Acute necrotizing encephalopathy associated with hemophagocytic syndrome.

Abstract:

:A 7-year-old female suddenly exhibited high fever and convulsions, and entered a semi-coma. She also had thrombocytopenia, elevated aminotransferase, prolonged prothrombin time and activated partial thromboplastin time, and hemophagocytes in the bone marrow. The brain magnetic resonance imaging revealed multiple low-intensity areas on the T1-weighted images, and high-intensity areas on the T2-weighted images bilaterally in the thalamus, the dorsal part of the pons, and the cerebellar white matter. The patient was diagnosed as having both acute necrotizing encephalopathy and hemophagocytic syndrome. Serum and cerebrospinal fluid interleukin-6 and tumor necrosis factor-alpha were elevated to the same high levels (serum:cerebrospinal fluid interleukin-6, 103:101 pg/mL; tumor necrosis factor-alpha 753:753 pg/mL). The clinical symptoms and the magnetic resonance imaging findings improved immediately after the administration of dexamethasone. These results suggest that the hypercytokinemia and the hyperpermeability of both the blood-brain barrier and the capillary walls of the central nervous system might be essential in the pathogenesis of acute necrotizing encephalopathy, and that early steroid therapy might be effective in these conditions.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Akiyoshi K,Hamada Y,Yamada H,Kojo M,Izumi T

doi

10.1016/j.pediatrneurol.2005.08.030

subject

Has Abstract

pub_date

2006-04-01 00:00:00

pages

315-8

issue

4

eissn

0887-8994

issn

1873-5150

pii

S0887-8994(05)00545-X

journal_volume

34

pub_type

杂志文章
  • Neonatal seizures: do they damage the brain?

    abstract::Seizures are an early sign of brain injury in newborns. These seizures are in most cases repetitive or associated with asymptomatic electrographic seizures. Despite the relative resistance of the immature brain to seizure-induced brain damage, there is more and more evidence that neonatal seizures impair normal brain ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pediatrneurol.2008.10.026

    authors: Thibeault-Eybalin MP,Lortie A,Carmant L

    更新日期:2009-03-01 00:00:00

  • Benign hereditary chorea improved on stimulant therapy.

    abstract::Stimulant therapy is usually contraindicated in patients with movement disorders such as tics or chorea. A young boy is reported who had benign hereditary chorea and attention deficit disorder, whose chorea, handwriting, and independent ambulation paradoxically improved with methylphenidate treatment. ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(96)00056-2

    authors: Friederich RL

    更新日期:1996-05-01 00:00:00

  • Traumatic spinal epidural hematoma of a 10-month-old male: a clinical note.

    abstract::Traumatic spinal epidural hematoma (TSEH) is rare in children. Only three cases of TSEH were documented in the pediatric literature. This clinical note presents an infant with TSEH but no risk factors. Without magnetic resonance imaging examination, children with TSEH and minor symptoms may be missed and under reporte...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/s0887-8994(00)00151-x

    authors: Alva NS

    更新日期:2000-07-01 00:00:00

  • Congenital cytomegalovirus infection and brain clefting.

    abstract:BACKGROUND:Human cytomegalovirus, a major cause of permanent neurodevelopmental disability in children, frequently produces intracranial abnormalities, including calcifications and polymicrogyria, in infants with congenital cytomegalovirus infections. This report describes the features of cerebral cortical clefting, in...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2013.11.005

    authors: White AL,Hedlund GL,Bale JF Jr

    更新日期:2014-03-01 00:00:00

  • Mycoplasma pneumoniae--associated transverse myelitis and rhabdomyolysis.

    abstract::Mycoplasma pneumoniae is a common cause of respiratory tract infection. Extrapulmonary manifestations of M. pneumoniae infection are also common. The present case is that of a previously healthy 4-year-old boy who displayed a novel simultaneous onset of both acute rhabdomyolysis and transverse myelitis associated with...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2008.10.009

    authors: Weng WC,Peng SS,Wang SB,Chou YT,Lee WT

    更新日期:2009-02-01 00:00:00

  • Psychogenic seizures in obsessive-compulsive disorder with poor insight: a case report.

