bluuebunny/crossref_metadata_2025 huggingface dataset:
Perinatal Cerebral Insults: Hemorrhage and Ischemia
Authors: Ment Laura R. , Duncan Charles C. , Stewart William B.
Published: March 2008

The incidence of major neurodevelopmental handicaps in the survivors of newborn special care has remained essentially unchanged over the past decade despite the development of sophisticated perinatal care. Preterm infants with parenchymal involvement of intraventricular hemorrhage and neonates of all gestational ages with cerebral infarction are believed to be at higher risk for both neurologic and developmental abnormalities than infants without these problems. Both lesions are thought secondary to alterations in cerebral blood flow to the developing brain, and many infants demonstrate evidence for both hemorrhage and ischemic insult.
Pituitary Adenomas in Pediatric Patients: Are They more Invasive?
Authors: Kanter Steven L. , Mickle Parker , Hunter Stephen B. , Rhoton Albert L.
Published: March 2008

Twenty patients with biopsy proven pituitary adenomas that became symptomatic prior to the age of 20 years are reviewed. Based on age, the patients are divided into prepubertal, pubertal, and postpubertal groups. An increased incidence of invasiveness was observed in those patients with onset of symptoms during the pubertal years compared to patients whose symptoms first occurred during the postpubertal years. Other series of pediatric pituitary adenomas in the English literature are reviewed, and ostensibly disparate conclusions are examined.
Positron Emission Tomographic Evaluations in the Diagnosis and Therapy of Multifocal Glioblastoma
Authors: Mineura Katsuyoshi , Yasuda Tsuneo , Kowada Masayoshi , Sakamoto Tetsuya , Ogawa Toshihide , Shishido Fumio , Uemura Kazuo
Published: March 2008

A pediatric case of multifocal glioblastoma is reported with special reference to positron emission tomography (PET) evaluations in the diagnosis and therapy. A PET scan employing 15O and 18F positron tracers disclosed a right thalamic lesion high in regional cerebral blood flow, cerebral blood volume and metabolic glucose rate, suggesting a malignant tumor. In contrast, a left frontal lesion with low regional cerebral blood flow, cerebral blood volume and metabolic glucose rate was presumed to be rather hypodynamic and hypometabolic. Histological examination of these isolated masses showed both to be glioblastomas, and, as predicted by the PET findings, there were minor histological distinctions. In repeated PET scans following postoperative combined radiochemotherapy, metabolic glucose rate values at the thalamic tumor decreased to about 50% the levels before therapy, although there was no distinguishable change in tumor size on the concurrent computerized tomography scans. This therapeutic efficacy on tumor metabolism was highly consistent with the period of clinical relief.
Long-Term Intellectual Prognosis of Hydrocephalus with Reference to 77 Children
Authors: Billard C. , Santini J. , Gillet P.G. , Nargeot M.C. , Adrien J.L.
Published: March 2008

The long-term intellectual prognosis of nontumoral hydrocephalus is described: 75% of the population have a full intellectual quotient greater than 70. But often, there is a heterogeneous pattern of the intellectual quotient with a great difference between verbal and performance results because of poor visuospatial skills. Disabilities of syntactical comprehension of language are also encountered. Sex, age, delay before shunting and frequency of shunt complications are not related to intelligence level or pattern. Meningitis and toxoplasmosis often result in a poor intelligence level, but other etiologies do not influence intelligence level or pattern. Visuospatial deficits, attention disorders and language disabilities are independent of the degree of hydrocephalus but are especially encountered when ventricular dilatation persists in spite of the operation.
Cerebral Perfusion Pressure in the High-Risk Premature Infant
Authors: Yordy Michael , Hanigan William C.
Published: March 2008

Continuous monitoring of the cerebral perfusion pressure (CPP) and arterial blood gases was performed in 13 high-risk preterm infants. CPPs were monitored with a Ladd® fiberoptic transducer. Blood gases were measured via umbilical artery lines. Monitoring was performed within 12 h following birth and continued for 48–72 h. The mean gestational age (± SD) was 30.6 ±3.1 weeks with a mean weight (± SD) of 1,326 ± 511 g. Initial mean CPP (± SD) was 37.3 ± 8.9 mm Hg with a mean initial intracranial pressure (ICP) of 2.7 ±1.8 mm Hg. During the trial period, a sustained CPP below 25 mm Hg for over 2 h was indicative of a poor neurological outcome. A sustained ICP over 7 mm Hg was indicative of intracranial hypertension, but occurred in relation to nursing events such as suctioning or positioning of the patient’s head. Seven infants demonstrated significant linear relationships between CPP or ICP and levels of arterial pCO2 or pH. The mean Apgar scores were significantly higher for this group, but the presence of this relationship did not indicate a better neurological outcome.
Management of Children with Primitive Neuroectodermal Tumors of the Posterior Fossa/Medulloblastoma
Authors: Packer Roger J. , Sutton Leslie N. , D’Angio Giulio , Evans Audrey E. , Schut Luis
Published: March 2008

