Insights from neuroradiology in acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated demyelinating disorder primarily observed in children, typically triggered by recent vaccination or viral infection. Although uncommon, there have been reports of ADEM occurring in adults, with varying radiological findings compared to pediatric cases. Distinguishing ADEM from other demyelinating disorders, such as multiple sclerosis (MS) and neuromyelitis optica (NMO), can pose a diagnostic challenge.

Acute disseminated encephalomyelitis and viral encephalitis: An unusual and misleading imaging.

Acute disseminated encephalomyelitis (ADEM) is a rare illness. It is characterized by different presentations like encephalopathy, seizures, hemiplegia, and visual symptoms. We present a patient who presented seizures and encephalopathy.

Safety and Health Care Use Following COVID-19 Vaccination Among Adults With Rheumatoid Arthritis: A Population-Based Self-Controlled Case Series Analysis.

Objective: To determine if coronavirus disease 2019 (COVID-19) vaccines were associated with adverse events of special interest (AESIs) and healthcare use among adults with rheumatoid arthritis (RA).

Methods: Among adults with RA who received at least 1 COVID-19 vaccine, a self-controlled case series (SCCS) analysis was conducted to evaluate relative incidence (RI) rates of AESIs (Bell palsy, idiopathic thrombocytopenia, acute disseminated encephalomyelitis, pericarditis/myocarditis, Guillain-Barré syndrome, transverse myelitis, myocardial infarction, anaphylaxis, stroke, deep vein thrombosis, pulmonary embolism, narcolepsy, appendicitis, and disseminated intravascular coagulation) in any 21-day period following vaccination compared to control periods. Secondary outcomes included emergency department (ED) visits, hospitalizations, and rheumatology visits.

[Weston-Hurst syndrome. A case report and literature review].

Background: Acute disseminated encephalomyelitis is an autoimmune and demyelinating disease. It is rare in adults. It has 3 main variants.

Antibody Deficiency in Patients with Biallelic KARS1 Mutations.

Biallelic KARS1 mutations cause KARS-related diseases, a rare syndromic condition encompassing central and peripheral nervous system impairment, heart and liver disease, and deafness. KARS1 encodes the t-RNA synthase of lysine, an aminoacyl-tRNA synthetase, involved in different physiological mechanisms (such as angiogenesis, post-translational modifications, translation initiation, autophagy and mitochondrial function). Although patients with immune-hematological abnormalities have been individually described, results have not been collectively discussed and functional studies investigating how KARS1 mutations affect B cells have not been performed.

An Observational Study of Multi-Faceted Demyelinating Disorders.

Background And Objectives: Idiopathic inflammatory demyelinating diseases of the central nervous system (IIDCDs) are wide-ranging disorders due to their similarities and differences. In order to address these conditions, studying their characteristics is essential. The endpoints of our study were to assess the incidence, presenting features, MRI findings, and predictors of disease progression of prevalent demyelinating disorders.

Language impairments in people with autoimmune neurological diseases: A scoping review.

Introduction: Autoimmune neurological diseases (ANDs) are a specific type of autoimmune disease that affect cells within the central and peripheral nervous system. ANDs trigger various physical/neuropsychiatric symptoms. However, language impairments in people with ANDs are not well characterized.

Phenotypic characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease in children: a single-center, retrospective study.

Objective: To analyze the clinical characteristics and follow-up data of children with different clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Methods: The basic demographic and clinical features, laboratory and imaging examination results, and follow-up data of 74 Chinese children with different phenotypes of MOGAD were retrospectively reviewed and analyzed.

Results: The male-to-female ratio in this cohort was 1:1.

Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure.

We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm HO. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES).

Neuro-Sweet Syndrome: A Diagnostic Conundrum.

Sweet Syndrome presents as acute fever, leucocytosis and characteristic skin plaques. It can involve many organ systems but rarely affects the nervous system. We report the case of a 51-year-old female that presented with fever, rash, headache and encephalopathy.

Pathogenesis, Clinical Features, and Treatment of Patients with Myelin Oligodendrocyte Glycoprotein (MOG) Autoantibody-Associated Disorders Focusing on Optic Neuritis with Consideration of Autoantibody-Binding Sites: A Review.

Although there is a substantial amount of data on the clinical characteristics, diagnostic criteria, and pathogenesis of myelin oligodendrocyte glycoprotein (MOG) autoantibody-associated disease (MOGAD), there is still uncertainty regarding the MOG protein function and the pathogenicity of anti-MOG autoantibodies in this disease. It is important to note that the disease characteristics, immunopathology, and treatment response of MOGAD patients differ from those of anti-aquaporin 4 antibody-positive neuromyelitis optica spectrum disorders (NMOSDs) and multiple sclerosis (MS). The clinical phenotypes of MOGAD are varied and can include acute disseminated encephalomyelitis, transverse myelitis, cerebral cortical encephalitis, brainstem or cerebellar symptoms, and optic neuritis.

