Role of physical therapy intervention in acute disseminated encephalomyelitis.

We reported a case of a school-going child, diagnosed with acute disseminated encephalomyelitis (ADEM) who presented with symptoms such as high fever, acute hemiplegia and ataxia and was referred for physiotherapeutic intervention. This case report aims to document the assessment and management of ADEM from the intensive care unit to the home setting by physical therapy. Also, the child developed ventilator-associated pneumonia and a right lower motor neuron facial injury for which the child was referred to paediatric physical therapy. Since then, continuing for 8 months has helped the child to be independent in all aspects of mobility with no complaints. The child showed improvement in WeeFIM scores and Sunnybrook facial grading after 99 sessions of intensive physical therapy for approximately 83 hours along with the home programme. It has been proven an efficient treatment method along with other medical lines of treatment for neurological impairment associated with ADEM.

The role of plasmapheresis in severe acute disseminated encephalomyelitis with clinical findings of transverse myelitis.

INTRODUCTION: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM. MATERIALS AND METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE. RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day. CONCLUSION: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.

Acquired Demyelinating Syndromes of the Central Nervous System in Children: The Importance of Regular Follow-up in the First Year After Onset.

AIM: We reviewed the clinical features of a sample of pediatric acquired demyelinating syndromes with the purpose of determining the appropriate protocol for follow-up after the first episode. METHODS: A multicenter retrospective observational study was conducted on a cohort of 40 children diagnosed with a first episode of acquired demyelinating syndrome over the period 2012-2021. Patients were evaluated with clinical and neuroradiologic assessment after 3, 6, and 12 months, with a median follow-up of 4.0 years. RESULTS: At the first acquired demyelinating syndrome episode, 18 patients (45%) were diagnosed with acute disseminated encephalomyelitis, 18 (45%) with clinical isolated syndrome, and 4 (10%) with multiple sclerosis. By month 12, 12 patients (30%) had progressed from an initial diagnosis of acute disseminated encephalomyelitis (2) or clinical isolated syndrome (10) to multiple sclerosis. Of these, 6 had clinical relapse and 6 radiologic relapse only. The first relapse occurred after a median of 3 months. Among the patients who had evolved toward multiple sclerosis, there was a prevalence of females (P = .014), higher oligoclonal bands positivity (P = .009), and older median age (P < .001) as compared with those who had remained stable. INTERPRETATION: Both clinical and radiologic follow-up of children with acquired demyelinating syndromes is crucial, especially during the first year after acute onset, for early identification of multiple sclerosis and prompt initiation of disease-modifying treatment to delay axonal damage and to limit disability.

[A case of anti-myelin oligodendrocyte glycoprotein antibody-positive multiphasic disseminated encephalomyelitis showing significant recovery after immunoadsorption plasmapheresis].

The patient is an 18-year-old female. She had a history of acute disseminated encephalomyelitis at the age of 6 and 7. She visited our hospital due to acute disturbance of consciousness, quadriplegia, and numbness of left upper and lower extremities. Brain MRI showed multiple DWI/FLAIR high-signal lesions in the bilateral cerebral hemispheres, cerebellum, and brainstem. Qualitative test indicated that serum anti-MOG antibodies was positive, and she was diagnosed with anti-MOG antibody-positive polyphasic disseminated encephalomyelitis. Intravenous mPSL pulse therapy was performed twice, but the symptoms worsened. As a second line treatment, plasma exchange was started. However, she developed transfusion related acute lung injury. Alternatively, she was treated with immunoadsorption plasmapheresis. Her symptoms were significantly improved. This case seems to be valuable because there are few reports showing effectiveness of immunoadsorption therapy on anti-MOG antibody-related diseases, especially for polyphasic disseminated encephalomyelitis.

Therapeutic plasma exchange as a treatment modality for steroid refractory acute disseminated encephalomyelitis - A case report.

The epidemiology of Dengue fever in the Indian subcontinent has been substantially increased. Acute disseminating encephalomyelitis is a rare complication of dengue and is characterized by multifocal white matter involvement and encephalopathy with neurological deficits. Treatment is usually steroids, IVIG and therapeutic plasma exchange (TPE). We report a case of 46 year old female patient who was a non responder to steroid treatment and successfully treated by TPE.

