Treatment Of Acute Disseminated Encephalomyelitis Research Articles

The Occurrence of Acute Disseminated Encephalomyelitis in SARS-CoV-2 Infection/Vaccination: Our Experience and a Systematic Review of the Literature.

The neurological complications of coronavirus disease 2019 (COVID-19) infection and the side effects of vaccination include immune-mediated diseases of the central nervous system (CNS) such as acute disseminated encephalomyelitis (ADEM). It is an acute-onset demyelinating disease that involves a rapid evolution and multifocal neurological deficits that develops following a viral or bacterial infection or, less frequently, following vaccination. Acute hemorrhagic leukoencephalitis (AHLE) is the hemorrhagic variant of ADEM that presents a more severe evolution which can be followed by coma and death. The objectives of this study consist in evaluating the diagnosis, clinical characteristics, imaging and laboratory features, evolution, and treatment of ADEM and AHLE following COVID-19 infection or vaccination. We performed a systematic review of the medical literature according to PRISMA guidelines that included ADEM cases published between 1 January 2020 and 30 November 2022 following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and vaccination and also included our own clinical experience regarding this pathology. A total number of 74 patients were diagnosed with ADEM, 45 following COVID-19 infection and 29 after a SARS-CoV-2 vaccine. A total of 13 patients (17.33%) presented AHLE. The moderate form of COVID-19 presented a positive correlation with AHLE (r = 0.691, p < 0.001). The existence of coma and AHLE was correlated with poor outcomes. The following more aggressive immunomodulatory therapies applied in severe cases were correlated with poor outcomes (major sequelae and death): therapeutic plasma exchange (TPE) treatment (r = 382, p = 0.01) and combined therapy with corticosteroids and TPE (r = 0.337, p = 0.03). Vaccinations are essential to reduce the spread of the COVID-19 pandemic, and the monitoring of adverse events is an important part of the strategic fight against SARS-CoV-2. The general benefits and the overall good evolution outweigh the risks, and prompt diagnosis is associated with a better prognosis in these patients.

Manic Episode in an Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating inflammatory disease. Corticosteroids are its main treatment. This case report presents a manic episode after taking corticosteroids during the treatment of ADEM. A 23 years old woman,that presented two month ago an episode of 40°C fever, fatigue and headache. The radiology imaging concluded to an ADEM. After taking corticosteroid she presented a manic episode which needed decreasing in corticosteroids, and the introduction of Olanzapine and Lamotrigine with a good evolution.

Acute disseminated encephalomyelitis

HintergrundDie akute disseminierte Enzephalomyelitis (ADEM) gehört zu den seltenen demyelinisierenden Erkrankungen, die meistens bei Kindern auftreten. ADEM gehört laut Leitlinien zu den Myelin-Oligodendrozyten-Glykoprotein(MOG)-assoziierten Krankheiten und manifestiert sich in der Regel nach febrilen Infektionen (auch nach SARS-CoV-2) oder, deutlich seltener, nach Impfungen.FragestellungInzidenz, Verlauf und klinische sowie radiologische Diagnostik sowie Entwicklung und Therapieoptionen von ADEM.Material und MethodeAnalyse und Auswertung der Literatur über ADEM sowie Analyse der bemerkenswerten Fälle und Leitlinien.ErgebnisseZu den ersten Anzeichen von ADEM gehören Fieber, Nausea bis zum Erbrechen sowie Kopfschmerzen und Meningismus sowie per definitionem eine Enzephalopathie, die am Anfang wenig ausgeprägt sein kann und sich meistens als Schläfrigkeit und Verwirrung manifestiert. Die radiologische Diagnose wird in der Magnetresonanztomographie (MRT) gestellt. Hier sind asymmetrisch verteilte, unscharf abgrenzbare, tumorsimulierende Läsionen supra- und infratentoriell abgrenzbar. In der akuten Phase nehmen die Läsionen meistens ringförmig Kontrastmittel auf und zeigen eine Diffusionsrestriktion. Spinaler Befall der grauen Substanz mit dem typischen H‑Muster mit Myelitis transversa ist nicht selten. Die ADEM hat meistens einen monophasischen Verlauf, wobei eine rekurrierende Form („relapsing ADEM“) in 1–20 % der Fälle zu erwarten ist. Bei der Behandlung kommen Steroide und in schweren Fällen Immunsuppressiva zum Einsatz.SchlussfolgerungADEM ist eine meist monophasische Erkrankung, deren Symptome nach einigen Wochen/Monaten abklingen sollten. Es ist wichtig, sie von anderen demyelinisierenden Krankheiten wie der multiplen Sklerose zu unterscheiden, um die nötige Therapie nicht zu verzögern.