Primitive neuroectodermal tumors of the posterior fossa/medulloblastoma (PNET-MB) are the most common malignant primary central nervous system tumors of childhood. Current approaches to therapy for children with PNET-MB are illustrative of both the advances which have been made in management of childhood brain tumors and the areas where increased understanding is needed. With the optimal use of surgery and radiotherapy, the majority of children with PNET-MB can be expected to be alive and free of disease 5 years after diagnosis. Based on various factors, including the age of patient, the extent of the disease at the time of diagnosis and histological parameters, it is possible to stratify patients with PNET-MB into ‘risk’ groups. It is unclear whether preoperative or postoperative factors are most predictive of outcome. Patients with factors predictive of a higher likelihood of disease relapse seem to benefit from treatment with chemotherapy. The amount of radiotherpy and the type and amount of chemotherapy which is most efficacious in controlling the disease has yet to be determined. Children surviving PNET-MB are at high risk for endocrinologic and neuropsychologic sequelae. Multiple prospective treatment protocols are now underway, attempting to determine which therapy is best at controlling disease without causing severe long-term damage. A multidisciphnary approach is needed for the treatment of children with PNET-MB.
Angioarchitecture in Experimental Hydrocephalus
Authors: Oka Nobuo , Nakada Jun-ichi , Endo Shunro , Takaku Akira
Published: March 2008

The angioarchitecture of microcorrosion casts in 12 hydrocephalic rats and 5 normal control rats was investigated by using scanning electron microscopy to observe the changes of the vascular bed in the cerebral mantle in hydrocephalus. Reduction in the number and caliber of the capillaries in the white matter was the most remarkable change in the hydrocephalic rats. This change increased in proportion to the severity of hydrocephalus. A similar change was observed in the basal ganglia. The change in the cortical capillaries was mild, but the cortical ‘palisade’ pattern had disappeared in some cases. Three-dimensional consideration of the angioarchitecture by using microvascular casts compared with microangiographic technique in rat hydrocephalus was discussed.
Direct Sagittal Computed Tomography of the Head in Childhood
Authors: Towbin Richard B. , Naidich Thomas P. , Ball, Jr. William S. , Ball, Jr. James B.
Published: March 2008

Direct sagittal computed tomography (CT) was performed on 47 children with pathology in the posterior fossa, orbit, parasellar region, face and ear. The direct sagittal projection provided additional information about the extent of disease and relationship of the pathologic process to the adjacent tissues in 43% of patients studied. Direct sagittal CT has improved spatial resolution over reformatted images. It is readily available, less costly than magnetic resonance and can provide similar useful anatomic information.
Treatment of Brain Tumors in Babies and Very Young Children
Authors: Duffner Patricia K. , Cohen Michael E.
Published: March 2008

Approximately 13% of brain tumors in childhood occur in children less than 2 years of age. Although the survivals of older children with certain forms of brain tumors have increased over the past 20 years, this treatment has been least effective in very young children with brain tumors. These poor survival rates may be due in part to the highly malignant nature of the neoplasms; the delay in diagnosis because of low index of suspicion; the large bulk of tumor found at presentation, and the reduction in radiation dosage to the central nervous system. Since standard treatment has produced both poor results and severe neurotoxicity, new approaches have been sought. A conservative approach is recommended in some children with optic gliomas and low-grade supratentorial astrocytomas. Radiation is deferred until the child reaches 3–4 years of age when he can better tolerate its effects on the CNS. Current therapy of malignant brain tumors of infancy is not only toxic, but inadequate. There are several centers which currently use postoperative chemotherapy and delayed radiation. Early results have been encouraging and neurotoxicity has been low.
Lyophilized Cadaver Dura Mater for Primary Repair of Myelomeningoceles
Authors: Park T.S. , Delashaw Johnny B. , Broaddus William C. , Vollmer Dennis G.
Published: March 2008

The authors have used lyophilized dura mater for primary repair of five large myelomeningoceles. Their preliminary experience suggests that lyophilized dura can be used safely in myelodysplastic neonates. Furthermore, the use of lyophilized dura simplifies significantly the surgical repair of myelomeningoceles.
Correlation between Delta and Spindle Components in Compressed Spectral Array during Nocturnal Sleep in Mentally Retarded Children
Authors: Shibagaki M. , Kiyono S. , Takeuchi T.
Published: March 2008

The correlation between the delta and spindle components in compressed spectral array in 96 mentally retarded children (from 3 months to 12 years of age) was studied throughout nocturnal sleep. In 57 (59.4%, group A) of 96 subjects, periodic changes of delta and spindle rhythm powers were noted in sleep EEG. In 29 subjects (30.2% group B), delta but not spindle rhythm powers were found, and in the remaining 10 subjects (10.4%, group C) neither delta nor spindle rhythm powers were found throughout nocturnal sleep. A significant increase in abnormal clinical EEG was found in groups B and C as compared with group A. A significant decrease in the developmental quotient (DQ) was found in groups B and C as compared with group A. A significant decrease in DQ was also found in group C as compared with group B.
Venous Malformation Mimicking a Posterior Fossa Tumor in a Young Child
Authors: Kutscher Martin L. , Shinnar Shlomo , Levan Anne-Marie , Llena Josefina , Shapiro Kenneth
Published: March 2008

Symptomatic venous malformations are uncommon at any age. They are extremely rare in the first decade of life. We describe a 3.5-year-old child with a cerebellar venous malformation presenting with a subacute course mimicking the more common posterior fossa tumors. The child’s symptoms were due to a posterior fossa hemorrhage; she subsequently did well. The clinical, CT, angiographic, and pathologic features are described. Venous malformations should be included in the differential diagnosis of young children with evidence of posterior fossa disease.
Anterior Spinal Artery Syndrome
Authors: Blennow Gösta , Starck Lena
Published: March 2008

