bluuebunny/crossref_metadata_2025 huggingface dataset:
Topical Review: Activated Remodeling and N-Methyl-D-Aspartate (NMDA) Receptors in Cortical Dysplasia
Authors: Yamanouchi Hideo
Published: June 2007

Cortical dysplasia is now recognized as one of the major etiologies causing intractable epilepsy in childhood. Dysplastic cortex displays cortical dyslamination, which is often associated with dysmorphic large neurons and less frequently with balloon cells. The dysmorphic large neurons are commonly located in the subcortical white matter and cerebral cortex, with enlarged nuclei with a single prominent nucleolus and showing aberrant cytoskeletal changes. I have shown that dysmorphic large neurons have several immature types of cytoskeletal proteins, such as the low-molecular-weight form of microtubule-associated protein 2 (MAP2) and MAP1B, which are involved in the outgrowth and modeling of neuronal processes in the immature brain. I have also reported that dysmorphic large neurons also have enhanced gene expression of growth-associated protein GAP43, which is a phosphoprotein enriched at presynaptic nerve terminals and is thought to be involved in axonal outgrowth and plasticity in synaptic connections. Finally, I have shown that the N-methyl-D-aspartate acid (NMDA) receptor R1 gene is up-regulated in the dysmorphic large neurons and nearly normal-sized neurons located in the dysplastic cortex. This evidence suggests that growth of neuronal processes and activated excitatory synaptic remodeling exist in the epileptic conditions of cortical dysplasia. ( J Child Neurol 2005;20:303—307).
Dynamic Statistical Parametric Mapping for Analyzing the Magnetoencephalographic Epileptiform Activity in Patients With Epilepsy
Authors: Shiraishi Hideaki , Stufflebeam Steven M. , Knake Susanne , Ahlfors Seppo P. , Sudo Akira , Asahina Naoko , Egawa Kiyoshi , Hatanaka Keisaku , Kohsaka Shinobu , Saitoh Shinji , Grant P. Ellen , Dale Anders M. , Halgren Eric
Published: June 2007

Our current purpose is to evaluate the applicability of dynamic statistical parametric mapping, a novel method for localizing epileptiform activity recorded with magnetoencephalography in patients with epilepsy. We report four pediatric patients with focal epilepsies. Magnetoencephalographic data were collected with a 306-channel whole-head helmet-shaped sensor array. We calculated equivalent current dipoles and dynamic statistical parametric mapping movies of the interictal epileptiform discharges that were based in the minimum-L2 norm estimate, minimizing the square sum of the dipole element amplitudes. The dynamic statistical parametric mapping analysis of interictal epileptiform discharges can demonstrate the rapid change and propagation of interical epileptiform discharges. According to these findings, specific epileptogenic lesion—focal cortical dysplasia could be found and patients could be operated on successfully. The presurgical analysis of interictal epileptiform discharges using dynamic statistical parametric mapping seems to be promising in patients with a possible underlying focal cortical dysplasia and might help to guide the placement of invasive electrodes. ( J Child Neurol 2005;20:363—369).
Molecular Diagnosis of Rett Syndrome
Authors: Huppke Peter , Gärtner Jutta
Published: June 2007

In 1999, mutations in the MECP2 gene were identified as the primary cause of Rett syndrome. MECP2 mutations can be found in 70% to 80% of all clinically defined Rett syndrome cases; in classic Rett syndrome, this frequency is even higher. In most cases, missense and nonsense mutations affecting functionally important domains can be found. Additionally, a hot spot for small deletions has been defined, and several gross rearrangements have also been described. Among female individuals with Rett syndrome, the spectrum of clinical phenotypes is broad, but most fulfill the diagnostic criteria. In contrast, male individuals with mutations in the MECP2 gene are rare, and only a minority have clinical symptoms resembling Rett syndrome. ( J Child Neurol 2005;20:732—736).
Newborn Screening and Prenatal Diagnosis for Rett Syndrome: Implications for Therapy
Authors: Amir Ruthie E. , Sutton V. Reid , Van den Veyver Ignatia B.
Published: June 2007

Most girls with Rett syndrome develop normally prior to the appearance of the typical symptoms. A presymptomatic phase is also observed in many inborn errors of metabolism that are included in newborn screening programs. Diagnostic testing for mutations or large genomic rearrangements involving methyl-CpG binding protein 2 gene ( MECP2) is highly sensitive and identifies mutations in up to 95% of female individuals with classic Rett syndrome. This has prompted some to ask whether MECP2 testing should be included in newborn and prenatal screening programs. We review current and evolving practices in these programs, emphasizing their relevance to Rett syndrome. The availability of a reliable test and the characteristic early latent phase, which creates a window of opportunity for early treatment, favor universal newborn screening for Rett syndrome. However, the high cost and the lack of an effective presymptomatic treatment make universal newborn screening for Rett syndrome impractical at present. In contrast, prenatal diagnosis should be offered to the parents of an affected child if the responsible mutation has been identified in the index case. ( J Child Neurol 2005;20:779—783).
Association of Duchenne Muscular Dystrophy With Autism Spectrum Disorder
Authors: Wu Joyce Y. , Kuban Karl C. K. , Allred Elizabeth , Shapiro Frederic , Darras Basil T.
Published: June 2007

