Diagnosis Of Acute Disseminated Encephalomyelitis Research Articles

Visual disturbances in a 12-year-old male patient with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome

Abstract: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare condition. Here, we present a case of a male patient with a very unique combination of symptoms that started at the age of 12. Transient symptoms manifested after 4 days of high fever and included lethargy, a loss of appetite, and an inability to recognize faces. After that, the patient was symptom-free for 4 years before he was hospitalized again with a working diagnosis of acute disseminated encephalomyelitis. The ophthalmic symptoms at this stage were photophobia and discrete horizontal nystagmus. One year later, the patient had another seizure and during hospitalization, predominantly displayed cerebral symptoms such as dystaxia, dysmetria, generalized muscle hypotrophy, and absent myotonic reflexes. Like before, an ophthalmic examination demonstrated a best-corrected visual acuity of 1.0 and a normal anterior segment. A fundus examination revealed a slightly rarefied retina, along with areas of perifoveal and parapapillary atrophy, in addition to increased vascular tortuosity. Optical coherence tomography scans of the macula and the optic disc depicted a mild thinning of the retinal layers. Visual field testing showed an isopter contraction with a predominant loss of the temporal visual field in both eyes. A genetic serum analysis was positive for mitochondrial mutation m.3243A>G, and a diagnosis of MELAS was confirmed. The treatment included anticonvulsive drugs and dietary precautions. Patients with MELAS syndrome can manifest a wide range of visual disturbances, meaning that a detailed ophthalmic examination is required.

Multimodal evoked potentials are useful for the diagnosis of pediatric acute disseminated encephalomyelitis.

The application of evoked potentials (EPs) to the diagnosis of acute disseminated encephalomyelitis (ADEM ) has not been investigated in detail. The aim of this study, therefore, was to analyze the value of multimodal EPs in the early diagnosis of pediatric ADEM. This was a retrospective study in which we enrolled pediatric ADEM patients and controls (Cs) from neurology units between 2017 and 2021. We measured indices in patients using brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials (SEPs), and then we analyzed their early diagnostic value in ADEM patients. The mean age of the ADEM group was 6.15 ± 3.28 years (range,1-12 years) and the male/female ratio was 2.1:1 The mean age of the Cs was 5.97 ± 3.40 years (range,1-12 years) and the male/female ratio was 1.3:1. As we used magnetic resonance imaging (MRI) as the diagnostic criterion, the sensitivity, specificity, and accuracy (κ was 0.88) of multimodal EPs were highly consistent with those of MRI; and the validity could be ranked in the following order with respect to the diagnosis of ADEM: multimodal Eps > single SEP > single VEP > single BAEP. Of 34 patients with ADEM, abnormalities in multimodal EPs were 94.12%, while abnormalities in single VEPs, BAEPs and SEPs were 70.59%,64.71%and 85.3%, respectively. We noted significant differences between single VEP/BAEPs and multimodal EPs (χ2 = 6.476/8.995,P = 0.011/0.003). The combined application of multimodal EPs was superior to BAEPs, VEPs, or SEPs alone in detecting the existence of central nerve demyelination, and we hypothesize that these modalities will be applicable in the early diagnosis of ADEM.

Diffi culties of early diagnosis of acute disseminated encephalomyelitis

Among the in fl ammatory diseases of the central nervous system, with progressive demyelination is acute disseminated encephalomyelitis. This disease proceeds with the development of diverse neurological symptoms, the formation of foci of demyelination. The etiology of the disease is not known for certain, but there is a connection with viral diseases. The clinical case considered in this article shows the rapid development of the disease with extensive demyelination, with the formation of foci in almost all brain structures in a short period of time, and a progressive deterioration of the patient’s condition against the background of ongoing therapy.