Acute Hemorrhagic Leukoencephalitis (AHLE): A Comprehensive Review on Causes, Symptoms, Link with COVID-19, Diagnosis, and Treatment.

Acute hemorrhagic leukoencephalitis (AHLE), also called Hurst disease, is a rare demyelinating disease of the central nervous system (CNS) marked by rapid progression and acute inflammation of the white matter. Due to the correlation in their suspected postinfectious autoimmune pathogenesis, it is regarded as the most severe form of acute disseminated encephalomyelitis (ADEM). Because this clinical scenario has a high mortality rate, aggressive and immediate treatment is required. Although the exact cause of AHLE is unknown, it usually occurs after a bacterial or viral infection, or, less frequently, after a measles or rabies vaccination. AHLE has been reported in patients with coronavirus disease 2019 (COVID-19) as a rare but serious neurological complication. However, due to the lack of evidence-based diagnostic criteria, diagnosis is difficult. The small number of cases described in the literature, which most likely reflects underreporting and/or low incidence, necessitates greater public awareness. Increased clinical suspicion and early imaging identification of this entity may allow clinicians to pursue more aggressive treatment options, potentially reducing fatal outcomes. This study focuses on symptoms and causes of AHLE, difference between AHLE and ADME, diagnosis and treatment of AHLE, and its link with COVID-19.

Acute disseminated encephalomyelitis.

ABSTRACT: Acute disseminated encephalomyelitis (ADEM) is a relatively rare autoimmune process that causes demyelination of the central nervous system. This condition primarily affects children under age 10 years and can produce symptoms including fever, vomiting, headaches, and altered mental status. Diagnostic criteria include encephalopathy (behavioral changes or altered mental status not explained by fever) and MRI findings of demyelination during the first 3 months of developing symptoms without subsequent new MRI findings. Patients can have full recovery within days to weeks if recognized and treated promptly. This article describes an ED visit and hospital course for a 3-year-old girl with headaches and fatigue who was diagnosed with and treated for ADEM.

Prognostic factors for functional recovery in children with moderate to severe acute disseminated encephalomyelitis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an immune-mediated encephalopathy with heterogeneous disease courses. However, clinical characteristics for a prognostication of functional recovery from acute episodes of ADEM remain limited. The study aims to characterize the clinical presentations and neuroimaging findings of children with poor functional recoveries from acute episodes of moderate to severe ADEM. METHODS: The multicenter retrospective cohort study included children under 18 years of age who presented with moderate to severe ADEM (modified Rankin Scale [mRS] ≥ 3 at nadir) from 2002 to 2019. Children were assigned to a good recovery group (mRS ≤ 2) and a poor recovery group (mRS ≥ 3) after mean 4.3 months of follow-up. The clinical presentations and the distribution of brain lesions on magnetic resonance imaging were compared between the two groups by the t-test for numerical variables and Fisher's exact test for categorical variables. Analyses of logistic regression were conducted and significant variables in the multivariate model were examined by the receiver operating characteristic curve for the prediction of functional recovery. RESULTS: Among the 73 children with moderate to severe ADEM, 56 (77%) had good functional recoveries and 17 (23%) showed poor functional recoveries. Children with poor recoveries had a lower rate of prodromal headache (12% vs. 39%, p = 0.04), and presented with higher proportions of dystonia (29% vs. 9%, p = 0.046), myoclonus (24% vs. 2%, p = 0.009), and cerebellar lesions on neuroimages (59% vs. 23%, p = 0.01). The multivariate analyses identified that a lack of prodromal headache (OR 0.1, 95% CI 0.005 - 0.7, p = 0.06) and the presentations of myoclonus (OR 21.6, 95% CI 1.7 - 874, p = 0.04) and cerebellar lesions (OR 4.8, 95% CI 1.3 - 19.9, p = 0.02) were associated with poor functional recoveries. These three factors could prognosticate poor outcomes in children with moderate to severe ADEM (area under the receiver operating characteristic curve 0.80, 95% CI 0.68 - 0.93, p = 0.0002). CONCLUSION: Nearly one-fourth of children with moderate to severe ADEM had a poor functional recovery from acute episodes, who were characterized by a lack of prodromal headache, the presentation of myoclonus, and the neuroimaging finding of cerebellar lesions. The clinical variables associated with poor functional recoveries could assist in the planning of immunotherapies during hospitalization for a better outcome in moderate to severe ADEM.

