Acute Encephalomyelitis Research Articles

ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM) ASSOCIATED WITH COVID-19

ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM) ASSOCIATED WITH COVID-19

An acute hemorrhagic leukoencephalitis (clinical case)

Hurst’s disease (acute hemorrhagic leukoencephalitis) is a rare fulminant demyelinating disease of the central nervous system. It was singled out as a separate nosological form in the middle of the 20th century. The etiology is not fully understood. According to statistics, the disease most often occurs after a viral or bacterial infection. Patients have an increase in body temperature up to febrile values, headaches, gastrointestinal disorders, cognitive impairments. When making a diagnosis, they are guided by the results of neuroimaging (computed tomography/magnetic resonance imaging), analysis of cerebrospinal fluid, and brain biopsy. Corticosteroids, acyclovir, antibiotics, immunoglobulins, plasmapheresis are used for treatment. The prognosis in patients with acute hemorrhagic leukoencephalitis is poor.

Adult Onset Acute Disseminated Encephalomyelitis (ADEM): A Comprehensive Case Repot

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated condition affecting the central nervous system, characterized by acute encephalopathy and multifocal neurological deficits. This disorder predominantly impacts children but here, we document a rare adult case, emphasizing the necessity for considering organic causes in psychiatric symptomatology [1 ; 2]. AIMS / OBJECTIVES: A detailed review of literature was conducted through PubMed focusing on the atypical presentations of ADEM and the incidence of organic diseases manifesting psychiatric complaints. This approach informed our diagnostic strategy and management of an unusual adult case of ADEM, underscoring the importance of maintaining a wide differential diagnosis in emergency medicine [3 ; 4] CASE DESCRIPTION: A 22-year-old female presented with generalized weakness, confusion, apathy, and reduced appetite, following an upper respiratory tract infection and a distressing dental visit. Her neurological examination revealed dysarthria, dysmetria, and ataxia, with a significant reduction in motor strength and generalized hyperreflexia. Laboratory tests were largely unremarkable except for elevated ESR and CRP. MRI findings were consistent with a demyelinating process, See Figure 1A and 1B, fulfilling the criteria for ADEM diagnosis [5 ; 6]. RESULTS: Results: Initiation of a steroid regimen led to significant clinical improvement, and by day 11 of hospitalization, there was a marked improvement in the patient's condition, enabling discharge with outpatient neurology and ophthalmology follow-ups. A six-week steroid taper was prescribed. Follow-up MRI confirmed resolution of the demyelinating lesions, confirming the effectiveness of the treatment protocol [7 ; 8]. DISCUSSION: This case underscores the complexity of diagnosing ADEM in adults, particularly when presenting with psychiatric symptoms. It highlights the critical importance of comprehensive evaluation in emergency settings to identify rare organic causes of psychiatric presentations. This report reinforces the need for awareness among clinicians to consider ADEM in differential diagnoses, ensuring timely and appropriate management for affected patients [9; 10; 11]. REFERENCES: 1. [Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S, Belman AL. Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology. 2016 Aug 30;87(9 Suppl 2):S38-45. doi 10.1212/WNL.0000000000002825. PMID: 27572859.] 2. [De Fino C, Nociti V, Modoni A, Bizzarro A, Mirabella M. An atypical case of acute disseminated encephalomyelitis associated with cytomegalovirus infection. Mult Scler Relat Disord. 2016 Jan;5:70-2. doi: 10.1016/j.msard.2015.11.003. Epub 2015 Nov 10. PMID: 26856946.] 3. [Kamel MG, Nam NT, Han NHB, El-Shabouny AE, Makram AM, Abd-Elhay FA, Dang TN, Hieu NLT, Huong VTQ, Tung TH, Hirayama K, Huy NT. Post-dengue acute disseminated encephalomyelitis: A case report and meta-analysis. PLoS Negl Trop Dis. 2017 Jun 30;11(6):e0005715. doi: 10.1371/journal.pntd.0005715. PMID: 28665957; PMCID: PMC5509372.] 4. [Obermeier PE, Karsch K, Hoppe C, Seeber L, Schneider J, Muhlhans S, Chen X, Tief F, Kaindl AM, Weschke B, Bottcher S, Diedrich S, Rath B. Acute Disseminated Encephalomyelitis After Human Parechovirus Infection. Pediatr Infect Dis J. 2016 Jan;35(1):35-8. doi: 10.1097/INF.0000000000000928. PMID: 26741581.] 5. [Neeki MM, Au C, Richard A, Peace C, Jaques S, Johansson J. Acute Disseminated Encephalomyelitis in an Incarcerated Adolescent Presents as Acute Psychosis: Case Report and Literature Review. Pediatr Emerg Care. 2019 Feb;35(2):e22-e25. doi: 10.1097/PEC.0000000000000919. PMID: 27749808.] 6. [Chaudhry LA, Babur W, Chaudhry GA, Al-Atawi FE, Robert AA. Acute disseminated encephalomyelitis: a call to the clinicians for keeping this rare condition on clinical radar. Pan Afr Med J. 2018 M

Acute Hemorrhagic Leukoencephalitis with Concurrent Retinal Vasculitis in an Elderly Japanese Patient: A Case Report.