    abstract::Psychogenic seizures or psychogenic nonepileptic seizures occur in various mental disorders. Obsessive-compulsive symptoms can also imitate epileptic partial seizures, but detailed observations of this phenomenon are rare in the literature. A girl of 13 years was referred to the Department of Child Psychiatry because ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(97)00135-5

    authors: Wolańczyk T,Bryńska A

    更新日期:1998-01-01 00:00:00

  • Effect of treatment of obstructive sleep apnea on seizure outcomes in children with epilepsy.

    abstract::A retrospective review of children with epilepsy and obstructive sleep apnea, treated surgically for their obstructive sleep apnea from January 2008-October 2010, was performed for age, sex, type of epilepsy, antiseizure medications, sleep-study data, and changes in seizure frequency. Twenty-seven subjects (median age...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2012.03.005

    authors: Segal E,Vendrame M,Gregas M,Loddenkemper T,Kothare SV

    更新日期:2012-06-01 00:00:00

  • Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation.

    abstract::Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptom...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(98)00036-8

    authors: Gherpelli JL,Azeka E,Riso A,Atik E,Ebaid M,Barbero-Marcial M

    更新日期:1998-08-01 00:00:00

  • Idiopathic unilateral paralysis of the palate in childhood.

    abstract::Idiopathic soft palate paralysis is an isolated clinical entity of unknown cause. Typical clinical features are sudden onset, rhinolalia, and nasal escape of fluids from the ipsilateral nostril. The disorder affects mainly male children at the ages of 2 to 3 years and resolves spontaneously. This report presents a 5-y...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2005.02.014

    authors: Alp H,Tan H,Altunkaynak S,Orbak Z

    更新日期:2005-08-01 00:00:00

  • Phenotypic differences between T-->C and T-->G mutations at nt 8993 of mitochondrial DNA in Leigh syndrome.

    abstract::This study reports on a patient with Leigh syndrome with a T-to-C mutation at nucleotide 8993 of mitochondrial deoxyribonucleic acid (T8993C). The authors reviewed 10 Leigh syndrome patients, including ours, with T8993C. Compared with 18 reported patients with Leigh syndrome caused by a T-to-G mutation at nucleotide 8...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(97)00187-2

    authors: Fujii T,Hattori H,Higuchi Y,Tsuji M,Mitsuyoshi I

    更新日期:1998-03-01 00:00:00

  • Cerebrovascular accidents following the Fontan operation.

    abstract::The Fontan operation is one of the most common cardiac operations for children with congenital heart disease beyond the first year of age. Although the surgical mortality of this procedure has improved over the past 2 decades, the neurologic outcome in this population is not well described. We performed a retrospectiv...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(95)00027-d

    authors: du Plessis AJ,Chang AC,Wessel DL,Lock JE,Wernovsky G,Newburger JW,Mayer JE Jr

    更新日期:1995-04-01 00:00:00

  • Brain death secondary to arteriovenous hemangioma of the forearm.

    abstract::We describe the association of brain death in an infant with a large cutaneous hemangioma of the forearm. The irreversible cerebral injury is presumed to be secondary to cerebral hypoperfusion as a result of a combination of systemic hypotension and a systemic vascular "steal" phenomenon caused by the cutaneous hemang...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(90)90083-d

    authors: Hamvas A,Perlman JM,Volpe JJ

    更新日期:1990-01-01 00:00:00

  • Successful endovascular treatment of cerebral arteriovenous fistula.

    abstract::Childhood intracranial varix is rare and has been associated mostly with vein of Galen fistula or arteriovenous malformation. We present one patient with intracranial arteriovenous fistula with concomitant giant varix in a child. We treated the patient with endovascular embolization and obtained complete closure of fi...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(02)00424-1

    authors: Hung PC,Wang HS

    更新日期:2002-10-01 00:00:00

  • Sleep abnormalities in children with Dravet syndrome.

    abstract:BACKGROUND:Mutations in the voltage-gated sodium channel SCN1A gene are responsible for the majority of Dravet syndrome cases. There is evidence that the Nav1.1 channel coded by the SCN1A gene is involved in sleep regulation. We evaluated sleep abnormalities in children with Dravet syndrome using nocturnal polysomnogra...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2014.01.017

    authors: Dhamija R,Erickson MK,St Louis EK,Wirrell E,Kotagal S

    更新日期:2014-05-01 00:00:00

  • Elevated cerebrospinal fluid opening pressure in a pediatric demyelinating disease cohort.

