Intranasal midazolam as a treatment of autonomic crisis in patients with familial dysautonomia.

abstract：:We report a patient with BH(4)-sensitive phenylketonuria. In neonatal screening, phenylalanine levels above 10 mg/dl were detected. In the tetrahydrobiopterin- (BH(4)) loading test, phenylalanine concentrations in serum fell significantly. Dihydropteridine reductase activity in blood, pterines, and neurotransmitters i...

journal_title：Pediatric neurology

authors： Lücke T,Illsinger S,Aulehla-Scholz C,Sander J,Das AM

更新日期：2003-03-01 00:00:00

abstract：:Patients with autosomal dominant Segawa disease (dopa-responsive dystonia) demonstrate excellent, sustained response to low-dose levodopa. In contrast, the development of levodopa limiting treatment dyskinesias is thought to support the diagnosis of other early-onset dystonia/parkinsonism syndromes. We describe an aty...

journal_title：Pediatric neurology

authors： López-Laso E,Beyer K,Opladen T,Artuch R,Saunders-Pullman R

更新日期：2012-06-01 00:00:00

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

authors： White AL,Hedlund GL,Bale JF Jr

更新日期：2014-03-01 00:00:00

abstract：OBJECTIVE:A single-center, single-blind, parallel-group, randomized clinical trial was performed to test the null hypothesis that adrenocorticotropic hormone is not superior to high-dose prednisolone for treatment of newly diagnosed West syndrome. METHODS:Newly diagnosed infants with West syndrome were randomized to r...

journal_title：Pediatric neurology

authors： Wanigasinghe J,Arambepola C,Sri Ranganathan S,Sumanasena S,Attanapola G

更新日期：2015-09-01 00:00:00

abstract：:Planning strategies to encourage students to pursue a career in pediatric neurology requires assessment of their attitudes and career choices. A structured 30-item questionnaire was designed to examine students' attitudes toward pediatric neurology. In the study, 161 final-year medical students (1999-2000), 20-26 year...

journal_title：Pediatric neurology

authors： Jan MM,Fida NM

更新日期：2002-08-01 00:00:00

abstract：:Musicogenic epilepsy is a relatively rare form of epilepsy. In its pure form, it is characterized by epileptic seizures that are provoked exclusively by listening to music. The usual type of seizure is partial complex or generalized tonic-clonic. Precipitating factors are quite specific, such as listening to only one ...

journal_title：Pediatric neurology

authors： Lin KL,Wang HS,Kao PF

更新日期：2003-05-01 00:00:00

abstract：:We report on a 16-year-old girl with muscular atrophy of a distal upper extremity (Hirayama disease). The disease progressed insidiously, and during our first examination, she exhibited weakness and wasting in the right hand, accompanied by cold paresis. No sensory disturbance was evident. A nerve conduction test reve...

journal_title：Pediatric neurology

authors： Hosokawa T,Fujieda M,Wakiguchi H,Oosaki Y

更新日期：2010-08-01 00:00:00

abstract：:Cloxazolam has been used mainly as an anxiolytic agent. The present study was designed to evaluate the effectiveness of cloxazolam as an add-on antiepileptic drug in patients with intractable epilepsy. A total of 32 patients with intractable epilepsy were treated with cloxazolam: 13 with generalized epilepsy, 15 with ...

journal_title：Pediatric neurology

authors： Kimura N,Fujii T,Miyajima T,Kumada T,Mikuni T,Ito M

更新日期：2010-12-01 00:00:00

abstract：:The distribution of leukomalacia and glial fibrillary acidic protein (GFAP)-positive glial cells in prenatal- and postnatal-onset leukomalacia were compared and diagnosed histologically in 128 autopsied infants and the different pathogeneses were examined. Prenatal-onset leukomalacia was diagnosed in 12 of 71 still-bi...

journal_title：Pediatric neurology

authors： Iida K,Takashima S,Takeuchi Y,Ohno T,Ueda K

更新日期：1993-01-01 00:00:00

abstract：:Central neurogenic hyperventilation refers to progressive tachypnea leading to hypocarbia and respiratory alkalosis caused by cortical disorders, initially reported in comatose patients with mainly pontine infarction. Central neurogenic hyperventilation in conscious patients is even rarer, numbering around 30 reported...

