Publication date: Available online 26 December 2016 Source:Seminars in Pediatric Neurology Author(s): Karen S. Carvalho, Tal Grunwald, Francesco De Luca The Endocrine system is a complex group of organs and glands that relates to multiple other organs and systems in the body with the ultimate goal of maintaining homeostasis. This complex network functions through hormones excreted by several glands and released in the blood, ultimately targeting different body tissues and modulating their function. Any primary disorders affecting the endocrine glands and altering the amount of hormones synthesized and released will lead to disruption in the functions of multiple organs. The central nervous system of a developing child is particularly sensitive to endocrine disorders. A variety of neurological manifestations have been described as features of several endocrine diseases in childhood. Knowledge of these manifestations may contribute to an early diagnosis of an endocrine disorder, especially when more typical features of that disorder have not manifested yet. In this review, we will discuss specific neurologic manifestations found in various endocrine disorders in children.
Neurological Complications of Nutritional Deficiencies
Publication date: Available online 1 March 2017 Source:Seminars in Pediatric Neurology Author(s): Aparna Polavarapu, Daphne Hasbani Vitamins and minerals have essential functions in the body, from signal transduction to acting as cofactors for numerous enzymatic processes. Nutritional deficiencies and excess of certain vitamins and minerals can have profound effects on the central and peripheral nervous systems from early development into adulthood. This article summarizes the role of various nutritional factors in the nervous system and the neurologic symptoms that can arise from deficiency or excess.
Publication date: Available online 23 December 2016 Source:Seminars in Pediatric Neurology Author(s): Lauren Weaver, Ayman Samkari Though the treatment of pediatric cancers has come a long way, acute and chronic effects of cancer are still affecting the life of many children. These effects may be caused not only by the malignancy itself but also by the interventions used for the purpose of treatment. This article focuses primarily on the indirect effects of pediatric cancers and their treatment on the central and peripheral nervous system. Chemotherapy, radiation, and stem cell transplantation cause an immune-compromised state and place the patient at risk of infection, the leading cause of mortality in pediatric cancer. The underlying cancer and the treatments also cause neurovascular changes that may lead to neurologic sequelae immediately or many years in the future. Chemotherapy and radiation have both immediate and long-term neurotoxic effects on the central and peripheral nervous system. Cancers may also trigger an immune response that damages nervous system components, leading to altered mental status, seizures, abnormal movements, and even psychosis. Knowledge of these effects can help the practitioner be more vigilant for the signs and symptoms of potential neurological complications during the management of pediatric cancers.
Pediatric Sudden Unexpected Death in Epilepsy: What Have we Learned from Animal and Human Studies, and Can we Prevent it?
Publication date: May 2016 Source:Seminars in Pediatric Neurology, Volume 23, Issue 2 Author(s): Rebecca L. Holt, Eric Arehart, Arsen Hunanyan, Nina A. Fainberg, Mohamad A. Mikati Several factors, such as epilepsy syndrome, poor compliance, and increased seizure frequency increase the risks of sudden unexpected death in epilepsy (SUDEP). Animal models have revealed that the mechanisms of SUDEP involve initially a primary event, often a seizure of sufficient type and severity, that occurs in a brain, which is vulnerable to SUDEP due to either genetic or antecedent factors. This primary event initiates a cascade of secondary events starting, as some models indicate, with cortical spreading depolarization that propagates to the brainstem where it results in autonomic dysfunction. Intrinsic abnormalities in brainstem serotonin, adenosine, sodium-postassium ATPase, and respiratory-control systems are also important. The tertiary event, which results from the above dysfunction, consists of either lethal central apnea, pulmonary edema, or arrhythmia. Currently, it is necessary to (1) continue researching SUDEP mechanisms, (2) work on reducing SUDEP risk factors, and (3) address the major need to counsel families about SUDEP.
The Episodic Syndromes That Maybe Associated with Migraines
Publication date: February 2016 Source:Seminars in Pediatric Neurology, Volume 23, Issue 1 Author(s): Diana Lebron, Elza Vasconcellos The “childhood periodic syndromes” have been renamed “the episodic syndromes that maybe associated with migraines”. These syndromes were initially considered precursors of migraines that only occurred in childhood; however recent literature suggests that the episodic syndromes can occur in adults with known migraine and does not necessarily present as a precursor. This review article discusses the recent literature regarding the episodic syndromes and potential treatments. These disorders are seen by multiple subspecialists, therefore it is important to recognize and use the same definitions, criteria and nomenclature. A collaborative and multidisciplinary approach is critical to characterize, manage and potentially improve outcomes.
Publication date: May 2016 Source:Seminars in Pediatric Neurology, Volume 23, Issue 2 Author(s): Phillip L. Pearl Vitamin-dependent epilepsies and multiple metabolic epilepsies are amenable to treatment that markedly improves the disease course. Knowledge of these amenably treatable severe pediatric epilepsies allows for early identification, testing, and treatment. These disorders present with various phenotypes, including early onset epileptic encephalopathy (refractory neonatal seizures, early myoclonic encephalopathy, and early infantile epileptic encephalopathy), infantile spasms, or mixed generalized seizure types in infancy, childhood, or even adolescence and adulthood. The disorders are presented as vitamin responsive epilepsies such as pyridoxine, pyridoxal-5-phosphate, folinic acid, and biotin; transportopathies like GLUT-1, cerebral folate deficiency, and biotin thiamine responsive disorder; amino and organic acidopathies including serine synthesis defects, creatine synthesis disorders, molybdenum cofactor deficiency, and cobalamin deficiencies; mitochondrial disorders; urea cycle disorders; neurotransmitter defects; and disorders of glucose homeostasis. In each case, targeted intervention directed toward the underlying metabolic pathophysiology affords for the opportunity to significantly effect the outcome and prognosis of an otherwise severe pediatric epilepsy.
Publication date: Available online 16 January 2017 Source:Seminars in Pediatric Neurology Author(s): Nandini Madan, Karen S. Carvalho This article focuses on the complex interactions between the cardiovascular and neurological systems. Initially we focus on neurological complications in children with Congenital Heart Disease both secondary to the underlying cardiac disease or complications of interventions. We discuss diagnosis and management of common syncope syndromes with emphases in Vasovagal Syncope. We review the diagnosis, classification and management of children and adolescents with Postural Orthostatic Tachycardia Syndrome. Last we discuss Long QT Syndrome and SUDEP, reviewing advance in genetics and current knowledge of pathophysiology of these conditions. This article attempts to provide an overview of these disorders with focus on pathophysiology, advances in molecular genetics and current medical interventions.