Abstract:
:While prognostic information for Dandy-Walker syndrome and non-progressive cerebellar ataxia/cerebellar hypoplasia is available, surprisingly scant literature reports are found for space-occupying posterior fossa arachnoid cysts (PFAC). We describe the outcome of patients with symptomatic PFAC shunted as infants. Only 11 children were seen over a 20-year period (1980-1999). Cyst location was retrocerebellar (n = 8), supravermian (n = 2) and in the cerebellopontine angle (n = 1). Motor and cognitive impairments in one child were interpreted as the result of neurosurgical complications (intraventricular and intracerebral haemorrhage). One 6-year-old boy had mild truncal ataxia and mild cognitive delay. The 9 other patients had no evidence of ataxia or clumsiness and had average, some even above average, cognitive ability and scholastic achievements. Epilepsy was not a feature. We conclude that the prognosis of PFAC for cognitive development and neurological signs is favourable.
journal_name
Neuropediatricsjournal_title
Neuropediatricsauthors
Boltshauser E,Martin F,Altermatt Sdoi
10.1055/s-2002-33674subject
Has Abstractpub_date
2002-06-01 00:00:00pages
118-21issue
3eissn
0174-304Xissn
1439-1899journal_volume
33pub_type
杂志文章相关文献
NEUROPEDIATRICS文献大全abstract::The content of free amino acids in the cerebrospinal fluid from 52 children in different age groups with febrile seizures were determined and compared to 88 age matched children without seizures. We found that the concentrations of some amino acids in CSF in the control group decreased slowly with age, reaching the co...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071277
更新日期:1989-08-01 00:00:00
abstract::Primary degeneration of the granular layer of the cerebellum is an autosomal recessive disorder exhibiting characteristic clinical features: hypotonia, strabismus, delayed motor development, nonprogressive ataxia, delayed language development with dysarthria and mental retardation. We studied fourteen children, seven ...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1073020
更新日期:1994-08-01 00:00:00
abstract::Acute inflammatory processes of the brain tissue and meninges caused by viruses are relatively common and may be caused by a number of different viral agents. The specific etiological agent is not identified in many instances. Most cases completely recover. The prognosis depends upon the severity of the clinical illne...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2006-924164
更新日期:2006-06-01 00:00:00
abstract::We report the management of refractory status epilepticus (SE) by using continuous intravenous infusions of lidocaine in a previously healthy 15-year-old girl with a "catastrophic encephalopathy" in whom a diagnosis of febrile infection-related epilepsy syndrome was supposed. One week after a banal pharyngitis and fev...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0034-1389898
更新日期:2015-02-01 00:00:00
abstract::Syringomyelia is an etiologically diverse affliction caused by disturbance of normal cerebrospinal fluid flow dynamics. An evaluation by comprehensive imaging, using advanced three-dimensional constructive interference in steady state and four-dimensional phase contrast imaging techniques, should be focused on finding...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1361921
更新日期:2014-02-01 00:00:00
abstract::Thiamine metabolism dysfunction syndrome type 2 is also known by other terms including: "SCL19A3 gene defect," "biotin-responsive basal ganglia disease" (BBGD), and "biotin-thiamine-responsive basal ganglia disease" (BTBGD). The worldwide incidence and prevalence of this disorder are unknown, but the syndrome has prim...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0037-1607191
更新日期:2018-04-01 00:00:00
abstract::Neurological examination and magnetic resonance imaging were performed in the neonatal period in 58 full-term infants who presented with hypoxic-ischaemic encephalopathy. The aim of this study was to evaluate the patterns of neurological abnormalities and their correlation to brain lesions on MRI. The prognostic value...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973465
更新日期:1999-04-01 00:00:00
abstract::Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARe) was originally described as a paraneoplastic disease with more than 50% cases involving a tumor. However, tumor incidence in anti-NMDARe in children is much lower. Herpes simplex virus-induced anti-NMDARe has been well-described; however, findings on Japane...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0038-1675607
更新日期:2019-04-01 00:00:00
abstract::The occurrence of mitochondrial mutations with primary pathogenic significance for Leber's hereditary optic neuropathy in patients with a multiple sclerosis-like phenotype and the preferential maternal transmission points to an involvement of the mitochondrial genome in conferring increased susceptibility to MS. To ev...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973583
更新日期:1998-12-01 00:00:00
abstract:: ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0038-1675237
更新日期:2019-02-01 00:00:00
abstract::Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of age, were followed up for 3 years both clinically and by using brain ma...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973503
更新日期:1999-10-01 00:00:00
abstract::We report a newborn with exaggerated startle reactions and stiffness of neonatal onset, the prototypical signs of hyperekplexia. Startle and flexor spasms, leading to apnoea, did not respond to treatment with clonazepam but did partially to sodium valproate. Molecular analysis of GLRA1 revealed no mutations. The incid...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2005-872877
更新日期:2005-12-01 00:00:00
abstract::Two children with acute transverse myelopathy following adenovirus and Borrelia Burgdorferi infections are presented. The diagnosis stems from the clinical presentation, the determination of specific antibodies in serum and the favorable response to penicillin treatment in the case of neuroborreliosis. Both children m...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071427
更新日期:1991-05-01 00:00:00
abstract::Functional MRI techniques were used to map the position of visual cortex in an awake and a sedated child with congenital anomalies of the posterior hemispheres. In one subject with cortical heterotopia, an activated cortex was found distinct from the structurally abnormal area detected on conventional MRI. In a sedate...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2000-15291
更新日期:2000-02-01 00:00:00