We hypothesize that Duchenne muscular dystrophy and autism spectrum disorder/pervasive developmental disorder co-occur with a greater than random frequency. In this study, we set out to reject the hypothesis that Duchenne muscular dystrophy and autism spectrum disorder/pervasive developmental disorder co-occur no more often than expected by chance. Two index cases and six additional boys with concomitant Duchenne muscular dystrophy and autism spectrum disorder were identified in a muscular dystrophy clinic that approximates the total number of Duchenne muscular dystrophy boys (158) in the state of Massachusetts. The rate of prevalence (6 of 158) was compared with the prevalence rate of autism spectrum disorder in boys in the general population (1.6 in 1000). We rejected the hypothesis that Duchenne muscular dystrophy and autism spectrum disorder co-occurrence was likely to be explained by chance ( P = .006). We identify a previously unrecognized association of Duchenne muscular dystrophy with autism spectrum disorder. Further work might elucidate the level of association between these two conditions, either at the genetic or at the protein level, and might clarify, at least partially, the neurobiologic mechanisms associated with autism spectrum disorder. ( J Child Neurol 2005;20:790—795).
Environmental Enrichment Reverses the Impaired Exploratory Behavior and Altered Gene Expression Induced by Early-Life Seizures
Authors: Koh Sookyong , Chung Hyokwon , Hongjing Xia  , Mahadevia Amit , Song Youngju
Published: June 2007

Behavioral problems, school failure, and memory impairment are common among children with epilepsy. Currently, no effective treatment exists to promote recovery and neuron regeneration after seizures. To investigate the efficacy of environmental enrichment in reversing early-life seizure-induced changes in exploratory behavior and gene expression, we injected postnatal day 20 to 25 rats with kainic acid or saline and placed them either singly in a cage or as a group of eight in an enriched environment for 7 to 10 days. Exploratory behavior was quantified in an open field, and hippocampal gene analysis was performed on oligonucleotide microarrays. Exploratory behavior in kainic acid isolated rats were decreased in open field, whereas kainic acid rats exposed to an enriched environment behaved similarly to controls ( n = 37, analysis of variance, P < .001). Correlated with an improvement in behavior, genes involved in synaptic plasticity and memory consolidation, such as Arc, Homer1a, and Egr1, were significantly increased in rats exposed to environmental enrichment. Real-time quantitative reverse transcriptase—polymerase chain reaction confirmed our microarray data on select genes. Our results provide an experimental basis for promoting enriching education programs for children with epilepsy. ( J Child Neurol 2005;20:796—802).
Tau, Phospho-Tau, and S-100B in the Cerebrospinal Fluid of Children With Multiple Sclerosis
Authors: Rostasy Kevin , Withut Esther , Pohl Daniela , Lange Peter , Ciesielcyk Barbara , Diem Ricarda , Gärtner Jutta , Otto Markus
Published: June 2007

Axonal injury and glial activation are an early neuropathologic event in adults with multiple sclerosis. To investigate whether markers of axonal injury and glial activation are already elevated in the cerebrospinal fluid of children with multiple sclerosis, we studied the cerebrospinal fluid of 25 children with multiple sclerosis and 67 controls for the presence of tau, phospho-tau, and S-100B proteins using specific enzyme-linked immunoabsorbent assays. In general, tau, phospho-tau, and S-100B protein levels did not differ significantly between groups. However, in a subgroup of nine children with multiple sclerosis, all of whom had prominent clinical symptoms at the time of lumbar puncture and radiologic disease activity, tau protein levels were significantly elevated when compared with other controls. These data indicate that axonal injury is not restricted to adult multiple sclerosis but can already occur in children with multiple sclerosis. ( J Child Neurol 2005;20:822—825).
Finger Cold-Induced Vasodilatation, Sympathetic Skin Response, and R—R Interval Variation in Patients With Progressive Spinal Muscular Atrophy
Authors: Arai Hidee , Tanabe Yuzo , Hachiya Yasuo , Otsuka Eiko , Kumada Satoko , Furushima Wakana , Kohyama Jun , Yamashita Sumimasa , Takanashi Jun-ichi , Kohno Yoichi
Published: June 2007

To elucidate autonomic function in spinal muscular atrophy, we evaluated finger cold-induced vasodilatation, sympathetic skin response, and R—R interval variation in 10 patients with spinal muscular atrophy: 7 of type 1, 2 of type 2, and 1 of type 3. Results of finger cold-induced vasodilatation, sympathetic skin response, and R—R interval variation were compared with those of healthy children. Finger cold-induced vasodilatation was abnormal in 6 of 10patients with spinal muscular atrophy; it was normal in the healthy children. The mean sympathetic skin response latency and amplitude did not differ significantly from those of the healthy children. Amplitudes of sympathetic skin response to sound stimulation were absent or low in all six patients with spinal muscular atrophy. No significant difference was found in the mean R—R interval variation of patients with spinal muscular atrophy and healthy children. Results show that some patients with spinal muscular atrophy have autonomic dysfunction, especially sympathetic nerve hyperactivity, that resembles dysfunction observed in amyotrophic lateral sclerosis. ( J Child Neurol 2005;20:871—875).
Identifying the Primary Epileptogenic Hemisphere from Electroencephalographic (EEG) and Magnetoencephalographic Dipole Lateralizations in Children With Intractable Epilepsy
Authors: Ochi Ayako , Otsubo Hiroshi , Iida Koji , Oishi Makoto , Elliott Irene , Weiss Shelly K. , Kutomi Tomoko , Nakayama Tojo , Sharma Roy , Chuang Sylvester H. , Rutka James T. , Snead Carter
Published: June 2007

