Journal of Pediatric Neurology back issues from January 2005:
Febrile seizures: the role of intermittent prophylaxis
Jan 01, 2005; ... Febrile seizure (FS) is the most common seizure type of the childhood and known as a benign condition which usually does not require any acute intervention or long-term therapy [1,2]. "No treatment" approach for febrile seizures was universally accepted and recommended between the periods 1930 ...
Sudden infant death syndrome. Review of infant and environmental stress factors on survival
Jan 01, 2005; ... Abstract. Preventable risk factors for sudden infant death syndrome include the infant's environment and response characteristics. Various infant and environmental factors thus modify the vital cardiocirculatory, respiratory and arousal controls in healthy infants. Similar changes in ...
Use of zonisamide in twelve patients with intractable seizures refractory to common anti-epileptic regimens
Jan 01, 2005; ... Abstract. This non-randomized, uncontrolled, single-center study was conducted to evaluate the effects of zonisamide on intractable epilepsy in a primarily pediatric population. Twelve patients ranging in age from 18 months to 23 years were included in the study. All patients were ...
Nocturnal enuresis: a review
Jan 01, 2005; ... Abstract. Enuresis is a common problem affecting a large child population. Though not psychogenic in nature, it is often associated with psychosocial problems. Structural abnormalities as a cause are rare. Common causative factors include developmental delay, genetic factors, ...
Intermittent clobazam in febrile seizures: an Indian experience
Jan 01, 2005; ... Abstract. The aim of this study is to find the efficacy and safety of intermittent clobazam in febrile seizures (FS). The study was a double blind placebo control trial. Sixty patients of age group 6 months to five years presenting with febrile seizures were finally randomized to ...
An open clinical trial of risperidone in children with seizures and hyperactivity
Jan 01, 2005; ... Abstract. Children with seizures may also have behavioral problems including hyperactivity. The management of hyperactivity in these cases is complicated by the fact that pharmacotherapeutic interventions may worsen the seizures. In this study 10 children with epilepsy and attention ...
Quetiapine in children and adolescents with subaverage IQ
Jan 01, 2005; ... Abstract. A retrospective study was conducted to assess the effectiveness and tolerability of quetiapine in children and adolescents with subaverage replaced with IQ. Through a chart review conducted in a specialized outpatient clinic, five patients were identified (three males, two ...
A particular type of epileptic seizures in infancy with cerebellar ganglioneurocytoma
Jan 01, 2005; ... Abstract. A 4-month-old female infant presented with paroxysmal clonic or tonic hemifacial contractions. Afterward, she sometimes had clonic hemibody contractions. Nystagmoid eyeball movements were also occasionally seen. These evolved to stereotypic clinical patterns and frequencies, ...
Hemicraniectomy to reduce raised intracranial pressure in a child with acute disseminated encephalomyelitis
Jan 01, 2005; ... Abstract. We report the case of a 14-year-old boy with acute disseminated encephalomyelitis who, rapidly deteriorated while on intravenous methylprednisolone. Emergency hemicraniectomy had to be done to control the raised intracranial pressure when the conventional methods failed to ...
Moyamoya in an infant with Down syndrome
Jan 01, 2005; ... Abstract. In moyamoya disease, the anterior blood circulation in the brain is occluded and the characteristic appearance of the disease is seen in angiography. Moyamoya disease may be primary or secondary to diseases or syndromes such as Down syndrome. A 20-month-old girl with Down ...
Pseudoseizures simulating status epilepticus: two cases of over-treatment in the emergency department and pediatric intensive care unit
Jan 01, 2005; ... Abstract. Pseudoseizures (PS) resemble epileptic seizures. They are strictly linked to personality disorders and may involve all ages, with a higher incidence during adolescence. Patients experience episodes of loss of consciousness, twitching or jerking, and unusual emotional states, ...
Familial isolated congenital nystagmus in a Turkish infant
Jan 01, 2005; ... Nystagmus is an involuntary, rhythmic oscillation of the eyes in which at least one phase is slow. Its congenital form may be associated with blindness, familial and idiopathic. In familial form, the pattern of inheritance may be autosomal recessive, autosomal dominant, or X linked [1,2] ....
