[Weston-Hurst syndrome. A case report and literature review]. / Síndrome de Weston Hurst. Reporte de un caso y revisión de la literatura.

Background: Acute disseminated encephalomyelitis is an autoimmune and demyelinating disease. It is rare in adults. It has 3 main variants. One of them is Weston-Hurst syndrome, also called acute hemorrhagic leukoencephalitis. The objective was to share the experience in the diagnostic and therapeutic approach of this rare disease, as well as make a review of the current bibliography, in order to collaborate in the knowledge of this disease. Clinical case: 27-year-old woman, with a viral respiratory infection 2 weeks prior to the development of a neurological syndrome characterized by paresthesia, motor deficit, status epilepticus and acute encephalopathy, progressing rapidly to coma, with evidence in MRI of diffuse hemorrhagic lesions in cerebral white matter with demyelination and peripheral edema. It was administered steroid treatment for 5 days, with improvement of symptoms, but with motor and sensory deficits persisting. Conclusion: Acute disseminated encephalomyelitis and its variants are rare entities, with an important range of differential diagnosis, which must be identified and quickly treated to avoid their lethal or disabling outcome.

Introducción: la encefalomielitis aguda diseminada es una enfermedad autoinmune y desmielinizante. Es rara en el adulto. Cuenta con tres variantes principales. Una de ellas es el síndrome de Weston Hurst, también conocido como leucoencefalitis hemorrágica aguda. El objetivo fue compartir la experiencia en el abordaje diagnóstico y terapéutico de esta rara enfermedad, así como hacer una revisión de la bibliografía actual, a fin de colaborar con el conocimiento de esta. Caso clínico: mujer de 27 años con cuadro de infección respiratoria viral 2 semanas previas al desarrollo de síndrome neurológico caracterizado por parestesias, déficit motor, estatus epiléptico y encefalopatía aguda, el cual progresó a estado de coma y evidenció en resonancia magnética lesiones difusas hemorrágicas en sustancia blanca cerebral con desmielinización y edema periférico. Se inició tratamiento con esteroides por 5 días con mejora de síntomas, aunque persistió el déficit motor y sensitivo. Conclusión: la encefalomielitis aguda diseminada y la variante hemorrágica de esta son entidades raras, con una importante gama de diagnóstico diferencial, que deben ser identificadas y tratadas de forma rápida para evitar su letal o incapacitante desenlace.

Acute disseminated encephalomyelitis in a patient with monkeypox: a case report and radiological findings.

Monkeypox is a zoonosis caused by a double-stranded DNA virus of the Poxviridae family. It currently represents a global epidemic given its contagion reported in more than 31 previously non-endemic countries. We present the case of a 30-year-old male patient from Peru with a diagnosis of monkeypox by PCR test, who manifested an initial clinical picture of asthenia, adynamia, and odynophagia, with the appearance of pustular lesions on the lower lip and in the genital region associated with motor and sensory deficit of the lower limbs with altered state of consciousness, with subsequent findings of acute disseminated encephalitis by brain magnetic resonance imaging. This entity corresponds to an infrequent complication with only one case reported in the literature. The importance lies in knowing the possible imaging findings to suspect the diagnosis and expand the knowledge about this current disease.

Levamisole-associated multifocal inflammatory encephalopathy: clinical and MRI characteristics, and diagnostic algorithm.

Levamisole-associated multifocal inflammatory encephalopathy (LAMIE) is a devastating adverse effect of levamisole (LEV) treatment. In Russia, people often use LEV without a doctor's prescription for anthelmintic prophylaxis. LAMIE often misdiagnosed as the first episode of MS or acute disseminated encephalomyelitis (ADEM). The aim of our study was to describe clinical, laboratory and morphological characteristics of LAMIE, magnetic resonance imaging (MRI) patterns and create an algorithm for the differential diagnosis. This study was a prospective observational study with retrospective analysis of cases. It was performed at two hospitals with ambulatory service for MS. We included 43 patients with LAMIE with follow-up was from 1 year to 5 years. Age was 19-68 y.o. with female predominance. The most typical manifestations of LAMIE were cerebellar, pyramidal and cognitive symptoms, and majority of patients had biphasic course of the disease. Three main types of MRI patterns were described: ADEM-like, MS-like, atypical demyelination. About 40% of patients had CSF specific oligoclonal bands synthesis, but only 20 % of them converted to MS during the period from 1 month until 2 years. The CSF albumin levels and immunoglobulin G index were elevated in LAMIE patients compared to reference values. We described results of brain biopsy in two cases. Therefore LAMIE should be considered in patients with demyelinating or inflammatory conditions with biphasic onset of the disease and variable MRI presentation.

