bluuebunny/crossref_metadata_2025 huggingface dataset:
Leucine-Rich Repeat Kinase 2 Colocalizes with α-Synuclein in Parkinson’s Disease, but Not Tau-Containing Deposits in Tauopathies
Authors: Perry George , Zhu Xiongwei , Babar Asim K. , Siedlak Sandra L. , Yang Qiwei , Ito Genta , Iwatsubo Takeshi , Smith Mark A. , Chen Shu G.
Published: March 2008

Background: Mutations in leucine-rich repeat kinase 2 (LRRK2) are thus far the most frequent genetic cause associated with autosomal dominant and idiopathic Parkinson’s disease. Objective:To examine whether LRRK2 is directly associated with the pathological structures of Parkinson’s disease, dementia with Lewy bodies, and other related disorders using highly specific antibodies to LRRK2. Results:LRRK2 antibodies strongly labeled brainstem and cortical Lewy bodies, the pathological hallmarks of Parkinson’s disease and dementia with Lewy bodies, respectively. We found that 20–100% (mean 60%) of α-synuclein-positive Lewy bodies contained LRRK2. While antibodies raised against various regions of LRRK2 were previously shown to label recombinant LRRK2 on Western blots, only antibodies raised against the N- and C-termini, but not the regions containing folded protein domains of LRRK2, immunolabeled Lewy bodies. In Alzheimer’s disease, Hirano bodies were found to contain LRRK2 and the neurofibrillary tangles in progressive supranuclear palsy remained unlabeled. Conclusions: Information on the cellular localization of LRRK2 under normal and pathological conditions will deepen our understanding of its functions and molecular pathways relevant to the progression of Parkinson’s disease and related disorders.
Polymorphisms at the Paraoxonase 1 L55M and Q192R Loci Affect the Pathophysiology of Alzheimer’s Disease: Emphasis on the Cholinergic System and Beta-Amyloid Levels
Authors: Leduc Valerie , Poirier Judes
Published: March 2008

Background: Paraoxonase 1 (PON1) functions to protect the cholinergic system against nerve gases and the organophosphate family of pesticides. Recent studies have shown that polymorphisms at the PON1 L55M and Q192R loci might affect individual susceptibility to experience-derived and environmental events such as the exposure to inhibitors of cholinesterase (ChEIs). Objective: ChEI therapy being the treatment of choice for mild-to-moderate Alzheimer’s disease (AD) patients, we determined whether genetic variations in the PON1 loci are associated with AD risk and whether they affect brain choline acetyltransferase (CHAT) activity, nicotinic receptor density, and β-amyloid (Aβ) levels in different regions of AD and age-matched control subjects. Methods: This pilot genetic study used a small cohort of brains from autopsy-confirmed AD patients and age-matched controls from the Douglas Hospital Brain Bank, Quebec, Canada. Results: The frequency of the M55M genotype at the PON1 L55M locus was found to be significantly increased in AD patients relative to age-matched controls (p < 0.05). Significant associations were observed between the PON1 L55M and Q192R polymorphisms and frontal cortex Aβ levels as well as CHAT activity and nicotinic receptor density in the temporal cortex. Conclusions: Our results suggest a prominent role for PON1 in the pathophysiology of common AD with a marked impact on the cholinergic system and Aβ levels in the brain.
Galanin Hyperinnervation Upregulates Choline Acetyltransferase Expression in Cholinergic Basal Forebrain Neurons in Alzheimer’s Disease
Authors: Counts Scott E. , He Bin , Che Shaoli , Ginsberg Stephen D. , Mufson Elliott J.
Published: March 2008