A 68-year-old Japanese man developed a fever, headache, hiccups, and altered consciousness. Brain magnetic resonance imaging (MRI) revealed a hemorrhagic lesion in the right temporal lobe and multiple high-intensity white matter lesions. A brain biopsy showed pathological findings consistent with acute disseminated encephalomyelitis (ADEM), suggesting a diagnosis of acute hemorrhagic leukoencephalitis (AHLE), an aggressive ADEM variant. The patient also developed myodesopsia and was diagnosed with retinal vasculitis, likely due to a hyperimmune state caused by AHLE. Corticosteroids enabled full recovery. Although AHLE is uncommon in elderly individuals, clinicians should be aware of its occurrence in this patient subgroup and recognize potential retinal manifestations associated with AHLE.

Efficacy and Safety of Therapeutic Plasma Exchange in Children with Neuroimmunological Disorders: A Limited Unicentral Study.

Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE's safety and feasibility data in these conditions. The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE's efficacy was studied in these patients. The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients. Patients with PNID tolerated therapeutic plasma exchange, which was a safe process.

The central vein sign in the differential diagnosis of multiple sclerosis

To carry out a clinical and radiological assessment of the central vein sign (CVS) as a diagnostic marker for multiple sclerosis (MS) and other demyelinating and non-demyelinating diseases with focal brain damage, using clinical and laboratory examination data, as well as MRI. The results of clinical and neuroradiological examination of 107 patients diagnosed with MS or with other diseases accompanied by focal brain damage according to MRI data were analyzed. CVS is a sensitive but low-specific diagnostic marker of MS. According to our data, the sensitivity and specificity of 40 and 50% of the threshold of perivenular lesions in the diagnosis of MS are the same and amount to 100% and 39.4%, respectively. Neither the type of MS course, nor the severity of the course, nor the intake of DMT (disease modifying treatment), affect the proportion of foci with CVS. The spread of the proportion of foci with CVS in patients with MS was 60-100%. The proportion of foci with CVS is below 40 and 50% of the threshold in patients with demyelinating and non-demyelinating diseases (NMOSD, migraine, systemic lupus erythematosus, Susak disease, CLIPPERS), which allows for differential diagnosis with MS. The proportion of foci with CVS comparable to MS in patients with acute disseminated encephalomyelitis, small vessel disease, as well as in patients with radiologically isolated syndrome does not allow using this symptom in the differential diagnosis of these conditions. The use of CVS as a diagnostic marker of MS is possible only in combination with the already existing diagnostic criteria of MS.

Authors' Response to Letter to the Editor: Acute Hemorrhagic Leukoencephalitis with Concurrent Retinal Vasculitis in an Elderly Japanese Patient: A Case Report

Authors' Response to Letter to the Editor: Acute Hemorrhagic Leukoencephalitis with Concurrent Retinal Vasculitis in an Elderly Japanese Patient: A Case Report

Modern pathogenetic treatment of rare demyelinating diseases

Rare demyelinating diseases are a group of diseases whose pathogenesis is based on the process of demyelination. This group of diseases includes acute multiple encephalomyelitis (ADEM), opticoneuromyelitis spectrum diseases (NMOSD) and anti-myelin-oligodendrocyte glycoprotein-associated diseases (MOG-antibodies-associated diseases - MOGAD). Recently, new biological drugs for pathogenetic therapy have been developed, which have shown their effectiveness and good tolerability in comparison with therapy with first- and second-line drugs. Aim of the study - analysis of modern possibilities of pathogenetic treatment of patients with ADEM, seronegative and seropositive patients with NMOSD. The analysis was carried out on the basis of English-language publications in PubMed published over the past five years. This review summarizes current ideas about the possibilities of pathogenetic treatment of rare diseases. The advantages of using ravulizumab over other representatives of a new biological therapy associated with the use of monoclonal antibodies are shown. The analyzed data allow us to conclude that there is a significant development of pathogenetic treatment options for ZSONM. However, the effectiveness of new therapeutic biological drugs is still limited due to the lack of a large amount of clinical data to confirm, which creates the need to continue analyzing the experience of their use.

