Acute Demyelinating Encephalomyelitis Research Articles

MOG Antibody-associated Hemorrhagic Acute Demyelinating Encephalo-myelitis (ADEM) with Elevated CSF IL-6 After Tdap Vaccination (P8-6.007)

MOG Antibody-associated Hemorrhagic Acute Demyelinating Encephalo-myelitis (ADEM) with Elevated CSF IL-6 After Tdap Vaccination (P8-6.007)

178 Complement 3 mediated Glomerulonephritis (CM -GN) in a patient with background of COVID, Meningitis, Klebsiella UTI-Sepsis, and Acute Demyelinating Encephalomyelitis (ADEM)

178 Complement 3 mediated Glomerulonephritis (CM -GN) in a patient with background of COVID, Meningitis, Klebsiella UTI-Sepsis, and Acute Demyelinating Encephalomyelitis (ADEM)

Subacute Partially Reversible Leukoencephalopathy Expands the Aicardi-Goutières Syndrome Phenotype.

To report a series of atypical presentations of Aicardi-Goutières syndrome. Clinical, neuroimaging, and genetic data. We report a series of six unrelated patients (five males) with a subacute loss of developmental milestones, pyramidal signs, and regression of communication abilities, with onset at ages ranging from 7 to 20 months, reaching a nadir after 4 to 24 weeks. A remarkable improvement of lost abilities occurred in the follow-up, and they remained with residual spasticity and dysarthria but preserved cognitive function. Immunization or febrile illness occurred before disease onset in all patients. CSF was normal in two patients, and in four, borderline or mild lymphocytosis was present. A brain CT scan disclosed a subtle basal ganglia calcification in one of six patients. Brain MRI showed asymmetric signal abnormalities of white matter with centrum semi-ovale involvement in five patients and a diffuse white matter abnormality with contrast enhancement in one. Four patients were diagnosed and treated for acute demyelinating encephalomyelitis (ADEM). Brain imaging was markedly improved with one year or more of follow-up (average of 7 years), but patients remained with residual spasticity and dysarthria without cognitive impairment. Demyelination relapse occurred in a single patient four years after the first event. Whole-exome sequencing (WES) was performed in all patients: four of them disclosed biallelic pathogenic variants in RNASEH2B (three homozygous p.Ala177Thr and one compound heterozygous p.Ala177Thr/p.Gln58*) and in two of them the same homozygous deleterious variants in RNASEH2A (p.Ala249Val). This report expands the phenotype of AGS to include subacute developmental regression with partial clinical and neuroimaging improvement. Those clinical features might be misdiagnosed as ADEM.

Case Report Presentation: Measles as the Cause of Cerebral Venous Sinus Thrombosis in Childhood

The major cause of stroke in children is cerebral venous sinus thrombosis (CVST), which may cause neurological impairment and even death. The most frequent causes of CVST in kids are infections, followed by anemia, and then dehydration. Despite the existence of a safe and reliable vaccination, measles is an infectious viral infection that continues to be a major cause of death in young children worldwide. With a high morbidity and death rate, measles may result in neurologic consequences, such as acute demyelinating encephalomyelitis (ADEM), coma, seizures, or obtundation. In this case, a 4-year-old Syrian girl with status epilepticus and an altered sensorium was brought to the emergency room and was later identified as having CVST. There have never been any reports of CVST with measles.

Seroprevalence of MOG and AQP4 Antibodies and Outcomes in an Indian Cohort of Pediatric Acquired Demyelinating Syndromes.

This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADSs). Children (6 months-15 years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary care hospital in North India. Children with proven nonimmune-mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, acute demyelinating encephalomyelitis (ADEM) (25) was the most common first clinical event followed by optic neuritis (ON) (20) and transverse myelitis (TM) (19; one child had ON and TM simultaneously [neuromyelitis optica spectrum disorders [NMOSDs]]), while two children had clinically isolated syndrome (CIS) apart from ON and TM. Fourteen (21.2%, confidence interval [CI] 11.3-31.1) tested positive for one antibody (12 [18.1%; 95% CI 10.5-25.5%] for MOG-Ab and 2 [3%; 95% CI 0-7.2%] for AQP4-Ab). None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was multiphasic disseminated encephalomyelitis (MDEM) (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95% CI 0.5-15.2%) were MOG-Ab positive and 2 AQP4-Ab positive, whereas 8/15 (53.3% [95% CI 28.1-78.6%]) with recurrent events (MDEM [2], ADEM-ON [4], recurrent ON [1], and recurrent TM [1]) were MOG-Ab positive. Hence, MOG-Abs are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Abs are rare in children with ADS.