Pediatric neurosurgery. High-yield database, (First Edition) edited by Raj Kumar and Vinita Sing
Jan 01, 2005; ... Pediatric neurosurgery. High-yield database, (First Edition) edited by Raj Kumar and Vinita Sing. Published in 2004 by Paras Medical Publisher, Hyperabad, India, ISBN: 81 8191 057 5, 420 pages, IR275 or $20. This yellow with linear lines and purple based book catches your eye immediately ...
In Memoriam: Sudden infant death syndrome research pionneer: André Kahn (1943-2004)
Jan 01, 2005; ... This issue of the Journal of Pediatric Neurology includes the latest paper on which I worked with my friend and colleague André Kahn and his team, the last he will have written. André Kahn was the Professor of Pediatrics at the Free University of Brussels (ULB), Head of the Department of ...
In Memoriam: André Kahn
Jan 01, 2005; ... With shock and deep sadness we received the news that Prof. André Kahn suddenly died on September 1, 2004. Andre has been one of the most eminent researchers in the field of sudden infant death syndrome (SIDS). Starting with a study on the effects of apneas on transcutaneous PO2 measurements in ...
Clinicopathological findings in mitochondrial disorders
Jan 01, 2005; ... Mitochondrial encephalomyopathies have shifted from being regarded just a few years ago as a category of rare metabolic diseases to a current recognition that they are much more common than previously thought, are a large and heterogeneous group of distinct diseases, and that they may occur not ...
Alternating hemiplegia of childhood
Jan 01, 2005; ... Abstract. We reviewed the etiologic aspects, clinical symptoms, complementary studies, differential diagnosis and treatment of alternating hemiplegia of childhood (AHC). AHC is an uncommon illness of uncertain pathophysiology that provokes crisis of transient hemiplegia affecting one ...
A clinicopathological and brain MRI study in Egyptian children with mitochondrial disorders
Jan 01, 2005; ... Abstract. This prospective study was undertaken to focus on the clinicopathological aspects as well as the value of brain magnetic resonance imaging (MRI) in children suffering from mitochondriopathies. Thirty-one patients [15 males (48.4%) and 16 females (51.6%)] gathered from the ...
Screening of mentally handicapped Egyptian children for fragile X syndrome using clinical, cytogenetic and molecular approaches
Jan 01, 2005; ... Abstract. To screen children with idiopathic mental retardation (MR) using a clinical Ten-Item Checklist (TIC) and to analyze high-risk fragile X syndrome (FXS) cases by cytogenetic and molecular genetic techniques. This study was conducted on 192 children with idiopathic MR enrolled ...
Cerebral birth anoxia, seizures and Kluver-Bucy syndrome: some observations
Jan 01, 2005; ... Abstract. Kluver-Bucy syndrome (KBS) includes increased oral exploration, placidity, indiscriminate hypersexuality and altered dietary hahits, reported to develop following insult to temporal lobes. Psychic blindness (inability to recognize familiar objects) and hypermetamorphosis ...
Risk factors for carbamazepine-induced leukopenia in children and adolescents
Jan 01, 2005; ... Abstract. Carbamazepine (CBZ) is a commonly used anticonvulsant agent, but it has been linked with different blood cell abnormalities. This study tried to determine some of the risk factors associated with CBZ-induced leukopenia in pediatric and adolescent age group. This nested-cohort ...
Mitoxantrone as rescue therapy in two children with worsening multiple sclerosis
Jan 01, 2005; ... Abstract. Multiple sclerosis (MS) is an inflammatory, demyelinating disease of the central nervous system of unknown etiology. In the acute phase. MS is treated with corticosteroids, whereas drugs, such as interferon-beta and glatiramer acetate, are currently used as continuous ...
Recurrent familial cerebellar syndrome associated with pyrexia
Jan 01, 2005; ... Abstract. Pyrexia associated recurrent familial cerebellar syndrome is reported. Three members in the family (father and his two sons) had this condition. All these used to get features of cerebellar dysfunction on having fever and the features would disappear when the fever came down ....
Clinical features of mitochondrial disease in a family
Jan 01, 2005; ... Abstract. Mitochondrial disorders, once thought to be relatively rare, are now thought to he the most prevalent metabolic disease. They represent a challenge to clinicians, especially in children, in whom clinical presentation and course show enormous variation. We report a respiratory ...