Tuberous sclerosis complex: a case report and literature review.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with different initial symptoms and complex clinical manifestations. A 14-year-old female patient presented with persistent fever and severe headache. Medical imaging examinations revealed multiple abnormal intracranial lesions.

Background rates of adverse events of special interest for COVID-19 vaccines: A multinational Global Vaccine Data Network (GVDN) analysis.

Background: The Global COVID Vaccine Safety (GCoVS) project was established in 2021 under the multinational Global Vaccine Data Network (GVDN) consortium to facilitate the rapid assessment of the safety of newly introduced vaccines. This study analyzed data from GVDN member sites on the background incidence rates of conditions designated as adverse events of special interest (AESI) for COVID-19 vaccine safety monitoring.

Methods: Eleven GVDN global sites obtained data from national or regional healthcare databases using standardized methods.

Juvenile multiple sclerosis: addressing epidemiology, diagnosis, therapeutic, and prognostic updates along with cognitive dysfunction and quality of life.

Juvenile multiple sclerosis (JMS) is a rare but significant subtype of multiple sclerosis (MS) that affects a small percentage of patients under the age of 10 and 3-5% of all MS patients. Despite its rarity, JMS poses unique challenges in terms of diagnosis, treatment, and management, as it can significantly impact a child or adolescent's physical, cognitive, and emotional development. JMS presents with a varying spectrum of signs and symptoms such as coordination difficulties and permanent cognitive dysfunctions and may include atypical clinical features such as seizures, acute disseminated encephalomyelitis, and optic neuritis, making diagnostic evaluations challenging.

Severe and rare neurological manifestations following COVID-19 infection in children: A Malaysian tertiary centre experience.

Introduction: Since the emergence of COVID-19, we have experienced potent variants and sub-variants of the virus with non-specific neurological manifestations. We observed a surge of the Omicron variant of COVID-19 patients with neurological manifestations where less cases of multisystem inflammatory syndrome in children (MIS-C) were reported. This article describes our experience of children with severe and rare neurological manifestations following COVID-19 infection.

COVID-19 and Crossed Eye: A Case Report and Literature Review.

This study aims to report a case of neuro-ophthalmic manifestation in a coronavirus disease 2019 (COVID-19) patient and a literature review of neuro-ophthalmological manifestation in COVID-19 patients. A 57-year-old male presented with headache, giddiness, and sudden onset of diplopia over two days after having a flu-like illness. Clinical examination revealed bilateral bizarre extraocular movement with right lower motor neuron facial nerve palsy.

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.

Radiologic-Pathologic Correlation of COVID-19-Associated Acute Disseminated Encephalomyelitis.

A 42-year-old woman presented with drooling, slurred speech, inability to walk and talk, and a recent positive COVID-19 test. She had two prior hospital admissions within the past week for similar symptoms with inconclusive evaluation. MRI of the brain demonstrated multifocal white matter hyperintense lesions on fluid-attenuated inversion recovery (FLAIR)/diffusion with variable enhancement.

Acute Disseminated Encephalomyelitis in a Six-Year-Old Child: A Case Report.

Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune demyelinating disorder that primarily affects the central nervous system (CNS). It is characterized by an acute inflammatory response targeting the myelin sheath surrounding nerve fibers in the brain and spinal cord. The exact mechanism of ADEM is not fully understood, but it is believed to involve an abnormal immune response that leads to the activation of immune cells and subsequent inflammation within the CNS.

Thiamine metabolism dysfunction syndrome 5 (THMD5) mimicking acute disseminated encephalomyelitis.

Thiamine pyrophosphate (TPP), the substrate of Thiamine pyrophosphate kinase (TPK), is an important cofactor in carbohydrate metabolism, specifically as a cofactor of the Pyruvate dehydrogenase complex (PDH) complex. The nervous system is particularly dependent on TPP due to its reliance on glucose metabolism. In this case, a four-year-old girl had a previously unreported pathogenic variant of the gene encoding TPK (TPK1) which presented as Thiamine metabolism dysfunction syndrome 5 (THMD5; OMIM 614458).

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.

Overlapping Epstein-Barr virus encephalitis and autoimmune glial fibrillary acidic protein astrocytopathy.

We describe three cases of overlapping Epstein-Barr virus (EBV) Encephalitis and Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy (GFAP-A). The three cases all presented with initial symptoms of fever, headache, coma, and posture tremor of the upper limbs, then followed by limb weakness and dysuria. All of the three cases were on ventilators.

Central nervous system complications in SARS-CoV-2-infected patients.

Objective: To investigate the clinical manifestations, treatment and prognosis of COVID-19-associated central nervous system (CNS) complications.

Methods: In this single-centre observation study, we recruited patients with COVID-19-associated CNS complications at the neurology inpatient department of the Eighth Affiliated Hospital, Sun Yat-Sen University (Futian, Shenzhen) from Dec 2022 to Feb 2023. Patients were analysed for demographics, clinical manifestations, cerebrospinal fluid properties, electroencephalographic features, neuroimaging characteristics, and treatment outcome.