    abstract:BACKGROUND:Cerebrospinal fluid opening pressure is elevated with central nervous system infection and vasculitis, but has not been studied in inflammatory demyelinating disease. This retrospective study sought to determine whether children with demyelinating disease demonstrate elevated cerebrospinal fluid opening pres...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2015.01.002

    authors: Narula S,Liu GT,Avery RA,Banwell B,Waldman AT

    更新日期:2015-04-01 00:00:00

  • Whole body hypothermia and oxidative stress in babies with hypoxic-ischemic brain injury.

    abstract::According to increasing evidence, hypothermia can significantly improve outcomes in term neonates manifesting asphyxic insult and hypoxic-ischemic encephalopathy. Oxidative stress plays a key role in hypoxic-ischemic and inflammatory brain injuries. We investigated the impact of hypothermia on oxidative stress in babi...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1016/j.pediatrneurol.2010.05.009

    authors: Perrone S,Szabó M,Bellieni CV,Longini M,Bangó M,Kelen D,Treszl A,Negro S,Tataranno ML,Buonocore G

    更新日期:2010-10-01 00:00:00

  • Visual-evoked potentials in the assessment of optic gliomas.

    abstract::The most effective method for the screening and monitoring of optic pathway gliomas in children with neurofibromatosis type 1 remains a dilemma. Children less than 6 years of age are the group at highest risk for the development of optic pathway gliomas. Although an annual ophthalmologic examination currently is recom...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(00)00230-7

    authors: Ng YT,North KN

    更新日期:2001-01-01 00:00:00

  • Cortical atrophy and cognitive performance in infantile nephropathic cystinosis.

    abstract::A group of children and adolescents with infantile nephropathic cystinosis underwent cognitive testing and were examined for cortical atrophy using magnetic resonance imaging or computed tomography. Ten of 11 patients demonstrated cortical atrophy. A consistent pattern of lower cognitive performance was found in patie...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(90)90004-k

    authors: Nichols SL,Press GA,Schneider JA,Trauner DA

    更新日期:1990-11-01 00:00:00

  • Clinical case of anti-N-methyl-D-aspartate receptor encephalitis in an 8-month-old patient with hyperkinetic movement disorder.

    abstract::This article describes an 8-month-old boy with the full clinical spectrum anti-N-methyl-d-aspartate receptor encephalitis. He was admitted to the hospital with involuntary orofacial head movements, behavioral changes, and fluctuation in consciousness. His examination showed tongue thrusting, decreased responsiveness, ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2012.12.032

    authors: Cantarín-Extremera V,Duat-Rodríguez A,González-Gutiérrez-Solana L,López-Marín L,Armangue T

    更新日期:2013-05-01 00:00:00

  • Electroencephalogram monitoring in critically ill children: indications and strategies.

    abstract::Continuous electroencephalographic monitoring often detects nonconvulsive seizures in critically ill children, but it is resource-intense and has not been demonstrated to improve outcomes. As institutions develop clinical pathways for monitoring, they should consider how seemingly minor variations may exert substantia...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2011.12.009

    authors: Gutierrez-Colina AM,Topjian AA,Dlugos DJ,Abend NS

    更新日期:2012-03-01 00:00:00

  • A Cross-sectional Survey of Growth and Nutritional Status in Children With Cerebral Palsy in West China.

    abstract:BACKGROUND:We describe the growth and nutritional status of children with cerebral palsy (2 to 18 years old) in West China and to explore the correlation between the nutritional status and age, gender, and gross and fine motor function. METHODS:We performed a cross-sectional survey of children registered as having cer...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2016.01.002

    authors: Wang F,Cai Q,Shi W,Jiang H,Li N,Ma D,Wang Q,Luo R,Mu D

    更新日期:2016-05-01 00:00:00

  • Anxiety and depressive symptoms in children presenting with a first seizure.

    abstract::We investigated whether children presenting with a first seizure experienced anxiety and depressive signs. Children (aged 7-17 years) with a first unprovoked afebrile seizure participated. These patients (mean age, 12 +/- 2.7 years S.D.; 14 female/8 male) completed the Revised Child Manifest Anxiety Scale (n = 22) and...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2008.07.005

    authors: Loney JC,Wirrell EC,Sherman EM,Hamiwka LD

    更新日期:2008-10-01 00:00:00

  • Calf muscle volume estimates: implications for botulinum toxin treatment?