The diagnosis of the anterior spinal artery syndrome can be reached with fair certainly by thorough clinical examination and knowledge of the underlying neuroanatomy. Sudden onset of para- or quadripareses, and dissociated sensory loss are the headmarks. Seven cases are reported who all had this diagnosis. The etiology is discussed with special reference to the possibility of a traumatic origin. The prognosis is variable, in spite of considerable initial impairment restitution in certain cases can be very good. Proposals for investigations are given.
Dandy-Walker Syndrome and Associated Anomalies
Authors: Golden Jeffrey A. , Rorke Lucy B. , Bruce Derek A.
Published: March 2008

This is a retrospective study of 28 infants and children with Dandy-Walker syndrome: 16 had a complete postmortem examination and 12 are currently being followed clinically. Eleven of sixteen in the first category had hydrocephalus; 10 of those 11 had other central nervous system malformations, and 6 also had visceral anomalies. Four of the five without hydrocephalus had complex cardiac malformations and other visceral anomalies whereas 1 died as a consequence of acute myelogenous leukemia. The 12 children still under surveillance manifest no clinical evidence of significant visceral or central nervous system malformation, although 1 has CT scan evidence of agenesis of the corpus callosum. The majority (9/12) of these children have normal intelligence although 40% have focal neurologic deficits or gait abnormalities and 60% have an abnormal EEG. Frequent association of other malformations and/or visceral anomalies in children with Dandy-Walker syndrome make it difficult to propose a unifying hypothesis relative to embryogenesis of the syndrome.
Dandy-Walker Syndrome Revisited
Authors: Maria Bernard L.M. , Zinreich James , Carson Benjamin C. , Rosenbaum Arthur E. , Freeman John M.
Published: March 2008

The Dandy-Walker syndrome is said to be associated with a high incidence of mental retardation and motor dysfunction leading some to suggest termination of the affected fetus in utero. Since this view seemed contrary to our experience, we reviewed 19 patients with the Dandy-Walker syndrome diagnosed from 1966 to 1983. Thirteen patients with Dandy-Walker syndrome were diagnosed before 6 months of age and followed for more than years (mean: 10 years). In the absence of other associated major abnormalities, 7 of 8 (88%) are functioning well and have attended regular schools. Two of these seven have had special help with isolated learning problems. None of these patients have significant motor disability. One additional patient with normal intellectual and motor function died of acute shunt malfunction. In the remaining 4 patients with Dandy-Walker-associated abnormalities, (75%) have severe intellectual retardation and spastic cerebral palsy. Five of six patients (83%) with Dandy-Walker syndrome diagnosed after 6 months of age are normal. The outcome of patients with Dandy-Walker syndrome appears far better than previously reported.
Intraventricular Hemorrhage in the Newborn Beagle Puppy
Authors: Johnson Dennis L. , Getson Pamela , Shaer Catherine , O’Donnell Regina
Published: March 2008

The term beagle puppy has been used extensively as an experimental model of intraventricular hemorrhage (IVH) in the preterm infant. Fourteen variables were selected which were considered theoretically important to both the model and the preterm infant, and the data were subjected to discriminant analysis. Use of only three variables in combination correctly predicted whether an animal would sustain germinal matrix hemorrhage: baseline blood pressure, PCO, and temperature. The model does not involve ischemia, which appears to be the sentinel but not singular insult in the IVH of preterm infants.
Neurosonographic and Biochemical Correlates of Periventricular Leukomalacia in Low-Birth-Weight Infants
Authors: Amato M. , Gambon R. , Von Muralt G. , Huber P.
Published: March 2008

The incidence of periventricular leukomalacia (PVL) was investigated by ultrasound in a group of 119 consecutively scanned low-birth-weight infants during a period of 3 years. The overall incidence of PVL was 6.7 % while the incidence of peri-intraventricular hemorrhage was 44.5%. Ultrasound evidence of posthemorrhagic lesions was seen early and related strongly to follow-up findings. Evidence of cystic degeneration appeared later. The presence of PVL was confirmed by computed tomography at 5 months of age and anticipated by serial measurements of creatine kinase brain isoenzyme performed in the first 60 h of life. A significant correlation was observed between neurosonographic findings of PVL and high cord blood values of enzymatic pattern (p < 0.02). Both were correlated with poor neurodevelopmental outcome at 12 months corrected age.
Thyroid Function in Children with Long-Term Anticonvulsant Treatment
Authors: Tanaka Kazuhiro , Kodama Soichi , Yokoyama Sumiyoshi , Komatsu Mikio , Konishi Hideki , Momota Keiko , Matsuo Tamotsu
Published: March 2008

Thyroid functions were studied in 287 children given long-term treatment with anticonvulsants. Of these 287 patients, 26 were treated with carbamazepine (CBZ) alone, 63 with phenobarbital (PB) alone, 66 with sodium valproate (VPA) alone and 132 with combination therapy. Serum triiodothyronine (T3) and thyroxine (T4) concentrations were decreased after more than 3 years of treatment with CBZ. The regression line of serum T4 concentrations against serum CBZ concentrations indicated a negative correlation. Serum T3 and T4 concentrations were decreased after more than 6 years of treatment with PB, although there was no statistical significance in the relationship between serum T4 and PB concentrations. There was no significant decrease in serum T3 or T4 concentration in patients treated with VPA. Serum-free T4 and thyroxine-binding globulin were decreased and serum thyrotropic hormone concentration was increased in patients with low serum T4 concentrations. We conclude that thyroid functions should be examined in children given long-term treatment with anticonvulsants.
Survival from Symptomatic Nemaline Myopathy in the Newborn Period
Authors: Roig Manuel , Hernández Miguel A. , Salcedo Salvador
Published: March 2008

