Abstract:
:Amyotrophic lateral sclerosis is a fatal neurodegenerative disease. Epidemiologic data suggest that malnutrition is a common feature in amyotrophic lateral sclerosis and being overweight or obese confers a survival advantage in this patient population. In amyotrophic lateral sclerosis mouse models, a high-fat diet has been shown to lead to weight gain and prolonged survival. However, little research has been conducted to test whether nutritional interventions might ameliorate the disease course in humans. Here we review the currently available evidence supporting the potential role of dietary interventions as a therapeutic tool for amyotrophic lateral sclerosis. Ultimately, determining whether a high-fat or ketogenic diet could be beneficial in amyotrophic lateral sclerosis will require large randomized, placebo-controlled clinical trials.
journal_name
J Child Neuroljournal_title
Journal of child neurologyauthors
Paganoni S,Wills AMdoi
10.1177/0883073813488669subject
Has Abstractpub_date
2013-08-01 00:00:00pages
989-92issue
8eissn
0883-0738issn
1708-8283pii
0883073813488669journal_volume
28pub_type
杂志文章abstract::We describe the clinical utility of echo-planar diffusion-weighted imaging in neonatal cerebral infarction. Eight full-term neonates aged 1 to 8 days referred for neonatal seizures were studied. Patients were followed for a mean of 17 months with detailed neurologic examinations at regular intervals. Head computed tom...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307380001500905
更新日期:2000-09-01 00:00:00
abstract::Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are two sides of the same coin. Both arise from mutations in the gene encoding myelin proteolipid protein. The disease spectrum for Pelizaeus-Merzbacher disease and spastic paraplegia type 2 is extraordinarily broad, ranging from a spastic gait in the...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/08830738030180090801
更新日期:2003-09-01 00:00:00
abstract::There are conflicting reports regarding the efficacy of trihexyphenidyl, an anticholinergic drug, for treatment of dystonia in cerebral palsy. The author hypothesized that trihexyphenidyl may be more effective in specific subgroups and performed a retrospective analysis of 31 children (8.2 ± 5.8 years) with dystonia f...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073810392582
更新日期:2011-07-01 00:00:00
abstract::Acute transverse myelitis is a clinical syndrome affecting the spinal cord, which is characterized by acute onset of motor, sensory, and autonomic dysfunction. Approximately 20% of cases of acute transverse myelitis occur in children. This review summarizes the current published literature on acute transverse myelitis...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/0883073812452916
更新日期:2012-11-01 00:00:00
abstract::Acute disseminated encephalomyelitis is a monophasic inflammatory demyelinating disease of the central nervous system involving the white matter, and to a lesser extent, the gray matter. Bilateral thalamic lesions have been reported in 12% of pediatric patients with acute disseminated encephalomyelitis. In most cases,...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808331364
更新日期:2009-08-01 00:00:00
abstract::Direct perfusion of specific regions of the central nervous system by convection-enhanced delivery is becoming more widely used for the delivery of compounds in the research and treatment of various neural disorders. In contrast to other currently available central nervous system delivery techniques, convection-enhanc...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/0883073808321064
更新日期:2008-10-01 00:00:00
abstract::Epilepsy is a very uncommon first manifestation of a neuroblastoma. A 5-month-old healthy infant presented with acute onset seizures and developmental regression. Extensive investigation was remarkable for urinary vanillylmandelic acid and homovanillic acid peaks. Abdominopelvic magnetic resonance imaging (MRI) disclo...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073812461946
更新日期:2014-01-01 00:00:00
abstract::The purpose of this survey and record review was to characterize emergency department management of unprovoked seizures and status epilepticus in children in Illinois. The survey was sent to 119 participating emergency departments in the Emergency Medical Services for Children program; responses were received from 103...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073814566626
更新日期:2015-10-01 00:00:00
abstract::Motor speed and accuracy are both affected in childhood dystonia. Thus, deriving a speed-accuracy function is an important metric for assessing motor impairments in dystonia. Previous work in dystonia studied the speed-accuracy trade-off during point-to-point tasks. To achieve a more relevant measurement of functional...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073815578526
更新日期:2015-10-01 00:00:00
abstract::The relationship between somatic growth and neurocognitive outcome was studied in a cohort of 136 children with intrauterine growth retardation. The children were followed up from birth to 9 to 10 years of age by annual measurements of growth parameters, neurodevelopmental evaluations, and IQ. The rate of catch-up for...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808331082
更新日期:2009-07-01 00:00:00
abstract::We sent questionnaires concerning the current therapy for West syndrome to 208 institutions at which pediatric care members of the Japan Epilepsy Society were working. Of these, 129 (62%) institutions responded. Vitamin B6 was the preferred first-line drug, followed by the combination of vitamin B6 and valproate or mo...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307380001500615
更新日期:2000-06-01 00:00:00
abstract::Ketogenic therapy is now an accepted treatment for pediatric and adult patients with medically refractory epilepsy.1-3 However, young adults treated with a ketogenic diet face unique challenges when transitioning to adult neurology providers.4 The variable acceptance of dietary therapy, paucity of nutritionists and ad...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073820938587
更新日期:2020-11-01 00:00:00
abstract::The epilepsies in childhood are classified as primary (or idiopathic) and secondary (or symptomatic). The primary epilepsies account for two thirds of all childhood epilepsies and are presumed to be genetically determined. In the remaining one third of cases, a neuropathologic lesion can be identified. This paper summ...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/088307389801300801
更新日期:1998-08-01 00:00:00
abstract::Acute cerebellitis is a rare condition often considered within the group of acute postinfectious cerebellar ataxia despite its distinctive clinical and imaging features. We retrieved clinical, laboratory, and follow-up data of 15 children diagnosed with acute cerebellitis in our department between 2011 and 2019. There...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073820901407
更新日期:2020-05-01 00:00:00
abstract::After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsycho...
