Abstract：
INTRODUCTION:Primary pseudotumor cerebri (PTC) in childhood is a rare but important differential diagnosis in children presenting with papilledema. It is defined as elevated cerebrospinal fluid (CSF) pressure of more than 20 cm H(2)O, normal CSF composition, and exclusion of underlying structural or systemic causes. Visual loss is a serious complication, which requires careful monitoring and management. PATIENTS AND METHODS:We conducted a retrospective chart review of 12 patients with primary PTC. The mean age at presentation was 8212 years, and there was a male-to-female ratio of 7:5. The aim of this study was to investigate the clinical features of primary PTC in children, and to highlight the different treatment options in normalizing intracranial pressure in these patients. RESULTS:In the majority of cases, children presented with headache. Four patients had no obvious symptoms and papilledema was found on routine eye examination. Obesity was uncommon and there was no distinct sex predilection. Acetazolamide was our drug of choice for the initial treatment. Furosemide and prednisone were used as second-line agents. Treatment was gradually decreased after resolution of the papilledema with exception of the two youngest children, who remained symptomatic. One child underwent ventricular-peritoneal shunting. DISCUSSION:The treatment goals of PTC are the relief of symptoms, and preservation of visual function. Acetazolamide is an effective first-line method of lowering raised intracranial pressure. In our study group especially the young children were difficult to treat. This might indicate an age-related difference in the etiology of PTC. When medical treatment remains ineffective and visual function deteriorates, surgical treatment should be considered.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Distelmaier F,Sengler U,Messing-Juenger M,Assmann B,Mayatepek E,Rosenbaum Tdoi
10.1016/j.braindev.2005.07.003keywords:
subject
Has Abstractpub_date
2006-04-01 00:00:00pages
190-5issue
3eissn
0387-7604issn
1872-7131pii
S0387-7604(05)00173-7journal_volume
28pub_type
杂志文章abstract：OBJECTIVES:To evaluate the outcome of ketogenic diets (KDs) in patients with glucose transport type 1 deficiency syndrome (GLUT1DS) in Japan. METHODS:A nationwide survey for GLUT1DS was conducted by sending questionnaires to board-certified pediatric neurologists nationwide to obtain clinical and laboratory data. RES...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2016.01.002
更新日期：2016-08-01 00:00:00
abstract：:To determine the effect of flunarizine therapy on patients with alternating hemiplegia of childhood (AHC), we sent a questionnaire by mail to council members of the Japanese Society of Child Neurology. We collected 28 AHC patients, and studied their clinical courses and the effects of drug therapy. All of the patients...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(01)00229-7
更新日期：2001-08-01 00:00:00
abstract：:A family in which three siblings were affected with severe cerebral malformations in association with ocular anomalies and muscle disease is reported. One sibling was diagnosed as having Fukuyama type congenital muscular dystrophy (FCMD) because he showed severe hypotonia with dystrophic findings on a muscle biopsy in...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(12)80154-9
更新日期：1992-09-01 00:00:00
abstract：BACKGROUND:The effect of rituximab on acute disseminated encephalomyelitis (ADEM) followed by recurrent optic neuritis (ON) is not yet known. PATIENT:We are reporting the case of a 4-year-old Japanese girl who was diagnosed with anti-myelin oligodendrocyte glycoprotein (MOG) antibody positive ADEM followed by recurren...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2018.03.011
更新日期：2018-08-01 00:00:00
abstract：:Sixty patients (age-range one month to 14 years) with other types of epilepsy than infantile spasms were treated with clonazepam. Disappearance of seizures and normalization of abnormal EEG with disappearance of seizures were recognized in 77% and 50%, respectively. Seizures disappeared in 71% of the patients with gen...