We report a patient with nemaline myopathy of sporadic genetical character, who presented with respiratory insufficiency in the neonatal period and has been followed for 4 years. The evolution towards progressive improvement is described in detail, particularly the therapeutic problems posed by the respiratory insufficiency and the alterations of swallowing. This is the first patient reported in the literature who has survived symptomatic nemaline myopathy presenting in the newborn period. The experience of this case may justify the use of exceptional therapeutic measures in other patients.
Current Management of Low-Grade Astrocytomas of Childhood
Authors: Sutton Leslie N.
Published: March 2008

Astrocytomas are the most frequently encountered brain neoplasms in the pediatric age group. Current management strategies consist of surgery, radiation therapy, chemotherapy and immunotherapy, although the optimal treatment is yet to be defined. It is hoped that new treatment modalities, and reassessment of current regimens will improve outcome, better in terms of survival and quality of life.
Subsarcolemmal Vermiform Deposits in Skeletal Muscle, Associated with Familial Cardiomyopathy: Report of Two Cases of a New Entity
Authors: Calderon Ana , Becker Laurence.E. , Murphy Gordon
Published: March 2008

Distinctive subsarcolemmal acid phosphatase-positive crescents were found in many muscle fibers of triceps muscle biopsies from 2 siblings with cardiomyopathy. The crescents appeared as dense osmophilic material in irregular vermiform arrays on electron microscopy. However, both patients had clinically normal skeletal muscle function. These 2 cases are believed to represent a rare familial myopathy affecting cardiac and skeletal muscle.
Slit-Ventricle Syndrome: Review of 15 Cases
Authors: McLaurin Robert L. , Olivi Alessandro
Published: March 2008

The slit-ventricle syndrome (SVS) has been the subject of diverse opinions and recommendations during the past 2 decades. In an effort to define the clinical features of SVS and to make recommendations concerning management we have reviewed 15 cases treated by a fairly uniform technique during the past 5 years. The syndrome consists of: (1) intermittent, but self-limiting episodes resembling shunt malfunction, usually lasting a few days, (2) nonfilling of the pumping device after compression, and (3) a slit-like ventricular system on CT scan. In all but 2 patients the initial shunt was performed in infancy. The mean interval from the initial shunt to treatment of SVS was 6 years. The age range at onset of SVS varied from 2 to 17 years with a mean of 7 years. All patients in this series were relieved of symptoms by placement of an antisiphon device and, in most patients, upgrading the valve resistance. Analysis of this series has led to the following conclusions: (1) SVS is a characteristic clinical entity, usually distinguishable from persistent shunt malfunction and from low-pressure headache, (2) the pathogenesis is intermittent obstruction of the ventricular catheter, (3) there is no good evidence that changes of brain compliance or La Place principles apply, and (4) placement of antisiphon device and upgrading valve resistance are effective treatments.
Fetal Subdural Hematoma: Diagnosis in utero
Authors: Mateos F. , Esteban J. , Ramos J.T. , Martin-Puerto M.J. , Miralles M. , Ozaita G. , Martínez-Portillo J.
Published: March 2008

A subdural hematoma was diagnosed antepartum in an infant by means of sonography. An elective caesarean section was performed because cephalopelvic disproportion, and later the hematoma was drained with a poor evolution due to basal ganglia and intraventricular hemorrhage. No etiological factor of bleeding was found. This is the third published case of fetal subdural hematoma diagnosed antepartum.
Insidious Hydrocephalus in the Preterm Newborn following Discharge from the Nursery
Authors: James Hector E. , Bejar R. , Merritt A.T. , Mannino F. , Vaucher Y. , Coen R.
Published: March 2008

Posthemorrhagic ventriculomegaly may be due to perinatal brain damage and consequent cerebral atrophy, or represent progressive hydrocephalus due to impairment of CSF flow and reabsorption. ‘Arrested’ hydrocephalus occurs when the CSF pathways are adequate and, hence, intracranial hypertension no longer exists. The differential diagnosis is often difficult and insidious progressive hydrocephalus should always be a concern in the high-risk preterm newborn. We report 10 preterm infants who were discharged with the diagnosis of arrested hydrocephalus (7) or cerebral atrophy (3), and who later developed progressive severe hydrocephalus 1.5–15 months after the discharge. Five had been treated with intermittent lumbar punctures, while 4 had not received any treatment prior to nursery discharge. One infant died prior to the shunt procedure. Progressive insidious hydrocephalus should be suspected in preterm infants with intracranial hemorrhage following discharge from the nursery.
Selective Posterior Rhizotomy for Treatment of Progressive Spasticity in Patients with Myelomeningocele
Authors: Storrs Bruce B.
Published: March 2008

This preliminary report describes two cases of progressive spasticity and scoliosis in children with myelomeningocele. Both patients had evidence of tethered spinal cord. Untethering of the spinal cord was successful in arresting the scoliosis but not the spasticity. Selective posterior rhizotomy was successful in resolving the spasticity in both cases. The technique of selective posterior rhizotomy is reviewed.
Cerebral Metastasis from Malignant Pulmonary Fibrous Histiocytoma
Authors: Epstein Nancy E. , Sundrani Shanker , Rosenthal Alan D.
Published: March 2008