Child with cytomegalovirus associated acute demyelinating encephalomyelitis: a case report

Cytomegalovirus (CMV) is a common virus that causes mild, self-limiting illness in immunocompetent individuals. However, CMV is also frequently associated with acute demyelinating encephalomyelitis (ADEM), which is an uncommon monophasic idiopathic inflammatory demyelinating disease. It affects both children and adults. This is a case of unfortunate boy with CMV infection which was complicated with ADEM. Prompt diagnosis and aggressive treatment had provided a good outcome in this patient. Bangladesh Journal of Medical Science Vol. 21 No. 03 July’22 Page: 749-753

COVID-19 vaccine associated demyelination & its association with MOG antibody

BackgroundChAdOx1-S (Covishield™/Vaxzervria, AstraZeneca) and BBV152 (Covaxin) SARS-CoV-2 vaccines are proven to be safe and effective, but rare complications have been reported. ObjectiveTo describe reports of central nervous system (CNS) demyelination following ChAdOx1-S and BBV152 vaccinations. Methods & ResultsWe report 29 (17 female; mean 38 years) cases of CNS demyelination; twenty-seven occurred in temporal association with ChAdOx1-S vaccine; two in association with BBV152 vaccine. Eleven patients had presentation with myelitis, six patients developed optic neuritis, five had acute demyelinating encephalomyelitis, three presented with brainstem demyelination, and four had multiaxial involvement. Myelin oligodendrocyte glycoprotein (MOG) antibodies were positive in ten patients. One patient with ADEM and tumefactive demyelinating lesions died after a prolonged intensive care unit stay and superimposed infection. As compared to the control group (87); the postvaccinial cases were found to have a significantly higher mean age, presence of encephalopathy (p value:0.0007), CSF pleocytosis (p value: 0.0094) and raised CSF protein (p value: 0.0062). ConclusionsIt is difficult to establish a causal relationship between vaccination and neurological adverse events such as demyelination. The temporal association with the vaccination and the presence of MOG antibodies raises the possibility of an immunogenic process triggered by the vaccine in susceptible individuals.

Radiological Manifestation of Neurological Complications in the Course of SARS-CoV-2 Infection.

Many reports suggest the SARS-CoV-2 infection may result in neurological complications. A wide spectrum of clinical syndromes have been reported, including both central and peripheral nervous system. Such symptoms may be a consequence of a direct viral injury, secondary to systemic inflammatory response, autoimmune processes, ischemic lesions or combination of these. Anosmia and dysgeusia are highly prevalent in the early stage of infection. Cerebrovascular events in patients with COVID-19 have also been documented with increasing frequency. Some cases of parainfectious autoimmune neurologic manifestations concurrent with active SARS-CoV-2 infection have been described, including hemorrhagic necrotizing encephalopathy, Guillain-Barré and Miller-Fisher syndromes. There are also a few reports documenting encephalitis and acute demyelinating encephalomyelitis (ADEM) in the course of COVID-19. There is also a growing number of cases of patients after recovery from COVID-19 with psychosomatic disorders, manifesting with memory disfunction, cognitive functions disorders, depression or other affective disorders, which may lead to a decrease of intellectual functions. Many of these neurological manifestations of the infection are possible to distinguish using radiological imaging techniques. It plays a very important role in evaluating the course of COVID-19 as well as diagnosing respiratory complications and choosing a proper management of infected patients. Similarly, radiological techniques play crucial role in identifying the cause of neurological symptoms connected to SARS-CoV-2 infection, being one of the most important elements of diagnostics. Especially in case of the presence of nervous system implication, using radiological imaging techniques to monitor the emerging onset of various symptoms is crucial to assess the severity and scope of involvement. Quick diagnostic process and identifying complications as fast as possible in order to implement specific treatment can be crucial to avoid long-term secondary conditions and accelerate the recovery period. In this review, we present the most important neurological complications that may occur in the course of SARS-CoV-2 infection and summarize their radiological manifestations.

Fulminant acute demyelinating encephalomyelitis (ADEM) with coexisting cerebral venous sinus thrombosis (CVT) in pregnency – An unusual presentation

Acute demyelinating encephalomyelitis (ADEM) is commoner in children than in adults. Pregnancy, a pro-thrombotic state is known to cause cerebral venous sinus thrombosis (CVT). ADEM is rare in pregnancy and its diagnosis can be challenging. Both ADEM and CVT occurring simultaneously in pregnancy are rare. We report such an unusual case.