journal_title：Brain & development
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：:Continuous and simultaneous registration of electroencephalogram (EEG) and heart rate (HR) pattern in preterm infants can give information about the functioning of central nervous system and the integrity of the autonomic nervous system. The developmental and behavioural state determine the pattern of EEG activity. A ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2005.01.007
更新日期：2005-12-01 00:00:00
abstract：:To investigate the development of the voluntary control of saccadic eye movement, we examined eye movements in 99 normal children (4-13 years of age). Subjects were asked to fixate a central light for 3-5 s. A target was then presented, either to its right or left. In visually guided saccades, the mean latencies of th...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(00)00101-7
更新日期：2000-05-01 00:00:00
abstract：:We describe three patients with the limb pain of complex regional pain syndrome (CRPS) in childhood with autonomic nervous system function involvement. Their autonomic nerve abnormality was non-invasively examined by means of laser doppler flowmetry (LDF) and a sympathetic skin response (SSR) test. In one it was resol...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(00)00174-1
更新日期：2000-10-01 00:00:00
abstract：:We present a unique 11-year-old girl showing clinical features of Rett-related disorder with distinctive facial features and multiple congenital anomalies including ocular hypertelorism, arched eyebrows, a broad nose, dental anomalies, congenital heart disease, truncal obesity, and epilepsy. A novel de novo mutation i...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2017.12.013
更新日期：2018-05-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(95)00168-9
更新日期：1996-05-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2006.09.013
更新日期：2007-06-01 00:00:00
abstract：:Neonatal focal cerebral arterial infarction has been rarely reported in the literature, in contrast to the watershed infarctions, which are common entities among asphyxiated infants. In neonatal postmortem series, thromboembolism was the commonest cause of cerebral arterial occlusion; the source of emboli was associat...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(12)80344-5
更新日期：1992-11-01 00:00:00
abstract：:Neurofibromatosis type 2 (NF2) is an autosomal dominant multiple neoplasia syndrome of the central nervous system. The aim of the present study was to characterize the clinical course of early onset NF2. The specific Japanese disease registry for NF2 in 2010 was analyzed retrospectively. The male:female ratio for the ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2013.01.007
更新日期：2014-02-01 00:00:00
abstract：:The electroencephalographic features and their evolutional changes with age were described in three cases of lissencephaly syndrome diagnosed by CT scan. The case with more severe lissencephaly displayed very similar EEG findings. In early or middle infancy when infantile spasms began, EEG showed very high amplitude (...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(79)80042-x
更新日期：1979-01-01 00:00:00
abstract：:A study of epileptic drop attacks (EDA) by simultaneous video-polygraphic recordings was carried out in one epileptic patient with myoclonic astatic seizures (Doose syndrome). EDA was shown to correspond to a burst of generalized bilaterally synchronous spike and wave complexes (GBSSW) at 3 Hz. Absence seizures were a...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(93)90070-o
更新日期：1993-05-01 00:00:00
abstract：:We report the case of an 11-year-old girl who developed slowly progressive atrophy of the left lower extremity. She suffered from mild dilated cardiomyopathy of unknown cause since 4years of age. When she was 7years old, her family noticed that her left extremity was thinner compared to the right one. Computed tomogra...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2009.11.005
更新日期：2010-04-01 00:00:00
abstract：:Both xeroderma pigmentosum group A (XPA) and Cockayne syndrome (CS) are rare autosomal disorders, have a genetic defect in the step of nucleotide repair, and involve various neurological abnormalities caused by progressive neurodegeneration. We performed comprehensive neuropathological analysis of five cases of XPA an...
journal_title：Brain & development
pub_type： 临床试验,杂志文章
doi：10.1016/s0387-7604(99)00033-9
更新日期：1999-07-01 00:00:00
abstract：:We report the 3rd case in the literature of a 3-year-old boy with D-2-hydroxyglutaric (D-2-HG) aciduria, who presented primarily generalized hypotonia and feeding difficulty during the neonatal period, with eventual development of generalized myoclonic seizures. Gas chromatographic analysis of urinary organic acids sh...