Within 3 months, a 13-year-old girl had two thoracotomies performed for resection of pulmonary primary and metastatic malignant fibrous histiocytoma (MFH). Seven months following the second operation, a right frontal cerebral metastasis was diagnosed as responsible for the sudden onset of a left hemiparesis. Although she recovered from the craniotomy, and tolerated radiation and chemotherapy, she succumbed 5 months later to recurrent pulmonary involvement. This case report uniquely records the spread of MFH to the brain in a child. The unusual appearance of this tumor in the pediatric age group and its rare presentation intracranially is explored within the context of the current pediatric literature.
Malignant Supratentorial Gliomas in Childhood
Authors: Prados Michael , Levin Victor
Published: March 2008

In this review, we try to review concisely the incidence, genetic predilections, classification, and diagnosis of supratentorial gliomas (astrocytomas, oligodendrogliomas, and mixed gliomas) in children. While surgery and irradiation present the most commonly used and valuable modes of treatment, chemotherapy is a valuable adjunctive therapy. Used either with surgery and radiation in primary untreated patients or at recurrence (progression), chemotherapy can increase useful life. The late effects of treatment are primarily endocrine deficiencies and intellectual impairment; nevertheless, they are not of a degree to preclude aggressive therapy. In order to improve therapy, and because of the small number of patients with supratentorial tumors, collaborative multi-institutional trials should be encouraged.
Management of Primary Intracranial Germ Cell Tumors of Childhood
Authors: Allen Jeffrey C.
Published: March 2008

Primary CNS germ cell tumors present several biologic and therapeutic concerns. Their predilection to midline locations (pineal and suprasellar) has discouraged until recently attempts at biopsy, much less radical surgical resection. Their heterogeneity (germinomas, embryonal carcinoma, endodermal sinus tumor, choriocarcinoma and malignant teratoma) has made uniform treatment planning unrealistic. Their rarity (<50 cases per year in USA) precludes a large institutional experience. More recently, with the use of microsurgical techniques, major resections are possible and accurate pretreatment histologic diagnosis can be established. Following surgery, patients should be staged with cerebrospinal fluid (CSF) cytology, serum and CSF tumor markers (α-fetoprotein and human β-chorionic gonadotrophin) and myelography. The majority of patients will present with disseminated disease and require craniospinal radiotherapy. Chemotherapy should eventually prove to be an effective adjuvant treatment modality, not only for the highly radiosensitive germinoma with a good prognosis (5-year survival >60%) following radiotherapy alone, but also for the radioinsensitive nongerminoma germ cell variants where longterm survival is unusual. Chemotherapy agents such as cyclophosphamide, cisplatin, vinblastine, VP-16 and bleomycin appear to be useful for patients with newly diagnosed and recurrent disease.
Pigmented Primitive Neuroectodermal Tumor with Multipotential Differentiation in Cerebellum (Pigmented Medullomyoblastoma)
Authors: Kalimo Hannu , Paljärvi Leo , Ekfors Tauno , Pelliniemi Lauri J.
Published: March 2008

A 5-year-old girl had a midline cerebellar primitive neuroectodermal tumor (PNET) with neuronal, astrocytic, rhabdomyoblastic and melanocytic differentiation (a variant of pigmented medullomyoblastoma). The tumor recurred following surgery and radiation therapy and the child died within 6 months. Autopsy revealed spread into cerebellar hemispheres, subarachnoidal space and brain stem. Neuronal differentiation was verified by electron microscopy and antiserum against neurofilament protein. Neoplastic astrocytes were identified by use of antisera to glial fibrillary acidic and S-100 proteins. Rhabdomyoblasts were identified by the use of antisera against skeletal muscle type 2 myosin and myoglobin, as well as by electron microscopy. This tumor is compared with 20 previously reported cases of cerebellar PNETs containing muscle, 2 of which showed a similar pattern of multipotential differentiation. The oncogenesis of these tumors is in dispute although origin of both the neuroectodermal and mesenchymal components from the neural crest (mesectodermal differentiation) is most widely accepted.
Sphenoid Wing Meningioma Causing a Proptosis in a Child
Authors: Heiferman Kenneth S. , Tomita Tadanori , Lissner Gary S. , Gonzalez-Crussi Frank
Published: March 2008

A sphenoid wing meningioma causing a progressive painless proptosis is reported in a 5-year-old boy. The diagnostic evaluation and surgical management of this case are reported. A review of the literature indicates this is the youngest patient with sphenoid wing meningioma.
Recurrent Brain Tumors in Children
Authors: Friedman Henry S. , Oakes Jerry
Published: March 2008

A significant cohort of children with central nervous system tumors will fail initial therapeutic intervention and demonstrate recurrent disease. Treatment of children with these recurrent tumors requires a comprehensive multidisciplinary approach, with consideration of both histologic classification and anatomic location of the tumor. The choice of the specific therapeutic modality selected should reflect institutional experience and seek-to advance the treatment of brain tumors in children. Children at Duke Medical Center with biologically indolent tumors and clinical or significant radiographic evidence of recurrence (low-grade/benign tumors with greater than 2-year recurrence-free interval) are usually treated by repeat surgical intervention. Children whose initial therapy did not include radiotherapy are considered for irradiation, while those previously irradiated are observed or occasionally considered for chemotherapy. Children with biologically aggressive tumors (high-grade lesions or early recurrence) are offered neurosurgical intervention if there is considerable mass effect, and subsequently enrolled on phase II chemotherapy protocols, reflecting an institutional commitment to exploring the role of this modality in the treatment of pediatric brain tumors. Children with recurrent brain tumors may derive benefit from this aggressive comprehensive approach, achieving a prolonged and quality survival.
Intracranial Arteriovenous Anomalies of Infancy: Modern Concepts
Authors: Godersky John C. , Menezes Arnold H.
Published: March 2008

