Bilateral thalamic encephalitis due to Epstein-Barr virus (EBV) is a rare and severe manifestation of EBV infection, often leading to rapid neurological deterioration and poor outcomes. We report the case of an 82-year-old man with a history of arterial hypertension and a previous herpes zoster infection who presented to the emergency department with high fever and acute neurological impairment evolving into coma (Glasgow Coma Scale, GCS 3). Cerebrospinal fluid (CSF) analysis revealed mild lymphocytic pleocytosis, elevated protein and normal glucose. EBV-DNA was detected by polymerase chain reaction (PCR) and serology confirmed positive anti-EBV IgM antibodies. Autoimmune and paraneoplastic panels, including anti-NMDA receptor, anti-GQ1b, anti-MOG, anti-AQP4 and anti-LGI1 antibodies, were negative. Magnetic resonance imaging (MRI) demonstrated bilateral thalamic hyperintensities on T2-weighted and FLAIR sequences, with restricted diffusion on DWI and corresponding hypointensity on ADC maps, without contrast enhancement. Angiography showed normal patency of the main intracranial vessels. Differential diagnoses such as autoimmune encephalitis, acute disseminated encephalomyelitis, Bickerstaff's brainstem encephalitis, vascular, metabolic and other viral causes were excluded. Despite prompt antiviral and corticosteroid therapy and intensive supportive management, the clinical outcome was poor. A comprehensive literature review was performed, highlighting key clinical and radiological findings from previously published cases of EBV-related bilateral thalamic encephalitis, to improve recognition and understanding of this rare and devastating condition.

Autoimmune diseases of the central nervous system in children present a significant diagnostic challenge due to their heterogeneous nature. The identification of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies has contributed to our understanding and classification of these disorders.This study aims to characterize the clinical, paraclinical, and neuroimaging findings in pediatric patients diagnosed with acquired demyelinating syndromes (ADS) and autoimmune encephalitis (AE).Conducted as a descriptive case series, this study retrospectively analyzed medical records of children under 18 years diagnosed with ADS or AE from January 2017 to February 2022. We employed basic descriptive statistics to analyze patient demographics, clinical presentations, laboratory findings, and neuroimaging results.A total of 71 cases were evaluated, with 80% classified as ADS and 20% as AE. Within ADS, acute disseminated encephalomyelitis and optic neuritis were the most prevalent at 19%, followed by pediatric-onset multiple sclerosis at 17%, and transverse myelitis at 16%. AE cases were predominantly seropositive (93%), with a vast majority (92%) showing anti-NMDAR antibodies compared with 8% with anti-MOG antibodies. Since MOG antibody measurement became available at our institution in 2020, many new cases have been diagnosed as MOGAD (MOG antibody-associated disease), a new entity with different clinical manifestations.In children demyelinating disorders are more frequent than autoimmune encephalitis. New biomarkers, such as anti-MOG antibodies, have led to a better understanding of pediatric autoimmune CNS diseases. The study underscores the necessity of continuous reevaluation of diagnostic criteria and treatment protocols considering emerging biomarkers in neuroimmunology.

As compared to adults, less is known about the effects of SARS-CoV-2 infection in children with neuroinflammatory diseases, such as multiple sclerosis (MS), anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD), and neuromyelitis optica spectrum disorder (NMOSD). Moreover, the effects of SARS-CoV-2 vaccination on pediatric demyelinating diseases is also lesser known. Here, we examine the effects of SARS-CoV-2 infection and vaccination on children with demyelinating diseases. Retrospective chart review was performed at a single tertiary pediatric center, including 116 children with demyelinating diagnoses (MOGAD, MS, NMOSD, transverse myelitis-TM, and acute disseminated encephalomyelitis). Descriptive statistics and logistic regression were used. The diagnoses included: MS: 56/116 = 48%, MOGAD: 32/116 = 28%, NMOSD: 15/116 = 13%, acute disseminated encephalomyelitis: 12/116 = 10%, and TM: 1/116 = 1%. The known COVID infection rate was 26% = 30/116. Three of 30 (10%) of those with known infection were hospitalized. None of those hospitalized required intubation or ICU admission. Of the 30 with known infection, two (7%) had neuroinflammatory events that occurred within four weeks of infection, both of which were their first clinical events for MS. SARS-CoV-2 vaccination was not associated with any new onset or relapse neuroinflammatory events in our cohort. Demyelinating diagnosis and anti-CD20 mAbs were associated with known SARS-CoV-2 infection. Two participants had an initial clinical MS event associated with infection. However, we found no association with SARS-COV-2 vaccination or infection with relapses and no long-term adverse outcomes with SARS-CoV-2 vaccination or infection. Future larger multicenter studies should prospectively compare how infection and vaccination affects children and adults with demyelinating diseases.

