Cognitive disabilities and long term outcomes in children with epilepsy: a tangled tail
Cognitive problems ranging from mild specific learning problems to profound intellectual disability (ID) are very common in children with epilepsy. For most affected patients there is good evidence that the cognitive problems are present at the onset of seizures and do not deteriorate over time. There is no evidence that a few seizures lead to cognitive deterioration. An exception may occur in children with epileptic encephalopathies, although this contention is not always easy to prove. Intellectual disability is a strong predictor of intractable epilepsy, and the greater the degree of the ID the greater the risk of medic...
Source: Seminars in Pediatric Neurology - October 6, 2017 Category: Neurology Authors: Carol Camfield, Peter Camfield Source Type: research
Neuroinflammation in Ischemic Pediatric Stroke
Over the last decades, the importance of inflammatory processes in pediatric stroke have become increasingly evident. Ischemia launches a cascade of events: activation and inhibition of inflammation by a large network of cytokines, adhesion and small molecules, protease, chemokines. There are major differences in the neonatal brain compared to adult brain, but developmental trajectories of the process during childhood are not yet well known. In neonatal stroke ischemia is the leading pathophysiology, but infectious and inflammatory processes have a significant input into the course and degree of tissue damage. (Source: Sem...
Source: Seminars in Pediatric Neurology - September 19, 2017 Category: Neurology Authors: Maja Steinlin Source Type: research
Novel Inflammatory Neuropathology in Immature Brain: (1) Fetal Tuberous Sclerosis, (2) Febrile Seizures, (3) α-B-crystallin, and (4) Role of Astrocytes
Though the term “inflammation” is traditionally defined as proliferation or infiltration of lymphatic cells of the lymphatic immune system and macrophages or as immunoreactive proteins including cytokines, interleukins and major histocompatibility complexes, recently recognized reactions to tissue injury also a re inflammation, often occurring in the central nervous system in conditions where they previously were not anticipated and where they may play a role in both pathogenesis and repair. We highlight 4 such novel inflammatory conditions revealed by neuropathologic studies: (1) inflammatory markers and cells in the ...
Source: Seminars in Pediatric Neurology - September 13, 2017 Category: Neurology Authors: Harvey B. Sarnat, Morris H. Scantlebury Source Type: research
Novel Inflammatory Neuropathology in Immature Brain: 1. Fetal Tuberous Sclerosis; 2. Febrile Seizures; 3. α-B-crystallin; 4. Role of Astrocytes
Though the term “inflammation” is traditionally defined as proliferation or infiltration of lymphatic cells of the lymphatic immune system and macrophages or as immunoreactive proteins including cytokines, interleukins and major histocompatibility complexes, recently recognized reactions to tissue injury also a re inflammation, often occurring in the CNS in conditions where they previously were not anticipated and where they may play a role in both pathogenesis and repair. We highlight four such novel inflammatory conditions revealed by neuropathological studies: 1) inflammatory markers and cells in the br ain of human...
Source: Seminars in Pediatric Neurology - September 13, 2017 Category: Neurology Authors: Harvey B. Sarnat, Morris H. Scantlebury Source Type: research
Cancer and Autoimmunity: Paraneoplastic Neurological Disorders Associated With Neuroblastic Tumors
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - September 1, 2017 Category: Neurology Authors: Wendy G. Mitchell, Franz Blaes Source Type: research
Cancer and Autoimmunity: Paraneoplastic Neurologic Disorders Associated with Neuroblastic Tumors
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome (OMS). They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks. (Source: Seminars ...