A Case of CNS Lymphoma Imitating Post-Vaccination ADEM (P14-13.003)

<h3>Objective:</h3> NA <h3>Background:</h3> A previously healthy 42-year-old male presented acutely with slurred speech, confusion and urinary incontinence several weeks after receiving a COVID booster and Tdap vaccination. <h3>Design/Methods:</h3> NA <h3>Results:</h3> Initial workup revealed multifocal enhancing T2 white matter hyperintensities in the bilateral frontal/parietal lobes, corpus callosum and bilateral midbrain, sparing the cerebellum and spinal cord. Contrast enhanced CT of the chest, abdomen and pelvis showed no masses or lymphadenopathy. Cerebrospinal fluid analysis showed a mild lymphocytic pleocytosis (12) with normal RBC, protein, glucose, negative meningitis/encephalitis PCR, cytology and autoimmune encephalitis antibodies. Serum testing was negative for HIV, RPR, NMO, MOG and antinuclear antibodies. Working diagnosis of acute disseminated encephalomyelitis was made and he was started on IV methylprednisolone 1g daily for 5 days followed by an oral steroid taper with a weekslong improvement in symptoms to his neurologic baseline followed by relapse. We repeated MRI brain which demonstrated mixed response to treatment and two repeat CSF analyses showed 2 WBC with negative cytology. Given symptom recurrence, 1g daily IV methylprednisolone was re-administered for 5 days with another brief improvement, followed by IVIG 2g/kg over 5 days. Despite aggressive immunotherapy, his symptoms continued to worsen, and he was referred for brain biopsy confirming the diagnosis of primary diffuse large B-cell lymphoma of the CNS. At the direction of neuro-oncology, the patient underwent 6 cycles of systemic high-dose methotrexate and rituximab and 2 cycles of temozolomide with PET/MRI evidence of complete response (no lesion enhancement). His performance status improved significantly, and he underwent autologous hematopoietic stem cell transplant for consolidative therapy. <h3>Conclusions:</h3> CNS lymphoma should be considered in the differential for contrast-enhancing FLAIR hyperintensities and can be steroid responsive. Brain biopsy should not be delayed in cases where CNS lymphoma is a leading differential as it can guide early disease management and help improve patient outcomes. <b>Disclosure:</b> Dr. Frank has nothing to disclose. Dr. Alalami has nothing to disclose. An immediate family member of Dr. Livingston has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. An immediate family member of Dr. Livingston has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. The institution of an immediate family member of Dr. Livingston has received research support from Vertex. The institution of an immediate family member of Dr. Livingston has received research support from Roche. The institution of an immediate family member of Dr. Livingston has received research support from AstraZeneca. The institution of an immediate family member of Dr. Livingston has received research support from Enanta. Dr. Hu has nothing to disclose. Dr. Shadbehr has nothing to disclose.

A Case of Viral Illness Complicated by Acute Disseminated Encephalomyelitis (ADEM)

We present a case of a 7 years old boy that developed impaired consciousness &amp; constant headache 5 days following a tonsillitis infection treated with augmentin, investigations such as CSF was ruling out encephalitis &amp; head MRI was supportive of the diagnosis of ADEM, however the Patient symptoms were controlled with IVIG, methylprednisolone and plasmapheresis which provided a good outcome, He also received acyclovir and ceftriaxone as an initial managment.

Re-examining the characteristics of pediatric multiple sclerosis in the era of antibody-associated demyelinating syndromes.

The discovery of anti-myelin oligodendrocyte glycoprotein (MOG)-IgG and anti-aquaporin 4 (AQP4)-IgG and the observation on certain patients previously diagnosed with multiple sclerosis (MS) actually have an antibody-mediated disease mandated re-evaluation of pediatric MS series. To describe the characteristics of recent pediatric MS cases by age groups and compare with the cohort established before 2015. Data of pediatric MS patients diagnosed between 2015 and 2021 were collected from 44 pediatric neurology centers across Türkiye. Clinical and paraclinical features were compared between patients with disease onset before 12 years (earlier onset) and ≥12 years (later onset) as well as between our current (2015-2021) and previous (<2015) cohorts. A total of 634 children (456 girls) were enrolled, 89 (14%) were of earlier onset. The earlier-onset group had lower female/male ratio, more frequent initial diagnosis of acute disseminated encephalomyelitis (ADEM), more frequent brainstem symptoms, longer interval between the first two attacks, less frequent spinal cord involvement on magnetic resonance imaging (MRI), and lower prevalence of cerebrospinal fluid (CSF)-restricted oligoclonal bands (OCBs). The earlier-onset group was less likely to respond to initial disease-modifying treatments. Compared to our previous cohort, the current series had fewer patients with onset <12 years, initial presentation with ADEM-like features, brainstem or cerebellar symptoms, seizures, and spinal lesions on MRI. The female/male ratio, the frequency of sensorial symptoms, and CSF-restricted OCBs were higher than reported in our previous cohort. Pediatric MS starting before 12 years was less common than reported previously, likely due to exclusion of patients with antibody-mediated diseases. The results underline the importance of antibody testing and indicate pediatric MS may be a more homogeneous disorder and more similar to adult-onset MS than previously thought.