We used electroencephalographic (EEG) and magnetoencephalographic dipole lateralizations to identify the primary epileptogenic hemisphere in 41 children with intractable localization-related epilepsy. We compared EEG and magnetoencephalographic dipole lateralizations, EEG ictal onsets, and magnetic resonance images (MRIs). Concordant lateralization of EEG and magnetoencephalographic dipoles (> 50% of each lateralizing to the same hemisphere) occurred in 34 patients, with EEG ictal onsets in the same hemisphere in 23 (68%) and concordant MRI lesions in 23 (68%). Focal resection in 16 of 20 patients resulted in a good surgical outcome. Of the seven children with nonconcordant magnetoencephalographic and EEG lateralizations, one (14%) had EEG ictal onset and one (14%) had MRI lesions that lateralized; none had surgery. The relationship between lateralized EEG and magnetoencephalographic dipoles forecasts surgical candidacy. Concordant lateralizations predict good seizure control after surgery by identifying the primary epileptogenic hemisphere. Discordant lateralizations signify an undetermined epileptogenic hemisphere and contraindicate surgery without further testing. ( J Child Neurol 2005;20:885—892).
Craniocervical Arterial Dissection in Children: Clinical and Radiographic Presentation and Outcome
Authors: Rafay Mubeen F. , Armstrong Derek , deVeber Gabrielle , Domi Trish , Chan Anthony , MacGregor Daune L.
Published: June 2007

Craniocervical arterial dissection is a recognized cause of arterial ischemic stroke in children. Whether children with craniocervical arterial dissection have dissection characteristics different from those of adults is unclear. A retrospective review of children, 1 month to 18 years of age, with dissection from two Canadian pediatric ischemic stroke registry centers was conducted. From 213 patients with arterial ischemic stroke, 16 (7.5%) were identified with dissection, 37.5% had warning symptoms, and 50% had a history of head or neck trauma. The clinical presentation included headache (44%), altered consciousness (25%), seizures (12.5%), and focal deficits (87.5%). Dissection involved extracranial vessels in 75% and anterior circulation in 56%. Follow-up included complete recovery in 43%, mild to moderate deficits in 44%, and severe deficits in 13%. Fourteen (87.5%) children received antithrombotic treatment. Follow-up angiography showed resolution of abnormalities in 60% of vessels. Total occlusion had the worst outcome for recanalization. In conclusion, the etiology of arterial dissection in the majority of children appears to be either trauma or idiopathic. Long-term angiography shows variable outcomes, depending on the initial findings. The relationship of angiographic outcomes with recurrent strokes requires further study in pediatric dissection. ( J Child Neurol 2006;21:8—16).
Early Clinical and Electrophysiologic Features of the Two Most Common Autosomal Recessive Forms of Charcot-Marie-Tooth Disease in the Roma (Gypsies)
Authors: Guergueltcheva Velina , Tournev Ivailo , Bojinova Veneta , Hantke Janina , Litvinenko Ivan , Ishpekova Boryana , Shmarov Alexander , Petrova Julia , Jordanova Albena , Kalaydjieva Luba
Published: June 2007

Our recent studies of the genetic epidemiology of neuromuscular disorders in Gypsies in Bulgaria have revealed that two private disorders, hereditary motor and sensory neuropathy type Lom and hereditary motor and sensory neuropathy type Russe, account for most cases of Charcot-Marie-Tooth disease in this population. In this study, we examined the clinical and electrophysiologic manifestations of the two disorders in childhood, aiming to identify the distinctive features that allow early differential diagnosis. The study included 13 patients, aged between 2 and 15 years. The childhood clinical manifestations of both neuropathies were similar, although they tended to be more severe in hereditary motor and sensory neuropathy type Lom. The nerve conduction velocities in hereditary motor and sensory neuropathy type Lom were lower than in hereditary motor and sensory neuropathy type Russe. Brainstem auditory evoked potentials were abnormal in hereditary motor and sensory neuropathy type Lom, even at an early age, and normal in hereditary motor and sensory neuropathy type Russe. Although electrophysiologic data provide a more reliable differentiation than clinical data, the definitive diagnosis should rely on genetic testing. ( J Child Neurol 2006;21:20—25).
Longitudinal Changes in Cortical Glucose Hypometabolism in Children With Intractable Epilepsy
Authors: Benedek Krisztina , Juhász Csaba , Chugani Diane C. , Muzik Otto , Chugani Harry T.
Published: June 2007