ACUTE DISSEMINATED ENCEPHALOMYELITIS

This study examines the new literature on acute disseminated encephalomyelitis (ADEM) or post- infectious encephalomyelitis (encephalomyelitis that develops after infection), and evidence- based information has been collected by studying its epidemiology, etiological causes, clinical course, diagnostic criteria, and management. Mainly people with ADEM genetic susceptibility are thought to have autoimmune disorders of the central nervous system as a result of environmental influences (infectious and non-infectious factors). MRI has been shown to be the most optimal neuroimaging method in the diagnosis of ADEM. One important point is that the features found on MRI of the brain can also help differentiate ADEM from multiple sclerosis. However, it is described that it is not possible to completely differentiate between the two diseases on the basis of MRI alone. In addition, Gadolinium contrast media (sometimes called a MRI contrast media, agents or ‘dyes’) and non-contrast MRI imaging of the brain once a year for several years in patients with ADEM and asymptomatic is recommended because it is important to rule out pathological lesions as well as multiple sclerosis. Obtaining cerebrospinal fluid (lumbar puncture) has not lost its relevance in ADEM, but it has been found that its sensitivity is not high due to the different results. In addition to multiple sclerosis, information was analyzed on criteria important in the differential diagnosis of infectious meningoencephalitis, sarcoidosis, vasculitis, progressive multifocal leukoencephalopathy (PML), and Behcet's syndrome. Because immune suppression is important in the treatment of ADEM, high-dose glucocorticoids are the basis of pharmacological therapy. It has been established that IVIG (Intravenous Immunoglobulin) and plasmapheresis can be used if treatment is not effective within 5 days. According to statistics, the prognosis of ADEM varied depending on age, clinical manifestations and course of the disease.

Multimodal treatment of acute disseminated encephalomyelitis in children

The chief treatment options for acute disseminated encephalomyelitis (ADEM) include methylprednisolone (MP), plasma exchange (PE), intravenous immunoglobulin (IVIG). However, there is no evidence-based recommendations for management of ADEM. To identify the prognosis in ADEM after use of different modalities of treatment, a 3-year prospective study of cases presenting with neurological features with a temporal relation to an infection or vaccination or with a presumed etiology as demyelination was undertaken. Investigations to identify the causative agent, magnetic resonance imaging of brain and spinal cord, cerebrospinal fluid studies, electrophysiological studies, blood tests to exclude metabolic and collagen vascular disorders were done. A standard protocol of steroids, failing which (Modified Rankin scale score of four or 5 at end of 3 weeks) IVIG or PE was given. One patient underwent hemicraniectomy. Cases were followed up for 1 year. Telephonic interview was done at 3rd and 5th year. Of the 32 cases (

The analysis on 12 cases of acute disseminated encephalomyelitis

Objective To study the clinical features and treatment of acute disseminated encephalomyelitis (ADEM). Methods All patients admitted with ADEM during May 1990 to Dec 2010 were included in the study. Clinical data of 12 cases with ADEM were reviewed and analysed. The diagnosis of ADEM was made based on the clinical presentation, suggestive MRI and auxiliary examination findings. All patients were treated with intravenous steroids or immunoglobulins (IVIg). Results The sample consisted of 10 men and 2 women. The oldest patient was 69 years old and the youngest was 6 years old. Six patients had definite upper respiratory tract infection preceded the onset of neurological symptoms, 3 patients had non⁃specific fever, 1 patient had measles vaccination, 1 patient had measles prior to the onset 4 months ago. No preceding illness and vaccination occurred in 1 patient. The common presenting symptoms were fever, nausea, vomiting, headache. Neurological manifestations included cranial nerve involvement (the abducent nerve was the most common cranial nerve involved), paralysis (include hemiplegia, quadriplegia, paraplegia), altered sensorium, bladder involvement (both incontinence and retention), meningeal irrigation sign and conscious disturbance. Conclusion Despite the serious manifestation, ADEM in patient has good immediate outcome. Early diagnosis and treatment should be emphasized. DOI：10.3969/j.issn.1672⁃6731.2012.02.015