Neuropsychiatry presentation of acute disseminated encephalomyelitis in a young adult

Acute disseminated encephalomyelitis (ADEM) is an autoimmune inflammatory demyelinating disease of the central nervous system (CNS) involving the spinal cord and white matter of the brain, usually affecting children and young adults after vaccination or an infection. Neuropsychiatric manifestations are common but are less frequently reported in literature. This case details a young man with no previous comorbidities, who presented with a range of neuropsychiatric manifestations including blank stares, catatonic-like symptoms, selective mutism, irritability, aggression, and visual hallucination. Magnetic resonance imaging showed focal abnormal signal intensity at the splenium of the corpus callosum, notably mildly hypointense on T1W, hyperintense on T2W/FLAIR with restricted diffusion on ADC/DWI. Minimal patchy enhancement was noted postcontrast. Subtle T2/FLAIR hyperintensities were also seen involving the right medial thalamus. He was diagnosed with monophasic ADEM and initiated on intravenous methylprednisolone 1 g daily; however, he developed potential steroid-induced psychosis and was subsequently changed to orodispersible olanzapine and intravenous immunoglobulin (IVIG) 0.4 g/kg/h for 5 days to which he responded satisfactorily. This case report highlights the importance of treating ADEM with steroids and immunoglobulins as it can result in high psychiatric and physical morbidity and mortality if untreated early.

Myelin oligodendrocyte glycoprotein (MOG) antibody positive disease presenting as acute disseminated encephalomyelitis and unilateral optic neuritis

The autoantibody against myelin oligodendrocyte glycoprotein (MOG-abs) is a recognized biomarker in acquired demyelinating syndromes. The diversity in clinical and neuroimaging phenotypes is still being understood in the paediatric population. The presenting clinical phenotype strongly depends on the age at onset. Acute disseminated encephalomyelitis (ADEM) and ADEM-like phenotypes are common in younger children, but optic neuritis (ON), often bilateral, is predominantly seen in females and older children and or adults.We present a 6.5-year-old boy with a rapid onset encephalopathy due to ADEM and a unilateral longitudinally affected optic neuritis detected on neuroimaging. His serum was positive for MOG-abs but negative for Aquaporin-4 antibodies. His cerebrospinal fluid was negative for oligoclonal bands. He gradually improved following treatment with intravenous high-dose methylprednisolone and five cycles of plasma exchange. Complete clinical recovery was achieved within twelve weeks of admission.Conclusion: Contrary to the usual older age of MOG-ON, and the bilateral involvement, this case illustrates a unilateral ON in a six-year-old boy occurring in conjunction with a MOG-ab positive encephalomyelitis.

A Case of Post-Infective Myelin-Oligodendrocyte Glycoprotein Antibody-Associated Disease

Anti-myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG)-associated disease (MOGAD) is an immune-mediated central nervous system inflammatory demyelinating disorder that has been widely recognized in recent years. Herein, we describe the case of a 27-year-old woman with MOGAD following an upper respiratory infection. The patient was hospitalized for quadriparesis and drowsiness. Brain and spine MRI revealed abnormalities in multiple sites. She was initially considered to have infectious encephalomyelitis or acute disseminated encephalomyelitis (ADEM). This case report of a young woman who presented with ADEM that led to a MOGAD diagnosis after upper respiratory infection can potentially aid in elucidating the pathophysiology of this disease.

A Case of Post-Infective Myelin-Oligodendrocyte Glycoprotein Antibody-Associated Disease

Anti-myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG)-associated disease (MOGAD) is an immune-mediated central nervous system inflammatory demyelinating disorder that has been widely recognized in recent years. Herein, we describe the case of a 27-year-old woman with MOGAD following an upper respiratory infection. The patient was hospitalized for quadriparesis and drowsiness. Brain and spine MRI revealed abnormalities in multiple sites. She was initially considered to have infectious encephalomyelitis or acute disseminated encephalomyelitis (ADEM). This case report of a young woman who presented with ADEM that led to a MOGAD diagnosis after upper respiratory infection can potentially aid in elucidating the pathophysiology of this disease.

