Acute disseminated encephalomyelitis followed by recurrent or monophasic optic neuritis in pediatric patients

Huppke, Peter; Rostásy, Kevin; Karenfort, Michael; Huppke, Brenda; Seidl, Rainer; Leiz, Steffen; Reindl, Markus; Gärtner, Jutta

doi:10.1177/1352458512466317

Cited by 139 publications

(151 citation statements)

References 18 publications

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“…34 Currently, active interest in the connection between serum anti-MOG antibodies and recurrent ON and ADEM-ON in children exists, but there is uncertainty about this relationship. 35 The pathologic significance of anti-MOG antibodies has yet to be defined.…”

Section: Neurology 87 (Suppl 2) August 30 2016 S55mentioning

confidence: 99%

“…37 Efficacy of prophylactic treatment for ADEM-ON is unknown. 34 CONCLUSION ON is a common presenting symptom in pediatric CNS demyelinating disorders. Clinical symptoms may be dramatic, especially in younger children, with severe decreases in visual acuity in more than half of children presenting with ON and a high likelihood of bilateral disease.…”

Section: Prophylactic Therapy In Recurrentmentioning

confidence: 99%

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Pediatric optic neuritis

et al. 2016

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Optic neuritis (ON) is a common presenting symptom in pediatric CNS demyelinating disorders and may be associated with dramatic visual loss. Knowledge regarding clinical presentation, associated diseases, therapy, and outcomes in ON in children has grown over the past decade. These studies have shown that younger children (,10 years of age) are more likely to present with bilateral ON and older children with unilateral ON. Furthermore, studies focusing on visual recovery have shown excellent recovery of high-contrast visual acuity in the majority of children, but functional and structural studies have shown evidence of irreversible injury and functional decline after ON in children. Although randomized controlled treatment trials have not been performed in children and adolescents with ON, standard of care suggests that the use of highdose pulse steroids is safe and likely effective. This article reviews current knowledge about the clinical presentation and management of pediatric ON, with attention to associated syndromes and evaluative tools that may inform diagnosis and interventions. Neurology ® 2016;87 (Suppl 2): S53-S58 GLOSSARY ADEM 5 acute disseminating encephalomyelitis; AQP4 5 aquaporin-4; HCVA 5 high-contrast VA; CRION 5 chronic relapsing inflammatory optic neuropathy; IVIg 5 IV immunoglobulin; LCLA 5 low-contrast letter acuity; LCVA 5 low-contrast VA; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NMOSD 5 NMO spectrum disorder; OCT 5 optical coherence tomography; ON 5 optic neuritis; ONTT 5 Optic Neuritis Treatment Trial; RNFL 5 retinal nerve fiber layer; TPE 5 therapeutic plasma exchange; VA 5 visual acuity; VEP 5 visual evoked potential; VF 5 visual field.

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Section: Neurology 87 (Suppl 2) August 30 2016 S55mentioning

confidence: 99%

Section: Prophylactic Therapy In Recurrentmentioning

confidence: 99%

Pediatric optic neuritis

et al. 2016

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“…A small percentage (≤ 10 %) of patients with ADEM will experience a biphasic course, with a subsequent second attack of ADEM-termed multiphasic ADEM [15,26]. Rarely, ADEM may be followed by monophasic or recurrent optic neuritis and may also precede or follow a diagnosis of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis [27,28].…”

Section: Ademmentioning

confidence: 99%

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

Brenton

Banwell

2016

Neurotherapeutics

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Acquired pediatric demyelinating diseases manifest acutely with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, or with various other acute deficits in focal or polyfocal areas of the central nervous system. Patients may experience a monophasic illness (as in the case of acute disseminated encephalomyelitis) or one that may manifest as a chronic, relapsing disease [e.g., multiple sclerosis (MS)]. The diagnosis of pediatric MS and other demyelinating disorders of childhood has been facilitated by consensus statements regarding diagnostic definitions. Treatment of pediatric MS has been modeled after data obtained from clinical trials in adult-onset MS. There are now an increasing number of new therapeutic agents for MS, and many will be formally studied for use in pediatric patients. There are important efficacy and safety concerns regarding the use of these therapies in children and young adults. This review will discuss acute management as well as chronic immunotherapies in acquired pediatric demyelination.