The Wide Variety of Acute Disseminated Encephalomyelitis in Children: A Clinical Perspective

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating inflammatory disorder of the central nervous system. It is characterized by encephalopathy, multifocal neurological deficits, and typical magnetic resonance imaging findings of widespread demyelinating lesions, predominantly involving the white matter of the brain and spinal cord. ADEM mainly affects children and is commonly associated with preceding viral and bacterial infections, and, rarely, vaccinations. Despite substantial advances in the understanding of the association of myelin oligodendrocyte glycoprotein antibody with recurrent forms of ADEM or other demyelinating conditions, specific etiologic agents or biological markers have not been identified. Therefore, the diagnosis of ADEM is still based on clinical and radiological findings and the exclusion of other conditions mimicking ADEM. However, a prompt diagnosis and adequate treatment are crucial because diagnostic delays or inappropriate treatment may lead to unwanted neurological sequelae in some children. There is no standardized treatment protocol for ADEM, but the use of corticosteroids, intravenous immunoglobulin, and plasmapheresis has been associated with good clinical outcomes. Adequate treatment has reportedly resulted in favorable outcomes, with full or almost full recovery in most children with ADEM, although some children may develop neurological sequelae, such as cognitive impairment and motor deficits. Further studies are needed to identify biological clues and optimal treatment protocols to minimize the incidence of neurological sequelae.

Post-Covid-19 Acute Disseminated Encephalomyelitis (ADEM) in a 27-year-old girl: Case Report

Acute disseminated encephalomyelitis (ADEM) is a monophasic, multifocal, demyelinating, autoimmune disease that affects the central nervous system (CNS). It usually occurs after a systemic infection, usually viral, including certain coronavirus infections. A 27-year-old girl presented with complaints of left interscapular pain, paresthesias and weakness in the ipsilateral upper limb. These symptoms followed paresthesias on the fingertips of her right hand the day before her admission. she was treated two weeks earlier for pneumonia with COVID-19. Her clinical pattern resulted in a moderate weakness of the left limbs associated with tactil and algic hypoesthesia in the lower left limb ascending until the C4 level in the left side. Magnetic resonance imaging (MRI) of the brain and spinal cord showed diffuse spontaneous hypersignals on fluid-attenuated inversion recovery (FLAIR) images at the cerebral level and on T2-weighted images at the spinal level. These imaging lesions coupled with the medical history of a recent COVID-19 infection led to the diagnosis of acute disseminated encephalomyelitis (ADEM) post covid-19. The clinical condition improved rapidly with intravenous (IV) corticosteroid therapy and IV immunoglobulin combined with physiotherapy. ADEM is a demyelinating autoimmune disease which is increasingly reported during this current corona virus pandemic.

Acute disseminated encephalomyelitis in children and adolescents - 20-year single-center experience in Serbia

Introduction/Objective. Acute disseminated encephalomyelitis (ADEM) is the most common demyelinating disease of the central nervous system in pediatric patients. We aimed to evaluate the clinical profile of children with ADEM and to discern prognostic factors for disease outcome. Methods. A 20-year retrospective?prospective study was conducted in a cohort with the diagnosis of ADEM. Results. The study included 36 patients, with range of follow-up period of 6?120 months (median of 26 months). Prior infection was reported in 72.2% of the patients. In the clinical presentation of the disease, motor deficit was most common (81.1%), followed by ataxia (77.8%). More than a third of patients had back and limb pain or abdominal visceral pain, which highly correlated with MRI findings of myelitis. Abnormal brain CT findings were evident in 22.2% of the patients, and this was associated with higher Expanded Disability Status Scale (EDSS) and quicker progression of the disease. Median EDSS was 0 at the most recent follow-up visit, in all the patients. EDSS 0?2.5 was verified in 29 (80.6%) of the patients, while three (8.3%) patients scored 7?9.5 at the last visit. Two patients had a lethal outcome. Conclusions. ADEM is a serious disease in pediatric patients, but with a good prognosis, which is illustrated by the fact that 80.6% of our patients had a complete or almost complete recovery.