Clinical Presentation and Outcomes of Acute Disseminated Encephalomyelitis in Adults Worldwide: Systematic Review and Meta-Analysis.

Background: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disorder that is often misdiagnosed. To improve early diagnosis, we performed a systematic review and meta-analysis of clinical features, outcomes for ADEM in adults. Methods: The PubMed, Embase, Web of Science and Cochrane Library databases were searched for studies reporting the clinical features of adults with ADEM between January 1990 and May 2021. A random-effects meta-analysis model was used to pool data on clinical features and functional outcomes. Results: Twelve studies examining 437 adults with ADEM met the inclusion criteria. Overall, the clinical features and diagnostic findings observed in more than two-thirds of the patients were white matter lesions [87.1%, 95% confidence interval (CI)=75-95.6], polyfocal onset (80.5%, 95% CI=50.5-98.9) and pyramidal signs (68.7%, 95% CI =40.0-91.9). The mortality rate was 7.8% (95% CI = 3.3-13.5), and the risk of residual deficits was 47.5% (95% CI = 31.8-63.4). Conclusions: Adults with ADEM had worse outcomes than children. Clinicians should maintain high clinical suspicion for patients presenting with certain clinical features and diagnostic findings.

Therapeutic plasma exchange in pediatric patients with acute demyelinating syndromes of the central nervous system: A single-center experience.

BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal treatment that can be used in adult and pediatric patients with acute demyelinating syndromes of the central nervous system. In this study, the efficacy and safety of TPE was evaluated in 10 pediatric patients who underwent TPE that were unresponsive to corticosteroid treatment. METHODS: Records of 10 pediatric patients who underwent TPE in our pediatric intensive care unit (PICU) between May 2017 and June 2020 were used. Expanded Disability Status Scale (EDSS), Gait Scale (GS), and Visual Outcome Scale (VOS) were applied to the patients before and after TPE. RESULTS: Of the 10 patients who underwent TPE, five were diagnosed with multiple sclerosis (MS), three with transverse myelitis (TM), and two with acute disseminated encephalomyelitis (ADEM). The median age of the patients was 13.3 years (IQR 8-15), and the median day from symptom onset to onset of TPE was 12.5 days (IQR 7-28). A total of 104 TPE sessions were performed successfully. While no complications were encountered in three patients during the sessions, the most common complication was hypofibrinogenemia. The decrease in EDSS and GS scores was found to be consistent with the clinical response of the patients. There was no statistically significant decrease in the VOS. CONCLUSIONS: With this study, we can say that TPE is a feasible, effective, and safe treatment modality in children with acute demyelinating syndromes of the central nervous system.

[Acute disseminated encephalomyelitis]. / Akute disseminierte Enzephalomyelitis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease that occurs predominantly in children. According to the guidelines, ADEM belongs to the myelin oligodendrocyte glycoprotein (MOG)-associated diseases and usually manifests after febrile infections (also after SARS-CoV-2) or postvaccinally. OBJECTIVES: Incidence, course and clinical, and as well, as radiological features and new developments and treatment of ADEM. METHODS: Analysis and review of the literature on ADEM and of notable cases and guidelines. RESULTS: The first signs of ADEM include fever, nausea and vomiting, headache and meningism as well as, by definition, encephalopathy, which usually manifests as drowsiness and confusion. The radiological diagnosis is made by magnetic resonance imaging (MRI). Here, the asymmetrically distributed, diffuse and tumefactive lesions can be located supra- and infratentorially. In the acute phase, the lesions usually show contrast enhancement and restricted diffusion. Spinal involvement of the gray matter with the typical H­pattern with myelitis transversa is not uncommon. ADEM has mostly a monophasic course, with a recurrent form ("relapsing ADEM") in 1-20% of cases. For treatment, steroids and in severe cases immunosuppressive drugs are used. CONCLUSIONS: ADEM is generally a monophasic disease whose symptoms usually last for a few weeks or months. It is crucial to differentiate ADEM from other demyelinating diseases, like for example multiple sclerosis, in order not to delay the proper treatment.