Background: Fibers containing galanin (GAL) enlarge and hyperinnervate cholinergic basal forebrain (CBF) nucleus basalis (NB) neurons in late-stage Alzheimer’s disease (AD), yet the physiological consequences of this phenomenon are unclear. Objective: To determine whether GAL hyperinnervation of cholinergic NB neurons modulates the expression of genes critical to cholinergic transmission [e.g. acetylcholine (ACh) metabolism and ACh receptors] in AD. Methods: Single-cell gene expression profiling was used to compare cholinergic mRNA levels in non-GAL-hyperinnervated NB neurons in tissue autopsied from cases classified as having no cognitive impairment (NCI) or late-stage AD (AD/GAL–) and in GAL-hyperinnervated (AD/GAL+) NB neurons from the same AD subjects. Results: AD/GAL+ cells displayed a significant upregulation in choline acetyltransferase (ChAT) mRNA expression compared to NCI and AD/GAL– cells. Conclusion: GAL fiber hyperinnervation of cholinergic NB neurons upregulates the expression of ChAT, the synthetic enzyme for ACh, suggesting that GAL regulates the cholinergic tone of CBF neurons in AD.
Human Recombinant Butyrylcholinesterase Purified from the Milk of Transgenic Goats Interacts with Beta-Amyloid Fibrils and Suppresses Their Formation in vitro
Authors: Podoly E. , Bruck T. , Diamant S. , Melamed-Book N. , Weiss A. , Huang Y. , Livnah O. , Langermann S. , Wilgus H. , Soreq H.
Published: March 2008

Background: In Alzheimer’s disease (AD), brain butyrylcholinesterase (BChE) co-localizes with β-amyloid (Aβ) fibrils. Aims: In vitro testing of the significance of this phenomenon to AD progress. Methods: A thioflavine T (ThT) fluorogenic assay, photo-induced cross-linking and quantifiable electron microscopy served to compare the effect on Aβ fibril formation induced by highly purified recombinant human BChE (rBChE) produced in the milk of transgenic goats with that of serum-derived human BChE. Results: Both proteins at 1:50 and 1:25 ratios to Aβ dose-dependently prolonged the ThT lag time and reduced the apparent rate of Aβ fibril formation compared to Aβ alone. Photo-induced cross-linking tests showed that rBChE prolonged the persistence of amyloid dimers, trimers and tetramers in solution, whereas Aβ alone facilitated precipitation of such multimers from solution. Transmission electron microscopy showed that rBChE at 1:100 to Aβ prevented the formation of larger, over 150-nm-long, Aβ fibrils and reduced fibril branching compared to Aβ alone as quantified by macro programming of Image Pro® Plus software. Conclusion: Our findings demonstrate that rBChE interacts with Aβ fibrils and can attenuate their formation, extension and branching, suggesting further tests of rBChE, with unlimited supply and no associated health risks, as a therapeutic agent for delaying the formation of amyloid toxic oligomers in AD patients.
Glutamatergic Approaches to the Treatment of Cognitive and Behavioural Symptoms of Alzheimer’s Disease
Authors: Francis Paul T.
Published: March 2008

The glutamatergic system has long been recognised for its role in learning and memory and recent studies indicate an early loss of glutamatergic synapses in Alzheimer’s disease (AD). Efforts to produce drugs which address changes in the glutamatergic system in AD are well advanced (e.g. memantine and drugs in development such as ampakines). Much less is known about the possible role of glutamate in non-cognitive behavioural changes; however, recent data from clinical trials suggest that memantine reduces agitation and aggressive behaviour in AD patients. In this context, it is important to help identify new treatment approaches to replace the use of antipsychotics in this vulnerable population.
Circannual Rhythm of Laboratory Test Parameters among Chronic Haemodialysis Patients
Authors: Yanai Mitsuru , Satomura Atsushi , Uehara Yuki , Murakawa Masaya , Takeuchi Makoto , Kumasaka Kazunari
Published: March 2008

Background/Aims: Seasonal variations in laboratory test results have been pointed out in dialysis patients. Although the mechanism for this phenomenon is not clear, this could result in changes in dialysis and medication prescriptions. We investigated the effect of the circannual rhythm on laboratory test parameters in chronic haemodialysis patients. Methods: Data of 38 laboratory test parameters were collected every month and analyzed for 150 stable haemodialysis patients, with non-linear sine wave regression and paired t test between data of peak and trough months. Results: Serum urea nitrogen, unsaturated iron binding capacity, lactate dehydrogenase, alkaline phosphatase, amylase, and neutrophil count showed significant circannual rhythms with high amplitudes. Additionally, serum creatinine, uric acid, chloride, calcium, phosphate, magnesium, total cholesterol, total protein, leucocyte count, mean corpuscular haemoglobin level, mean corpuscular haemoglobin concentration, and platelet count showed significant circannual rhythms with little amplitudes. Conclusions: The circannual rhythm of laboratory test parameters could be attributed to seasonal variations in food intake. Awareness of these variations should be taken into account in the interpretation of laboratory results.
Comparison of Effects of Total Enteral versus Total Parenteral Nutrition on Ischemia/Reperfusion-Induced Heart Injury in Rats
Authors: Hagiwara S. , Iwasaka H. , Shingu C. , Noguchi T.
Published: March 2008