Clinicoradiological and prognostic features of COVID-19-associated acute disseminated encephalomyelitis

IntroductionThe Covid-19 pandemic has resulted in a spark in interest in the subject given the high exposure rate to viral antigens in the form of infections and vaccines. It is expected that acute disseminated encephalomyelitis (ADEM) cases see a rise in incidence during this period. Given the plethora of Covid-19-related central nervous system (CNS) involvement, it is important to be aware of the varied presentations of ADEM. Case reportsIn this paper, we report 3 cases of ADEM following Covid-19 infection. Patients presented with polyfocal neurological symptoms 6 to 18 days after respiratory symptoms onset. The diagnosis of Covid-19 was made based on nasal swab reverse transcriptase-polymerase chain reaction (RT-PCR) and chest computerized tomography (CT). DiscussionThese cases illustrate both classic and atypical presentations requiring exclusion of a spectrum of CNS conditions to be able to retain the diagnosis of ADEM. Consequently, we stress the importance of context, clinical examination and MRI findings in the differentials. In addition, we discuss workup, and particularly, the indication of brain biopsy. Also, the paper discusses options in therapy and the prognosis. The prognosis of covid-associated ADEM is dependent on the extent of pathology intrinsic to ADEM and the intrication of the prognosis of Covid-19 infection. ConclusionThe key message in these 3 cases is that clinicians should have a low threshold of suspicion of ADEM in the Covid-19 context, adopt appropriate workup strategies, and initiate adequate treatment for better outcomes.

Diagnostic odyssey of acute disseminated encephalomyelitis in children

We aimed to determine whether acute disseminated encephalomyelitis (ADEM) diagnosis in children is delayed, and if so, to identify the clinical risk factors of delayed diagnosis. Standardised data were collected from children with ADEM from 2003 to 2020. Overall diagnostic delay (time between symptom onset and ADEM diagnosis), physicians’ delay (between the first medical visit and ADEM diagnosis), and patients’ delay (between symptom onset and the first medical visit) were analysed. Thirty ADEM patients were identified, including 16 (54%) with neurological deficits at discharge. Overall, physicians’, and patients’ delays were 9 (interquartile range [IQR] 6–20.5), 5.5 (IQR 3–14), and 4 (IQR 2–8) days, respectively. Overall delay was significantly associated with physicians’ delay, but not with patients’ delay. There were 61 misdiagnoses among 25 (83%) patients, while 5 (17%) were diagnosed correctly at the first visit. The misdiagnoses of common respiratory and gastrointestinal infection and aseptic meningitis were associated with overall and/or physicians’ delay. Later onset of specific neurological features suggestive of ADEM was associated with all three diagnostic delays. A unique diagnostic odyssey exists in ADEM. Several clinical risk factors were associated with the diagnostic delay.

Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Leukoencephalitis Following COVID-19: Systematic Review and Meta-synthesis.