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“…Anti-MOG antibodies occur more frequently in children than in adults and have been described (using different assays which, as noted above, may affect findings) in pediatric patients with a variety of autoimmune demyelinating disease phenotypes, including those of ADEM, MS, 32,[35][36][37] relapsing ADEM, relapsing ON, 38,39 relapsing ADEM-ON, 40 and relapsing ON-longitudinally extensive TM (NMO-like). 36,37 The normal function of MOG, which resides at the cell surface of oligodendrocytes, is not well understood.…”

Section: Cns-directed Antibodies In Pediatricmentioning

confidence: 99%

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

et al. 2016

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Elucidating pathophysiologic mechanisms underlying the spectrum of pediatric-onset CNS demyelinating diseases, particularly those that may distinguish multiple sclerosis (MS) from other entities, promises to both improve diagnostics and guide more-informed therapeutic decisions. Observations that pediatric-and adult-onset MS share the same genetic and environmental risk factors support the view that these conditions represent essentially the same illness manifesting at different ages. Nonetheless, special consideration must be given when CNS inflammation manifests in early life, at a time when multiple organs (including immune and nervous systems) are actively maturing. CSF analysis in pediatric-onset MS points to chronic CNS inflammation, supported by observations from limited pathologic material available for study. Emerging results implicate abnormalities in both effector and regulatory T cell subsets, and potentially immune senescence, in children with MS. Although CNS-directed antibodies (including antibodies recognizing myelin antigens; Kir4.1) can be documented in pediatric-onset MS, their pathophysiologic significance (as in adults) remains unclear. This is in contrast to the presence of serum and/or CSF antibodies recognizing aquaporin-4, which, when measured using validated cell-based assays, supports the diagnosis of a neuromyelitis optica spectrum disorder, distinct from MS. Presence of anti-myelin oligodendrocyte glycoprotein antibodies documented with similar cell-based assays may also be associated with pathophysiologically distinct disease phenotypes in children. The substantial impact of pediatriconset MS on normal brain development and function underscores the importance of elucidating both the immunobiology and neurobiology of disease. Ongoing efforts are aimed at developing and validating biological measures that define pathophysiologically distinct monophasic and chronic forms of pediatric CNS demyelination. Neurology ® 2016;87 (Suppl 2):S12-S19 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; AQP4 5 aquaporin-4; BBB 5 blood-brain barrier; CBA 5 cell-based assay; EDSS 5 Expanded Disability Status Scale; MBP 5 myelin basic protein; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NMOSD 5 NMO spectrum disorder; OCB 5 oligoclonal band; ON 5 optic neuritis; OPC 5 oligodendrocyte precursor cell; TM 5 transverse myelitis.There is presently limited insight into the pathophysiologic mechanisms underlying childhoodonset inflammatory demyelinating diseases. Because these disorders occur in the context of the developing immune and nervous systems, understanding the implications to both disease mechanisms and efficacy and safety profiles of potential therapeutics will help guide optimal management approaches for these children and adolescents. Elucidating the biology of pediatric-onset multiple sclerosis (MS) may provide key insights into earliest targets and processes involved in disease pathogenesis as well as into the broader spectrum of CNS...

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Acute disseminated encephalomyelitis followed by recurrent or monophasic optic neuritis in pediatric patients

Abstract: ADEM followed by ON is a rare but distinct clinical phenotype among pediatric patients. Further studies are needed to allow recommendations on treatment or prognosis.

Cited by 139 publications

References 18 publications

Pediatric optic neuritis

Pediatric optic neuritis

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

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