[An 11 year old woman with myelin-oligodendrocyte glycoprotein antibody showing various phenotypes of central nervous system disorders in one year].

An 11-year-old woman with myelin-oligodendrocyte glycoprotein (MOG) antibody developed cortical encephalitis twice, followed by acute disseminated encephalomyelitis (ADEM) and optic neuritis in one year. Although optic neuritis was refractory after corticosteroid therapy, plasma exchange was effective and complete remission was achieved. We considered that episodes of cortical encephalitis, ADEM and optic neuritis occurred in the present patient can be included in MOG IgG-associated disorders. Also, we recommend plasma exchange for refractory MOG IgG-associated optic neuritis, even in pediatric patient.

Acute Hemorrhagic Leukoencephalitis - A Rare but Fatal Form of Acute Disseminated Encephalomyelitis - Complicated by Brain Herniation: A Case Report and Literature Review.

BACKGROUND Acute hemorrhagic leukoencephalitis (AHLE) is a very rare fulminant post-infectious demyelinating disease of the CNS. We report an atypical presentation of AHLE involving unique brain areas 2 weeks following a viral upper-respiratory tract infection (URTI). Early diagnosis and proper management improve the prognosis of this disease, and AHLE can have a very poor prognosis and high mortality rate. CASE REPORT A 52-year-old male patient was referred for deteriorating consciousness 2 weeks after a viral URTI. An initial brain CT scan showed multiple patchy bilateral and diffuse hypodense areas including the cerebellar, occipital, parietal, and frontal lobes. The diagnostic workup also included CSF analysis and MRI of the brain, which revealed multiple areas of hemorrhagic involvement. Management included broad-spectrum antibiotics, acyclovir, mannitol, steroids, and plasmapheresis. On the fifth day of admission, brain CT showed severe diffuse edema and brain herniation. Unfortunately, despite prompt aggressive treatment measures, within 48 hours the patient died due to centrally-mediated hemodynamic instability. CONCLUSIONS We report a rare case of AHLE with a unique presentation and extensive unusual involvement of regions of periventricular and subcortical white matter, cerebellum, and midbrain. Early diagnosis along with appropriate management measures and intensive care can help decrease morbidity and mortality; therefore, prompt referral and high-level care should be sought for all patients who present with acute deteriorating consciousness. We hope that this report can help future studies to better characterize this rare disease and provide further guidance regarding prognosis and management.

Lactant amb torticoli aguda. És sempre una entitat benigna? / Lactante con tortícolis aguda. ¿És siempre una entidad benigna? / Infant with acute torticollis. Is it always a benign entity?

Introducció. L’encefalomielitis aguda disseminada (EMAD) és una malaltia inflamatòria immunomediada desmielinitzant del sistema nerviós central. Associa simptomatologia neurològica multifocal i encefalopatia. Afecta fonamentalment la població infantil, amb una incidència anual estimada en menors de catorze anys del 0,64/100.000. El diagnòstic de l’EMAD és d’exclusió. Cas clínic. Lactant de quatre mesos, sana i amb neurodesenvolupament adequat, és portada a urgències per desviació lateral dreta de cap i coll. Associa quadre catarral sense febre. A les 48 hores de l’ingrés desenvolupa oftalmoplegia, sialorrea i nivell d’alerta alternant. Atesa l’aparició de simptomatologia neurològica, es deriva a centre de referència on s’amplia l’estudi etiològic. La ressonància magnètica (RM) cerebroespinal mostra alteració d’intensitat de senyal de tàlems, nuclis lenticulars i tronc de l’encèfal esquerre. Davant la sospita d’EMAD, rep tractament amb glucocorticoides i gammaglobulina. També rep biotina i tiamina a l’espera de l’estudi metabòlic. La RM cerebroespinal al cap de deu dies mostra reducció de les lesions. La recuperació dels dèficits neurològics dos mesos després és completa. Comentaris. La clínica, l’evolució i els estudis complementaris són suggestius d’EMAD. Les lesions típiques d’aquesta entitat a la RM afecten la substància blanca subcortical profunda de forma bilateral i asimètrica. També hi pot haver afectació de ganglis de base, tàlem i tronc de l’encèfal. El diagnòstic diferencial inclou encefalitis infecciosa, esclerosi múltiple i metabolopaties. La primera línia de tractament són els bols de glucocorticoides. La majoria de casos presenta recuperació completa. L’interès del cas exposat rau en l’edat de presentació. (AU)