    abstract::An optimal botulinum toxin dose may be related to the volume of the targeted muscle. We investigated the suitability of using ultrasound and anthropometry to estimate gastrocnemius and soleus muscle volume. Gastrocnemius and soleus muscle thickness was measured in 11 cadaveric human legs, using ultrasound. Lower leg l...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2007.05.019

    authors: Bandholm T,Sonne-Holm S,Thomsen C,Bencke J,Pedersen SA,Jensen BR

    更新日期:2007-10-01 00:00:00

  • Isolated sympathetic failure with autoimmune autonomic ganglionopathy.

    abstract::A 16-year-old boy had a gradual onset of post-exercise myalgia with progressive fatigue and dizziness. He had bradycardia (37 beats/minute) with low supine and normal standing norepinephrine levels (56 and 311 pg/mL, respectively). He had absent sympathetically mediated vasoconstrictor responses during Valsalva maneuv...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2010.05.003

    authors: Fischer PR,Sandroni P,Pittock SJ,Porter CB,Lehwald LM,Raj SR

    更新日期:2010-10-01 00:00:00

  • Life expectancy of children with cerebral palsy.

    abstract::Risk factors for mortality of young children with cerebral palsy were studied using a sample of 12,709 children aged 0.5-3.5 years with cerebral palsy who had received services from the State of California between 1980 and 1995. The most powerful prognostic factors for survival were simple functional items: mobility a...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(97)00172-0

    authors: Strauss DJ,Shavelle RM,Anderson TW

    更新日期:1998-02-01 00:00:00

  • Methylphenidate effects on global and complex measures of EEG.

    abstract::Methylphenidate (MPH) effects on global and complex measures of electroencephalography were examined in boys with attention-deficit-hyperactivity disorder between the ages of 9 and 11 years. Electroencephalogram (EEG) data were collected separately from the administration of a continuous performance task and were eval...

    journal_title:Pediatric neurology

    pub_type: 临床试验,杂志文章

    doi:10.1016/s0887-8994(99)00052-1

    authors: Lubar JF,White JN Jr,Swartwood MO,Swartwood JN

    更新日期:1999-09-01 00:00:00

  • Obstetrical brachial plexus palsy.

    abstract::Obstetrical brachial plexus palsy, one of the most complex peripheral nerve injuries, presents as an injury during the neonatal period. The majority of the children recover with either no deficit or a minor functional deficit, but it is almost certain that some will not regain adequate limb function. These few cases m...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pediatrneurol.2007.09.013

    authors: Zafeiriou DI,Psychogiou K

    更新日期:2008-04-01 00:00:00

  • ACTH therapy in infantile spasms: relationship between dose of ACTH and initial effect or long-term prognosis.

    abstract::The relationship between the dose of ACTH and the initial effect was investigated in 41 children with infantile spasms. More than 0.015 mg (0.6 IU)/kg/day of ACTH was needed for a good initial response of seizures and electroencephalographic abnormalities. The relationship between the dose of ACTH and long-term progno...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(90)90114-g

    authors: Ito M,Okuno T,Fujii T,Mutoh K,Oguro K,Shiraishi H,Shirasaka Y,Mikawa H

    更新日期:1990-07-01 00:00:00

  • Spontaneous intracerebral hematomas in juvenile diabetic ketoacidosis.

    abstract::Ketoacidosis is one of the common complications of Type I insulin-dependent diabetes mellitus. Several neurologic (cerebral) deficiencies have been associated with diabetic ketoacidosis, including cerebral edema with increased intracranial pressure resulting in coma; partial and generalized seizures; and cerebrovascul...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(86)90011-1

    authors: Atluru VL

    更新日期:1986-05-01 00:00:00

  • Multiplex ligation-dependent probe amplification to subtelomeric rearrangements in idiopathic intellectual disability in Colombia.

    abstract:BACKGROUND:A cause cannot be determined in 30% to 50% of patients with intellectual disability. Determining the etiology of intellectual disability is important and useful for pediatric neurologists, geneticists, pediatricians, and patients' families because it allows assessment of recurrence risk, appropriate genetic ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2013.10.017

    authors: Medina A,Piñeros L,Arteaga C,Velasco H,Izquierdo A,Giraldo A,Espinosa E

    更新日期:2014-03-01 00:00:00