Rhombencephalitis, neuromyelitis optica and hypothyroidism following Coxsackie virus infection
Jan 01, 2005; ... Abstract. A 7-year-old girl presented with swallowing and respiratory difficulty necessitating artificial ventilation. Her cranial magnetic resonance imaging revealed T2 hyperintensity in the brainstem and cerebrospinal fluid polymerase chain reaction was positive for Coxsackie B4 virus ...
Prolonged QT syndrome triggered by swimming: A mimicking cause of seizures not to be missed
Jan 01, 2005; ... Abstract. Epilepsy can sometimes be mimicked in children by uncommon conditions. We present a 6-year-old boy with congenital deafness and long QT syndrome (Jervell and Lange-Nielsen syndrome) triggered by swimming. He had bradycardia and a history of early seizures and deaths in the ...
Dissemination of intramedullary anaplastic ependymomas
Jan 01, 2005; ... Abstract. Two cases of 6 years old and 20 years of ages were operated for intramedullary anaplastic ependymomas. A gross total microsurgical excision of their tumors was achieved in both the cases, barring the infiltrated nerve roots of one case (6 years old, child). Standard doses of ...
Cysticercosis of temporalis muscle: a case report
Jan 01, 2005; ... Abstract. A 16 years aged female presented with gradually increasing swelling of left temporalis muscle associated with episodic headache. Computerized tomography scan of head revealed a defined enhancing soft tissue mass in left temporal region without an intracranial extension and bone ...
Relevance of the blood-brain barrier on compartmental pharmacokinetics of paracetamol
Jan 01, 2005; ... Abstract. Cerebrospinal fluid and plasma pharmacokinetics of paracetamol in one full term neonate are described. The observed impact of the blood-brain barrier of paracetamol on the compartmental pharmacokinetics and the pharmacodynamic relevance of this observation are ...
Adrenoleukodystrophy: Diffusion MRI findings
Jan 01, 2005; ... An 8-year-old boy with laboratory evidence of adrenal insufficiency, and an established diagnosis of X-linked adrenoleukodystrophy was referred to our institution for a follow-up magnetic resonance imaging (MRI) examination. Besides T1 and T2-weighled sequences, a diffusion MRI protocol was ...
Guillain-Barré syndrome following bee sting
Jan 01, 2005; ... Guillain-Barré syndrome is an acute demyelinating disease of peripheral nerves characterized clinically by progressive weakness [1]. Herein, we report on a child with Guillain-Barré syndrome, which developed following bee sling because of very rare presentation. A previously healthy ...
The Epilepsies - Seizures, Syndromes and Management based on the ILAE classifications and practice parameter guidelines
Jan 01, 2005; ... The Epilepsies - Seizures, Syndromes and Management based on the ILAE classifications and practice parameter guidelines. C. P. Panayiotopoulos, Baldon Medical Publishing, Oxford, 2005. ISBN 1-904218-34-2. Recent events in the United Kingdom have highlighted the necessity of properly ...
The Kleine-Levin syndrome
Jan 01, 2005; ... Why write an editorial on a rare and enigmatic disorder, which is difficult to diagnose, lacks a definite laboratory test and is hardly mentioned in the classical text books i.e., 18 sentences in Bradley's Neurology in Clinical Practice [1], and 15 words including the two stating the name of the ...
The Kleine-Levin syndrome
Jan 01, 2005; ... Abstract. As so often in uncommon syndromes difficulties in diagnosis are a problem, especially when symptoms are strange and suggest a psychiatric disorder. Such an example is the Kleine-Levin syndrome. It occurs mainly during adolescence, and may last for a number of years, but the ...
Somatosensory and MRI findings in children with cerebral palsy: correlations and discrepancies with clinical picture
Jan 01, 2005; ... Abstract. The aim of this study was to determine if there is any association between the findings of somatosensory evoked potentials (SEPs), magnetic resonance imaging (MRI) findings and the severity of motor deficits and cognitive impairments in children with spastic cerebral palsy ...
