A Novel Phenotype in a Previously Described Epilepsy-Aphasia Disorder.

abstract：:A 13-year-old boy with Lennox-Gastaut syndrome characterized by absence, myoclonic, complex-partial, and secondarily generalized tonic-clonic seizures, presents with progressive obtundation and loss of motor and verbal skills over a 2-day period. Initial evaluation revealed therapeutic phenytoin serum concentrations. ...

journal_title：Seminars in pediatric neurology

authors： Gidal BE,Seltz JN,Rutecki P

更新日期：1999-09-01 00:00:00

abstract：:Ethical issues in the critical care unit frequently arise in children with neurological problems. These ethical issues frequently challenge our medical management of such cases and can be quite problematic. This article reviews key ethical issues that may arise including informed consent, futility, justice/rationing, ...

journal_title：Seminars in pediatric neurology

authors： Shevell M

更新日期：2004-06-01 00:00:00

abstract：:A brain, a neural structure located in the head, differs from a ganglion by the following characteristics: (1) a brain subserves the entire body, not just restricted segments; (2) it has functionally specialized parts; (3) it is bilobar; (4) commissures and neurons form the surface with axons in the central core; (5) ...

journal_title：Seminars in pediatric neurology

authors： Sarnat HB,Netsky MG

更新日期：2002-12-01 00:00:00

abstract：:Infections of the central nervous system are a significant cause of neurologic dysfunction in resource-limited countries, especially in Africa. The prevalence is not known and is most likely underestimated because of the lack of access to accurate diagnostic screens. For children, the legacy of subsequent neurodisabil...

journal_title：Seminars in pediatric neurology

authors： Mallewa M,Wilmshurst JM

更新日期：2014-03-01 00:00:00

abstract：:Many genetic conditions can result in ataxia. Careful clinical, radiologic, and genetic evaluation permits the specific diagnosis of many of these conditions, which in turn facilitates medical care for the patient and genetic counseling for the patient and family. Predictive, prenatal, and carrier testing are possible...

journal_title：Seminars in pediatric neurology

authors： Nance MA

更新日期：2003-09-01 00:00:00

abstract：:Herpes simplex virus type 2 (HSV-2) is known to cause acute retinal necrosis (ARN). The availability of HSV-2-specific polymerase chain reaction tests for diagnostic analysis has greatly increased our ability to discriminate ARN caused by HSV-2 from ARN caused by either herpes simplex virus type 1 or varicella zoster ...

journal_title：Seminars in pediatric neurology

authors： Grose C

更新日期：2012-09-01 00:00:00

abstract：:Scoliosis, common in preadolescent females is a finding which warrants investigation in any other age group or if the clinical or neuroimaging features are atypical even in the preadolescent female. The cause of scoliosis are numerous but include several important conditions some of which are treatable and some of whi...

journal_title：Seminars in pediatric neurology

authors： Barnes PD

更新日期：1996-09-01 00:00:00

abstract：:Many inherited metabolic diseases or inborn errors of metabolism (IEM) cause movement disorders in children. This review focuses on chorea, dystonia, myoclonus, tremor, and parkinsonism. Broad neurometabolic categories commonly responsible for pediatric movement disorders include mitochondrial cytopathies, organic aci...

journal_title：Seminars in pediatric neurology

authors： Christensen CK,Walsh L

更新日期：2018-04-01 00:00:00

abstract：:Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various ence...

journal_title：Seminars in pediatric neurology

authors： Shbarou R,Mikati MA

更新日期：2016-05-01 00:00:00

abstract：:Creatine deficiency syndromes are a group of disorders of creatine (Cr) synthesis and transport characterized by intellectual disability, language delay, epilepsy, autism spectrum disorder, and movement disorders secondary to decrease of Cr concentration in the brain. Synthesis defects are treatable, therefore an earl...

journal_title：Seminars in pediatric neurology

authors： Fons C,Campistol J

更新日期：2016-11-01 00:00:00

abstract：:The discovery of new cytogenetic and molecular genetic techniques and principles has been explosive in recent years. A secure diagnosis based on molecular evidence has become possible for many syndromes previously only clinically defined, which has helped enormously in predicting children's developmental progress, in ...