In children with partial epilepsy, there is increasing evidence to suggest that not all cortical regions showing glucose hypometabolism on positron emission tomography (PET) represent epileptogenic cortex but that some hypometabolic areas might be the result of repeated seizures. Most of the supportive data, however, have come from cross-sectional imaging studies. To evaluate longitudinal changes in cortical glucose hypometabolism, we compared two sequential [18F]fluorodeoxyglucose (FDG) PET scans performed 7 to 44 months apart in 15 children with intractable nonlesional partial epilepsy. The extent of hypometabolic cortex on the side of the electroencephalography-verified epileptic focus and its changes between the two PET scans were measured and correlated to clinical seizure variables. The change in seizure frequency between the two PET scans correlated positively with the change in the extent of cortical glucose hypometabolism ( r = .8, P < .001). Most patients with persistent or increased seizure frequency (one or more seizures per day) showed enlargement in the area of hypometabolic cortex on the second PET scan. In contrast, patients whose seizure frequency had decreased below daily seizures between the first and second PET scans showed a decrease in the size of the hypometabolic cortex. These results support the notion that the extent of cortical glucose hypometabolism on PET scanning can undergo dynamic changes, and these are, at least partly, related to the frequency of seizures. The findings have implications on how aggressively persistent seizures should be treated in children. ( J Child Neurol 2006;21:26—31).
Recurrent Headache in Chinese Children: Any Agreement Between Clinician Diagnosis and Symptom-Based Diagnoses Using the International Classification of Headache Disorders (Second Edition)?
Authors: Chan Tracy Y.P. , Wong Virginia
Published: June 2007

There has been a lack of published data on the pattern of recurrent headache in Chinese children. The validity of the International Classification of Headache Disorders criteria has not been evaluated in Chinese children. We performed a retrospective medical record review of 124 children aged < 18 years with an International Classification of Diseases coding of headache followed up in a general outpatient clinic in a university-based hospital over a 3-year period (2000—2002). The aims of our study were to (1) study the pattern of recurrent headache in Chinese children and (2) study any agreement between clinical diagnoses made by our board-certified pediatricians and symptom-based diagnoses using the second edition of the International Classification of Headache Disorders (International Classification of Headache Disorders—II). The most common type was unclassified headache (70.2%), followed by infrequent episodic tension-type headache (24.2%) and migraine without aura (5.6%). A family history of headache or migraine was more commonly found in children with infrequent episodic tension-type headache or migraine without aura. ( P = .0109) The co-occurrence of abdominal pain with infrequent episodic tension-type headache was 30%; for unclassified headache, it was 19.5%. Dysmenorrhea occurred in 7.1% of girls with infrequent episodic tension-type headache and 8.6% of girls with unclassified headache. However, migraine without aura was not associated with abdominal pain or dysmenorrhea. Children with migraine without aura were more frequently referred to child neurologists ( P = .0207) and admitted ( P = .0000). Neurologic investigations, including electroencephalography, computed tomography, or magnetic resonance imaging of the brain, were performed in less than 30% of cases. Abnormal results were found in only seven cases; with two referred to a neurosurgeon and none requiring surgical intervention. Thus, by using the clinical diagnosis of our board-certified pediatricians as the standard, the sensitivity and specificity of International Classification of Headache Disorders—II—based definition of migraine without aura was 23.1% and 93.4%, respectively, and for infrequent episodic tension-type headache, it was 37.5% and 76%, respectively. The typical characteristics of migraine tend to emerge later and might have led to underdiagnosis of the younger age group, with a higher rate of referral and inpatient management. The new edition of the International Classification of Headache Disorders criteria is still restrictive in clinical practice and might not be able to reflect current pediatric practice. Further studies with a defined study period or recurrent headache might be more useful in analyzing the use of these new International Classification of Headache Disorders criteria in the diagnosis of recurrent headache in children. ( J Child Neurol 2006;21:132—138); DOI 10.2310/7010.2006.00013
Gillespie Syndrome: Two Further Cases
Authors: Donald Kirsten A. , Grotte Rhianne , Crutchley Antony C. , Wilmshurst Jo M.
Published: June 2007

We describe two unrelated male children with Gillespie syndrome, a rare genetic disorder consisting of cerebellar ataxia, partial aniridia, and psychomotor delay. One was more severely affected than the other, but neither had evidence of neuroregression. Partial aniridia was a key diagnostic marker, present at birth in both patients. Neurocognitive impairment and cerebellar ataxia were severe in one and mild in the other. The genetics of this condition remain undefined. Although believed to be an autosomal recessive condition, no clear, single candidate gene has been identified. ( J Child Neurol 2006;21:337—340; DOI 10.2310/7010.2006.00052).
Walking With Modafinil and Its Use in Diplegic Cerebral Palsy
Authors: Hurst Daniel L. , Lajara-Nanson Walter A. , Lance-Fish Margie E.
Published: June 2007

A retrospective review of pediatric patients with spastic cerebral palsy was undertaken at Texas Tech University Health Sciences Center covering a period from January 1, 2000, until December 31, 2003. One hundred twenty pediatric patients were identified in the Texas Tech University Health Sciences Center child neurology clinic with spastic cerebral palsy. Fifty-nine patients of this group received modafinil treatment for cerebral palsy. Twenty-nine of the 59 patients were noted to have an improving gait on modafinil. Six of these modafinil-treated patients improved from no ambulation or only assisted ambulation to unassisted ambulation. This varied from taking a few steps without holding on to walking down the hall without assistance. Two patients with spastic diplegia secondary to prematurity have had a dramatic improvement in gait during the first 6 months after starting modafinil. Two other patients with spastic diplegia not included in this group of six patients taught themselves to stand up and walk while in water unassisted. During this same time period, only three non—modafinil-treated patients with mild cerebral palsy were noted with gait improvements, but not to the dramatic extent of the modafinil-treated group. A nonambulatory 5-year-old child, who presented for a requested wheelchair prescription because the mother had given up all hope of her child ever walking, is now taking independent steps unassisted after starting modafinil. Modafinil, a central nervous system stimulant, appears to improve tone and ambulation in spastic diplegic cerebral palsy. ( J Child Neurol 2006;21:294—297; DOI 10.2310/7010.2006.00086).
Evaluation of the Influence of Antiepileptic Therapy on Antioxidant Enzyme Activity and Lipid Peroxidation in Erythrocytes of Children With Epilepsy
Authors: Sobaniec Wojciech , Solowiej Elzbieta , Kulak Wojciech , Bockowski Leszek , Smigielska-Kuzia Joanna , Artemowicz Barbara
Published: June 2007

