scholarly journals Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 280
Author(s):  
Serena Massa ◽  
Adriana Fracchiolla ◽  
Cosimo Neglia ◽  
Alberto Argentiero ◽  
Susanna Esposito
Keyword(s):  
Demyelinating Disease ◽  
Clinical Laboratory ◽  
Acute Disseminated Encephalomyelitis ◽  
Future Research ◽  
Term Outcome ◽  
Adequate Treatment ◽  
Long Term Outcome ◽  
Immune Mediated ◽  
Therapeutic Decisions ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory demyelinating disease of the central nervous system (CNS) that usually affects children and young adults after an infection or vaccination. The presence of several conditions mimicking ADEM, added to the lack of specific biomarkers, makes diagnosis potentially hard. Prompt diagnosis is necessary to start adequate treatment to improve the clinical course and long-term outcome. Because of its heterogeneity in both clinical presentation and course, challenges remain in establishing the most appropriate therapeutic approach in each patient. The aim of this review is to provide an update on management of this disease with a focus on acute treatment and to give suggestions for future research. We showed that there are currently no guidelines that help clinicians manage ADEM and therapeutic decisions are often made on a case-by-case basis. Further studies are necessary to identify clinical, laboratory, and instrumental criteria that could be correlated with outcomes and guide clinicians in choosing when and what treatment should be given in each case.

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

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

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.

scholarly journals Acute Disseminated Encephalomyelitis Following Typhoid Fever: A Case Report

2014 ◽  
Vol 9 (4) ◽  
pp. 55-58
Author(s):  
R Adhikari ◽  
A Tayal ◽  
PK Chhetri ◽  
B Pokhrel
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Typhoid Fever ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Medical Sciences ◽  
Cerebellar Syndrome ◽  
Immune Mediated ◽  
The Central Nervous System

The involvement of central nervous system in children with typhoid fever is common. Acute disseminated encephalomyelitis is a rare immune mediated and demyelinating disease of the central nervous system that usually affects children. We report a 7-year-old child with typhoid fever who developed acute cerebellar syndrome due to acute disseminated encephalomyelitis.Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 55-58 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10237

Long-Term Outcomes of Patients with Hydrocephalus Secondary to Tectal Plate Glioma versus Idiopathic Aqueductal Stenosis: Results from a Single Center

2021 ◽  
pp. 1-11
Author(s):  
Hamid Reza Niknejad ◽  
Melissa Frederickx ◽  
Emiel Salaets ◽  
Jurgen Lemiere ◽  
Lieven Lagae ◽  
...  
Keyword(s):  
Aqueductal Stenosis ◽  
Maximum Diameter ◽  
Benign Tumors ◽  
Term Outcome ◽  
Adequate Treatment ◽  
Long Term Outcome ◽  
Tectal Plate ◽  
The Mean

<b><i>Introduction:</i></b> Tectal plate gliomas (TPG) constitute a distinct entity of benign tumors of the brain stem which show an indolent clinical course. Adequate treatment of secondary hydrocephalus is undoubtedly a major factor in the outcome. However, little is known about to what degree the tumor itself determines the long-term outcome of these patients. <b><i>Methods:</i></b> We retrospectively analyzed and compared the clinical and radiological data of 16 pediatric TPG patients with data of 12 pediatric idiopathic aqueductal stenosis (IAS) patients treated in our center from 1988 to 2018. For both groups, we assessed the long-term outcome in terms of hydrocephalus management, and for the TPG group, we assessed tumor growth during follow-up. In a separate prospective part of the study, we performed a neuropsychological evaluation in a subgroup of patients using a standardized testing battery, covering intelligence, learning, memory, executive functions, and an inventory on depression. <b><i>Results:</i></b> In the TPG group, the mean clinical and radiological follow-up was 84 and 70 months, respectively. On average, the maximum diameter of the tumor increased by 11% (<i>p</i> = 0.031) and the estimated tumor volume with 35% (<i>p</i> = 0.026) on radiological follow-up. The fronto-occipital horn ratio (FOHR) decreased by 23% on average after treatment. In the IAS group, the mean clinical and radiological follow-up was 117 and 85 months, respectively. In this group, the FOHR decreased by 21% on average. Neurocognitive testing revealed significant higher scores in the TPG group on global intelligence (TPG = 109, IAS = 85.5, <i>U</i> = 3, <i>p</i> &#x3c; 0.01, <i>z</i> = −2.71), performance (TPG= 100, IAS = 85, <i>U</i> = 7, <i>p</i> = 0.03, <i>z</i> = −2.2), and verbal intelligence (TPG = 122, IAS = 91.5, <i>U</i> = 2, <i>p</i> &#x3c; 0.00, <i>z</i> = −2.87) as well as working memory (TPG = 109.5, IAS = 77, <i>U</i> = 0.5, <i>p</i> = 0.01, <i>z</i> = −2.46). <b><i>Conclusion:</i></b> Our results suggest that the long-term outcome in TPG patients is acceptable and that cognition is substantially better preserved than in patients with IAS. This puts the idea of a significant contribution of the tumoral mass to disease outcome on the long term in question. Adequate and prompt management of hydrocephalus is the most important factor in long-term cognitive outcome.