journal_title:Journal of child neurology
pub_type: 杂志文章,多中心研究
doi:10.1177/088307380001500510
更新日期:2000-05-01 00:00:00
abstract::Recently described, the congenital Zika syndrome caused by the Zika virus has many features of other congenital infections. This case series study reports 22 infants with congenital Zika syndrome in Brazil who developed infantile spasms during their first year of life. The median age of infantile spasms onset was 4.3 ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073818780105
更新日期:2018-09-01 00:00:00
abstract::Alexander disease is a leukodystrophy caused by dominant missense mutations in the gene encoding the glial fibrillary acidic protein. Individuals with this disorder often present with a typical neuroradiologic pattern including white matter abnormalities with brainstem involvement, selective contrast enhancement, and ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073816673263
更新日期:2017-02-01 00:00:00
abstract::Two children with isolated congenital anosmia, a rare syndrome of deficient restricted neuronal migration, are presented with early diagnosis confirmed by standardized smell testing and detailed neuroimaging studies. Recognition of this disorder and its spectrum of presentations provides important insights into the mo...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307389801300404
更新日期:1998-04-01 00:00:00
abstract::Facioscapulohumeral muscular dystrophy is one of the most prevalent muscular dystrophies in the world, resulting from the deletion of tandem repeats on chromosome 4q35. Extramuscular associations include sensorineural hearing loss, mental retardation, and epilepsy. These manifestations are commonly found in those with...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.2310/7010.2006.00054
更新日期:2006-03-01 00:00:00
abstract::Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset...
journal_title:Journal of child neurology
pub_type: 临床试验,杂志文章
doi:10.1177/0883073807302614
更新日期:2007-05-01 00:00:00
abstract::We describe the brain magnetic resonance imaging (MRI) findings in eight neonates with tuberous sclerosis complex to further delineate the spectrum of characteristic findings in these patients. In addition to the previously described characteristic brain MRI findings in neonates, which included cortical tuber, transma...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738060210042301
更新日期:2006-04-01 00:00:00
abstract::Paroxysmal kinesigenic dyskinesia is a rare episodic movement disorder that can be isolated or associated with benign infantile seizures as part of choreoathetosis syndrome. Mutations in the PRRT2 gene have been recently identified as a cause of paroxysmal kinesigenic dyskinesia and infantile convulsion and choreoathe...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073814556887
更新日期:2015-09-01 00:00:00
abstract::Autosomal inherited mitochondrial diseases have been of increasing interest among clinicians and mitochondrial research groups because these diseases are caused through a secondary effect on the mitochondrial DNA. It was thought that the genetic stability of mitochondrial DNA relies on the accuracy of DNA polymerase g...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808324539
更新日期:2009-04-01 00:00:00
abstract::Acute infantile bilateral striatal necrosis is a rarely described acute neurological syndrome associated with radiological findings. Its etiology and pathogenic mechanisms are unknown. Clinically, the syndrome usually follows respiratory illnesses and presents with an array of neurological findings, including axial at...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073807304194
更新日期:2007-10-01 00:00:00
abstract::Neurofibromatosis type 1 is associated with executive dysfunctions and comorbidity with attention-deficit hyperactivity disorder (ADHD) in 30% to 50% of children. This study was designed to clarify the neurocognitive phenotype observed in neurofibromatosis type 1 by testing the hypothesis that children with neurofibro...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073813517001
更新日期:2014-10-01 00:00:00
abstract::The purpose of this study was to examine the psychiatric characteristics of children with Prader-Willi syndrome in Korea, focusing particularly on their behavioral problems and obsessive-compulsive spectrum symptoms. Fourteen patients with Prader-Willi syndrome, together with their parents, underwent a psychiatric int...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738050200021001
更新日期:2005-02-01 00:00:00
abstract::Rett syndrome is a sporadic disorder (except for a few familial cases) occurring in 1 in 10,000 to 1 in 23,000 girls worldwide. It is associated with profound mental and motor handicap. About 90% of cases involve a mutation in the methyl-CpG binding protein 2 gene (MECP2). The role of this gene in the pathogenesis of ...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/08830738050200090901
更新日期:2005-09-01 00:00:00
abstract::Mitochondrial cytopathies are a group of heterogeneous disorders characterized by multisystem involvement. Renal involvement in mitochondrial cytopathies is usually manifested as tubular dysfunction owing to impaired energy metabolism; however, a few cases with glomerular changes have also been reported. Herein we rep...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738050200011304
更新日期:2005-01-01 00:00:00
abstract::Metabolic disorders constitute an important cause of neurologic disease, including infantile epilepsy. The inability to characterize seizures and epilepsy syndromes precisely in infants impedes the recognition of features suggestive of specific underlying metabolic and neurodegenerative etiologies. Classification syst...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:
更新日期:2002-12-01 00:00:00
abstract::An epileptic syndrome of benign nocturnal childhood occipital epilepsy with excellent prognosis is described. The syndrome is characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. There may be marching to involve the head and limbs, ending with a generalized tonic-cl...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307388900400107
更新日期:1989-01-01 00:00:00