The aim of this study was to evaluate the influence of antiepileptic therapy on antioxidant enzyme activity and lipid peroxidation in the erythrocytes of children with epilepsy. For this purpose, the activity of superoxide dismutase, glutathione peroxidase, and glutathione reductase and the malondialdehyde concentration in 61 healthy children and 90 children with epilepsy were measured. The activities of all of these enzymes were insignificantly higher, whereas the malondialdehyde concentration was significantly lower in the patients treated with carbamazepine monotherapy. In patients treated with valproate monotherapy, the activities of all enzymes were insignificantly lower, whereas the malondialdehyde concentration was insignificantly higher. In patients treated with polytherapy, the activity of superoxide dismutase was insignificantly lower, whereas the activity of glutathione peroxidase and glutathione reductase was insignificantly higher and the malondialdehyde concentration was lower. There were differences in glutathione reductase activity between the valproate monotherapy group and both the carbamazepine monotherapy and polytherapy groups and in malondialdehyde concentrations between the carbamazepine and valproate groups. The results indicate that the oxidant-antioxidant balance in children with epilepsy is modified by antiepileptic therapy. (J Child Neurol 2006;21:558–562; DOI 10.2310/7010.2006.00115).
Administration of Intravenous Tissue Plasminogen Activator in a Pediatric Patient With Acute Ischemic Stroke
Authors: Shuayto Marwan I. , Ivan Lopez J. , Greiner Frank
Published: June 2007

Intravenous tissue plasminogen activator is the only therapy approved by the US Food and Drug Administration (FDA) for the treatment of acute ischemic strokes. The National Institutes of Health (NIH)—sponsored study that led to the approval of tissue plasminogen activator to be used in acute ischemic strokes included only individuals 18 years of age and older. We report a case of a pediatric patient who suffered a dominant-hemisphere acute ischemic stroke who was treated with intravenous tissue plasminogen activator. Our patient's symptoms began to resolve 4 hours after treatment, and he had a complete recovery after 8 hours. The clinical outcome was excellent and without complications. Tissue plasminogen activator administration can be safe and effective in younger patients. Randomized, controlled, double-blind studies are needed. (J Child Neurol 2006;21:604— 606; DOI 10.2310/7010.2006.00142).
False-Positive Metaiodobenzylguanidine Scan for Neuroblastoma in a Child With Opsoclonus-Myoclonus Syndrome Treated With Adrenocorticotropic Hormone (ACTH)
Authors: McGarvey Cynthia K. , Applegate Kimberly , Lee Nadine Deanie , Sokol Deborah K.
Published: June 2007

We describe the case of a 2-year-old girl with opsoclonus-myoclonus syndrome treated with chronic adrenocorticotropic hormone (ACTH) in which a metaiodobenzylguanidine scan showed abnormal radiotracer uptake in the left adrenal gland region, interpreted as the site of an occult neuroblastoma. As this finding was not corroborated by previous or subsequent metaiodobenzylguanidine scans or by computed tomography (CT) or magnetic resonance imaging (MRI), we attribute the finding to being a false-positive result from adrenal hyperplasia owing to chronic use of ACTH and not to neuroblastoma. Metaiodobenzylguanidine scintigraphy is an extremely important nuclear medicine examination tool used for the evaluation and staging of pediatric neuroblastoma. We highlight the need for cautious interpretation of metaiodobenzylguanidine as a screening tool for neuroblastoma in patients treated with ACTH. (J Child Neurol 2006;21:606—610; DOI 10.2310/ 7010.2006.00143).
Stroke Associated With Central Nervous System Vasculitis After West Nile Virus Infection
Authors: Alexander John J. , Lasky Andrew S. , Graf William D.
Published: June 2007

We report the association of West Nile virus infection, isolated vasculitis, and stroke in a 9-year-old girl. West Nile virus is of growing epidemiologic importance and should be considered in the differential diagnosis of stroke etiologies, especially during late summer and in patients with a history of exposure in areas where West Nile virus transmission is present. (J Child Neurol 2006;21:623—625; DOI 10.2310/7010.2006.00148).
Diffusion Tensor Imaging of the Corticospinal Tract Following Cerebral Hemispherectomy
Authors: Wakamoto Hiroyuki , Eluvathingal Thomas J. , Makki Malek , Juhasz Csaba , Chugani Harry T.
Published: June 2007