Oligoclonal bands, age 11-17 years, occipital lesion, and female sex differentiate pediatric multiple sclerosis from acute disseminated encephalomyelitis: A nationwide cohort study.

BACKGROUND: Our aim was to propose criteria to distinguish multiple sclerosis (MS) from acute disseminated encephalomyelitis (ADEM) at onset based on age at onset, sex, cerebrospinl fluid (CSF)-specific oligoclonal bands, and MRI. METHODS: A neuroradiologist undertook retrospective evaluation of the baseline magnetic resonance imaging (MRI) in a nationwide cohort of children with medical record-validated MS (n = 67) and monophasic ADEM (n = 46). Children with ADEM had at least 5 years of follow-up for relapse. We used forward stepwise conditional logistic regression to develop our criteria based on age at onset, sex, CSF-specific oligoclonal bands, and MRI. We undertook sensitivity analyses using children with ADEM including encephalopathy and polyfocal neurological deficits and in those with onset between 11 and 17 years of age. We estimated accuracy statistics from our criteria and all previously proposed MRI criteria to distinguish MS and ADEM. RESULTS: The best performing criteria to differentiate MS from ADEM were scoring at least three points in the following categories: presence of CSF-specific oligoclonal bands (2 points), occipital lesion (1 point), age 11-17 years (1 point), female sex (1 point). These criteria gave highly reliable discrimination with sensitivity of 95% (95% CI=89%-100%), specificity of 100% (95% CI=100%-100%), and area under the curve of 98% (95% CI=95%-100%). The best performing MRI criteria had area under the curve of 84% (95% CI=78%-91%). Previously proposed MRI criteria had the following areas under the curve: Callen (75%), KIDMUS (82%), and McDonald 2017 criteria (68%). CONCLUSION: Combining sex, age at onset, CSF-specific oligoclonal bands, and MRI gives highly reliable differentiation between pediatric MS and monophasic ADEM at onset.

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.

Magnetic resonance imaging criteria at onset to differentiate pediatric multiple sclerosis from acute disseminated encephalomyelitis: A nationwide cohort study.

BACKGROUND: MRI of the nervous system is the critical in distinguishing pediatric MS from acute disseminated encephalomyelitis (ADEM). Our aim was to propose MRI criteria to distinguish MS from monophasic ADEM based on the first MRI and to validate previously proposed MRI criteria. METHODS: A neuroradiologist undertook retrospective evaluation of the MRI at the first demyelinating event in children (<18 years) with medical record-validated MS and ADEM in Denmark during 2008-15. We used forward stepwise logistic regression to identify MRI categories that differed significantly between MS and ADEM. We estimated accuracy statistics for all MRI criteria to distinguish MS from ADEM. RESULTS: The monophasic ADEM cohort (n=46) was nationwide and population-based during 2008-15; the median age at onset of 5.3 years (range 0.8‒17.2) and children had at least five years of follow-up to ensure a monophasic disease course. Children with MS (n=67) had a median age at onset of 16.3 years (range 3.3‒17.9). Having at least two categories best distinguished MS from monophasic ADEM by an area under the curve of 83% to 89%: (a) corpus callosum long axis perpendicular lesion; (b) only well-defined lesions; (c) absence of basal ganglia or thalamus lesion OR, (a) corpus callosum long axis perpendicular lesion; (b) only well-defined lesions; (c) absence of diffuse large lesions; (d) black holes. The Callen, KIDMUS, and IPMSSG criteria performed well. The McDonald 2017, Barkhof, MAGNIMS, and Verhey criteria had poorer performance. CONCLUSION: This study provides Class II evidence that MRI has good performance in differentiating MS from monophasic ADEM at onset.

Acute disseminated encephalitis in an adult patient addicted to heroin. Neuropathological, neuroradiological and clinical features.