journal_title：Pediatric neurology

authors： Shahar E,Postovsky S,Bennett O

更新日期：2004-04-01 00:00:00

abstract：BACKGROUND:Neurogenic pulmonary edema may be a complication of multiple neurological processes. Although there is debate regarding the underlying pathophysiology, the recognition of neurogenic pulmonary edema is vitally important because of the high-potential for mortality and need for treatment of the underlying disor...

journal_title：Pediatric neurology

authors： Sweeney M,Rubin J,Hopkins SE

更新日期：2014-09-01 00:00:00

abstract：:Leigh syndrome, caused by dysfunction in mitochondrial energy metabolism, is an inherited, heterogeneous, and progressive neurodegenerative disorder of infancy and childhood. From 1983 to August 2006, 14 cases diagnosed with Leigh syndrome were studied in terms of characteristic neuroimaging findings and abnormal mito...

journal_title：Pediatric neurology

authors： Lee HF,Tsai CR,Chi CS,Lee HJ,Chen CC

更新日期：2009-02-01 00:00:00

abstract：:Neonatal group B streptococcus meningitis causes neurologic morbidity and mortality. Cerebrovascular involvement is a common, poorly studied, and potentially modifiable pathologic process. We hypothesized that imaging patterns of focal brain infarction are recognizable in neonatal group B streptococcal meningitis. A c...

journal_title：Pediatric neurology

authors： Hernández MI,Sandoval CC,Tapia JL,Mesa T,Escobar R,Huete I,Wei XC,Kirton A

更新日期：2011-04-01 00:00:00

abstract：:Transcranial magnetic stimulation and motor performance tests were used to study the correlation between corticospinal maturation and actual motor performance in a group of young school children (n = 10, mean age = 7 years, age range = 6-9 years). The results were compared with normal adults (n = 10, mean age = 24 yea...

journal_title：Pediatric neurology

authors： Heinen F,Fietzek UM,Berweck S,Hufschmidt A,Deuschl G,Korinthenberg R

更新日期：1998-09-01 00:00:00

abstract：:The authors investigated by immunohistochemistry the distribution of protective protein in human tissues. Immunoreactivity was observed in the cytoplasm, revealing a granular pattern and cell type specificity. The most intense staining was observed in the large neurons of brain, distal and collecting tubular cells of ...

journal_title：Pediatric neurology

authors： Sohma O,Mizuguchi M,Takashima S,Satake A,Itoh K,Sakuraba H,Suzuki Y,Oyanagi K

更新日期：1999-03-01 00:00:00

abstract：:Brain plasticity refers to its ability to recover after damage. Visual field plasticity is not well recognized. We report a 12-year-old female who first presented with recurrent seizures and was subsequently found to have a large, right occipital cortical dysplasia on magnetic resonance imaging. Her visual field by Go...

journal_title：Pediatric neurology

authors： Kong CK,Wong LY,Yuen MK

更新日期：2000-09-01 00:00:00

abstract：:To evaluate the efficacy of piracetam therapy, 76 children with breath-holding spells admitted to the Outpatient Clinic of Dicle University Medical Faculty Paediatrics Department and Bakirköy State Hospital, Paediatrics Department between 1988 and 1990 and 1991 and 1996, respectively, were included in this placebo-con...

journal_title：Pediatric neurology

authors： Donma MM

更新日期：1998-01-01 00:00:00

abstract：:Assumption of the vertical position and independent walking are potentially hazardous motor milestones in the developing infant. It has been presumed that the parachute reactions evolved to protect infants from injury during this developmental stage. To determine the relationship between the appearance of the upper an...

journal_title：Pediatric neurology

authors： Jaffe M,Kugelman A,Tirosh E,Cohen A,Tal Y

更新日期：1994-07-01 00:00:00

abstract：:Amiel-Tison neurologic assessment at term has recently been updated for clinical application. Experience in this field, in addition to a better understanding of pathophysiologic characteristics of the immature brain, has taught us that an increased precision in assessing central nervous system function in the neonate ...

journal_title：Pediatric neurology

authors： Amiel-Tison C

更新日期：2002-09-01 00:00:00

abstract：BACKGROUND:Neonatal cerebral infarction is a relatively common cause of neonatal seizures, with an incidence of at least 1:4000 live births and is associated with a high incidence of neurological sequelae. However, the pathophysiological mechanisms and predisposing factors responsible for neonatal infarction are not fu...

