What Is New In Pediatric CNS Demyelination?

Administrator: Silvia Tenembaum

Diagnosing MOGAD in pediatric patients. Updated diagnostic criteria from an International Panel.
Silvia Tenembaum

An international panel of experts has recently proposed diagnostic criteria for MOG antibody-associated disease (MOGAD) in which the presence of MOG-IgG is a core criterion. According to these criteria, MOGAD is typically associated with acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis, and unlike multiple sclerosis and AQP4-IgG-seropositive NMOSD, in which multiple clinical attacks characterize relapsing forms of disease, individuals with MOGAD can have either a monophasic or relapsing course. Optic neuritis is by far the most common onset feature, particularly among adults, while ADEM with or without concomitant optic nerve involvement is the typical first manifestation in children, particularly before the age of 11 years. Transverse myelitis is another common presentation. Less common presentations include cerebral cortical encephalitis (often with seizures), brainstem and cerebellar demyelinating attacks, tumefactive brain lesions, cerebral monofocal, and polyfocal CNS deficits associated with demyelinating lesions, cranial neuropathies, and progressive white matter damage (leukodystrophy-like pattern). Histopathological features of MOGAD differ from those of multiple sclerosis or NMOSD, as do its imaging features, treatment responses, and clinical outcomes. We aim to introduce and discuss the main clinical and neuroimaging presenting phenotypes of MOGAD.

 

Key MRI diagnostic features of MS, MOGAD and NMOSD
Thais Armangue

Magnetic resonance imaging (MRI) is central in the diagnostic workup of children with suspected CNS demyelination given its high sensitivity to detect signs of neuroinflammation involving optic nerves, brain-brainstem, or spinal cord. Differences in patterns of brain and spinal cord lesions between relapsing-remitting MS, AQP4 antibody– positive NMOSD, and MOGAD can be identified. In MS, white matter lesions tend to affect specific brain regions, such as the periventricular and juxtacortical white matter, the corpus callosum, and the infratentorial areas, whereas in AQP4-NMOSD, brain abnormalities are frequently located in areas with high AQP4 expression such as peri-ependymal lesions surrounding the ventricles or involving corticospinal tracts. Patients with MOGAD can show large, ill-defined, or defined lesions, mostly located in the deep gray matter and the cerebellar peduncles. Longitudinally extensive transverse myelitis is the hallmark of AQP4- NMOSD with predilection for the cervical cord, whereas in MS, multiple, short-segment lesions are common. In MOGAD, cord lesions often affect the lower thoracic cord and conus and tend to be longitudinally extensive in the acute stage. In addition, we will also review in this session the ability of MRI to exclude a wide range of differential diagnosis in pediatric patients.
 

Progress in Pediatric MS Treatment: Real World Evidence with High-impact therapies
Lekha Pandit

Individuals with pediatric onset-MS tend to have higher relapse rates, greater accumulation of new MRI lesions, and greater long-term cognitive impairment compared with adult-onset MS. Treating children with MS is challenging given its high disease activity and lack of safety and efficacy data in this age group, for most disease-modifying therapies (DMTs). The recent development of randomized controlled MS trials in youth has led to the first agents formally approved for the treatment of pediatric onset MS. We have entered a new era of knowledge and treatment in the pediatric population and ongoing pediatric trials are expected to further inform clinical management. We aimed to discuss real-world evidence of the impact of initial treatment with newer and highly effective therapies compared with the conventional approach using injectable DMTs in children with MS on the relapse rate, development of new MRI lesions, and disability progression.  In addition, we will review the risks and side-effect profiles associated with the newer immunotherapies and provide practical monitoring recommendations for their use.