scholarly journals Measuring attachment and parental bonding in psychosis and its clinical implications

2014 ◽  
Vol 25 (2) ◽  
pp. 142-149 ◽  
Author(s):  
S. Mathews ◽  
J. Onwumere ◽  
S. Bissoli ◽  
M. Ruggeri ◽  
E. Kuipers ◽  
...  
Keyword(s):  
Attachment Theory ◽  
Attachment Style ◽  
Attachment Styles ◽  
Parental Bonding ◽  
Insecure Attachment ◽  
Self Report ◽  
Future Research ◽  
Clinical Implications ◽  
Term Outcome ◽  
Long Term Outcome

Background.Attachment theory proposes that psychological functioning and affect regulations are influenced by the attachment we form with others. Early relationships with parents or caregivers lay the foundations for attachment styles. These styles are proposed to influence how we relate to others during our life can be modified by the relationships and events we experience in our lifespan. A secure attachment style is associated with a capacity to manage distress, comfort with autonomy and the ability to form relationships with others, whereas insecure attachment can lead to dysfunctional relationships, emotional and behaviour avoidance. Attachment theory provides a useful framework to inform our understanding of relationship difficulties in people with psychosis. This paper aims to complement recent systematic reviews by providing an overview of attachment theory, its application to psychosis, including an understanding of measurement issues and the clinical implications offered.Method.A narrative review was completed of the measures of attachment and parental bonding in psychosis. Its clinical implications are also discussed. The paper also explores the link between insecure attachment styles and illness course, social functioning and symptomatology. The following questions are addressed: What are the key attachment measures that have been used within the attachment and psychosis literature? What are the results of studies that have measured attachment or parental bonding in psychosis and what clinical implications can we derive from it? What are some of the key questions for future research from these findings in relation to the onset of psychosis research field?Results.The most commonly used measures of attachment in psychosis research are reviewed. Self-report questionnaires and semi-structured interviews have mainly been used to examine attachment styles in adult samples and in recent years comprise a measure specifically developed for a psychosis group. The review suggests that insecure attachment styles are common in psychosis samples. Key relationships were observed between insecure, avoidant and anxious attachment styles and psychosis development, expression and long-term outcome.Conclusions.Attachment theory can provide a useful framework to facilitate our understanding of interpersonal difficulties in psychosis that may predate its onset and impact on observed variability in outcomes, including treatment engagement. Greater attention should be given to the assessment of attachment needs and to the development of interventions that seek to compensate for these difficulties. However, further investigations are required on specifying the exact mechanisms by which specific attachment styles impact on the development of psychosis and its course.