Encephalitis in the Pediatric Population

1. Stephen J. Falchek, MD* 1. *Division of Pediatric Neurology, Alfred I. duPont Hospital for Children/Thomas Jefferson University, Wilmington, DE, and Philadelphia, PA. * Abbreviations: ADEM: : acute disseminated encephalomyelitis CEP: : California Encephalitis Project CNS: : central nervous system CPP: : cerebral perfusion pressure CSF: : cerebrospinal fluid HSV: : herpes simplex virus IVIG: : intravenous immune globulin MS: : multiple sclerosis PCR: : polymerase chain reaction WNV: : West Nile virus Management of encephalitis, which can be fatal, requires understanding of a broad range of causative agents, pathophysiologic mechanisms, clinical syndromes, and outcomes. After completing this article, readers should be able to: 1. Know the many causes of encephalitis, including infectious, parainfectious, and noninfectious disorders. 2. Recognize the clinical features of encephalitis. 3. Be able to conduct a diagnostic investigation of a patient with encephalitis by using clinical and laboratory criteria. 4. Know the anticipated clinical course of different types of encephalitis. 5. Be familiar with the modalities used to treat encephalitis. 6. Understand the factors that influence the outcome in a patient who has encephalitis. The broad definition of the term “encephalitis,” that is, inflammation of the brain , necessitates acknowledgment of the enormous inclusivity of the topic. The most common interpretation of the term implies a direct invasion of the brain by an infectious pathogen, most commonly, viral, fungal, or parasitic. The topic also includes examples of meningitis mediated by bacteria or other agents, which can produce extrameningeal symptoms such as lethargy or seizures, in which case, the combined term “meningoencephalitis” is used. There are also many examples of encephalitis not due to direct central nervous system (CNS) infections. Inflammatory processes due to an acute or chronic illness can result in an acute immune-mediated encephalitis, such as acute disseminated encephalomyelitis (ADEM), lupus cerebritis, and paraneoplastic syndromes. Agents or conditions that produce slowly progressive CNS symptoms, such as tertiary syphilis or “slow viruses” (the prion protein encephalopathies), also are considered examples of encephalitis. Table 1 lists only a limited number of the many pathogens and pathologic conditions that can cause either acute or subacute encephalitis. In this discussion, …

HIGH-DOSE, RAPID-INFUSION IVIG IN POSTVACCINATION ACUTE DISSEMINATED ENCEPHALOMYELITIS

IV immunoglobulin (IVIG) has been shown to be advantageous in the management of a variety of autoimmune neurologic conditions.1,2 As of yet, however, there is no standard treatment for acute disseminated encephalomyelitis (ADEM). Rapid diagnosis and the immediate initiation of effective therapy are essential for recovery. This clinical note adds to the growing body of literature demonstrating the impressive efficacy of high-dose, rapid-infusion IVIG in the treatment of ADEM. ### Case report. Seven days after he arrived in Iraq, a 20-year-old Caucasian service member developed an ascending, circumferential numbness in his lower extremities that progressed over 24 hours to profound muscle weakness. Physical examination showed decreased sensation up to the nipple line, significant lower extremity weakness, and symmetric hyperreflexia with 3–4 beat clonus. Catheterization produced 900 mL of urine. The patient required intubation within hours of presentation. There was neither recent illness nor animal or insect bites. Twelve days prior to presentation, he received primary smallpox immunization. He received a typhoid inoculation 2 days later. Forty-eight hours after presentation, his MRI showed multifocal diffuse T2 hyperintensity throughout his brain and spinal cord. CSF contained 27 leukocytes, 40 erythrocytes, protein …

Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions

Pediatric inflammatory demyelinating central nervous system diseases comprise monofocal and potentially monophasic disorders like optic neuritis and transverse myelitis, the multifocal, self-limiting disorder of acute disseminated encephalomyelitis, and multifocal chronic diseases like relapsing neuromyelitis optica and multiple sclerosis. This review discusses characteristics of these disorders with focus on epidemiology and treatment of acute disseminated encephalomyelitis, neuromyelitis optica and multiple sclerosis. An international consensus group very recently defined diagnostic criteria for pediatric multiple sclerosis and acute disseminated encephalomyelitis. Immunological studies on pediatric inflammatory demyelinating disorders revealed possible disease-related humoral and T-cellular pathomechanisms. The recently identified biomarker for neuromyelitis optica, aquaporin-4-autoantibody, was detected in children with relapsing neuromyelitis optica with a similar frequency as in adult neuromyelitis optica patients. Clinically relevant, there is growing evidence that disease-modifying treatments are well tolerated and effective in the pediatric age group also. Recent studies on pediatric inflammatory demyelinating central nervous system diseases have contributed to current awareness that these disorders are not restricted to the adult age group, and that some of them carry an unfavorable long-term prognosis. Growing knowledge will hopefully enable more timely diagnoses and more specifically tailored therapies in the near future, with the goal of improving outcomes in this young patient group.

Acute disseminated encephalomyelitis: an acute hit against the brain

In this review, the possible etiology, clinical characteristics, diagnosis, and treatment of acute disseminated encephalomyelitis (ADEM) are discussed. ADEM is a para- or postinfectious autoimmune demyelinating disease of the central nervous system and has been considered a monophasic disease. The highest incidence of ADEM is observed during childhood. Over the last decade, many cases of multiphasic ADEM have been reported. The occurrence of relapses potentially poses a diagnostic dilemma for the treating physician, as it may be difficult to distinguish multiphasic ADEM from multiple sclerosis (MS). Many retrospective patient studies have thus focused on the clinical and paraclinical features of ADEM and have attempted to define specific diagnostic criteria. Additionally, several experimental models have provided insight with respect to the pathogenic relation of an infectious event and subsequent demyelinating autoimmunity. Capitalizing on experience based on a large body of well characterized patient data collected both cross-sectionally and longitudinally, pharmacotherapy has been improved and mortality and comorbidities due to ADEM have been reduced. Unfortunately, the pathogenic events that trigger the initial clinical attack, and possibly pave the way for ongoing relapsing disease, remain unknown. Clinically applicable diagnostic criteria are still lacking.

Combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the treatment of acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system that is associated with significant morbidity and mortality. Early recognition of the disease is of paramount importance; however, treatment options are limited because only case reports and small series are available in the literature. We report a case of a 42 year-old previously healthy man, whom we treated successfully with a combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the course of the disease.

Early Diagnostic Magnetic Resonance Imaging in Acute Disseminated Encephalomyelitis

We report two cases and review the literature concerning the importance of early magnetic resonance imaging (MRI) of the brain as a guide for the early diagnosis and treatment of acute disseminated encephalomyelitis (ADEM). A nonspecific term, ADEM refers to an acute disease that is postinfectious, parainfectious, postvaccinal, or of an unknown precipitating factor. Often when there is clinical suspicion of ADEM, MRI is not done before significant morbidity and mortality occur, despite the existence of adequate treatments. Primary care physicians should be aware of the importance of early MRI in ADEM.

Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin

Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids. We describe two patients with ADEM in whom treatment with IV methylprednisolone coincided with deterioration in their clinical status. They were subsequently treated with IV immunoglobulin and exhibited dramatic clinical improvement, with return to their previous level of functioning.

Pathogenesis, diagnosis, and treatment of acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis is considered a monophasic, inflammatory demyelinating disorder of the central nervous system. A temporal relationship usually exists between the onset of neurologic symptoms and an infection or a vaccination. A viral exanthem facilitates the diagnosis. Some heterogeneity exists with regard to etiology and clinical course of this disease. Immunosuppression is considered the treatment of choice.