Development of a Standardized MRI Scoring Tool for CNS Demyelination in Children

Verhey, Leonard H.; Branson, Helen M.; Laughlin, Suzanne; Shroff, Manohar; Benseler, Susanne M.; Feldman, B. M.; Streiner, David L.; Sled, John G.; Banwell, Brenda

doi:10.3174/ajnr.a3382

Cited by 14 publications

(9 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 98 , 99 Further demyelinating differential diagnoses are acute disseminated encephalomyelitis (ADEM) or progressive multifocal leukoencephalopathy. 100 , 101 In case of early-onset vasculitis, STING-associated vasculopathy with onset in infancy (SAVI) defined as an autoinflammatory disease with vasculopathy, interstitial lung disease, and ulcerative skin lesions caused by sporadic/dominant mutation in the TMEM173 gene, should be considered. 102 In addition, deficiency of adenosine deaminase type 2 (DADA2), an autosomal recessive disease, representing with lacunar stroke, vasculitic peripheral neuropathy, livedo racemosa and systemic inflammation is a possible differential diagnosis to PACNS with manifestation in early childhood.…”

Section: Differential Diagnosismentioning

confidence: 99%

Primary angiitis of the central nervous system: diagnosis and treatment

Beuker

Schmidt

Strunk

et al. 2018

Ther Adv Neurol Disord

104

View full text Add to dashboard Cite

Primary angiitis of the central nervous system (PACNS) represents a rare inflammatory disease affecting the brain and spinal cord. Stroke, encephalopathy, headache and seizures are major clinical manifestations. The diagnosis of PACNS is based on the combination of clinical presentation, imaging findings (magnetic resonance imaging and angiography), brain biopsy, and laboratory and cerebral spinal fluid (CSF) values. PACNS can either be confirmed by magnetic resonance angiography (MRA)/conventional angiography or tissue biopsy showing the presence of typical histopathological patterns. Identification of PACNS mimics is often challenging in clinical practice, but crucial to avoid far-reaching treatment decisions. In view of the severity of the disease, with considerable morbidity and mortality, early recognition and treatment initiation is necessary. Due to the rareness and heterogeneity of the disease, there is a lack of randomized data on treatment strategies. Retrospective studies suggest the combined administration of cyclophosphamide and glucocorticoids as induction therapy. Immunosuppressants such as azathioprine, methotrexate or mycophenolate mofetil are often applied for maintenance therapy. In addition, the beneficial effects of two biological agents (anti-CD20 monoclonal antibody rituximab and tumour necrosis factor-α blocker) have been reported. Nevertheless, diagnosis and treatment is still a clinical challenge, and further insights into the immunopathogenesis of PACNS are required to improve the diagnosis and management of patients. The present review provides a comprehensive overview of diagnostics, differential diagnoses, and therapeutic approaches of adult PACNS.

show abstract

Section: Differential Diagnosismentioning

confidence: 99%

Primary angiitis of the central nervous system: diagnosis and treatment

Beuker

Schmidt

Strunk

et al. 2018

Ther Adv Neurol Disord

104

View full text Add to dashboard Cite

show abstract

“…A standardised MRI scoring method has been proposed for assessment of paediatric multiple sclerosis scans acquired in clinical practice. 61 The importance of standardised MRI acquisition protocols was emphasised in an analysis of imaging data from the US paediatric multiple sclerosis network. 31 …”

Section: Mri In Paediatric Multiple Sclerosismentioning

confidence: 99%

Multiple sclerosis in children: an update on clinical diagnosis, therapeutic strategies, and research

Waldman¹,

Ghezzi²,

Bar‐Or³

et al. 2014

The Lancet Neurology

132

119

View full text Add to dashboard Cite

The clinical features, diagnostic challenges, neuroimaging appearance, therapeutic options, and pathobiological research progress in childhood—and adolescent—onset multiple sclerosis have been informed by many new insights in the past 7 years. National programmes in several countries, collaborative research efforts, and an established international paediatric multiple sclerosis study group have contributed to revised clinical diagnostic definitions, identified clinical features of multiple sclerosis that differ by age of onset, and made recommendations regarding the treatment of paediatric multiple sclerosis. The relative risks conveyed by genetic and environmental factors to paediatric multiple sclerosis have been the subject of several large cohort studies. MRI features have been characterised in terms of qualitative descriptions of lesion distribution and applicability of MRI aspects to multiple sclerosis diagnostic criteria, and quantitative studies have assessed total lesion burden and the effect of the disease on global and regional brain volume. Humoral-based and cell-based assays have identified antibodies against myelin, potassium-channel proteins, and T-cell profiles that support an adult-like T-cell repertoire and cellular reactivity against myelin in paediatric patients with multiple sclerosis. Finally, the safety and efficacy of standard first-line therapies in paediatric multiple sclerosis populations are now appreciated in more detail, and consensus views on the future conduct and feasibility of phase 3 trials for new drugs have been proposed.

show abstract

“…Generally, the brain MRI of demyelination in children has one of the following four patterns: poorly demarcated changes in white and/or gray matter, well-demarcated white matter changes, confluent lesions in white matter, nonspecific small (< 0.3 cm) lesions or nothing. These lesions more commonly involve certain regions of juxtacortical and cortical gray matter, deep and periventricular white matter, thalamus, corpus callosum, basal ganglia, cerebellum, and brainstem (8). In orbital MRI, optic nerve lesions can be found by thin (2-3mm) fat suppression imaging, that can be achieved with fat-saturated fast-spin echo technique or fat-saturated T1-weighted imaging following contrast enhancement (9)(10)(11)(12).…”

Section: Resultsmentioning

confidence: 99%

Neuroimaging in Pediatric Optic Neuritis: A Narrative Review

2020

View full text Add to dashboard Cite

Context: Pediatric optic neuritis can occur in isolation or in association with neuroinflammatory disorders. Abnormal orbital and cranial Magnetic Resonance Imaging were reviewed in literature diagnosed as Pediatric Optic Neuritis primarily presented with visual problems. Evidence Acquisition: A PubMed literature search was accomplished using the following search terms; Neuroimaging, Pediatric, Optic Neuritis, Multiple Sclerosis, and Magnetic Resonance Imaging. Results: Poorly demarcated changes in white and/or gray matter, well-demarcated white matter changes, confluent lesions in white matter, and nonspecific small lesions or nothing in certain regions of the brain are the most common patterns of children with optic neuritis. Optic nerve lesions can be revealed by the thin fat suppression imaging technique. Contrast-enhanced sequences, especially by short tau inversion recovery, allow differentiation of particular highsignal intensity foci in the optic nerve and newly formed active lesions from inactive lesions. Conclusion: Brain imaging should be performed in all patients, if possible, during the following two weeks after the initial diagnosis. The cranial neuroimaging can predict multiple sclerosis development in pediatric patients with demyelinating brain lesions.

show abstract

Development of a Standardized MRI Scoring Tool for CNS Demyelination in Children

Cited by 14 publications

References 30 publications

Primary angiitis of the central nervous system: diagnosis and treatment

Primary angiitis of the central nervous system: diagnosis and treatment

Multiple sclerosis in children: an update on clinical diagnosis, therapeutic strategies, and research

Neuroimaging in Pediatric Optic Neuritis: A Narrative Review

Contact Info

Product

Resources

About