journal_title：Seminars in pediatric neurology

authors： Macmillan C

更新日期：1998-03-01 00:00:00

abstract：:Numerous neurosurgical approaches are available for children presenting with hypothalamic hamartomas (HHs) associated with severe epilepsy. A concern regarding the impairment of short-term memory after resective surgery is promoting the exploration of less invasive alternatives like radiosurgery. Gamma knife radiosurg...

journal_title：Seminars in pediatric neurology

authors： Régis J,Scavarda D,Tamura M,Villeneuve N,Bartolomei F,Brue T,Morange I,Dafonseca D,Chauvel P

更新日期：2007-06-01 00:00:00

abstract：:Infants with congenital infections are at high risk for developmental disabilities. Because of this increased-risk status, they require comprehensive, longitudinal follow-up that should begin in the neonatal period with sensory, developmental, and neuromotor assessments as well as neuroradiological imaging. Reassessme...

journal_title：Seminars in pediatric neurology

authors： Williamson WD

更新日期：1994-09-01 00:00:00

abstract：:Intracranial pressure (ICP) and skull volume are intricately related. Craniosynostosis alters skull volume, and the many forms of craniosynostosis complicate the relationship to ICP even further. Patients with single-suture synostosis are less likely to experience elevated ICP than patients in whom multiple sutures, c...

journal_title：Seminars in pediatric neurology

authors： Bristol RE,Lekovic GP,Rekate HL

更新日期：2004-12-01 00:00:00

abstract：:Advances in infant cardiac surgery have resulted in a dramatic decline in mortality rates; however, neurological morbidity remains an important concern. The effectiveness of a number of interventional strategies to prevent or minimize brain injury during open heart surgery are currently being investigated. This articl...

journal_title：Seminars in pediatric neurology

authors： du Plessis AJ,Johnston MV

更新日期：1999-03-01 00:00:00

abstract：:The incidence and sex distribution of spinal cord injury (SCI) changes with age. Motor vehicle accidents, bicycle accidents, sports accidents, and violence are major causes in the pediatric population. Pulmonary complications may be severe and life-threatening in the acute phase. Chronically, the degree of ventilatory...

journal_title：Seminars in pediatric neurology

authors： Zidek K,Srinivasan R

更新日期：2003-06-01 00:00:00

abstract：:Migraine is the most frequent type of headache in children. In the 1980s, scientists first hypothesized a connection between migraine and mitochondrial (mt) disorders. More recent studies have suggested that at least some subtypes of migraine may be related to a mt defect. Different types of evidence support a relatio...

journal_title：Seminars in pediatric neurology

authors： Yorns WR Jr,Hardison HH

更新日期：2013-09-01 00:00:00

abstract：:Pediatric neurologists frequently encounter patients who present with significant musculoskeletal pain that cannot be attributed to a specific injury or illness, which can often be defined as pain amplification syndrome (PAS). PAS in children and adolescents is the result of a heightened pain sensitivity pathway, whic...

journal_title：Seminars in pediatric neurology

authors： Namerow LB,Kutner EC,Wakefield EC,Rzepski BR,Sahl RA

更新日期：2016-08-01 00:00:00

abstract：:The heterogeneous childhood limb-girdle muscular dystrophies have originally been defined as a group of autosomal recessive and dominant diseases with progressive weakness and wasting of shoulder and pelvic-girdle muscles. Over the last 12 years, the underlying genetic defects for many of the diseases have been identi...

journal_title：Seminars in pediatric neurology

authors： Straub V,Bushby K

更新日期：2006-06-01 00:00:00

abstract：:Newborn screening has evolved from a single test for a single metabolite to a test that detects more than 90 metabolites on a single blood spot. In the past decade, the panel of newborn-screening disorders has rapidly expanded and will continue to grow as more is discovered about the human genome. It continues to be a...