Management of Acute Demyelinating Attacks in the Pediatric Population: A Swiss Consensus Statement

Background and methods: Acquired demyelinating syndromes (ADS) encompass distinct entities and occur in approximately 1/100,000 children. While the use of high dose intravenous corticosteroids is well-established, agreement on steroid taper and type of second line therapy is lacking. A comprehensive, unified and standardized treatment approach is crucial in the management of patients with rare diseases. Therefore, this study performed from July 2018 to June 2020 aimed at developing a national consensus on the management of ADS in the pediatric population using the Delphi approach. Consensus was defined as agreement in >75%. Designated Neuropediatricians with an expertise in the management of pediatric neuroinflammatory diseases in all university and cantonal hospitals of Switzerland were included. The response rate was 100%. Results: High-dose i.v. methylprednisolone (20–30 mg/kg/die for 5 days) is the first line treatment irrespective of the distinct entity of the ADS. An oral steroid taper is recommended in acute demyelinating encephalomyelitis (ADEM) and in neuromyelitis optica spectrum disorder (NMO-SD). However, in the latter more in the sense of bridging. The choice of second line treatment depends on the entity of ADS: in optic neuritis (ON) and ADS due to relapsing remitting multiple sclerosis, first line treatment should be repeated, whereas plasma exchange is recommended in NMO-SD, ADEM and transverse myelitis. Conclusions: A national guideline allowing for a more unified approach in the management of pediatric ADS will enhance future research in this field, making data more comparable. The definition of inadequate treatment response to first line therapy remains a challenge and requires future research.

Acute Leukoencephalopathy with Restricted Diffusion in an Infant with Severe COVID-19 and Dengue Coinfection Progressing to West Syndrome.

COVID-19 pandemic is increasingly being recognized in infants and some develop cytokine storm mediated tissue damage. We report 5-month-old infant presenting with fever, refusal of feeds, developing altered sensorium and convulsions during the hospital course, tested positive for SARS-CoV2 RT-PCR in second week of illness. Her serology was also Dengue positive. She had features of cytokine storm and her MRI Brain suggested acute demyelinating encephalomyelitis (ADEM). She was treated with high-dose methylprednisolone followed oral prednisolone, under antibiotics cover. Infant improved gradually over 3 weeks duration following a stormy hospital course. On follow-up, infant showed delayed motor milestones with epileptic spasms and hysparrhthymia on EEG, progressing to develop secondary West syndrome. Features of acute encephalopathy, hypercytokinemia and restricted diffusion on DWI–MRI, with post-encephalopathic epilepsy, pointed to a differential of ADEM—acute leukoencephalopathy with restricted diffusion (ALERD) as the primary diagnosis; establishing ALERD as a possible neurological complication of COVID-19 infection in infants.Timeline of events. There is a demonstrable fall in the inflammatory markers with clinical improvement following the start of intravenous methylprednisolone. Epileptic spasms and developmental delay with hypsarrhthymia noted on follow-up, suggestive of secondary West syndrome.

Plasma Exchange in Refractory Acute Disseminated Encephalomyelitis Associated with COVID-19: Technical Challenges in a Developing Country

AbstractEvaluation of acquired demyelinating syndrome (ADS) without diagnostic biomarkers results in diagnostic and therapeutic challenges in pediatric population. Immune-mediated ADS of childhood responds well to steroid and intravenous immunoglobulin (IVIg) and in refractory cases with plasma exchange. Novel coronavirus disease 2019 (COVID-19) coinfection in such cases imposes technical challenges in management. An 11-year-old girl with quadriparesis and loss of vision and a magnetic resonance imaging (MRI) brain showing acute demyelinating encephalomyelitis (ADEM) and cerebrospinal fluid examination being noncontributory responded well with plasma exchange after failing steroid and IVIg is described. Coinfection with COVID-19 mandating personal protection in a temperate country imposed technical challenges in her management.

Acute Demyelinating Encephalomyelitis (ADEM) following rabies vaccination

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system (CNS). Its diagnosis can be challenging due to having varied clinical presentations, including a range of motor, speech, cognitive, and behavioral changes that can vary in frequency and intensity, and there being no strictly defined diagnostic criteria for it in adults. Here we present a 58-year-old White male who developed ADEM following an uncommon cause (i.e., rabies vaccination), which was difficult to diagnose due to atypical manifestations.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Management.

Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis (ADEM) and cortical encephalitis. Even though sometimes the clinical picture of this condition is similar to the presentation of neuromyelitis optica spectrum disorder (NMOSD), most experts consider MOGAD as a distinct entity with different immune system pathology. MOG is a molecule detected on the outer membrane of myelin sheaths and expressed primarily within the brain, spinal cord and also the optic nerves. Its function is not fully understood but this glycoprotein may act as a cell surface receptor or cell adhesion molecule. The specific outmost location of myelin makes it a potential target for autoimmune antibodies and cell-mediated responses in demyelinating processes. Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. In adults, the disease course is multiphasic and subsequent relapses increase disability. In children ADEM usually presents as a one-time incident. Luckily, acute immunotherapy is very effective and severe disability (ambulatory and visual) is less frequent than in NMOSD. A critical element of reliable diagnosis is detection of pathogenic serum antibodies MOG with accurate, specific and sensitive methods, preferably with optimized cell-based assay (CBA). MRI imaging can also help in differentiating MOGAD from other neuro-inflammatory disorders. Reports on randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment. In this review, we present up-to-date clinical, immunological, radiographic, histopathological data concerning MOGAD and summarize the practical aspects of diagnosing and managing patients with this disease.

Etiological spectrum, clinico-radiological profile and treatment outcomes of longitudinally extensive transverse myelitis – A prospective study from Northwest India

ObjectivesTo study etiological, clinical and radiological profile and treatment outcomes in patients of longitudinally extensive transverse myelitis (LETM). MethodsThis was a prospective study conducted at Dr. S.N. medical college, Jodhpur, India, of patients presenting with LETM between March 2018 to March 2020. ResultsOur study included 32 patients (median age 32 years, female to male ratio of 2.5: 1). Neuromyelitis optica spectrum disorders (NMOsd) was the most common diagnosis affecting 14 patients followed by 5 patients of idiopathic myelitis, 4 patients of para-infectious myelitis, 3 patients of multiple sclerosis (MS), 3 patients of acute demyelinating encephalomyelitis (ADEM) and 1 patient each of myelin oligodendrocyte glycoprotein (MOG) antibody disease, sarcoidosis and mixed connective tissue disorder (MCTD). All the patients of NMOsd were positive for aquaporin 4 (AQP4) antibody. Spinal MRI showed central pattern of involvement in NMOsd and eccentric pattern of involvement in MS. All the patients of para-infectious myelitis were varicella myelitis. Twenty-eight (87.5%) patients had good clinical recovery at 6 months. ConclusionAlthough, LETM is classically associated with NMOsd, it can have multiple etiologies. Identifying the etiology is important for long term treatment and prognosis which varies according to the disease. Patients in our study presented with relapses and severe disease but had good clinical recovery with treatment.

Myelin oligodendrocyte glycoprotein-antibody-associated disorder: a new inflammatory CNS demyelinating disorder.

Myelin oligodendrocyte glycoprotein (MOG) is an oligodendrocytopathy resulting in demyelination. We aimed to determine the frequency of MOG-associated disorders (MOGAD), its various clinical phenotypes, and imaging characteristics. All patients with MOGAD were included. Description of the various clinical phenotypes, investigation profile, therapeutic response, differences between pediatric and adult-onset neurological disorders, determination of poor prognostic factors was done. The study population consisted of 93 (M:F = 45:48) (Pediatric:40, Adult-onset:47, Late-onset:7) patients with a median age of 21years. Among the 263 demyelinating episodes; 45.8% were optic neuritis (ON), 22.8% were myelopathy, 17.1% were brainstem, 7.6% were acute demyelinating encephalomyelitis(ADEM), 4.2% were opticomyelopathy and 2.3% with cerebral manifestations. There was exclusive vomiting in 24.7% prior to onset of clinical syndrome, none of them had area postrema involvement. ADEM was exclusively seen in pediatric patients. Poor prognostic indicators included: (i) incomplete recovery from an acute attack, (b) brainstem syndrome, (c) ADEM with incomplete recovery, (d) MRI suggestive of leukodystrophy pattern, (e) severe ON, (f) ADEMON. The Spectrum of MOG-associated disorders is wider affecting the brain (grey and white matter) and the meninges. There are various clinical phenotypes and MRI patterns, recognition of which may help in the determination of therapeutic strategies, and long-term prognosis.

Clinical phenotype, radiological features, and treatment of myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) optic neuritis.