Background and ObjectivesSince the onset of the COVID-19 pandemic, a growing number of reports have described cases of acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (AHLE) following infection with COVID-19. Given their relatively rare occurrence, the primary objective of this systematic review was to synthesize their clinical features, response to treatments, and clinical outcomes to better understand the nature of this neurologic consequence of COVID-19 infection.MethodsPatients with a history of COVID-19 infection were included if their reports provided adequate detail to confirm a diagnosis of ADEM or AHLE by virtue of clinical features, radiographic abnormalities, and histopathologic findings. Cases purported to be secondary to vaccination against COVID-19 or occurring in the context of a preexisting relapsing CNS demyelinating disease were excluded. Case reports and series were identified via PubMed on May 17, 2021, and 4 additional cases from the authors' hospital files supplemented the systematic review of the literature. Summary statistics were used to describe variables using a complete case analysis approach.ResultsForty-six patients (28 men, median age 49.5 years, 1/3 >50 years old) were analyzed, derived from 26 case reports or series originating from 8 countries alongside 4 patient cases from the authors' hospital files. COVID-19 infection was laboratory confirmed in 91% of cases, and infection severity necessitated intensive care in 67%. ADEM occurred in 31 cases, whereas AHLE occurred in 15, with a median presenting nadir modified Rankin Scale score of 5 (bedridden). Anti-MOG seropositivity was rare (1/15 patients tested). Noninflammatory CSF was present in 30%. Hemorrhage on brain MRI was identified in 42%. Seventy percent received immunomodulatory treatments, most commonly steroids, IV immunoglobulins, or plasmapheresis. The final mRS score was ≥4 in 64% of patients with adequate follow-up information, including 32% who died.DiscussionIn contrast to ADEM cases from the prepandemic era, reported post–COVID-19 ADEM and AHLE cases were often advanced in age at onset, experienced severe antecedent infection, displayed an unusually high rate of hemorrhage on neuroimaging, and routinely had poor neurologic outcomes, including a high mortality rate. Findings are limited by nonstandardized reporting of cases, truncated follow-up information, and presumed publication bias.

Immediate and Short-term Outcome of Acute Disseminated Encephalomyelitis (ADEM) after corticosteroid therapy

Background : Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system (CNS) occurring predominantly but not exclusively in children. The presence of severe disturbance of consciousness together with multifocal neurological symptoms and signs especially in a child under 10 years of age; favours a diagnosis of ADEM. Neuroimaging is essential investigation for establishing the diagnosis of ADEM. High-dose steroid treatment frequently results in a dramatic clinical improvement.&#x0D; Objective: This study aimed to describe our experience with Acute Disseminated Encephalomyelitis (ADEM) focusing on the clinical features, neuroimaging, and outcome after treatment with high-dose steroid.&#x0D; Method: This retrospective observational study was conducted in children who were admitted as inpatients in Paediatric Neurosciences Department of Dhaka Shishu Hospital (DSH) with the diagnosis of ADEM during the period of 2017- 2019. All patients got high dose IV methylprednisolone for 5 days followed by 2 weeks tapering dose of steroid. Outcome was observed on the 7th day of treatment and 6 months after discharge from hospital.&#x0D; Result: A total of 16 patients who were diagnosed as ADEM were included in this study. Twelve children (75%) had a recent upper respiratory tract illness. Patients presented most often with motor deficits (88%) and secondly with altered consciousness (63% ) followed by seizure ( 50% ). MRI brain was the preferable neuroimaging which mostly (41.7%) showed multifocal symmetrical demyelination patch. On the 7th day of steroid treatment, significant improvement in motor function and level of consciousness occurred (p values were 0.001% and 0.014% respectively). After 6 months of discharge from hospital; no motor deficit, age-appropriate cognitive level and no seizure in 71%, 57% and 71% respectively.&#x0D; Conclusion: High dose corticosteroid treatment in ADEM is significantly effective in improvement of motor impairment and consciousness level on immediate follow up.&#x0D; Northern International Medical College Journal Vol.11 (2) Jan 2020: 468-470

Acute Disseminated Encephalomyelitis (ADEM) in Children: A Case report

Acute Disseminated Encephalomyelitis (ADEM) is an inflammatory disease that causes demyelination and affects the white matter of the brain and spinal cord through an immune response. The patient, an eight-year-old Iranian Kurdish boy with a height of 125 cm and a weight of 24 kg, complained from fever for three days which didn’t respond to acetaminophen medication. After three days, the symptoms developed into headache, vomiting, and decreased consciousness. The patient was admitted to the emergency department of Imam Hasan hospital in Bojnourd, Iran, in July 2019. LP diagnostic testing, Wright Agglutination test, Brain CT, and MRI examination were performed. Finally, a diagnosis of acute disseminated encephalomyelitis (ADEM) was confirmed. Following the ADEM diagnosis, methylprednisolone 500 mg was administered immediately and continued for five days. This case study suggests that MRI is the most effective method of diagnosis for ADEM, and high-dose methylprednisolone is the treatment of choice for this syndrome.

