate degree from UFRJ, specialized in advanced magnetic resonance techniques and demyelinating diseases; Adjunct Professor of Radiology at the Fluminense Federal University (UFF); Radiologist at the DASA group – RJ and member of the DASA Institute of Teaching and Research.

References:

Fadda G, Armangue T, Hacohen Y et al. Pediatric multiple sclerosis and antibody-associated demyelination: clinical, imaging and biological considerations for diagnosis and care. Lancet Neurol 2021;20:136-49. It hurts:

  • 10.1016/S1474-4422(20)30432-4 Pohl D, Alper G, Van Haren K et al. Acute disseminated encephalomyelitis. Updates on an inflammatory CNS syndrome. Neurology 2016;87(Suppl2):S38-S45. It hurts: 10.1212/WNL.0000000000002825
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