Introducción. La encefalomielitis aguda diseminada (EMAD) es una enfermedad inflamatoria inmunomediada desmielinizante del sistema nervioso central. Asocia sintomatología neurológica multifocal y encefalopatía. Afecta fundamentalmente la población infantil con una incidencia anual estimada en menores de 14 años del 0,64/100.000. El diagnóstico de EMAD es de exclusión. Caso clínico. Lactante de 4 meses, sana y con adecuado neurodesarrollo, es traída a urgencias por desviación lateral derecha de cabeza y cuello; asocia cuadro catarral sin fiebre. A las 48 horas del ingreso desarrolla oftalmoplejía, sialorrea y nivel de alerta alternante. Dada la aparición de sintomatología neurológica, se deriva a centro de referencia donde se amplía el estudio etiológico. La resonancia magnética (RM) cerebroespinal muestra alteración de intensidad de señal de tálamos, núcleos lenticulares y tronco del encéfalo izquierdo. Ante la sospecha de EMAD, recibe tratamiento con glucocorticoides y gammaglobulina. También recibe biotina y tiamina a la espera del estudio metabólico. La RM cerebroespinal a los 10 días muestra reducción de las lesiones. La recuperación de los déficits neurológicos dos meses después es completa. Comentarios. La clínica, evolución y estudios complementarios son sugestivos de EMAD. Las lesiones típicas de esta entidad en la RM afectan la sustancia blanca subcortical profunda de forma bilateral y asimétrica. También puede haber afectación de ganglios de base, tálamo y tronco del encéfalo. El diagnóstico diferencial incluye encefalitis infecciosa, esclerosis múltiple y metabolopatías. La primera línea de tratamiento son bolus de glucocorticoides. La mayoría de casos presenta recuperación completa. El interés del caso expuesto radica en la edad de presentación. (AU)

Introduction. Acute disseminated encephalomyelitis (ADEM) is a demyelinating immune mediated inflammatory disease affecting the central nervous system. It associates multifocal neurological symptoms and encephalopathy. It predominantly affects children, and its annual cumulative incidence in children under 14 years is 0.64/100,000. The diagnosis of ADEM is one of exclusion. Case report. A previously healthy 4-month-old infant with adequate neurodevelopment was seen in the emergency department due to acute onset of right lateral deviation of head and neck, associated with cold symptoms without fever. 48 hours after admission, the infant developed ophthalmoplegia, sialorrhea and alternating alert level. Given the development of neurological symptoms, she was referred to a tertiary hospital for evaluation. Cerebrospinal magnetic resonance imaging (MRI) showed altered signal intensity of the thalamus, lenticular nuclei, and left brainstem. ADEM was suspected and glucocorticoids and gamma globulin were administered. She also received biotin and thiamine while awaiting results of a metabolic panel. Cerebrospinal MRI at 10 days showed improvement of the lesions, and a full recovery was reached after two months. Comments. The clinical presentation, diagnostic studies, and clinical evolution are suggestive of ADEM. Typical MRI lesions involve deep subcortical white matter bilaterally and asymmetrically. There may also be involvement of the basal ganglia, thalamus, and brainstem. Differential diagnosis includes infectious encephalitis, multiple sclerosis, and metabolic diseases. First line treatment are glucocorticoids. Most cases have complete recovery. The interest of the case lies in the age of presentation. (AU)

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

BACKGROUND AND OBJECTIVES: Since 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. METHODS: Patients 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. RESULTS: Forty-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. DISCUSSION: In 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.