journal_title：Brain & development
pub_type： 杂志文章,评审
doi：10.1016/0387-7604(94)00123-f
更新日期：1995-03-01 00:00:00
abstract：:The common factor causing congenital arthrogryposis is lack of fetal movements. This can result from a large number of disorders. They may be neuropathic, affecting the brain, the spinal cord, or the peripheral nerves; they may be abnormalities of the muscles, such as myasthenia gravis, congenital muscular dystrophies...
journal_title：Brain & development
pub_type： 杂志文章,评审
doi：10.1016/s0387-7604(98)00037-0
更新日期：1998-10-01 00:00:00
abstract：:In order to study the validity of disintegrative psychosis (DP) as defined in ICD-9, we compared the natural history of somatic morbidity of 13 patients given this diagnosis in childhood with a control group of 39 patients with infantile autism (IA) matched for gender, age, IQ and social class. Average follow-up time ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(99)00045-5
更新日期：1999-10-01 00:00:00
abstract：:Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a ra...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2018.02.001
更新日期：2018-06-01 00:00:00
abstract：:We performed neurophysiological studies in 12 patients with the Leigh syndrome (6 pathologically confirmed and 6 clinically diagnosed). The results are compared with data derived from a literature survey of 173 Leigh syndrome patients. We found no positive contribution of neurophysiological studies towards the diagnos...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(86)80004-3
更新日期：1986-01-01 00:00:00
abstract：:Two cases of status epilepticus are reported, whose seizures responded well to the injection of flunitrazepam. One patient had generalized tonic clonic seizures and the other had partial seizures. The improvement of their condition was confirmed by both clinical and electroencephalographic examinations. There were no ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(88)80066-4
更新日期：1988-01-01 00:00:00
abstract：:Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter. Either an antenatal environmental incident or a genetic origin could be responsible...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(00)00173-x
更新日期：2000-12-01 00:00:00
abstract：:We evaluated the cyclic alternating pattern (CAP) during the first year of life in order to obtain information on the maturation of arousal mechanisms during NREM sleep and to provide normative data for CAP parameters in this age range (5-16months). Eleven healthy children (mean age 7.9±3.3months, seven boys) were stu...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2010.07.008
更新日期：2011-05-01 00:00:00
abstract：:We report an autopsy case of bathtub drowning in epilepsy. A 26-year-old female with mental retardation had been treated for refractory epilepsy. Her younger sister found her floating supine in the bathtub 45 min after starting bathing. Neuropathological examination revealed cerebral cortical dysplasia in the precentr...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(97)00064-8
更新日期：1997-11-01 00:00:00
abstract：BACKGROUND:Rett syndrome is a severe genetic neurodevelopmental disorder mainly affecting females. The aim of this study was to describe pubertal development in a population-based cohort of females with Rett syndrome. METHODS:To assess pubertal trajectory we used six waves of data provided by parents of girls and wome...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2012.11.007
更新日期：2013-11-01 00:00:00
abstract：PURPOSE:The study aims to detect the polymorphisms in uridine diphosphate glucuronyl transferase (UGT) 2B7∗2 and investigate the corresponding effects on the blood concentrations of valproic acid (VPA) and carbamazepine (CBZ). METHODS:A chemiluminescence immunoassay analyzer was used to detect the plasma concentration...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2017.09.004
更新日期：2018-02-01 00:00:00
abstract：:Organic acidemias (OAs) have been detected worldwide in symptomatic patients using gas chromatography mass spectrometry. We diagnosed 188 Asian cases of OAs by analysis of urinary organic acids and investigated their clinical onset and outcome. Methylmalonic acidemia (MMA) was most common (74 cases), followed by propi...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2004.04.004
更新日期：2005-01-01 00:00:00
abstract：:A case of precocious puberty due to postmeningitic hydrocephalus was presented. Concentrations of serum gonadotropins and estradiol were found to be elevated, and the response of LH to LHRH was also high, similar to that in the late pubertal stage. CT scanning demonstrated marked dilatation of the third ventricle. It ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(83)80047-3
更新日期：1983-01-01 00:00:00