ObjectivesTo evaluate the demographic, clinical, laboratory, and prognostic data of children with acute disseminated encephalomyelitis with respect to anti-myelin oligodendrocyte glycoprotein (MOG) antibody status.MethodsAcute disseminated encephalomyelitis patients (n = 245) from 24 centers followed up between 2010 and 2022 were evaluated retrospectively. The short- and long-term outcome characteristics (disease severity and course, clinical relapse, and recovery rates) were assessed. Incomplete clinical recovery was defined as modified Rankin Score ≥1 or the presence of epilepsy. Univariant and multivariant analysis were performed for outcome characteristics.ResultsThe mean age at diagnosis was 6.3 ± 3.8 (0.5-17.7) years and the median follow-up was 22 (3-132) months. The outcome characteristics were evaluated in 180 of 245 patients (73.4%) with at least 12 months' follow-up. Twenty-three patients (12.6%) relapsed. The multivariable logistic regression analysis revealed the following clinical parameters as predictors of relapse: sex, visual impairment, and ataxia at initial presentation. Incomplete clinical recovery (n = 42/180, 23.3%) was associated with the presence of seizures on admission and the need for an intensive care unit. Anti-MOG antibody positivity was not associated with an increased risk of relapse (25% vs 13.1%, P = .164) or incomplete clinical recovery (P = .511).ConclusionThe nationwide cohort presented further supports the typically monophasic nature of acute disseminated encephalomyelitis, and a high rate of complete recovery. The presence of certain symptoms in the acute period may assist the clinician in estimating the outcome.

MOGAD is a demyelinating syndrome with the presence of antibodies against myelin oligodendrocyte glycoprotein, which is, next to multiple sclerosis and the neuromyelitis optica spectrum, one of the manifestations of the demyelinating process, more common in the pediatric population. MOGAD can take a variety of clinical forms: acute disseminated encephalomyelitis (ADEM), retrobulbar optic neuritis, often binocular (ON), transverse myelitis (TM), or NMOSD-like course (neuromyelitis optica spectrum disorders), less often encephalopathy. The course may be monophasic (40-50%) or polyphasic (50-60%), especially with persistently positive anti-MOG antibodies. Very rarely, the first manifestation of the disease, preceding the typical symptoms of MOGAD by 8 to 48 months, is focal seizures with secondary generalization, without typical demyelinating changes on MRI of the head. The paper presents a case of a 17-year-old patient whose first symptoms of MOGAD were focal epileptic seizures in the form of turning the head to the right with the elevation of the left upper limb and salivation. Seizures occurred after surgical excision of a tumor of the right adrenal gland (ganglioneuroblastoma). Then, despite a normal MRI of the head and the exclusion of onconeural antibodies in the serum and cerebrospinal fluid after intravenous treatment, a paraneoplastic syndrome was suspected. After intravenous steroid treatment and immunoglobulins, eight plasmapheresis treatments, and the initiation of antiepileptic treatment, the seizures disappeared, and no other neurological symptoms occurred for nine months. Only subsequent relapses of the disease with typical radiological and clinical picture (ADEM, MDEM, recurrent ON) allowed for proper diagnosis and treatment of the patient both during relapses and by initiating supportive treatment. The patient's case allows us to analyze the multi-phase, clinically diverse course of MOGAD and, above all, indicates the need to expand the diagnosis of epilepsy towards demyelinating diseases: determination of anti-MOG and anti-AQP4 antibodies.