To review the clinical characteristics, radiological manifestations and treatment of myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) optic neuritis. Serum antibodies to MOG have recently been found to be a biomarker of MOG-IgG-associated disorder (MOGAD), a demyelinating disease distinct from both multiple sclerosis (MS) and aquaporin-4-IgG neuromyelitis optica spectrum disorder (AQP4-IgG-positive NMOSD). The phenotype of MOGAD is broad and includes optic neuritis, transverse myelitis, and acute demyelinating encephalomyelitis (ADEM). Optic neuritis is the most common presentation in adults, whereas ADEM is the most common presentation in children. Clinical characteristics suggestive of MOG-IgG optic neuritis include recurrent optic neuritis, prominent disc edema, and perineural enhancement of the optic nerve on magnetic resonance imaging. Although the nadir of vision loss is severe with MOG-IgG optic neuritis, the recovery is typically better than AQP4-IgG optic neuritis and therefore has a favorable overall prognosis. Patients with relapsing disease will often need chronic immunotherapy. Rituximab, azathioprine, mycophenolate mofetil, and monthly intravenous immune globulin are the most commonly utilized treatments. MOGAD is a unique entity that is separate from both MS and AQP4-IgG-positive NMOSD. Recognition of the clinical and radiologic features allow for the correct diagnosis. Future randomized trials will determine the optimal treatment for MOGAD.

Acute Demyelinating Encephalomyelitis(ADEM) Presenting With Dystonia

Introduction: Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune inflammatory demyelinating disease of the central nervous system that predominantly involves the white matter of the brain and spinal cord(1). It is a rare disease affecting both children and adults (2). In the majority of cases ADEM usually appears after vaccinations or viral/bacterial infections Methods: This was a case study of two patients with ADEM presenting with dystonia. The study was conducted at King Khalid Hospital in Hail, Saudi Arabia. Results: MRI studies showed subcortical white matter lesion in the right frontal region in patient 1, and a right thalamic lesion in patient 2. Conclusion: We conclude that the lesions detected by the MRI were responsible for the limb dystonia. We believe that the lesions seriously disrupted the cortical-striatal pathways in the right cerebral hemisphere, leading to hemi-dystonia.

Neuropathie optique dans le syndrome des anticorps anti MOG positif

The diagnosis of optic neuritis (ON), or inflammation of the optic nerve, is based on clinical findings: first marked by rapidly progressive visual decline associated with eye pain accentuated by eye movements; abnormalities of color perception and/or contrast sensitivity may also be reported. In this case, inflammatory neuropathies are associated with anti-MOG antibodies. MOGs, oligodendrocytic glycoproteins involved in the production of myelin, were identified nearly three decades ago in association with demyelinating ON. The first series were reported in children following demyelinating neurological manifestations, particularly in ADEM (acute demyelinating encephalomyelitis) or multiple sclerosis (MS) [1]. Anti-MOGs are associated with neuropathies in the phenotypic setting of the neuromyelitis optica (NO) spectrum, and anti-Aquaporin 4antibodies (AQP4) are negative by definition. Thus, anti-MOG could explain up to 30% of cases of seronegative optic neuritis; their presence thus represents a significant diagnostic aid for the clinician, especially during a first neurological episode [1]. The first short published series in AQP4-/MOG+populations revealed primarily ophthalmological involvement with a good prognosis for recovery [1]. Knowledge of these antigens is important; it may permit not only an understanding of the physiopathology but also the stratification of patients in terms of prognosis and response to treatment [2]. Thus, the early diagnosis of anti-MOG positive ON must prompt aggressive initial treatment and a more or less maintenance therapy to prevent recurrence. The role of the ophthalmologist remains paramount, since most cases present with purely ocular involvement. We report herein the clinical, ophthalmological, laboratory and radiological data for 25patients (45eyes) managed between February 2011and January 2017. All of our patients had optic neuritis associated with anti-MOG antibodies. All patients underwent the following testing: -Visual acuity; -Humphrey and/or Goldmann visual field; -Non-mydriatic fundus photography; -Optic disc OCT; -3Tesla orbital-cerebral MRI with and without contrast; -Standard and immunological laboratory testing for anti-MOG and anti AQP4antibodies by Western Blot and ELISA. The male: female ratio of the population was 0.92 (13women and 12men). The average age at onset was 35.68years (15to 60years); 40% of the subjects were between 31and 40years old. The initial symptoms leading to consultation were mostly visual acuity (80%) and pain (88%). Involvement was bilateral in 80% of cases (5unilateral). Initial visual acuity was poor; 52% of eyes were less than or equal to count fingers. The course was favorable however, with visual acuity returning to 10-12/10after 6months of follow-up (84% of eyes). Orbital/cerebral MRI with attention to the visual pathways revealed involvement of the anterior visual pathways with gadolinium uptake in 92% of cases. Of the 35eyes initially considered affected, the main initial diagnoses were: -36% retro-bulbar optic neuritis (RBON); -40% anterior optic neuritis (AON); -24% other; of which 16% were initially diagnosed as acute anterior ischemic optic neuropathy (AAION). 96% of patients received corticosteroid treatment in the acute phase. 16% required plasma exchange sessions. Maintenance therapy was proposed for only 36% of the population. Optic neuritis is a pathology frequently encountered in ophthalmology; a good knowledge of symptoms and clinical signs is essential for early diagnosis and optimal management. The identification of autoantibodies, including anti-MOG antibodies, is important for patient management and is part of the required testing for all cases of optic neuritis, in order to adapt the treatment of the acute episode and to provide maintenance therapy to avoid recurrence.

