Successful Treatment of Acute Disseminated Encephalomyelitis (ADEM) by Prompt Usage of Immunoglobulins – Case Report and Review of the Literature

Buličić, Ana Repić; Filipović-Grčić, Petar; Jadrijević, Eni; Jurinović, Pavao; Titlić, Marina

doi:10.20471/acc.2019.58.01.22

Cited by 3 publications

(3 citation statements)

References 45 publications

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“…There are currently no randomized placebo-controlled trials establishing the best treatment of ADEM, which is mainly based on expert opinions and observational studies [ 28 ] and is analogous to the treatment of MS [ 29 ]. The standard is a nonspecific immunosuppressive therapy, including corticosteroids (CSs), intravenous immunoglobulins (IVIGs), and plasma exchange (PE).…”

Section: Treatmentmentioning

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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Section: Treatmentmentioning

confidence: 99%

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

et al. 2021

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“…MS lesions being fairly distinct can be seen in subcortical and periventricular areas. However, 22% of ADEM patients will have periventricular lesions that aren't distinguishable from MS [13]. MRI shows large, confluent FLAIR lesions in varying locations with edema, space occupancy in AHLE.…”

Section: Discussionmentioning

confidence: 99%

Acute hemorrhagic leukoencephalitis in a young female with excellent response to corticosteroids

Nasim,

Fasrina,

Bowattage

et al. 2023

Ceylon Med. J.

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Background: Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant entity of acute disseminated leukoencephalitis (ADEM) that has a rapidly progressive polysymptomatology corresponding to areas of brain affected. It’s characterized by rapid cognitive decline, coma or even rapid death. It is postulated to be a post infective immune mediated monophasic demyelinating disease. Diagnosis is clinical supported by CSF and MRI findings. It is challenging to differentiate the disease from MDEM (multiphasic demyelinating encephalomyelitis) and multiple sclerosis during the initial presentation.Case presentation: Here we report a case of a 36-yearold Sri Lankan Sinhalese lady who presented with seizure following febrile illness followed by gross right sided hemiparesis later progressing to mutism and cognitive decline. Her CSF showed pleocytosis 12/cumm with 5% polymorphs, red cells, mildly high proteins with normal glucose and positive oligoclonal bands. MRI showed evidence of multiple focal supra and infratentorial signal abnormalities and gyral thickening with hemorrhages, largest lesions being in left frontal region with mass effect but no herniation. She was diagnosed with AHLE and treated with high dose IV methyl prednisolone later converting to oral prednisolone tapering regime. She was continued on intravenous cefotaxime and acyclovir as per meningoencephalitis. Improvement with steroids was dramatic with complete neurological recovery, minimal sequel and no recurrences at 6 months follow up.Conclusion: The case is a representation of a very rare entity of an infrequently reported disease which needs high degree of clinical suspicion with early imaging for aggressive and early immunosuppressive treatment that may hinder progression to commonly described sequel including coma, death or residual neurology. The predictions on future MDEM or MS on first presentation and overlap are areas that need further exploration.

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“…Typically treatment is initiated with high dose intravenous steroids for the first three to five days and transitioned to oral steroids for three to six weeks in a tapering manner to prevent relapse. Other modalities, such as intravenous immunoglobulin and plasma exchange have also been used successfully if there is no improvement with high-dose steroid use [ 15 , 20 ].…”

Section: Discussionmentioning

confidence: 99%

Acute Disseminated Encephalomyelitis Following Chemoradiotherapy in an Adult Patient With Nasopharyngeal Cancer

Esakia¹,

Antia²,

Janelidze³

et al. 2021

Cureus

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Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disorder predominantly affecting children. It typically follows a viral illness or vaccination. We present a case of a 34-year-old white male treated with chemo-radiotherapy for nasopharyngeal cancer who developed ADEM. Prompt initiation of intravenous steroids led to the resolution of symptoms and normalization of the brain imaging. We hypothesized that direct brain tissue damage by chemotherapy and radiation therapy, combined possibly with a viral infection, triggered an immune inflammatory response and subsequent demyelination.

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Successful Treatment of Acute Disseminated Encephalomyelitis (ADEM) by Prompt Usage of Immunoglobulins – Case Report and Review of the Literature

Cited by 3 publications

References 45 publications

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

Acute hemorrhagic leukoencephalitis in a young female with excellent response to corticosteroids

Acute Disseminated Encephalomyelitis Following Chemoradiotherapy in an Adult Patient With Nasopharyngeal Cancer

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