A variety of cerebrovascular abnormalities may become manifest in the first years of life. We present our recent experience with 7 children, ages 1 week to 7 months of age, who presented with congestive heart failure and progressive head enlargement (3), heart failure alone (1) and head enlargement alone (2). The anomalies include vein of Galen aneurysm (3), complex arteriovenous fistula (2) and posterior fossa dural arteriovenous malformations (2). Computed tomographic scanning was invaluable in delineating the site of the lesion while arterial digital subtraction angiography was utilized to obtain the high-quality images, reduced dye volume and rapid filming. Direct surgical intervention was accomplished in 5 cases, while embolization was utilized in 2. One child underwent direct partial occlusion and embolization. Congestive heart failure resolved in 2 of 3 operated cases and improved in those undergoing embolization. Hydrocephalus improved in 2 cases with the elimination of the fistula. Morbidity included seizures and a hemorrhagic infarction in 1 case. Neurologic outcome was satisfactory and there was no surgical mortality.
Choroid Plexus Tumors in Children: Immunohistochemical and Scanning-Electron-Microscopic Features
Authors: Felix I. , Phudhichareonrat S. , Halliday W.C. , Becker L.E.
Published: March 2008

The 30 choroid plexus tumors recorded in a large pediatric tumor registry were reviewed. The 22 choroid plexus papillomas and 8 choroid plexus carcinomas represented 2.1% of all brain tumors in the registry. The patients (19 boys, 11 girls) were aged 3 months to 12 years (mean: 2 years 9 months). All tumors and four samples of normal choroid plexus were examined with conventional histology, 9 tumors were examined with electron microscopy and 24 using immunohistochemical techniques with antisera against glial fibrillary acidic protein, S-100 protein, epidermal cytokeratin, internal organ cytokeratin and carcinoembryonic antigen. Seven of the tumors (1 carcinoma and 6 papillomas) had some positive cells with antiserum against glial fibrillary acidic protein. Twenty-seven of the tumors (90%) and all normal choroid plexus samples were positive with antiserum to S-100 protein. Some tumor cells from both types of tumor and the normal choroid plexus reacted positively with internal organ cytokeratin antiserum, but all tissue samples were nonreactive with epidermal cytokeratin antiserum. Ultrastructurally, both papillomas and carcinomas displayed cytoplasmic intermediate filaments. Transmission and/or scanning electron microscopy revealed cilia in all choroid plexus papillomas. Frequently, the cilia had an abnormal arrangement, varying from 5 + 0 to 8 + 1. This study demonstrates that tumor cells of choroid plexus origin are characterized by the presence of S-100 protein and internal organ cytokeratin antiserum. Cilia, either normal or abnormal, are consistently identified in papillomas but rarely and with difficulty in carcinomas. Carcinoembryonic antigen is seen in choroid plexus carcinomas but not papillomas.
Vitamin A-Induced Suppression/Enhancement of Protein Glycosylation and Neurulation
Authors: Ersahin Yusuf , Higbee Russell G. , Vanden Hoek T. , McLone David G. , Knepper Paul A.
Published: March 2008

Glycoconjugates play major roles in many cellular functions, e.g. cell migration and cell-to-cell adherence, which are involved in neurulation. The maternal administration of vitamin A on gestation day 8.5 and 9.0 resulted in a high percentage of primary and secondary neurulation defects in gestation day 12 mouse embryos. The neuroepithelium of normal and abnormal embryos was analyzed by one- and two-dimensional sodium dodecyl sulfate polyacrylamide gel electrophoresis and one-dimensional Western blots using concanavalin A (Con A) and peroxidase-conjugated wheat germ agglutinin (WGA) lectins. In vitamin A abnormal embryos, WGA binding was decreased to glycoproteins with apparent molecular weights of 15,000 and 30,000 daltons on Western blots, whereas in vitamin A normal embryos, WGA binding was increased to these glycoproteins on Western blots. Computer-aided fluorescence microscopy using fluorescein isothiocyanate (FITC)-conjugated lectins on 1-µm araldite plastic sections indicated a decrease in FITC-WGA binding to the free surface of nonneurulated neuroepithelium. These results suggest: (1) vitamin A administration may have induced a suppression of WGA-binding carbohydrate residues on 15,000- and 30,000-dalton glycoproteins in abnormal embryos, and (2) modification in the type, amount, and distribution of glycoconjugates may provide a basis for the cellular mechanisms of abnormal development of the neural tube.
Neurobehavioral Sequelae of Minor Head Injuries in Children
Authors: Farmer Mychelle Y. , Singer Harvey S. , Mellits David , Hall David , Charney Evan
Published: March 2008

The incidence of neurobehavioral sequelae in children who have sustained minor head injury is controversial. Following an emergency room visit, behavioral symptoms in 247 children with mild head injuries were compared to those in 280 children with trauma to other regions of the body. Serial telephone interviews showed that complaints of irritability, clinging behavior and sleep disturbances were common in both groups, though headaches were a more frequent problem in the head-trauma patients. Virtually all symptoms were transient. Our results demonstrate a high incidence of behavioral sequelae in children after minor head injury and suggest that physicians should counsel parents about this brief functional morbidity.
Current Management of Brain Stem Gliomas
Authors: Edwards Michael S.B. , Prados Michael
Published: March 2008