Acute Disseminated Encephalomyelitis

2012 ◽  
Vol 27 (11) ◽  
pp. 1408-1425 ◽  
Author(s):  
Gulay Alper
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Protein Levels ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Elevated Protein ◽  
Demyelinating Lesions ◽  
Diagnostic Difficulties ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory and demyelinating disorder of the central nervous system, commonly preceded by an infection. It principally involves the white matter tracts of the cerebral hemispheres, brainstem, optic nerves, and spinal cord. Acute disseminated encephalomyelitis mainly affects children. Clinically, patients present with multifocal neurologic abnormalities reflecting the widespread involvement in central nervous system. Cerebrospinal fluid may be normal or may show a mild pleocytosis with or without elevated protein levels. Magnetic resonance image (MRI) shows multiple demyelinating lesions. The diagnosis of acute disseminated encephalomyelitis requires both multifocal involvement and encephalopathy by consensus criteria. Acute disseminated encephalomyelitis typically has a monophasic course with a favorable prognosis. Multiphasic forms have been reported, resulting in diagnostic difficulties in distinguishing these cases from multiple sclerosis. In addition, many inflammatory disorders may have a similar presentation with frequent occurrence of encephalopathy and should be considered in the differential diagnosis of acute disseminated encephalomyelitis.

scholarly journals Acute disseminated encephalomyelitis following spider bite

2020 ◽  
Vol 40 (1) ◽  
pp. 38-40
Author(s):  
Petra Bago Rožanković ◽  
Maristela Stojić ◽  
Jasna Badžak
Keyword(s):  
Clinical Presentation ◽  
Demyelinating Disease ◽  
Immunological Response ◽  
Immunoglobulin Therapy ◽  
Acute Disseminated Encephalomyelitis ◽  
Cross Reactivity ◽  
Pathophysiological Mechanism ◽  
Radiological Features ◽  
The Central Nervous System ◽  
Spider Bite

Acute disseminated encephalomyelitis (ADEM) is an inflammatory, demyelinating disease of the central nervous system that occurs as an immunological response to a viral or bacterial infection or an immunization. We describe a patient with clinical presentation and radiological features of ADEM that appeared after a spider bite. Corticosteroid therapy did not produce satisfied treatment response. The patient recovered after five days of immunoglobulin therapy. The cross-reactivity between spider toxin and myelin could explain pathophysiological mechanism of demyelination. ADEM should be considered as a possible complication of a spider bite.

P1813CLINICAL CHARACTERIZATION OF CONGENITAL SOLITARY FUNCTIONING KIDNEY IN CHILDREN

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Francesca Becherucci ◽  
Francesca Guzzi ◽  
Luigi Cirillo ◽  
Alessandra Bettiol ◽  
Arianna Zuccato ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Compensatory Hypertrophy ◽  
Functional Adaptation ◽  
Renal Scintigraphy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Functioning Kidney

Abstract Background and Aims Congenital anomalies of the kidney and urinary tract (CAKUT) are the major cause of chronic kidney disease and end-stage kidney disease in childhood. Solitary functioning kidney (SFK) is part of the spectrum of CAKUT. Congenital SFK is mainly due to unilateral renal agenesis (URA) and multi-cystic dysplastic kidney (MCDK). With the implementation of routine fetal ultrasound screening, SFK is increasingly recognized before birth, significantly raising the number of patients referred to paediatric nephrology units for clinical monitoring during childhood. Understanding the pathophysiology of SFK is pivotal for guiding the clinical management and informing long-term outcome. However, observational studies performed in children with SFK are controversial, especially about the need, methodology and timing of functional assessment. This may be at least in part due to the fact that different models of SFK, including congenital and acquired after unilateral nephrectomy, are often grouped together. The aim of this study was to assess the clinical, laboratory and functional features of congenital SFK caused by URA and MCDK in children, with a particular focus on the role of renal scintigraphy in estimating kidney function during childhood and adolescence. Method We retrospectively collected clinical, laboratory and instrumental records of all consecutive pediatric patients (aged 0-18 years) affected by congenital SFK caused by URA or MCDK referred to the Nephrology and Dialysis Unit of Meyer Children’s Hospital of Florence (Italy) from 1992 to 2019. Patients with unilateral kidney hypodysplasia were excluded. In particular, we reviewed data from ultrasound scanning and sequential renal scintigraphy over time. URA and MCDK were compared for clinical features, long-term course and outcome. Results A total of 155 patients with congenital SFK were included in the study and divided in two groups according to the cause of SFK (URA, n=100; MCDK, n=55). The median length of follow-up was 47 and 45 months, respectively. Male sex and ethnicity were equally distributed in the two groups. Prenatal diagnosis was more frequent in MCDK group. We did not observe either preterm birth or low birth weight in patients enrolled. Overall, the clinical features were not statistically different between the two groups. In particular, SFK associated CAKUT, including vesicoureteral reflux, occurred at a comparable frequency. Also, measurement of kidney length by ultrasound scanning, which is often considered suggestive of compensatory hypertrophy, did not differ between groups. Although renal clearance from sequential renal scintigraphy appeared not statistically different between URA and MCDK, the latter seems to reach complete functional adaptation more rapidly and earlier in the first two years of life. Conclusion The clinical course and long-term outcome of SFK has been a topic of extensive debate. Due to poor-quality of data (unclear inclusion/exclusion criteria, lack of uniformity in data collection and outcome definition), generalization of findings from observational studies to all patients with SFK could be inappropriate. Congenital SFK could represent the most unbiased group to analyze and this study provides a thorough clinical characterization of a large and strictly selected cohort. Insights from sequential renal scintigraphy suggest a different trend in reaching single kidney complete functional adaptation in URA and MCDK. These results could potentially reveal significant differences in the pathophysiologic mechanisms of reaching compensatory hypertrophy and functional adaptation by the solitary kidney in the two models. Whether confirmed in larger cohorts, these findings could provide important implications for follow-up planning, informing the need, methodology and timing for function assessment, tailoring the clinical management and understanding long-term prognosis.