abstract::Computed tomography (CT) of the brain has been used to evaluate potential etiologies of hemiplegic cerebral palsy in a population-based group of 28 children born preterm, in whom an obvious postnatal cause of hemiplegia could be excluded. The CT findings were classified according to a system so constructed to reflect ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071417
更新日期:1991-02-01 00:00:00
abstract::The aims of the present study were: to examine the quality of life (QOL) of parents of children with cerebral palsy (CP) and to establish the possible effect of behaviour problems on their QOL. One-hundred children with CP, aged between 4 and 10 years, and both their parents were included in the study. Both parents co...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0030-1262841
更新日期:2010-06-01 00:00:00
abstract::Four cases of hypomelanosis of Ito with typical skin depigmentation and various noncutaneous findings were described. This neurocutaneous syndrome has been well documented by clinical investigations, especially computerized tomography and magnetic resonance imaging of the brain. The literature is surveyed giving speci...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071300
更新日期:1989-11-01 00:00:00
abstract::B cell depletion with the anti-CD20-antibody rituximab is widely considered treatment of choice for long-term immunotherapy in aquaporin-4 (AQP4)-antibody positive neuromyelitis optica spectrum disorder (NMOSD). However, up to 30% of patients suffer from relapses despite complete B cell depletion. In these cases, the ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0039-1684004
更新日期:2019-06-01 00:00:00
abstract::We investigated two brothers with Scheie syndrome whose only complaint was exercise intolerance. In the quadriceps muscle biopsy of both patients, between the normal muscle fibres an increased number of markedly swollen periodic acid-Schiff-positive fibroblasts were seen. Ultrastructurally, these cells showed an accum...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2001-13873
更新日期:2001-04-01 00:00:00
abstract::A forme fruste Rett variant female with partially preserved speech remnants is described. She was first seen by the author at an age of 4 years. She then presented with an unspecific syndrome of moderate mental retardation. At follow-up when aged 32 she had successively through the years developed a number of Rett cha...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-979730
更新日期:1995-04-01 00:00:00
abstract:AIM: Attention deficit with or without hyperactivity (AD[H]D) is a common comorbidity of neurofibromatosis type 1 (NF 1). We tested the hypothesis that permanent medication with methylphenidate can improve cognitive functioning in children with NF 1 and comorbid AD(H)D. PATIENTS AND METHOD: We retrospectively analyzed...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0034-1368117
更新日期:2014-08-01 00:00:00
abstract::Alpers-Huttenlocher syndrome (AHS) is a very rare autosomal recessive disorder. AHS is caused by homozygous or compound heterozygous mutations in the nuclear gene encoding mitochondrial DNA polymerase gamma (POLG, chromosome 15q25). Most patients become symptomatic before the age of 2 years. We report 3 patients who w...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0031-1287812
更新日期:2011-10-01 00:00:00
abstract:PURPOSE OF THE STUDY:To collect data on both the natural history of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in children and their response to treatment. STUDY DESIGN:Retrospective multicentre study, using standardised criteria for the evaluation of hospital records and semi-quantitative scorin...
journal_title:Neuropediatrics
pub_type: 杂志文章,多中心研究
doi:10.1055/s-2007-973489
更新日期:1999-08-01 00:00:00
abstract::The definition and the clinical picture of arthrogryposis multiplex congenita (AMC) are discussed and the associated anomalies, deformities and syndromes are listed. It is stressed that isolated congenital contractures in (progressive) neuromuscular disorders may be manifestations of AMC, dependent on the moment of th...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1059546
更新日期:1983-02-01 00:00:00
abstract::Positron Emission Tomography (PET) with 2-deoxy-2 [18F]-fluoro-D-glucose provides a measure of functional brain activity, particularly in the dendritic field. In CLN3 (juvenile neuronal ceroid lipofuscinosis or juvenile Batten disease, with fingerprint inclusions) hypometabolism slowly spreads from calcarine to anteri...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973675
更新日期:1997-02-01 00:00:00
abstract:PURPOSE:The term clinical functional magnetic resonance imaging (fMRI) describes an examination with direct clinical impact on the patient. Interpretation of clinical fMRI especially in children, however, is often difficult due to suboptimal data quality. The current gold standard is standardized visual evaluation. To ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0032-1324731
更新日期:2012-10-01 00:00:00
abstract::Forty-four patients aged from one month to 16 years suffering from arterial stroke were carefully studied for any hereditary and acquired risk factors for stroke. No physiologic anticoagulant deficiency or antiphospholipid syndrome was found. Two patients had mitochondrial disease (MELAS). Six patients had migraineous...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1073026
更新日期:1994-10-01 00:00:00
abstract::Congenital horizontal gaze paralysis is a rare disorder which may be associated with severe scoliosis of early onset. We present the clinical findings of two sisters with this syndrome. The additional finding of a unique pericentric inversion of chromosome 12 is considered to be incidental. Every child with congenital...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973565
更新日期:1998-08-01 00:00:00
abstract::We describe a rare case of diffuse leptomeningeal oligodendrogliomatosis associated with the human herpes virus 6 variant A (HHV-6 A). A 2-year-old boy presented with progressive neurological symptoms and hydrocephalus. The patient had a VP shunt placement but did not fully recover. HHV-6 A was detected in both CSF an...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2002-36739
更新日期:2002-10-01 00:00:00
abstract:PURPOSE:Ketogenic diets (KDs) are used to treat epilepsies resistant to pharmacotherapy or some inborn errors of metabolism. For prolonged anesthesia, use of balanced electrolyte solutions (BESs) supplemented with 0.5% glucose has been advocated to maintain ketosis while preventing hypoglycemia. Unfortunately, there is...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0040-1715634
更新日期:2020-12-01 00:00:00