Phenotype of five patients with dopa-responsive dystonia and mutations in GCH1
Jan 01, 2005; ... Abstract. Autosomal dominant dystonia with diurnal variation, also known as DOPA-responsive dystonia (DRD, Segawa syndrome; MIM#128230), can be caused by mutations in the GTP cyclohydrolase I gene GCH1 on chromosome 14q22.1-q22.2. Reports on patients with thoroughly characterized DRD ...
X-linked West Syndrome and mental retardation
Jan 01, 2005; ... Abstract. X-linked infantile spasms associated with mental retardation have been recognized as a syndrome with an identified gene: ARX. We report a family with eight affected males. Mental retardation was present in all, seven also had epilepsy. In the index case, onset took place in the ...
Cutaneous thoracic hemangioma and internal vascular anomalies
Jan 01, 2005; ... Abstract. We report a male infant with a small thoracic strawberry hemangioma associated with other vascular anomalies: agenesis of the left vertebral artery and hypoplasia of the left carotid artery, and an intestinal hemangioma 45 cm in length removed at 1 1/2 months of age. He also ...
Clinical findings of tuberous sclerosis in Turkish children1
Jan 01, 2005; ... Abstract. We aimed to define clinical characteristics and laboratory findings of tuberous sclerosis in 17 patients. Age at presentation varied from 5 days to 13 years. Male to female ratio was 10/7. Genetic transmission was documented in 23.5% of cases. The most frequent presentation of ...
Compulsive behavior and coprolalia after cerebral malaria
Jan 01, 2005; ... Abstract. Neuro-pyschiatric symptoms such as chorea, tics, and obsessive-compulsive disorders have been documented with group A, beta-hemolytic Streptococcus, Mycoplasma pneumoniae and Herpes simplex infections, presumably through autoimmune damage to basal ganglia. No such association ...
Severe La Crosse meningoencephalitis in children: report of two cases1
Jan 01, 2005; ... Abstract. La Crosse virus is an arbovirus of the California Serogroup (Family Bunyaviridae), transmitted by the "tree-hole mosquito," Aedes triseriatus. La Crosse encephalitis cases occur annually from July to October. The majority of cases are asymptomatic, only a minority (< 4%) ...
Severe myocardial injury in an infant with hemorrhagic shock and encephalopathy syndrome
Jan 01, 2005; ... Abstract. Hemorrhagic shock and encephalopathy syndrome is a severe disease that affects previously healthy infants and associated with significant mortality and neurologic morbidity. It is characterized by sudden onset of shock, convulsion and coma, bleeding due to severe coagulopathy, ...
Hypoglycemia associated with brain damage: a case report
Jan 01, 2005; ... Abstract. We report a case of a 7-month-old infant with serial magnetic resonance imaging (MRI) confirmation of the neonatal hypoglycemia induced brain damage. Cranial MRI on day 17 revealed T1 and T2 prolongation of cortical and white matter in the parietal and occipital lobes ...
ARX and other single genes in X-linked mental retardation: revisiting a population-based study
Jan 01, 2005; ... It has been calculated that monogenic genes for X-linked mental retardation (XLMR) account for <10% of male MR [1]. XLMR can he classified as syndromic, referring to conditions with distinct neurological, physical or biochemical abnormalities, or nonsyndromic (unspecified or nonspecific) ....
Neurological "soft signs" in children and adolescents
Jan 01, 2005; ... 1. Introduction Neurological "soft signs" (NSS) are defined as minor abnormalities in the neurological examination in subjects with no other features of fixed or transient neurologic disorders [1]. These clinical disabilities are often associated with behavior, coordination and learning ...
Jeu de miroirs between medical illustration and art
Jan 01, 2005; ... It is 40 years since Angelman syndrome was first described [1]. Without any reference to this anniversary in medical literature, the starting line of a powerful contemporary poem states that 'Dopey has Angelman's' [2]. This appears to constitute a further avatar in the complex interplay between ...
Childhood multiple sclerosis
Jan 01, 2005; ... Abstract. Childhood multiple sclerosis (MS) is a rare demyelinating autoimmune disease with different risk factors and clinical features than adult onset MS. Onset of MS is extremely uncommon in early childhood, particularly in those less than 10 years of age. The overall prevalence of ...