Brain stem gliomas constitute 10–20% of central nervous system tumors in children. Management of these tumors remains controversial. In general, the prognosis for long-term survival is poor, but brain stem gliomas represent a heterogeneous population of tumors, some of which have a better prognosis than others. Improvements in diagnostic imaging techniques, particularly the availability of magnetic resonance imaging, provide the means to differentiate among these populations. Advances in radiation therapy may provide the means to improve survival. In this article we review the reported series and the present state of the art for the diagnosis and management of children with brain stem gliomas.
‘Slit-Ventricle Syndrome’: Etiology and Treatment
Authors: Epstein Fred , Lapras Claude , Wisoff Jeffrey H.
Published: March 2008

Between 1970 and 1986, 20 patients were treated for the ‘Slit-ventricle syndrome’. Six patients had intermittent proximal shunt malfunction and 14 children had increased intracranial pressure with normal shunt function. All of the children in the second group had a relatively small calvarium. Treatment consisted of proximal shunt revision in the first group and a calvarial expansion procedure in the second. The authors discuss the differential diagnosis of the slit-ventricle syndrome and offer guidelines to appropriate treatment.
Role of Magnetic Resonance Imaging and Three-Dimensional Computerized Tomography in Craniovertebral Junction Anomalies
Authors: Pappas Conrad T.E. , Rekate Harold L.
Published: March 2008

The surgical management of craniovertebral junction abnormalities depends on accurate radiological identification of the static and dynamic anatomical relationships. Four cases are presented to show the importance of flexion/extension magnetic resonance imaging and three-dimensional computerized tomography scanning in the evaluation of these relationships. With these techniques, anterior and/or posterior decompressive procedures can be appropriately planned to reduce and stabilize craniovertebral abnormalities. Long-term neural deficits can be reversed by these procedures.
Choroid Plexus Papillomas of Neonates, Infants and Children
Authors: Tomita Tadanori , McLone David G. , Flannery Ann M.
Published: March 2008

Case descriptions of 17 patients with choroid plexus papillomas of neonates, infants and children are presented. Fourteen (82%) were diagnosed and treated during the first 24 months of life (5 were in the neonatal period). Choroid plexus papillomas were located in the lateral ventricle in 11 (bilateral in 1), the third ventricle in 4, both the lateral and third ventricle in 1 and the fourth ventricle in 1. All patients were evaluated by computed tomography. All tumors were excised and histologically verified. There were no surgical or case mortalities. Ten patients needed permanent shunting postoperatively. Follow-up observations show that 13 patients exhibit normal neurological and psychomotor development. Three are retarded and have seizure disorders. One is hemiparetic but normal otherwise. Aggressive surgical resection and appropriate management of associated hydrocephalus shoud be performed for the patients with choroid plexus papillomas.
Sylvian Fissure Meningioma in a Child: Case Report and Review of the Literature
Authors: Silbergeld Daniel , Berger Mitchel , Griffin Brian
Published: March 2008

A case report of a Sylvian fissure meningioma in a 4-year-old female is presented. Childhood meningiomas are discussed, and the literature is reviewed with respect to the location of the lesion in the Sylvian fissure. The role of radiotherapy following subtotal resection of meningiomas is also described.
Ependymomas in Children
Authors: Kun Larry E. , Kovnar Edward H. , Sanford Robert A.
Published: March 2008

Ependymomas originate throughout the central nervous system (CNS), representing 10–12% of CNS tumors in children. The majority occur in children less than 5 years of age. Tumors occur most often in the 4th ventricular region. Current controversies regarding ependymomas include histologic categorization or ‘differentiation’. It is estimated that approximately 25% of childhood tumors are appropriately categorized as anaplastic or ‘high-grade’ lesions. Surgical resection is curative for tumors originating in the cauda equina; recent data suggest that surgery alone has achieved disease control in 75% of intramedullary tumors. For intracranial ependymomas, postoperative irradiation has been shown to improve survival in series dating from the 1960–1980 era. Debate regarding the necessary volume for radiation therapy (i.e. local treatment or craniospinal irradiation) centers on the reported rate of neuraxis seeding. An overall incidence of 12%has been documented in the literature, with clinicohistologic correlation suggesting that anaplastic or high-grade infratentorial ependymomas are associated with the highest frequency of subarachnoid spread. Recent reports of chemotherapy show response to several single agents, with no clear documentation that adjuvant chemotherapy has improved survival to date. Current studies utilizing preirradiation chemotherapy address tumor response and ultimate control in a sequence of surgery, chemotherapy, and irradiation primarily in very young children or those with anaplastic or high-grade tumors.
Parietal Cephaloceles: Radiographic and Magnetic Resonance Imaging Evaluation
Authors: Curnes John T. , Oakes Jerry
Published: March 2008

Three patients with parietal cephaloceles underwent evaluation and treatment at Duke University Medical Center between 1984 and 1987. All presented within the first 2 years of life with painful swelling near the vertex of the head. All patients had skull films and computed tomography, and two underwent magnetic resonance imaging (MRI). All 3 children had associated hindbrain deformities; two with Dandy-Walker malformation, the third with a Chiari II malformation. Each child eventually developed hydrocephalus. MRI is the procedure of choice to evaluate these patients, providing direct sagittal imaging of the posterior fossa and craniocervical junction, and displaying communication of the cephalocele with intracranial structures as well as associated venous vascular anomalies.
Ventricular Volume Regulation: A Mathematical Model and Computer Simulation
Authors: Rekate Harold L. , Brodkey Jason A. , Chizeck Howard J. , El Sakka W. , Ko Wen H.
Published: March 2008