journal_title：Pediatric neurology

authors： Kanda K,Sato A,Abe D,Nishijima S,Ishigami T

更新日期：2016-09-01 00:00:00

abstract：:Visual evoked potentials (VEPs) were recorded in 32 children (ages 4 months to 5 years) who were clinically diagnosed as being cortically blind. None of the children had visual or neurologic abnormalities prior to the precipitating insult which included surgery (N = 15), trauma (N = 3), infectious disease (N = 5), hyp...

journal_title：Pediatric neurology

authors： Taylor MJ,McCulloch DL

更新日期：1991-03-01 00:00:00

abstract：:A patient with X-linked adrenoleukodystrophy exhibited a phenotype of neurofibromatosis 1. He had large and multiple café-au-lait spots, and had elevated serum levels of very long chain fatty acids. The patient's mother and elder sister also had X-linked adrenoleukodystrophy. This case represents novel manifestations ...

journal_title：Pediatric neurology

authors： Yamada H,Izumi T

更新日期：2009-09-01 00:00:00

abstract：:It was the purpose of this study to compare the efficacy and side effects of lamotrigine (LTG) and vigabatrin (VGB) as add-on therapy in epilepsies of childhood resistant to conventional drugs. Retrospective analysis of the medical charts and electroencephalograms of 134 children (LTG 57, VGB 77) was performed conside...

journal_title：Pediatric neurology

authors： Dimova PS,Korinthenberg R

更新日期：1999-11-01 00:00:00

abstract：BACKGROUND:Clinically mild encephalopathy with a reversible splenial lesion (MERS) is the second commonest cause of encephalopathy. Several pathogens have been detected in patients with MERS type 2, such as influenza A and B, but little is known about the proportion of cases of MERS type 2 with this pathogenesis. Human...

journal_title：Pediatric neurology

authors： Sano F,Fukao T,Tamaru K,Kanemura H,Inukai T,Aihara M

更新日期：2020-12-01 00:00:00

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

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

更新日期：1999-09-01 00:00:00

abstract：:The object of this study was to investigate the potential association of infections, especially group A hemolytic streptococcal infection, with the abrupt onset/exacerbation of tics or obsessive-compulsive behaviors. A structured clinical interview was used to evaluate 80 consecutive children, 5-17 years of age, with ...

journal_title：Pediatric neurology

authors： Singer HS,Giuliano JD,Zimmerman AM,Walkup JT

更新日期：2000-05-01 00:00:00

abstract：:Pregabalin is a new antiepileptic drug that acts at presynaptic calcium channels, modulating neurotransmitter release. We report on treating consecutive children with severe drug-resistant epilepsy in a prospective, open-label, add-on trial. Nineteen children (63% male) aged 4-15 years (mean, 9.7; S.D., 2.9) were incl...

journal_title：Pediatric neurology

authors： Jan MM,Zuberi SA,Alsaihati BA

更新日期：2009-05-01 00:00:00

abstract：:Mitochondrial oxidation of a variety of substrates produces the bulk of energy requirements for most cell types. Impairment of oxidative metabolism may result in a broad spectrum of clinical signs and symptoms. A disorder of oxidative metabolism should be suspected when an unexplained association of signs and symptoms...

journal_title：Pediatric neurology

authors： Breningstall GN

更新日期：1993-03-01 00:00:00

abstract：:Alexia without agraphia is a rare disconnection syndrome characterized by the loss of reading ability with retention of writing and verbal comprehension. We report a patient who developed alexia without agraphia after undergoing a biopsy for a malignant glioma involving the left thalamus. A 15-year-old right-handed ma...

journal_title：Pediatric neurology

authors： Tamhankar MA,Coslett HB,Fisher MJ,Sutton LN,Liu GT

更新日期：2004-02-01 00:00:00

abstract：:Oxcarbazepine, a carbamazepine analog, was approved for use as an antiepileptic agent in the United States in 2000. A search of the United States Food and Drug Administration's Adverse Event Reporting System identified nine cases of oxcarbazepine-associated angioedema in pediatric patients aged 16 years and younger. W...

journal_title：Pediatric neurology

authors： Knudsen JF,Flowers CM,Kortepeter C,Awaad Y

更新日期：2007-08-01 00:00:00