Acute disseminated encephalomyelitis (ADEM) is an immune demyelinating central nervous system (CNS) disorder, characterized by monophasic new onset neurological symptoms including encephalopathy, combined with neuroradiological evidence of multifocal demyelination. ADEM is extremely rarely diagnosed and is much more common in children and adolescents than in adults. The aim of this study is to present an extremely rare case of ADEM in a heroin-addicted patient with a very difficult diagnostic course. The results of the magnetic resonance imaging (MRI) examination in this patient were inconclusive. Fungal abscesses or inflammatory lesions of an unclear nature were suspected especially in a patient with impaired immunodeficiency. In view of the constantly deteriorating condition of the patient with disturbed consciousness and the unclear aetiology, the lack of effective treatment, a decision was made to perform a bilateral stereotactic biopsy and aspiration of brain abnormalities in order to obtain a neuropathological specimen and begin with the causal treatment. Neuropathological examination revealed the presence of Creutzfeldt-Peters cells characteristic of ADEM. Treatment with methylprednisolone significantly improved the patient's general and neurological condition. To our knowledge, the above case is the first in the world literature in which ADEM has been confirmed by bilateral stereotaxic aspiration for the treatment of symptoms of increased intracranial pressure as a lifesaving procedure. Neuropathological confirmation allowed for the implementation of appropriate treatment, which resulted in complete recovery. Moreover, this case is interesting because ADEM was diagnosed in a patient addicted to heroin, where opportunistic inflammation of a fungal aetiology was considered in the first place.

SARS-CoV-2-associated acute disseminated encephalomyelitis: a systematic review of the literature.

The literature on cases of acute disseminated encephalomyelitis (ADEM) associated with SARS-CoV-2 infection has been rapidly increasing. However, the specific clinical features of ADEM associated with SARS-CoV-2 (SARS-CoV-2-ADEM) have not been previously evaluated. We screened all articles resulting from a search of PubMed and Web of Science databases looking for reports of ADEM published between December 01, 2019, and June 5, 2021. Of the 48 ADEM cases identified from 37 studies, 34 (71%) had ADEM while 14 (29%) were of AHLE. RT-PCR for SARS-CoV-2 was positive in 83% (n = 19) of patients. 26 patients (54%) were male, and 18 patients (38%) were female, with a male to female sex ratio of 1.4:1; median age was 44 (1.4-71) years. 9 patients (19%, 9/48) were children. Of the 9 children patients, their median age was 9 years (range 1.4-13 years), 6 patients (67%) were female, and 2 patients (22%) were male, with a female to male sex ratio of 3:1.39 patients (81%) was performed CSF analysis. PCR for SARS-CoV-2 tested positive in 3 patients (14%, 3/22) on CSF sample. 31 (64%) of patients had a poor outcome on discharge from hospital. Five (10%) patients died in hospital. Compared to classic ADEM, SARS-CoV-2-ADEM have a more longer duration between the onset of the antecedent infective symptoms and the start of ADEM symptoms, the older age distribution of the patients, relatively poor outcome, a lower full recovery rate, a more frequently brain lesions involved the periventricular white matter and corpus callosum, and less frequently affected the deep gray matter. Taken together, the present comprehensive review reveals that although rare, ADEM can be associated with SARS-CoV-2 infection. SARS-CoV-2-ADEM seems to share most features of classic ADEM, with moderate discrepancies from the classical ADEM.

Clinical and neuroimaging findings in MOGAD-MRI and OCT.

Myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are rare in both children and adults, and have been recently suggested to be an autoimmune neuroinflammatory group of disorders that are different from aquaporin-4 autoantibody-associated neuromyelitis optica spectrum disorder and from classic multiple sclerosis. In-vivo imaging of the MOGAD patient central nervous system has shown some distinguishing features when evaluating magnetic resonance imaging of the brain, spinal cord and optic nerves, as well as retinal imaging using optical coherence tomography. In this review, we discuss key clinical and neuroimaging characteristics of paediatric and adult MOGAD. We describe how these imaging techniques may be used to study this group of disorders and discuss how image analysis methods have led to recent insights for consideration in future studies.

Gist of Zika Virus pathogenesis.