Source: Seminars in Pediatric Neurology - September 1, 2017 Category: Neurology Authors: Wendy G. Mitchell, Franz Blaes Tags: Issue 3 from Dr. Michale Pranzatelli Source Type: research
Autoimmune Epilepsies
Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, and/or other focal neurologic deficits. Traditional neurologic diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - September 1, 2017 Category: Neurology Authors: Anusha K. Yeshokumar, Carlos A. Pardo Source Type: research
Introduction
“Although the way ahead is full of pitfalls and difficulties, this is indeed an exhilarating prospect…. The highlights of tomorrow are the unpredictibilities of today.” (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - August 25, 2017 Category: Neurology Authors: Michael R. Pranzatelli Source Type: research
The spectrum of inflammatory acquired demyelinating syndromes in children
Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, while recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, MRI features, outcomes and serum biomarkers in these disorders. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - August 22, 2017 Category: Neurology Authors: R.F. Neuteboom, C. Wilbur, E.D. Van Pelt, M. Rodriguez, E.A. Yeh Source Type: research
Neonatal epileptic encephalopathy caused by de novo GNAO1 mutation misdiagnosed as atypical Rett syndrome: Cautions in interpretation of genomic test results
Epileptic encephalopathies are childhood brain disorders characterized by a variety of severe epilepsy syndromes that differ by the age of onset and seizure type. Until recently, the cause of many epileptic encephalopathies was unknown. Whole exome or whole genome sequencing has led to the identification of several causal genes in individuals with epileptic encephalopathy, and the list of genes has now expanded greatly. Genetic testing with epilepsy gene panels is now done quite early in the evaluation of children with epilepsy, following brain imaging, electroencephalogram, and metabolic profile. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - August 16, 2017 Category: Neurology Authors: Brittany Gerald, Keri Ramsey, Newell Belnap, Szabolcs Szelinger, Ashley L. Siniard, Chris Balak, Megan Russell, Ryan Richholt, Matt De Both, Ana M. Claasen, Isabelle Schrauwen, Matthew J. Huentelman, David W. Craig, Sampathkumar Rangasamy, Vinodh Narayana Source Type: research
Immunotherapeutics in Pediatric Autoimmune Central Nervous System Disease: Agents and Mechanisms
Beyond the major advances produced by careful clinical-radiological phenotyping and biomarker development in autoimmune central nervous system disorders, a comprehensive knowledge of the range of available immune therapies and a deeper understanding of their action should benefit therapeutic decision-making. This review discusses the agents used in neuroimmunology and their mechanisms of action. First-line treatments typically include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while for severe disease second-line “induction” agents such as rituximab or cyclophosphamide are used. (Source: Seminars ...
Source: Seminars in Pediatric Neurology - August 16, 2017 Category: Neurology Authors: Margherita Nosadini, Stefano Sartori, Suvasini Sharma, Russell C. Dale Source Type: research
Immunotherapeutics in Pediatric Autoimmune CNS Disease: Agents and Mechanisms
Beyond the major advances produced by careful clinical-radiological phenotyping and biomarker development in autoimmune CNS disorders, a comprehensive knowledge of the range of available immune therapies and a deeper understanding of their action should benefit therapeutic decision-making. This review discusses the agents used in neuroimmunology and their mechanisms of action. First-line treatments typically include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while for severe disease second-line ‘induction′ agents such as rituximab or cyclophosphamide are used. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - August 16, 2017 Category: Neurology Authors: Margherita Nosadini, Stefano Sartori, Suvasini Sharma, Russell C. Dale Source Type: research
Neonatal epileptic encephalopathy caused by de novo GNAO1 mutation misdiagnosed as atypical Rett syndrome: Cautions in interpretation of genomic test results
Epileptic encephalopathies are childhood brain disorders characterized by a variety of severe epilepsy syndromes that differ by the age of onset and seizure type. Until recently, the cause of many epileptic encephalopathies was unknown. Whole exome or whole genome sequencing has led to the identification of several causal genes in individuals with epileptic encephalopathy, and the list of genes has now expanded greatly. Genetic testing with epilepsy gene panels is now done quite early in the evaluation of children with epilepsy, following brain imaging, electroencephalogram (EEG), and metabolic profile. (Source: Seminars i...
Source: Seminars in Pediatric Neurology - August 16, 2017 Category: Neurology Authors: Brittany Gerald, Keri Ramsey, Newell Belnap, Szabolcs Szelinger, Ashley L. Siniard, Chris Balak, Megan Russell, Ryan Richholt, Matt De Both, Ana M. Claasen, Isabelle Schrauwen, Matthew J. Huentelman, David W. Craig, Sampathkumar Rangasamy, Vinodh Narayana Source Type: research
A Neuroprimer: Principles of Central Nervous System Immunity
Despite longstanding perceptions, robust innate and adaptive immune responses occur within the central nervous system (CNS) in response to infection and tissue damage. Although necessary to control infection, immune responses can lead to severe CNS pathology in the context of both viral infection and autoimmunity. Research into how the central nervous and immune systems communicate has accelerated over the past 20 years leading to a better understanding of pathways controlling immune activation and neuroinflammation that have guided the approval of new disease-modifying therapies to treat CNS immunopathology, particularly ...
Source: Seminars in Pediatric Neurology - August 14, 2017 Category: Neurology Authors: Gregory P. Owens Source Type: research
Autoantibody-Associated Movement Disorders in Children: Proven and Proposed
Movement disorders secondary to autoantibodies in children represent a rapidly expanding group of conditions. Once considered to be limited to poststreptococcal Sydenham's chorea or rare cases of childhood systemic lupus erythematosus, a variety of antibody-related movement abnormalities are now seen as part of noninfectious autoimmune encephalitis or within an expanding list of postinfectious disorders. In this article, several proposed autoantibody-mediated movement disorders in children are reviewed. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - August 14, 2017 Category: Neurology Authors: Harvey S. Singer Source Type: research