Micturition Disturbance: A Predominant Feature of Acute Disseminated Encephalomyelitis

AbstractUrinary retention in children can have varied etiology ranging from anatomic to functional causes. Functional causes include neuropathies, including rare central nervous system (CNS) involvement, such as acute disseminated encephalomyelitis (ADEM). ADEM is usually preceded by acute viral infection and commonly presents with multifocal neurological deficits. The ADEM diagnosis is mainly based on the clinical presentation and its correlation with characteristic CNS findings on magnetic resonance imaging (MRI). Here, we report an adolescent boy who presented with acute urinary retention preceded by an acute febrile illness. The patient was worked up thoroughly, including an MRI of the brain and spine, which revealed ADEM's characteristic features. The bladder function was evaluated periodically. The voiding dysfunction persisted for a long time. Treatment with steroids and other supportive therapy ultimately lead to a complete recovery over 6 months.

Characteristics and Predictors of Disease Course in Children Initially Presenting with Acute Disseminated Encephalomyelitis (ADEM)

Background: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system (CNS), characterized by new onset polyfocal neurologic symptoms with encephalopathy and multifocal demyelination, typically occurring in early childhood. The initial diagnosis of ADEM can be challenging, as up to 20% of children with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD) are initially diagnosed with ADEM. Objective:To describe characteristics of patients with ADEM vs. recurrent demyelinating syndromes (MS, NMOSD, other) at the time of initial presentation and identify features at disease onset of presentations consistent with ADEM associated with monophasic demyelinating disease. Methods: This is a multicenter observational cohort study of children with a demyelinating disease diagnosis of ADEM, multiphasic ADEM, MS, and NMOSD who were followed at 12 regional pediatric MS referral centers in the US Network of Pediatric MS Centers. Descriptive statistics were used to report patient characteristics, clinical/imaging presenting features and subsequent outcomes. Logistic regression was used to predict features associated with a monophasic course. Results: As of July 2019, 872 pediatric patients with a final diagnosis of ADEM (n=89), MS (n= 664) or NMOSD (n=119) were identified. The mean +/- standard deviation follow-up for all patients was 5·7 +/- 3·1 years. ADEM patients were the youngest with mean age at first event 5·4 +/- 3·7 years (p < 0.001) and male predominance (62%) (p < 0·001). Severe clinical symptoms at onset were more frequent in ADEM (55% vs. 35% NMOSD and 15% MS, p < 0·001). After 2 years of follow-up, 78% (93/119) of patients initially diagnosed with ADEM retained this diagnosis (ADEM to ADEM), while 9·2% were later reclassified as MS, 3·3% as NMOSD and 7·5% as demyelinating disease not otherwise specified (DDNOS). In univariable logistic regression, younger age at first event was associated with retaining ADEM diagnosis, while presentation with optic neuritis and gadolinium enhancement on brain MRI were associated with ADEM reclassification to MS, NMOSD, or DDNOS at 2 years of follow-up. In multivariable analysis, older age at first event (OR 1·16 [95% CI 1·00 – 1·33] for 1-year increase, p = 0·04), presenting with optic neuritis (OR 15·31 [95% CI 3·27 – 71·66], p <0·001) and presence of gadolinium enhancement on brain MRI at onset (OR 6·80 [95% CI 2·03 – 22·78], p = 0·0019) were associated with reclassification of ADEM to MS, NMOSD or DDNOS within 2 years. Conclusion: Children who remain classified as ADEM vs. those who are reclassified as other demyelinating disorders are younger at onset, and less likely to have optic neuritis or gadolinium-enhancing lesions at disease onset. Funding: None to declare. Declaration of Interest: None to declare. Ethical Approval: The ethics committees of participating institutions approved this study. Parents and participants signed consent forms and assent forms when required by each center’s institutional review board prior to enrollment.

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.