Visual outcome measures in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)

BackgroundMyelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) comprises various age-dependent clinical phenotypes and may be monophasic, multiphasic, or chronic. Optic neuritis (ON) is a common manifestation and frequently appears in combination with other MOGAD phenotypes, particularly in young children. Despite permanent structural damage to the retinal nerve fiber layer (RNFL), children often experience complete visual recovery. AimsTo analyze the progression and impact of MOGAD on the visual system of pediatric patients independently of the history of ON. MethodsThis retrospective study included children who met specific criteria: myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG) seropositivity, acute presentation of MOGAD, and written general consent. Main outcome measures were global peripapillary retinal nerve fiber layer (pRNFL) thickness, and near and distance visual acuity, analyzed using descriptive statistics. ResultsWe identified 10 patients with median age of 7.7 years at first event: 7 patients manifested with acute disseminated encephalomyelitis (ADEM) (with ON 5/7, ADEM only 1/7, with transverse myelitis (TM) 1/7), 2 with isolated ON, and 1 patient with neuromyelitis optica spectrum disorder (NMOSD)-like phenotype with ON. Among ON patients, 5/8 were affected bilaterally, with 3 initially diagnosed with unilateral ON but experiencing subsequent involvement of the fellow eye. None of the patients without previous ON showed a deterioration of visual acuity and, if evaluated, a reduction of the pRNFL. ConclusionMost pediatric MOGAD-ON patients in our cohort presented with acute vison loss and optic disc edema. All patients achieved complete visual recovery, independent of number of relapses or initial visual loss. The pRNFL thickness decreased for several months and stabilized at reduced levels after 12 months in the absence of further relapses. MOGAD may not have subclinical/’silent’ effects on the visual system, as visual acuity and pRNFL were not affected in patients without ON.

How immune system impact the nervous system

Immune system and nervous system are both important. Adaptive immune system and innate immune system are two categories of the immune system. Both types of system have a lot of organs that has different types of function. Both the CNS and the PNS share this characteristic. The nervous system disorders have some relationship with the immune cells being too activated. The T-cells, B-cells, macrophages, and glial cells will become to activate and accidently kill some cells in the nervous systems to cause the diseases. However, these types of disorders will have a varying cause and the scientists have to spend more studying on that. Autoimmune encephalitis (AE), Acute disseminated encephalomyelitis (ADEM) and Multiple sclerosis (MS) are both common disease with a huge prevalence around the world. This research paper has found out the treatment and pathogenesis of these diseases. The main cause of these types of diseases are the disorders of immune cells.

Encephalitis is an Important Phenotype of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Diseases: A Single-Center Cohort Study

BackgroundMyelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is considered a demyelinating disease of the central nervous system, but an increasing number of encephalitis cases associated with MOG antibodies have been reported recently. MethodsThis was a single-center, retrospective study. All data for pediatric patients with MOGAD diagnosed at Beijing Children's Hospital from January 2017 to January 2022 were collected. Clinical characteristics and outcomes were analyzed, and treatment responses were compared between the rituximab (RTX) and mycophenolate mofetil (MMF) groups. ResultsA total of 190 patients (age range: 5 months to 16 years; median age: 7.2 years; females: 97) were included in this study. The phenotypes of the first attack included acquired demyelinating syndromes (105 [55%]), encephalitis other than acute disseminated encephalomyelitis (82 [43%]), and isolated meningitis (3 [2%]). After a median follow-up of 30.4 months (interquartile range: 14.8-43.7), 64 (34%) patients had relapses. Fifty-one of the 64 (80%) patients who had relapse received maintenance therapy, including MMF (41), RTX (11), maintenance intravenous immunoglobulin (two), and tocilizumab (two). The annualized relapse rates decreased significantly after treatment in both the RTX and MMF cohorts (P < 0.05); however, there were no significant differences between the two groups (P = 0.56). A total of 178 (94%) patients had complete (175 patients) or almost complete (three patients) recovery (modified Rankin scale [mRS] < 2), and 12 had moderate to severe deficits (mRS ≥ 2). ConclusionsThe spectrum of pediatric MOGAD is broader than previously reported and includes demyelinating syndromes and encephalitis. Encephalitis is an important initial phenotype observed in pediatric patients with MOGAD.

A rare neurological complication of COVID-19: Pediatric Miller Fisher Syndrome. A case report

The SARS-CoV-2 pandemic has significantly transformed the world. While it was initially viewed as a respiratory virus, it has now been found to cause cardiovascular, gastrointestinal, and psychological complications. Moreover, the nervous system (NS) is also greatly affected. Research has identified dizziness, headaches, as well as disturbances in one's taste and smell abilities as the most frequent symptoms of NS involvement. Even more significant changes may occur in patients who get infected with SARS CoV-2, such as the development of acute cerebrovascular disorders (stroke), multiple sclerosis, acute disseminated encephalitis, Guillain-Barré syndrome, encephalitis, and myelitis. It is important to note these potential complications and monitor patients closely. A retrospective study conducted in Wuhan, China revealed that CNS (central nervous system) involvement occurred three times more frequently than PNS (peripheral nervous system) involvement. This emphasizes the critical importance of studying and describing CNS manifestations. This case report depicts Miller Fisher syndrome in a 5-month-old infant after SARS CoV-2 infection and explores literature on pediatric cases and potential pathogenic mechanisms.