Following cerebral hemispherectomy, the corticospinal tract is believed to undergo reorganizational changes, which can induce enhanced function of the contralateral motor pathway and mediate partial recovery of motor function. The aim of this study was to use diffusion tensor imaging to investigate the effects of hemispherectomy on the corticospinal tract, with particular attention to the corticospinal tract contralateral to the resection. Diffusion tensor imaging would presumably detect microstructural abnormalities through quantitative measurements of the fiber tract integrity and orientation. Four patients with anatomic hemispherectomy and three patients with subtotal hemispherectomy were examined and compared with age-matched normal controls. Apparent diffusion coefficient and fractional anisotropy values were measured in regions along the corticospinal tract: internal capsule, cerebral peduncle, rostral pons, midpons, and caudal pons. None of the patients with anatomic hemispherectomy or subtotal hemispherectomy showed significant changes in either apparent diffusion coefficient or fractional anisotropy values in the corticospinal tract contralateral to the resected hemisphere, whereas increased apparent diffusion coefficient and decreased fractional anisotropy were observed in the ipsilateral rostral pons, midpons, and caudal pons of all patients with anatomic hemispherectomy, as well as in the ipsilateral cerebral peduncle of one patient with subtotal hemispherectomy. Increased apparent diffusion coefficient values were also noted in the ipsilateral internal capsule of the same patient. This study revealed no evidence of significant reinforcement of the contralateral corticospinal tract in patients with hemispherectomy, at least from diffusion tensor imaging measurements, but suggests that wallerian degeneration most likely occurs in the ipsilateral motor pathway. (J Child Neurol 2006;21:566—571; DOI 10.2310/7010.2006.00150).
Treatment of Children and Adolescents With Tics and Tourette Syndrome
Authors: Gilbert Donald
Published: June 2007

Tics, patterned movements distinct from stereotypies, myoclonus, and other hyperkinetic movements, are quite common in children, particularly among those with developmental and psychiatric disorders. Thus, tics can indicate the presence of atypical neurodevelopment or broader difficulties with cognition or mood. Tics are also the cardinal feature of Tourette syndrome, a childhood-onset neurobehavioral disorder characterized by a chronic inability to suppress or an urge to perform patterned, repetitive movements. Patients with Tourette syndrome most commonly have, in addition to tics, symptoms of inattention, hyperactivity, obsessiveness, or anxiety. Achieving the most effective treatment of a child with tics is contingent on proper diagnosis of the movement disorder and thorough assessment for other problems, followed by consideration of both nonpharmacologic and pharmacologic interventions for any and all symptoms interfering with the child's function and quality of life. This review focuses primarily on the diagnosis and medical treatment of tics in children and adolescents with Tourette syndrome. (J Child Neurol 2006;21:690—700; DOI 10.2310/ 7010.2006.00161).
Tic Disorders: Neural Circuits, Neurochemistry, and Neuroimmunology
Authors: Harris Kendra , Singer Harvey S.
Published: June 2007

The neuroanatomy and neurochemistry underlying tic disorders are thought to involve corticostriatothalamocortical circuits and dysregulation of their component neurotransmitter systems. Tourette syndrome is a tic disorder that begins in childhood and follows a waxing and waning course of tic severity. Although it is generally believed to have a genetic component, its etiology has not been fully elucidated. The clinical entity pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) has led some to suggest that the pathophysiology of tics in some individuals might involve a postinfectious autoimmune component. We review the neural circuits and neurochemistry of Tourette syndrome and evaluate the evidence for and against a role for autoimmunity in the expression of tics. (J Child Neurol 2006;21:678—689; DOI 10.2310/7010.2006.00166).
Obsessive-Compulsive Disorder in Tourette Syndrome
Authors: Goodman Wayne K. , Storch Eric A. , Geffken Gary R. , Murphy Tanya K.
Published: June 2007

Several lines of evidence suggest a meaningful association between obsessive-compulsive disorder and Tourette syndrome, including comorbidity, phenomenologic overlap, evidence from family and genetic studies, and the possible role of basal ganglia circuitry in both conditions. Obsessive-compulsive behaviors occur frequently in patients who have Tourette syndrome and tend to have a later onset than tics. Despite commonalities, the approaches to treating tics and obsessive-compulsive symptoms are actually quite distinct. A specialized form of cognitive behavior therapy and pharmacotherapy with a potent serotonin reuptake inhibitor are the two established first-line therapies for obsessive-compulsive disorder. An adequate trial of a serotonin reuptake inhibitor is 10 to 12 weeks in duration at doses near the upper end of the recommended range for age and weight. Cases of obsessive-compulsive disorder that do not sufficiently improve with serotonin reuptake inhibitors might benefit from adjunctive low-dose antipsychotic (eg, risperidone) medication whether or not tics are present. Warnings about an increased risk of suicidality among children and adolescents taking antidepressants for pediatric depression extend to those taking the medications for obsessive-compulsive disorder, but the risk-to-benefit ratio is more favorable in this latter population because several serotonin reuptake inhibitors have been shown to be efficacious in obsessive-compulsive disorder. (J Child Neurol 2006;21:704— 714; DOI 10.2310/7010.2006.00169).
Co-occurring Psychiatric Disorders in Children and Adolescents With Tourette Syndrome
Authors: Gaze Catherine , Kepley Hayden O. , Walkup John T.
Published: June 2007