Central Nervous System Disease and Adenoviruses

Research Article| March 01 2019 Central Nervous System Disease and Adenoviruses AAP Grand Rounds (2019) 41 (3): 35. https://doi.org/10.1542/gr.41-3-35 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Central Nervous System Disease and Adenoviruses. AAP Grand Rounds March 2019; 41 (3): 35. https://doi.org/10.1542/gr.41-3-35 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: adenoviruses, central nervous system dysfunction, adenovirus infections Source: Schwartz KL, Richardson SE, MacGregor D, et al. Adenovirus-associated central nervous system disease in children. J Pediatr. 2018 6 November [published online ahead of print]; doi: https://doi.org/10.1016/j.jpeds.2018.09.036Google Scholar Investigators from The Hospital for Sick Children (SickKids) in Toronto conducted a retrospective observational study to characterize adenovirus-associated central nervous system (CNS) disease in children. Three data sources were used: the SickKids microbiology and encephalitis databases (which include children who had been admitted to SickKids during 2000–2016 and 1996–2016, respectively) and a literature search. Children in the SickKids databases were eligible for the study if they were previously healthy; were 1 month to 18 years old; had neurologic symptoms; did not have a preexisting neurologic disorder; and had adenovirus detected by direct fluorescent antibody staining, culture, or polymerase chain reaction (PCR). Child cases identified from the literature search were eligible if they had a neurologic diagnosis other than febrile seizures and had microbiologic or serologic evidence of acute adenovirus infection. Clinical and demographic characteristics of identified children were abstracted from each data source. Using SickKids microbiology data, investigators determined the frequency and spectrum of neurologic complications of adenovirus disease. Using SickKids encephalitis data, investigators determined the frequency of adenovirus among children with encephalitis. Cases identified by literature search were then pooled with children identified in the SickKids databases (excluding those with febrile seizures) to characterize the adenovirus-associated CNS disease spectrum further. Multivariable logistic regression was used to identify risk factors associated with death or neurologic impairment. Of the 977 cases of adenovirus infection in the SickKids microbiology database, 32 (3.3%) had neurologic symptoms. Of these, 26 had febrile seizures, 4 had encephalitis, 1 had acute demyelinating encephalomyelitis, and 1 had aseptic meningitis. Of the 431 children in the SickKids encephalitis database, 8 (1.9%) had adenovirus (including the 4 with encephalitis identified in the SickKids microbiology database). There were 16 studies identified by the literature search involving 38 children, resulting in a pooled total of 48 cases. Of these, the median age was 2 years, and 40% were female. The most common prodromal symptoms were upper respiratory illness (48%), vomiting (22%), and diarrhea (19%). Hepatitis developed during hospitalization in 40%. There were 10 (21%) deaths, and 8 (17%) had permanent neurologic impairment. Children who developed seizures during their illness course had greater odds of death or permanent neurologic impairment (adjusted OR, 7.3; 95% CI, 1.69–31.45). The investigators conclude that adenovirus is a rare cause of CNS disease in children but can be fatal. Dr Brady has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device. Adenoviruses are common, accounting for at least 5%–10% of respiratory infections in children.1 Vomiting and diarrhea are often present concurrently.2 A specific viral etiology is usually not sought in children with these self-limited symptoms, so the incidence of adenovirus infections is difficult to ascertain. In the current study, the authors reviewed rare CNS manifestations of... You do not currently have access to this content.