journal_title：Seminars in pediatric neurology

authors： Copeland S

更新日期：2008-09-01 00:00:00

abstract：:Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with variable expression. The complications are age specific. Neurologic complications include tumors of the peripheral nerves, nerve roots, and plexi; spinal cord compression; dural ectasias; learning disabilities; attention deficit; headaches; seizures...

journal_title：Seminars in pediatric neurology

authors： Tonsgard JH

更新日期：2006-03-01 00:00:00

abstract：:This article contains a discussion and detailed pictorial account of diagnostic and neurosurgical therapeutic procedures used to help pediatric patients who have medically intractable epilepsy. The operations discussed are placement of sphenoidal electrodes, strips and grids, temporal and extratemporal resections, cor...

journal_title：Seminars in pediatric neurology

authors： Iskandar BJ,Ramirez LF

更新日期：2000-09-01 00:00:00

abstract：:In this review, we discuss the important pathological lesions observed in temporal lobectomies and neocortical resections performed for medically refractory seizures in children. A higher percentage of pediatric cases appear to be "lesional" with computed tomography (CT) and magnetic resonance imaging (MRI) and abnorm...

journal_title：Seminars in pediatric neurology

authors： Jay V,Becker LE

更新日期：1995-09-01 00:00:00

abstract：:Magnetic resonance imaging (MRI) has contributed dramatically to our understanding of the newborn with neurologic problems. Recently developed magnetic resonance techniques, such as fetal MRI and MR spectroscopy, offer additional insight into normal and pathologic processes affecting the fetal and neonatal CNS. This a...

journal_title：Seminars in pediatric neurology

authors： Bangert BA

更新日期：2001-06-01 00:00:00

abstract：:The discipline of childhood movement disorders is an exciting new area of specialization within the field of pediatric neurology. Establishing a formal educational system is an ongoing challenge that should include the standardization of movement definitions, the development of a formal classification system for exist...

journal_title：Seminars in pediatric neurology

authors： Singer HS

更新日期：2011-06-01 00:00:00

abstract：:Intermediate filament (IF) proteins are cytoplasmic and nuclear cytoskeletal proteins. Of the ~70 IF proteins, nearly 12 are found in the nervous system, where their expression is largely cell-type specific. Astrocytes express glial fibrillary acidic protein (GFAP), whereas different neuron types contain neurofilament...

journal_title：Seminars in pediatric neurology

authors： Quick Q,Paul M,Skalli O

更新日期：2015-03-01 00:00:00

abstract：:The protein surplus myopathies have emerged as a newly recognized subgroup of morphologically defined myopathies within the spectrum of congenital myopathies because of the accumulation of protein aggregates, some of them mutant proteins. Currently, nosologic, including molecular criteria include desmin-related myopat...

journal_title：Seminars in pediatric neurology

authors： Goebel HH,Borchert A

更新日期：2002-06-01 00:00:00

abstract：:Recurrent headaches in children are most often migraines and are based in a genetic predisposition with a low headache threshold. As with any pain experience, there is a large emotional component associated with an attack of migraines that grows in amplitude as the headaches become more frequent and resistant to medic...

journal_title：Seminars in pediatric neurology

authors： Farmer K,Dunn D,Scott E

更新日期：2010-06-01 00:00:00

abstract：:The interaction between sleep and headache or migraine is powerful and an elevated comorbidity between these 2 disorders has been reported in either adults or children. This comobidity is linked to common neurophysiological and neuroanatomical substrates that are genetically based strongly. The first reports on this r...

journal_title：Seminars in pediatric neurology

authors： Dosi C,Figura M,Ferri R,Bruni O

更新日期：2015-06-01 00:00:00

abstract：:Alpers disease is a mitochondrial depletion syndrome characterized by psychomotor retardation, intractable epilepsy, and liver failure. Polymerase gamma (POLG) gene mutations are a known cause of the disease. We describe a case in which a 14-month-old female presented with epilepsia partialis continua evolving into ge...

journal_title：Seminars in pediatric neurology

authors： Cardenas JF,Amato RS

更新日期：2010-03-01 00:00:00