Myelin oligodendrocyte glycoprotein antibody‐associated disorders: An overview

AbstractIn recent years, there is growing evidence of associations between antibodies against myelin oligodendrocyte glycoprotein (MOG) and several phenotypes of acute inflammatory demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis, brainstem, and cerebral cortical encephalitis, called MOG antibody associated disorders (MOGAD). Monophasic course is known in about half of cases especially in pediatric onset ADEM and optic neuritis, mainly in cases with transient positivity of MOG antibody. Pathological features of MOGAD are considered as acute demyelinating lesions with CD4 dominant cell infiltrations, the deposition of humoral immunity, perivascular inflammation and perivenous demyelination, which is distinct from multiple sclerosis. Now the diagnosis of MOGAD is based on the international panel criteria of MOGAD launched in 2023, which the diagnostic frameworks are three parts, including MOGAD‐specific clinical features, MOG antibody positivity, and the exclusion of other diseases. The prognosis of MOGAD patients is considered relatively mild, but the problem is refractory relapsing cases. For its prevention, there are no approved drugs, but oral tapering corticosteroids, immunosuppressants such as azathioprine and mycophenolic mofetil, rituximab, and the maintenance intravenous immunoglobulin are recommended, and now there are a few clinical trials of promising biological drugs already approved in other neurological disorders.

Acute disseminated encephalomyelitis following the COVID-19 Vaccine Sinopharm in low- and middle-income country: a case report

Introduction and importance: Coronavirus disease 2019 (COVID-19) is a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and various vaccines against it have been developed. Acute disseminated encephalomyelitis (ADEM) is a disease of the central nervous system that cause inflammation and demyelination and manifests as a multi-symptom acute neurological condition. Although infections are usually the cause of ADEM, vaccines may cause 5–10% of cases. Case presentation: A 40-year-old woman had received a second dose of the Sinopharm COVID-19 vaccine 4 months before her visit and experienced sudden gait imbalance and vertigo a day after her vaccination, which lasted for more than a month. On examination, no signs of skin bruising or bleeding were observed, and her vital signs were within the normal range. On neurological assessment, the patient had a Glasgow Coma Scale score of 14/15 (E4V5M5), had normal pupil size and light reaction, normal fundus, normal deep tendon reflexes and bilateral extensor plantar response. Meningeal symptoms were absent, and SARS-CoV-2 RNA tests using NAAT (Nucleic Acid Amplification Test) were negative. Development of central nervous system (CNS) manifestations during the recovery phase of fever, along with typical MRI findings; the diagnosis of para-infectious ADEM with COVID-19 vaccination was made. After the treatment with methylprednisolone sodium succinate injection, the patient showed improvement. Clinical discussion: ADEM associated with post-vaccinations is a rare condition. There has been growing evidence that shared epitopes between neuronal proteins and SARS-CoV-2 antigens may trigger autoimmune reactions against the CNS through molecular mimicry as its pathogenesis. Conclusion: We suggest the need for a strict vaccine safety monitoring system and post-vaccine monitoring and surveillance.

The risk of acute disseminated encephalomyelitis following COVID-19 vaccination in Korean population-based study

The risk of acute disseminated encephalomyelitis following COVID-19 vaccination in Korean population-based study

Microglia influence immune responses and restrict neurologic disease in response to central nervous system infection by a neurotropic murine coronavirus.

Intracranial (i.c.) inoculation of susceptible mice with a glial-tropic strain of mouse hepatitis virus (JHMV), a murine coronavirus, results in an acute encephalomyelitis followed by viral persistence in white matter tracts accompanied by chronic neuroinflammation and demyelination. Microglia serve numerous functions including maintenance of the healthy central nervous system (CNS) and are among the first responders to injury or infection. More recently, studies have demonstrated that microglia aid in tailoring innate and adaptive immune responses following infection by neurotropic viruses including flaviviruses, herpesviruses, and picornaviruses. These findings have emphasized an important role for microglia in host defense against these viral pathogens. In addition, microglia are also critical in optimizing immune-mediated control of JHMV replication within the CNS while restricting the severity of demyelination and enhancing remyelination. This review will highlight our current understanding of the molecular and cellular mechanisms by which microglia aid in host defense, limit neurologic disease, and promote repair following CNS infection by a neurotropic murine coronavirus.