A mathematical model of ventricular volume regulation based on fluid mechanical principles has been constructed using a systems engineering approach. The parameters used in the model are based on clinical observation, laboratory investigation, and presumptions that will be tested later. The model was constructed to be the basis of a computer simulation. Using the computer simulation, information obtained from the literature and laboratory hypotheses regarding pathophysiology, several enigmatic conditions were tested. The model predicted that overproduction of cerebrospinal fluid, as in the case of choroid plexus papilloma, could by itself lead to distention of the ventricular system. In simulating pseudotumor cerebri, if cerebrospinal fluid absorption at the arachnoid villi is impaired and the brain itself is rendered incompressible by swelling, intracranial pressure rises and ventricular volume diminishes. Conversely, in normal-pressure hydrocephalus, if cerebrospinal fluid flow is restricted between the spinal and cortical subarachnoid spaces and the brain is made more compressible, the ventricular volume increases with minimal increases in intracranial pressure. This mathematical model and its associated computer simulation is useful in predicting the behavior of the volume of the cerebral ventricles to a variety of pathological phenomena.
Neurogenic Arthrogryposis multiplex congenita: Clinical and Muscle Biopsy Findings
Authors: Adams Coleen , Becker Laurence E. , Murphy Gordon
Published: March 2008

Thirteen cases of arthrogryposis multiplex congenita without evidence of spinal muscular atrophy, congenital muscular dystrophy, or structural myopathy were reviewed. Family history, consanguinity, pregnancy, delivery, number and severity of contractures, and outcome were evaluated. Laboratory investigations had been performed and a biopsy of muscle from an affected limb had been examined histochemically and by electron microscopy. Five biopsies showed fiber type 1 predominance and three had type 2 predominance. Patterns of fiber-type predominance may have resulted from altered neural influence leading to impaired maturation of type 1 or 2 motor units. Nine patients had been followed up for 3–8 years. Two were still not walking at age 8 years. For the 6 who were walking, the mean age at ambulation was 4.7 years. There was no deterioration in power. Muscle biopsy is recommended in arthrogryposis multiplex congenita. Identification of a probable neurogenic arthrogryposis multiplex congenita is important because the condition is not progressive and is apparently not transmitted genetically.
Cerebral Hemiatrophy – Correlation of Human with Animal Experimental Data
Authors: Ford Lisa , De Courten-Myers Gabrielle M. , Mandybur Thaddeus , Myers Ronald E.
Published: March 2008

We report the neuropathologic findings in a 63-year-old white male with a history of birth asphyxia, cerebral palsy, seizures and mild mental retardation in conjunction with similar brain pathologic findings in animal models of perinatal asphyxia. The human case showed a left cerebral hemispheric hemiatrophy associated with an extensive ulegyria involving all cerebral lobes on that side and a single microscopic focus of cortical atrophy in the right hemisphere. Among a large number of experimental perinatal asphyctic exposures only an occasional animal, like the human case described, showed unilateral hemispheric injury with softening and necrosis if examined early and ulegyria with hemispheric hemiatrophy if examined late. The present paper suggests that perinatal asphyxia under specific pathophysiologic conditions may cause unilateral brain injury. Our experimental studies suggest the specific condition of perinatal asphyxia potentially causing unilateral or asymmetrical brain damage is marked hypoxemia combined with substantial reductions in blood pressure but without circulatory collapse. Given these conditions, the asymmetry of the brain damage likely reflects fetal head position within the gravitational field relative to the heart. With disturbed cerebral blood flow autoregulation from asphyxia, the gravitational field likely accentuates the ischemia of those brain areas most elevated above the level of the heart. Thus, we postulate head position may play a pivotal role in defining brain regions that are damaged in hypotensive perinatal asphyxia. This interpretation may affect the intensive care of hypoxemic, hypotensive newborns aimed at minimizing the risk of brain damage.
Use of the ‘H’ Reflex Recovery Curve in Selective Posterior Rhizotomy
Authors: Storrs Bruce B. , Nishida Takashi
Published: March 2008

Selective posterior rhizotomy is a safe and effective treatment in carefully selected patients. It produces objective and lasting changes in muscle tone and function without serious sensory complications. The Η’ reflex recovery curve technique allows objective evaluation of sensory rootlets and a physiologic rationale for the selection of abnormal rootlets. In 95 patients this technique has provided good results without significant sensory compromise.
Pituitary Stalk Agenesis: Magnetic Resonance Imaging of ‘Ectopic Posterior Lobe’ with Surgical Correlation
Authors: Kaufman Bruce A. , Kaufman Benjamin , Mapstone Timothy B.
Published: March 2008

A patient with anterior pituitary deficiencies, without diabetes insipidus, was found to have a retrochiasmatic mass in association with a large middle fossa cyst on CT and MRI scans, and no tissue in the sella (‘empty sella’). At surgery, the pituitary stalk was absent. The retrochiasmatic mass was biopsied and shown to be consistent with pituitary stalk. Recent descriptions of MR visualization of an ‘ectopic’ posterior pituitary lobe are discussed in relation to this case of agenesis of the pituitary stalk and posterior lobe.