Cerebral MRI findings in neonatal hypoglycemia
Jan 01, 2005; ... Abstract. Metabolic disturbances such as anoxia and hypoglycemia may adversely alter the development of the neonatal brain. While rapid and appropriate diagnosis with adequate therapy has a good prognosis; delayed detection and/or inappropriate therapy increases the risk of the ...
Prevalence of motor abnormalities among healthy 5-year-old children
Jan 01, 2005; ... Abstract. The prevalence of motor examination abnormalities among a general pediatric population is unknown. We determined the frequency of motor abnormalities noted at five years of age during a neurologic examination by a child neurologist. As part of a follow-up study of neonatal ...
Pediatric neurologists in Saudi Arabia: An audit of current practice
Jan 01, 2005; ... Abstract. Pediatric neurological disorders are common and constitute up to 30% of children seen in general pediatrics. Information about the availability and practice of pediatric neurologists is vital for strategic planning of management of neurological disorders. The aim of this study ...
Psychiatric morbidity in Nigerian adolescents with epilepsy
Jan 01, 2005; ... Abstract. To investigate the rate and type of psychiatric diagnosis in a group of Nigerian adolescents with epilepsy. Adolescents with epilepsy (n = 166) aged 12 to 18 years and healthy controls (n = 170) and their adult caretakers were interviewed with the youth and parent versions of ...
Treatment of infantile spasms, a Venezuelan experience
Jan 01, 2005; ... Abstract. We analyzed the treatment of 25 children with infantile spasm, 52% were males and 48% females. Of the cases studied 80% were symptomatic and 20% cryptogenic. Twenty-three children were treated with synthetic adrenocorticotropic hormone (ACTH), in 15 children the ACTH was ...
Maternal and neonatal MTHFR-C677T mutation: A case report
Jan 01, 2005; ... Abstract. Homozygosis for the thermolabile variant (C677T) of the methylene-tetrahydropholate reductase (MTHFR) gene has been related to a high incidence of neural tube defects, pre-eclampsia, retardation in fetal growth and hyperhomocysteinemia. The latter represents a known risk factor ...
Axonal involvement with white matter abnormalities in merosin-positive congenital muscular dystrophy: A new association
Jan 01, 2005; ... Abstract. An 18-month-old infant who presented with delayed motor development, hypotonia and absent deep tendon reflexes and normal cognitive development was diagnosed to have merosin-positive congenital muscular dystrophy (MP-CMD) on the basis of raised serum levels of creatine kinase, ...
Neuroenteric cyst: A rare cause of childhood hemiparesis
Jan 01, 2005; ... Abstract. Neuroenteric cysts are rare congenital anomalies derived from the displaced endodermal tissue at around third embryonic week. These are commonly encountered in the mediastinum and rarely seen in the spinal canal in the intradural extramedullary and at times in intramedullary ...
Supratentorial metastases and routes of dissemination of medulloblastoma: Report of two cases
Jan 01, 2005; ... Abstract. Two children of 4 and 18 years of ages developed multiple supratentorial metastases at 3 years and 8 months follow up respectively, following their gross total microsurgical excision of medulloblastomas. Both the children received standard doses of craniospinal irradiation ...
Central nervous system manifestations of Mycoplasma pneumoniae: Report of two children
Jan 01, 2005; ... Abstract. Mycoplasma pneumoniae is a common infection in the early adolescent period, presenting most commonly as an upper respiratory tract infection with variable severity. Extrapulmonary manifestations include neurologic, hematologic, hepatic, and cardiac involvement. Hemiparesis and ...
Suprasellar arachnoid cyst with precocious puberty
Jan 01, 2005; ... Abstract. A 9-year-old boy with precocious puberty was presented with severe headache and vomiting, despite having a functioning ventriculoperitoneal shunt, from the age of one year for hydrocephalus. Magnetic resonance imaging revealed a tense suprasellar arachnoid cyst indenting the ...
Epidermoid tumor in the fourth ventricle
Jan 01, 2005; ... The present patient is a 19-year-old girl who presented with headaches, and vertigo. On a magnetic resonance imaging (MRI) examination, a suspicious, small mass-like lesion was evident on T1-and T2weighted images. No hydrocephalus was noted. There was no contrast enhancement. FLAIR (fluid ...