Evaluation of demographic, clinical and paraclinical characteristics of children with acute disseminated encephalomyelitis

Introduction: Acute disseminated encephalomyelitis (ADEM) is an immunologically mediated inflammatory demyelinating disorder that commonly occurs following a viral infection or vaccination. Objectives: This study aimed to evaluate the demographic, clinical and paraclinical characteristics of children with ADEM. Patients and Methods: In a retrospective study, all children who had a definite diagnosis of ADEM were included in the study. Demographic information (including age and gender), epidemiological features (season of onset, history of previous vaccination and previous infection), clinical signs of ADEM, paraclinical features and also clinical outcomes were extracted from patients’ records and entered into a researcher-made checklist. All data were collected, classified and entered into SPSS version 21 for statistical analysis. Results: A total of 27 patients with ADEM were studied out of which, 16 (59.3%) were male and 11 (40.7%) were female. The mean age of patients was 5.21 ± 4.37 years, the youngest of which was three months and the oldest patient had 13 years old. The highest incidence (33.3%) was observed in autumn and 66.7% of patients had a history of recent infection. Electroencephalographic (EEG) findings were abnormal in 55.6% of patients. The most common clinical finding of abnormal EEG was motor disorders (51.8%). Among those who conducted MRI, 71.4% of them had cortical lesions in the brain. In the majority of cases (51.9%), the treatment achieved with intravenous immunoglobulin (IVIG) alone and complete recovery. Conclusion: The ADEM in this region has relatively similar epidemiological features to those studied worldwide and the favorable treatment of our cases has led to a satisfactory percentage of complete clinical recovery.

Neuropsychological outcome in cases with acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory and demyelinating disorder of the central nervous system. There have been a few studies in recent years on the fact that these cases have neurocognitive impairment. The purpose of this study is to evaluate the neurocognitive outcome and quality of life in cases with ADEM. Eleven cases who were on follow-up between 2008 and 2017 were included in the study, systemic, neurological and psychiatric examinations were done. All magnetic resonance images were re-evaluated. The neuropsychiatric evaluation was performed by clinical examination and psychometric scales; (1) The Pediatric Quality of Life Inventory 4.0, (2) Child Behavior Checklist, (3) Children`s Depression Inventory, (4) The Wechsler Intelligence Scale for Children-Revised and (5) Continuous Performance Test. The cases in our study underwent neuropsychiatric evaluation 3-42 months after the diagnosis of ADEM had been established. Nine cases (81.8%) fully recovered without neurologic deficit. One case (9.1%) had a psychiatric disorder. During follow-up, cognitive and psychiatric problems were encountered in half of the cases (54.5%). Most of the cases with basal ganglia involvement (80%) displayed attention deficit and cognitive problems. In particular, cases with basal ganglia involvement should be followed carefully in terms of attention and cognitive problems.

C-53 The Challenges of Neuropsychological Rehabilitation for Children Diagnosed with Multiple Sclerosis (MS) Due to Complex Diagnostic Factors

Abstract Objective With an incidence of 9.83 per million children per year, paediatric onset multiple sclerosis (MS) is rare. It does, however, present challenges for differential diagnosis alongside other demyelinating syndromes, such as Acute Disseminated Encephalomyelitis (ADEM) and various recurrent acquired demyelinating conditions, making diagnosis complex in this population. We highlight the different trajectories related to diagnosis and the impact of this on the child, family and wider healthcare and education systems surrounding them. Method Serial neuropsychological assessment data (Delis-Kaplan Executive Function System; Wechsler Intelligence Scale for Children, Children’s Memory Scale, Behavior Rating Inventory of Executive Function) is presented for two children with an initial diagnosis of ADEM; one subsequently was diagnosed with MS later altered to Myelin Oligodendrocyte Glycoprotein (MOG) antibody associated relapsing demyelination. Results Case 1: presents with average IQ and memory, significant deficits in executive function, including behavioural regulation and hyperactivity. Diagnostic journey indicated as recurrent ADEM leading to diagnosis of MS at 16 years old, later changed to MOG antibody associated relapsing demyelination. Case 2: presents significant deficits in IQ and memory and executive function following ADEM diagnosis at young age. Conclusions We consider how recent advances in medical investigation have uncovered numerous demyelinating syndromes impacting on the challenge of diagnosis of ADEM, MS and other demyelinating conditions. We question whether we are meeting the neuropsychological needs of children/adolescents with ADEM, MS and other demyelinating conditions in paediatric neuropsychology practice due to limited understanding of the complex and differential diagnoses and discuss whether ADEM and MS form a diagnostic continuum.