COVID-19-associated Acute Disseminated Encephalomyelitis-like Disease in 2 Children.

BACKGROUND: The coronavirus disease 2019 pandemic was caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Although the predominant clinical presentation is a respiratory disease, neurologic manifestations are being recognized increasingly. CASE REPORT: We report 2 children 9 years of age who developed acute disseminated encephalomyelitis-like disease associated with SARS-CoV-2. Seizures and encephalopathy were the main central nervous system symptoms. The cerebrospinal fluid analysis performed within the first week of disease onset showed elevated protein in both children with normal cell count and no evidence of infection including negative SARS-CoV-2 by antibody and polymerase chain reaction. Brain magnetic resonance imaging revealed T2A, fluid-attenuated inversion recovery cortical and subcortical hyperintensity without restricted diffusion consistent with acute disseminated encephalomyelitis-like disease. They received methylprednisolone followed by therapeutic plasma exchange. One of them showed complete clinical improvement and resolution in magnetic resonance imaging findings. The other developed laminar necrosis in brain magnetic resonance imaging and showed significant clinical improvement after therapeutic plasma exchange. He was positive for positive SARS-CoV-2 antibody in cerebrospinal fluid on day 55 of admission. They were both positive for SARS-CoV-2 antibodies in serum after 2 weeks. CONCLUSIONS: Our two cases highlight the occurrence of acute disseminated encephalomyelitis-like disease as a postinfectious/immune-mediated complication of SARS-CoV-2 infection.

Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient.

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that typically presents in childhood and is associated with encephalopathy and multifocal brain lesions. Although ADEM is thought to be a post-infectious disorder, the etiology is still poorly understood. ADEM is often a monophasic disorder, in contrast to other demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorder. With increasing awareness, understanding, and testing for myelin oligodendrocyte glycoprotein antibodies, this disease is now known to be a cause of pediatric ADEM and also has the potential to be relapsing. Diagnostic evaluation for ADEM involves neuroimaging and laboratory studies to exclude potential infectious, inflammatory, neoplastic, and genetic mimics of ADEM. Acute treatment modalities include high-dose intravenous corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin. Long-term outcomes for ADEM are generally favorable, but some children have significant morbidity related to the severity of acute illness and/or manifest ongoing neurocognitive sequelae. Further research related to the optimal management of pediatric ADEM and its impact on prognosis is needed. This review summarizes the current knowledge of the pathogenesis, epidemiology, clinical features, diagnostic evaluation, treatment approaches, and outcomes in pediatric ADEM.

Clinical Features, Treatment Strategies, and Outcomes in Hospitalized Children With Immune-Mediated Encephalopathies.

BACKGROUND: Autoimmune encephalitis (AE) and acute disseminated encephalomyelitis (ADEM) are immune-mediated brain conditions that can cause substantial neurological sequalae. Data describing the clinical characteristics, treatments, and neurological outcomes for these conditions are needed. METHODS: This is a single-center retrospective review of children diagnosed with AE or ADEM over a nine-year period with discharge outcomes measured by the Modified Rankin Score. RESULTS: Seventy-five patients (23 with ADEM and 52 with AE) were identified. Patients with ADEM had a higher percentage of abnormal magnetic resonance imaging findings (100% vs 60.8%; P < 0.001) and a shorter time from symptom onset to diagnosis (6 vs 14 days; P = 0.024). Oligoclonal bands and serum and cerebrospinal fluid inflammatory indices were higher in patients with AE. Nearly all patients received corticosteroids followed by plasmapheresis or intravenous immunoglobulin, and treatment strategies did not differ significantly between groups. Second-line immune therapies were commonly used in patients with AE. Finally, patients with AE had trends toward longer hospital lengths of stay (21 vs 13 days) and a higher percentage of neurological disability at hospital discharge (59.6% vs 34.8%). CONCLUSIONS: Although patients with ADEM and AE may have similar presenting symptoms, we found significant differences in the frequency of imaging findings, symptom duration, and laboratory and cerebrospinal fluid profiles, which can assist in distinguishing between the diagnoses. Patients in both groups were treated with a combination of immunomodulating therapies, and neurological disability was common at hospital discharge.