Zika virus (ZIKV) is a mosquito-borne neurotropic flavivirus. ZIKV infection may lead to microcephaly in developing fetus and Guillain-Barré Syndrome (GBS) like symptoms in adults. ZIKV was first reported in humans in 1952 from Uganda and the United Republic of Tanzania. Later, ZIKV outbreak was reported in 2007 from the Yap Island. ZIKV re-emerged as major outbreak in the year 2013 from French Polynesia followed by second outbreak in the year 2015 from Brazil. ZIKV crosses the blood-tissue barriers to enter immune-privileged organs. Clinical manifestations in ZIKV disease includes rash, fever, conjunctivitis, muscle and joint pain, headache, transverse myelitis, meningoencephalitis, Acute Disseminated Encephalomyelitis (ADEM). The understanding of the molecular mechanism of ZIKV pathogenesis is very important to develop potential diagnostic and therapeutic interventions for ZIKV infected patients.

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.

Neuroimaging Findings in Rabies Encephalitis.

BACKGROUND AND PURPOSE: Rabies encephalitis is a near-fatal zoonotic disease that is usually diagnosed on clinical grounds in conjunction with characteristic history. Owing to its rapidly progressive nature, imaging is seldom performed, and hence a description of imaging findings in rabies encephalitis is anecdotal and limited. METHODS: We describe MRI findings in eight confirmed rabies cases that presented to our institute over the last 21 years. RESULTS: Most of the patients' imaging patterns are in concordance with the described literature. However, we hereby demonstrate the involvement of novel structures like dentate nuclei, cranial nerves, and meninges besides the hot cross bun sign in the pons and the presence of diffusion restriction in many gray and white matter structures of the brain. CONCLUSION: Knowledge of the broad imaging spectrum of rabies may expedite the diagnosis, especially the paralytic form, which is prone to clinical misdiagnosis as Guillain-Barre syndrome or acute disseminated encephalomyelitis.

Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features.

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

Covid-19 systemic infection exacerbates pre-existing acute disseminated encephalomyelitis (ADEM).

Acute disseminated encephalomyelitis (ADEM) is an uncommon diagnosis in adults. It is known to be due to an abnormal immune response to a systemic infection rather than direct viral invasion to the central nervous system. There have been few reports of ADEM diagnosed in the setting of COVID-19 systemic infection. However, we report a case of Coxsackie induced ADEM that remitted but got exacerbated by COVID-19 infection. The patient contracted the COVID-19 infection shortly after being discharged to a rehabilitation facility. Direct COVID-19 neuroinvasion was ruled out via CSF PCR testing for the virus. The patient responded well to pulse steroid therapy and plasmapheresis in both occasions. We hypothesize that COVID-19 infection can flare-up a recently remitted ADEM via altering the immune responses. It is known now that COVID-19 infection can produce cytokine storming. Cytokine pathway activation is known to be involved in the pathology of ADEM. Caution regarding discharging immune suppressed patient to the inpatient rehabilitation facility should be made in the era of COVID-19 pandemic.

Listeria rhombencephalitis mimicking acute disseminated encephalomyelitis in a patient without predisposing medical conditions.

Listeria rhombencephalitis (L. rhombencephalitis) is an uncommon form of central nervous system infection caused by Listeria monocytogenes (LM). It often occurs to immunocompetent individuals. Here, we described the case of a 45-year-old female patient without medical histories, who presented for high-grade fever, headache, and focal neurological manifestations. She was initially empirically diagnosed with acute disseminated encephalomyelitis (ADEM) because of clinical symptoms, acute clinical course, and neuroimaging. However, the biochemical analysis of cerebral spinal fluid (CSF) questioned the diagnosis of ADEM. The final diagnosis of L. rhombencephalitis was based on CSF culture for LM. Thus, L. rhombencephalitis should be preferentially and empirically considered for a patient with significantly elevated lactic acid and moderately increased red cells in CSF at early time, accompanied with rapidly progressive neurological dysfunctions involved in the brain stem.

Acute Disseminated Encephalomyelitis with Seizures and Myocarditis: A Fatal Triad.

Autoimmune pathology of acute disseminated encephalomyelitis (ADEM) is generally restricted to the brain. Our objective is to expand the phenotype of ADEM. A four-year-old girl was admitted to the pediatric emergency room of a university medical center five days after a common upper respiratory tract infection. Acute symptoms were fever, leg pain, and headaches. She developed meningeal signs, and her level of consciousness dropped rapidly. Epileptic seizure activity started, and she became comatose, requiring intubation and mechanical ventilation. Serial brain magnetic resonance imaging (MRI) illustrated the fulminant development of ADEM. Treatment escalation with high-dose corticosteroids, immunoglobulins, and plasma exchange did not lead to clinical improvement. On day ten, the patient developed treatment-refractory cardiogenic shock and passed away. The postmortem assessment confirmed ADEM and revealed acute lymphocytic myocarditis, likely explaining the acute cardiac failure. Human metapneumovirus and picornavirus were detected in the tracheal secrete by PCR. Data sources-medical chart of the patient. This case is consistent with evidence from experimental findings of an association of ADEM with myocarditis as a postinfectious systemic autoimmune response, with life-threatening involvement of the brain and heart.

Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody-associated acute demyelinating syndromes.

AIM: To describe cognitive abilities through the evaluation of academic difficulties in children with acute demyelinating syndromes (ADS) and myelin oligodendrocyte glycoprotein (MOG) antibodies. METHOD: This was an observational, retrospective study of a French paediatric cohort that included children aged 18 years and younger. Clinical, biological, and imaging data were collected and academic outcome was measured. RESULTS: Seventy-six children were included in the study with a mean (SD) follow-up of 4 years 7 months (6y 4mo). Median age at disease onset was 9 years 1 months (interquartile range=4y 7mo-13y 11mo; 36 females, 40 males). Thirty-six children relapsed and 20 had academic difficulties at the last follow-up. Academic difficulties, as well as deep grey matter and putaminal lesions (p=0.047 and p=0.006 respectively), were significantly more prevalent in children aged 10 years and younger (p=0.02). Using univariate binary regression analysis, we found that age at disease onset of 10 years and younger (odds ratio [OR] 3.72 [95% confidence interval {CI} 1.19-11.64]; p=0.024), acute disseminated encephalomyelitis at disease onset (OR 52.5 [95% CI 5.97-461.4]; p<0.001), and deep grey matter lesions (OR 17.33 [95% CI 3.87-77.72]; p<0.001) were associated with academic difficulties. INTERPRETATION: MOG antibody-associated ADS have distinct clinical and radiological patterns that are age-dependent. Indirect cognitive evaluation through academic difficulties was prevalent in younger children and is associated with specific clinical and magnetic resonance imaging factors that need to be considered earlier on when assessing this patient population.

COVID-19-associated acute disseminated encephalomyelitis (ADEM).

A 51-year-old woman with COVID-19 infection developed coma and an impaired oculocephalic response to one side. MRI of the brain demonstrated acute multifocal demyelinating lesions, and CSF testing did not identify a direct cerebral infection. High-dose steroids followed by a course of IVIG was administered, and the patient regained consciousness over the course of several weeks. As more patients reach the weeks after initial infection with COVID-19, acute disseminated encephalomyelitis should be considered a potentially treatable cause of profound encephalopathy or multifocal neurological deficits.

Relationship Between Pregnancy and Acute Disseminated Encephalomyelitis: A Single-Case Study.

The relationship between pregnancy and autoimmune diseases is unclear. This study investigated the possible role of local immune changes and the activation state of the HMGB1/TLR4/Nf-κB/IL-6 pathway at the maternal-fetal interface during pregnancy in the pathogenesis of acute disseminated encephalomyelitis (ADEM). Clinical data and blood samples of a patient with ADEM were collected to observe the dynamic changes in lymphocyte populations after an abortion. The expression of HMGB1, TLR4, Nf-κB, AQP4, IL-2, IL-4, IL-6, and TNF-α in the fetal membrane and placenta was compared between the patient with pregnancy-related ADEM and a woman with a normal pregnancy using Real-time qPCR and western blotting (WB). The patient was diagnosed with ADEM in the early stage of pregnancy after showing limb weakness symptoms. In the third month of gestation, the symptoms worsened, with a disturbance of consciousness and breathing. After the abortion, the patient relapsed with vertigo and visual rotation. Analysis of lymphocyte subsets by flow cytometry showed that B lymphocytes increased, while natural killer T lymphocytes decreased. WB and Real-time qPCR showed that the expression levels of HMGB1, TLR4, Nf-κB, AQP4, and IL-6 in the fetal membrane and placenta were higher in the patient with pregnancy-related ADEM than in the woman with a normal pregnancy, while those of IL-2 were lower in the patient than in the woman with a normal pregnancy. The local immune changes and the activation of the HMGB1/TLR4/Nf-κB/IL-6 pathway at the maternal-fetal interface may be related to the pathogenesis of ADEM.