Relapsing demyelinating syndromes (RDS) in children encompass a diverse spectrum of entities including multiple sclerosis (MS) acute disseminated encephalomyelitis (ADEM), aquaporin-4 antibody associated neuromyelitis optica spectrum disorder (AQP4-NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOG-AD). In addition to these, there are "antibody-negative" demyelinating syndromes which are yet to be fully characterized and defined. The paucity of specific biomarkers and overlap in clinical presentations makes the distinction between these disease entities difficult at initial presentation and, as such, there is a heavy reliance on magnetic resonance imaging (MRI) findings to satisfy the criteria for treatment initiation and optimization. Misdiagnosis is not uncommon and is usually related to the inaccurate application of criteria or failure to identify potential clinical and radiological mimics. It is also notable that there are instances where AQP4 and MOG antibody testing may be falsely negative during initial clinical episodes, further complicating the issue. This article illustrates the typical clinico-radiological phenotypes associated with the known pediatric RDS at presentation and describes the neuroimaging mimics of these using a pattern-based approach in the brain, optic nerves, and spinal cord. Practical guidance on key distinguishing features in the form of clinical and radiological red flags are incorporated. A subsection on clinical mimics with characteristic imaging patterns that assist in establishing alternative diagnoses is also included.

/pdf/relapsing-demyelinating-syndromes-in-children-a-practical-29na76ptr9.pdf

Relapsing Demyelinating Syndromes in Children: A Practical Review of Neuroradiological Mimics

Background Antibodies to myelin oligodendrocyte glycoprotein (MOG) are being increasingly detected in patients with non-MS demyelination many of whom have the neuromyelitis optica spectrum disorders (NMOSD) phenotype. Cortical involvement or seizures are rare in AQP4 IgG NMO. Having encountered one MOG IgG+ve patient with seizures we systematically reviewed all our patients. Method Retrospective review of the clinical and MRI data of all MOG IgG +ve cases (n=28) and 100 patients with AQP4 IgG+ve NMOSD for seizures and cortical lesions. Results Only 1/100 NMOSD with AQP4 IgG had seizures (1%)-a 43-year old woman with epilepsy, her first seizure 5 years before the onset of NMOSD. Brain MRI was normal. 4/28 patients with MOG IgG had seizures (14%) that occurred along with an episode of demyelination. All 4 had cortical lesions and relapsing demyelination and are on immunosuppressants. 3 continue on antiepileptic drugs. Conclusion Patients with MOG IgG associated disease are more likely to have seizures (P

https://jnnp.bmj.com/content/jnnp/87/12/e1.41.full.pdf

SEIZURES AND CORTICAL LESIONS IN MOG IgG ASSOCIATED SYNDROMES

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SEIZURES AND CORTICAL LESIONS IN MOG IgG ASSOCIATED SYNDROMES