Diagnostic Considerations in Acute Disseminated Encephalomyelitis and the Interface with MOG Antibody

Santoro, Jonathan D.; Chitnis, Tanuja

doi:10.1055/s-0039-1693152

Cited by 22 publications

(46 citation statements)

References 48 publications

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“…We observed that sequelae occurred in patients with a more complex clinical onset (visual, motor, behavioural impairment): of those, only few patients received IV immunoglobulins. This is in line with literature [ 69 ].…”

Section: Discussionsupporting

confidence: 94%

Management of pediatric post-infectious neurological syndromes

et al. 2021

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Background Post-Infectious Neurological Syndromes (PINS) are heterogeneous neurological disorders with post or para-infectious onset. PINS diagnosis is complex, mainly related to the absence of any recognized guidelines and a univocal definition. Aim of the study To elaborate a diagnostic guide for PINS. Materials and methods We retrospectively analysed patients younger than 14 years old admitted to Bambino Gesù Children’s Hospital in Rome for PINS from December 2005 to March 2018. Scientific literature using PubMed as research platform was analysed: the key words “Post-Infectious Neurological Syndromes” were used. Results A polysymptomatic presentation occurred in a percentage of 88% of the children. Motor signs and visual disturbances the most observed symptoms/signs were the most detached, followed by fever, speech disturbances, sleepiness, headache and bradipsychism. Blood investigations are compatible with inflammation, as a prodromal illnesses was documented in most cases. Normal cerebral spinal fluid (CSF) characteristics has been found in the majority of the study population. Magnetic resonance imaging (MRI) was positive for demyelinating lesions. Antibiotics, acyclovir and steroids have been given as treatment. Discussion We suggest diagnostic criteria for diagnosis of PINS, considering the following parameters: neurological symptoms, timing of disease onset, blood and CSF laboratory tests, MRI imaging. Conclusions We propose criteria to guide clinician to diagnose PINS as definitive, probable or possible. Further studies are required to validate diagnostic criteria.

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Section: Discussionsupporting

confidence: 94%

Management of pediatric post-infectious neurological syndromes

et al. 2021

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“…Typical cerebral MRI lesions were detected in 11 (91.7%) children with MOG-abs and in eight (66.7%) children without MOG-abs, which is similar to what has been reported in previous studies. This finding suggests that children with anti-MOG-ab-associated ADEM have an increased rate of large, bilateral, and widespread lesions, particularly in the cerebral and spinal cord areas ( 6 , 22 ). The most common locations of lesions in both groups were in the supratentorial white matter and thalamus/basal ganglia, which were consistent with the findings of a previous study conducted by Baumann et al ( 23 ).…”

Section: Discussionmentioning

confidence: 94%

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

et al. 2020

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Objective: To compare the clinical and neuroimaging characteristics of anti-myelin oligodendrocyte glycoprotein antibody (MOG-ab) negative and positive pediatric acute disseminating encephalomyelitis (ADEM) patients.Methods: Clinical characteristics, neuroimaging features, ancillary examination results, and outcomes of pediatric ADEM patients were retrospectively reviewed between February 2016 and July 2019.Results: Among 37 pediatric ADEM patients, 24 patients (11 girls and 13 boys) fulfilled the inclusion criteria. The median age was 72 (range 19–156) months, and the median follow-up duration was 20 (range 12–48) months. Children with ADEM and MOG-abs presented with increased ataxia, reduced bladder/rectum dysfunction, and paralysis compared to children without MOG-abs. An important finding was that no significant differences existed in age at symptom onset, sex ratio, time from immunotherapy to clinical improvement and clinical recovery, or modified Rankin Scale (mRS) at the last follow-up. More typical cerebral MRI lesions were detected in patients with ADEM and MOG-abs than in children without MOG-abs [11/12 (91.7%) vs. 8/12 (66.7%)]. Cerebellar lesions were higher in ADEM patients with MOG-abs (7/12, 58.3%) than in those without MOG-abs (2/12, 16.7%). While seven children had abnormal spinal MRI findings (7/12, 58.3%) and five had longitudinally extensive transverse myelitis (LETM) (5/12, 41.7%) per group, the coexistence of spinal dysfunction and abnormal spinal MRI was lower in ADEM with MOG-abs (2/12, 16.7%) than in children without MOG-abs (7/12, 58.3%). Clinical improvement was achieved 1 week after immunotherapy. Most children in both groups achieved clinical recovery within 3 months after immunotherapy, although two (16.7%) patients with ADEM and MOG-abs had persistent neurological sequelae at the last follow-up.Conclusion: MOG-abs-positive ADEM is a major subtype of pediatric ADEM. Ataxia is the most common clinical presentation in pediatric ADEM and MOG-abs. Children with ADEM and MOG-abs have similar patterns of lesions characterized by large, bilateral, widespread lesions, as well as more cerebellar lesions than children without MOG-abs. Most spinal lesions were subclinical in pediatric ADEM with MOG-abs. A favorable prognosis can be achieved for pediatric ADEM regardless of the MOG-abs status. However, some patients with MOG-abs are likely to have more severe neurological sequelae.

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“…In most cases, ADEM has a monophasic course and is self-limiting, with return to neurological baseline within 3 months after the onset of symptoms. Occasionally, a subset of ADEM patients with relapsing disorders, including recurrent DEM (RDEM), multiphasic DEM (MDEM), neuromyelitis optica spectrum disorders (NMOSD), and multiple sclerosis have been reported [ 13 ]. The clinical presentation is heterogeneous.…”

Section: Insights In the Acute Disseminated Encephalomyelitis (Adem)mentioning

confidence: 99%

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

et al. 2021

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Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory demyelinating disease of the central nervous system (CNS) that usually affects children and young adults after an infection or vaccination. The presence of several conditions mimicking ADEM, added to the lack of specific biomarkers, makes diagnosis potentially hard. Prompt diagnosis is necessary to start adequate treatment to improve the clinical course and long-term outcome. Because of its heterogeneity in both clinical presentation and course, challenges remain in establishing the most appropriate therapeutic approach in each patient. The aim of this review is to provide an update on management of this disease with a focus on acute treatment and to give suggestions for future research. We showed that there are currently no guidelines that help clinicians manage ADEM and therapeutic decisions are often made on a case-by-case basis. Further studies are necessary to identify clinical, laboratory, and instrumental criteria that could be correlated with outcomes and guide clinicians in choosing when and what treatment should be given in each case.

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Diagnostic Considerations in Acute Disseminated Encephalomyelitis and the Interface with MOG Antibody

Cited by 22 publications

References 48 publications

Management of pediatric post-infectious neurological syndromes

Management of pediatric post-infectious neurological syndromes

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

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