More than half of all children and adolescents with Tourette syndrome show evidence of psychiatric comorbidity, exhibiting symptoms of attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder, and other anxiety and mood disorders. Although the prevalence of co-occurring conditions varies depending on the clinical setting, it is crucial for clinicians to be familiar with these disorders because they are often more impairing than tics and can influence the initial treatment choice. Left untreated, these conditions can negatively affect important developmental outcomes, such as academic and social functioning. We review the most common co-occurring disorders, the relationship of these co-occurring disorders to Tourette syndrome, and treatment recommendations for co-occurring conditions when tic symptoms are present. (J Child Neurol 2006;21:657—664; DOI 10.2310/7010.2006.00170).
Neurologic and Radiologic Manifestations of Three Girls Surviving Acute Carbon Monoxide Poisoning
Authors: Hon Kam-lun Ellis , Yeung Wai-lan , Ho Chi-hang Assunta , Leung Wing-kwan Alex , Li Albert Martin , Chu Winnie Chiu-wing , Chan Yu-leung
Published: June 2007

We report the neurologic and radiologic manifestations of three adolescent girls with acute carbon monoxide poisoning. The girls were found collapsed and unconscious in a bathroom where liquid petroleum gas was being used as heating fuel. As hyperbaric oxygen therapy was not available locally, they only received oxygen supplementation via nasal cannula (4 L/minute) as treatment in the first 2 days. On transfer to a tertiary center in Hong Kong, evolving neurologic manifestations of visual acuity and field deficits, confusion, and focal motor weaknesses were observed. Focal infarctions were evident in cerebral computed tomography in one patient and cortical lesions on magnetic resonance imaging in all three patients. [18F]Fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed additional decreased metabolism in the basal ganglia in two patients, which was typical of carbon monoxide poisoning. The neurologic deficits resolved completely at 3 weeks after the exposure, but psychologic symptoms succeeded. This report serves to alert clinicians to the varied neuro-ophthalmologic manifestations and psychologic impairment even with the same duration of carbon monoxide poisoning. PET might be more sensitive in detecting cerebral injuries specific for carbon monoxide poisoning. (J Child Neurol 2006;21:737—741; DOI 10.2310/7010.2006.00172).
Remission Associated With Human Herpesvirus Infection in West Syndrome
Authors: Iwasaki Toshiyuki , Nonoda Yutaka , Ishii Masahiro
Published: June 2007

Generally, West syndrome is an intractable epileptic syndrome in infancy, although spontaneous remission has been reported in some cases. An immunologic response to infection might be one of the factors involved in the remission of West syndrome, but the mechanisms remain unknown. On the other hand, exanthema subitum is a common disease occurring in infancy with the characteristics of fever and rash. Two kinds of human herpesvirus, 6 and 7, have been isolated as causal agents of exanthema subitum. We experienced one symptomatic case and three cryptogenic cases of West syndrome that showed spontaneous remission. In the symptomatic case, the subject showed a temporary remission; however, in the other cases, the remissions were long term. In the present study, we report the patients' improvement and electroencephalographic (EEG) findings. In all of our cases, hypsarrythmia disappeared on the EEG findings, the human herpesvirus 6 IgG antibodies increased in all four cases, and the herpesvirus 7 IgG antibodies increased in two cases. We postulate that the remission of the four cases proceeded from infection by exanthema subitum. The changes in serum antibody values suggest that the spontaneous remission of West syndrome was related to human herpesvirus 6 and 7 infections. (J Child Neurol 2006;21:886—890; DOI 10.2310/ 7010.2006.00193).
Megalocornea and Mental Retardation Syndrome: Clinical and Instrumental Follow-Up of a Case
Authors: Margari Lucia , Presicci Anna , Ventura Patrizia , Buttiglione Maura , Dicuonzo Franca , Lattarulo Caterina , Perniola Tommaso
Published: June 2007

Megalocornea—mental retardation syndrome, otherwise known as Neuhauser syndrome, is a rare autosomal recessive disorder. Only 36 cases have been reported in the literature. We describe the clinical and instrumental follow-up, lasting 5 years, of a case showing the typical features of the syndrome, associated with transient hypothyroidism, epilepsy, cerebral palsy with choreoathetotic movements, and brain malformation. Our report might help better delineate the phenotype and natural history of the syndrome. (J Child Neurol 2006;21:893—896; DOI 10.2310/ 7010.2006.00202).
Topical Review: Fetal Testosterone and Sex Differences in Typical Social Development and in Autism
Authors: Knickmeyer Rebecca Christine , Baron-Cohen Simon
Published: June 2007

Experiments in animals leave no doubt that androgens, including testosterone, produced by the testes in fetal and/or neonatal life act on the brain to induce sex differences in neural structure and function. In human beings, there is evidence supporting a female superiority in the ability to read nonverbal signals, specific language-related skills, and theory of mind. Even more striking than the sex differences seen in the typical population is the elevated occurrence of social and communicative difficulties in human males. One such condition, autism, occurs four times more frequently in boys than in girls. Recently, a novel theory known as the ``extreme male brain'' has been proposed. It suggests that the behaviors seen in autism are an exaggeration of typical sex differences and that exposure to high levels of prenatal testosterone might be a risk factor. In this article, we argue that prenatal and neonatal testosterone exposures are strong candidates for having a causal role in sexual dimorphism in human behavior, including social development, and as risk factors for conditions characterized by social impairments, particularly autism spectrum conditions. (J Child Neurol 2006;21:825—845; DOI 10.2310/7010.2006.00213).
Recurrent Nocturnal Tongue Biting in a Child With Hereditary Chin Trembling
Authors: Goraya Jatinder S. , Virdi Verinderjit , Parmar Veena
Published: June 2007