Relapse rates and long-term outcome in primary angiitis of the central nervous system

2019 ◽  
Vol 266 (6) ◽  
pp. 1481-1489 ◽  
Author(s):  
Simon Schuster ◽  
Ann-Kathrin Ozga ◽  
Jan-Patrick Stellmann ◽  
Milani Deb-Chatterji ◽  
Vivien Häußler ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
Relapse Rates

Participation of Ethnically Diverse Personnel in State-Federal Vocational Rehabilitation Agencies

2006 ◽  
Vol 37 (1) ◽  
pp. 30-37 ◽  
Author(s):  
Madan M. Kundu ◽  
Alo Dutta ◽  
Sylvia Walker
Keyword(s):  
Vocational Rehabilitation ◽  
Culturally Diverse ◽  
Ethnically Diverse ◽  
Substantial Improvement ◽  
Future Research ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Diverse Backgrounds ◽  
Rehabilitation Personnel

The rate of employment among those with disabilities, especially from culturally diverse backgrounds, is extremely low and the prevalence of poverty rather high compared to the general population. However, the long-term outcome of consumers of the state-federal vocational rehabilitation (VR) system continues to be in need of substantial improvement. The fact that the ethnographic composition of the direct service professionals and administrators employed by the system does not represent that of the country complicates the matter even further. The current study, involving 7,862 professionals from 38 VR agencies, was designed to ascertain the demographic composition of rehabilitation personnel in: (1) State Offices, (2) District/Regional Offices, and (3) Evaluation/Training Facilities. It was found that the participants were mostly Caucasians, females, did not have degrees in rehabilitation, and possessed non-rehabilitation related certification/licensure. The representation of people with disabilities was extremely low. Recommendations for future research, continued federal funding of rehabilitation and related programs, and possible changes in existing policies were made.

Acute disseminated encephalomyelitis in two renal transplant patients: is there a role for Epstein-Barr virus reactivation?

2013 ◽  
Vol 19 (9) ◽  
pp. 1222-1225 ◽  
Author(s):  
N Caucheteux ◽  
A Maarouf ◽  
L Daelman ◽  
O Toupance ◽  
S Lavaud ◽  
...  
Keyword(s):  
Epstein Barr Virus ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Virus Reactivation ◽  
Barr Virus ◽  
Transplant Patients ◽  
The Central Nervous System ◽  
Epstein Barr ◽  
Magnetic Resonance Imaging Mri ◽  
Epstein Barr Virus Reactivation

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system, usually occurring after a vaccination or infectious disease. It has been exceptionally described in transplanted patients. The pathophysiology remains incompletely understood. We report the clinical, biological and magnetic resonance imaging (MRI) presentation and evolution of two kidney-transplanted patients with ADEM associated with local Epstein-Barr virus (EBV) reactivation. ADEM may occur in transplanted patients with favorable evolution. Its pathophysiology is uncertain, and the implication of EBV is discussed.

Sign in / Sign up

Export Citation Format

Share Document