A 13-month-old boy presented with repeated episodes of tongue biting during sleep. On evaluation, he was found to have hereditary chin trembling, a rare autosomal dominant condition characterized by continuous or intermittent tremulous activity of the mentalis muscle. This is the first report of this kind from India. The tongue biting appeared to be the result of parasomnia. Treatment with clonazepam was very effective; it completely ameliorated the symptom of tongue biting but had no effect on chin trembling. (J Child Neurol 2006;21:985—987; DOI 10.2310/ 7010.2006.00220).
Headache as a Sole Manifestation in Nonconvulsive Status Epilepticus
Authors: Ghofrani Mohammad , Mahvelati Farhad , Tonekaboni Hassan
Published: June 2007

Nonconvulsive status epilepticus can present with several manifestations, many of which might not be obvious. The most important for the diagnosis of nonconvulsive status epilepticus is the electroencephalogram (EEG) pattern. This is a case report of a 9-year-old boy with severe and continuous headache. He received chemotherapy for histiocytosis and was diagnosed at 3.5 years. He had no evidence of central nervous system histiocytosis involvement or drug toxicity. He was diagnosed with nonconvulsive status epilepticus. Headache and EEG anomaly disappeared completely when anticonvulsant therapy was begun. Headache and seizure disorder can coexist, but this might be the first report of nonconvulsive status epilepticus with headache as a sole manifestation. (J Child Neurol 2006;21:981— 983; DOI 10.2310/7010.2006.00243).
Spontaneous Subarachnoid Hemorrhage: Assessment of Prognosis and Initial Management in the Intensive Care Unit
Authors: Thie Andreas , Spitzer Klaus , Kunze Klaus
Published: June 2007

Spontaneous subarachnoid hemorrhage (SAH) is asso ciated with high morbidity and mortality. Primary con servative or preoperative management in the intensive care unit aims at prevention, early detection, and treat ment of complications. In this article we review the literature on the value of initial clinical and laboratory findings in predicting complications and outcome after SAH. Current conservative management of SAH is briefly discussed.
An Economic Analysis of Extracorporeal Membrane Oxygenation
Authors: Pearson Gail Denise , Lou Short Billie
Published: June 2007

Financial considerations, in concert with clinical effec tiveness, are of increasing importance in the assessment of technological innovations. One such innovation, ex tracorporeal membrane oxygenation (ECMO), is now in use at over twenty centers nationwide to treat new borns with severe, acute lung disease. Use of ECMO therapy for one year at Children's Hospital National Medical Center, Washington, DC, in a population of pa tients with persistent pulmonary hypertension of the newborn (PPHN) is reported, comparing outcome and financial considerations with a similar group of infants treated conventionally prior to ECMO. A historical con trol group of infants with severe PPHN showed that before ECMO was available the survival rate in this criti cally ill population was only 21%. With ECMO, infants with the same clinical characteristics have an 80% chance of survival. Analysis of hospital and physician charges for these two groups (pre-ECMO and ECMO) reveals that ECMO therapy is about 2% less expensive than conventional treatment. When only survivors in each group are compared, ECMO is 43% less costly. These differences are attributable to reductions in aver age length of hospital stay with ECMO therapy, and they are conservative in that they do not take into considera tion the marked economic advantage to society of avert ing unnecessary deaths.
Analytic Reviews : Hypervolemic Hemodilution in Acute Ischemic Stroke
Authors: Wood James H. , Kee David B.
Published: June 2007

In patients with acute ischemic stroke, reversibility of the neurologic deficits depends on the depth and dura tion of the ischemic insult. The rapid elevation of cere bral blood flow (CBF) above ischemic thresholds may minimize or prevent infarction. Reduction of blood vis cosity has been shown to augment CBF and improve cerebral neuronal activity after stroke. Hypervolemic hemodilution decreases hematocrit, increases blood volume, augments cardiac output, and, in randomized clinical trials, improves the long-term outcome in acute ischemic stroke. Similarly, administration of drugs that decrease plasma fibrinogen and increase erythrocyte flexibility reportedly causes a decrease in the incidence of recurrent ischemia after an initial transient ischemic attack. Thus, the pharmacologic manipulation of blood viscosity appears to be effective in managing patients with acute ischemic stroke.
Metabolic Alkalosis
Authors: Rimmer Jeffrey M. , Gennari F. John
Published: June 2007

Metabolic alkalosis is a common acid-base disturbance in critically ill patients. In this review we discuss the approach to diagnosis and management of this disorder; particular emphasis is given to the causes most com monly responsible for alkalosis in critical care medicine. We present rules for (1) identifying the presence of metabolic alkalosis, ( 2 ) determining whether the disor der is simple or complicated by a second acid-base dis turbance, and (3) determining the cause: The causes are subdivided into three major groups: Chloride-respon sive, chloride-resistant, and alkali administration. The pathogenesis of each type of alkalosis is discussed sep arately, although we stress that more than one cause may be responsible in critically ill patients. The patho logical consequences of metabolic alkalosis and ap proaches to treatment are reviewed. The major issues relating to the critically ill patient are (1) identification and removal of exogenous sources of alkali, (2) iden tification and minimization of HCl losses or selective NaCl losses, and (3) maneuvers to reduce serum HCO 3 concentration without producing extracellular fluid volume overload.