Background: The long-term effect of nutrition on cardiac function remains to be elucidated. One possible link is the newly discovered gastric hormone ghrelin, which has been reported to be cardioprotective. Aim: The present study examined whether total enteral nutrition (TEN) and total parenteral nutrition (TPN) differ in their modulation of ghrelin production and their effects on cardiac function after ischemia/reperfusion injury. Methods: Rats received isocaloric parenteral or enteral nutrition through implanted vascular catheters or gastrostomy tubes. TEN was administered in a conventional (TEN-C) or immunonutrition (TEN-I) form. After 7 days, serum ghrelin levels were determined by enzyme-linked immunosorbent assays and myocardial function was assessed using the Langendorff isolated heart technique. Results: TEN-I animals had significantly higher plasma ghrelin levels than the other groups. After ischemia/reperfusion injury, left ventricular developed pressure decreased in animals receiving TPN when compared to animals receiving TEN-I. Animals receiving TPN also had significant reductions in their maximal rates of increase and decrease in left ventricular pressure when compared to animals receiving TEN-I (unpaired t test, p < 0.05). Conclusion: TEN-I increases serum levels of ghrelin, which protects cardiac function after ischemic/reperfusion injury. Because TEN-I more effectively protects cardiac function, we recommend it for long-term nutritional support.
SGK1-Dependent Upregulation of Connective Tissue Growth Factor by Angiotensin II
Authors: Hussain Azeemudeen , Wyatt Amanda W. , Wang Kan , Bhandaru Madhuri , Biswas Raja , Avram Diana , Föller Michael , Rexhepaj Rexhep , Friedrich Björn , Ullrich Susanne , Müller Gerhard , Kuhl Dietmar , Risler Teut , Lang Florian
Published: March 2008

Angiotensin II has previously been shown to trigger fibrosis, an effect involving connective tissue growth factor (CTGF). The signaling pathways linking angiotensin II to CTGF formation are, however, incompletely understood. A gene highly expressed in fibrosing tissue is the serum- and glucocorticoid-inducible kinase SGK1. The present study explored whether SGK1 is transcriptionally regulated by angiotensin II and participates in the angiotensin II-dependent regulation of CTGF expression. To this end, experiments have been performed in human kidney fibroblasts and mouse lung fibroblasts from gene-targeted mice lacking SGK1 (sgk1–/–) and their wild-type littermates (sgk1+/+). In human renal fibroblasts, SGK1 and CTGF protein expression were enhanced by angiotensin II (10 nM) within 4 h. In sgk1+/+ mouse fibroblasts, SGK1 transcript levels were significantly increased after 4 h of angiotensin II treatment. Angiotensin II stimulated both transcript and protein abundance of CTGF in fibroblasts from sgk1+/+ mice, effects significantly blunted in fibroblasts of sgk1–/– mice. In conclusion, angiotensin II stimulates the expression of SGK1, which is in turn required for the stimulating effect of angiotensin II on the expression of CTGF. Thus, SGK1 presumably contributes to the profibrotic effect of angiotensin II.
Hyponatremia Revisited: Translating Physiology to Practice
Authors: Hoorn Ewout J. , Zietse Robert
Published: March 2008

The complexity of hyponatremia as a clinical problem is likely caused by the opposite scenarios that accompany this electrolyte disorder regarding pathophysiology (depletional versus dilutional hyponatremia, high versus low vasopressin levels) and therapy (rapid correction to treat cerebral edema versus slow correction to prevent osmotic demyelination, fluid restriction versus fluid resuscitation). For a balanced differentiation between these opposites, an understanding of the pathophysiology of hyponatremia is required. Therefore, in this review an attempt is made to translate the physiology of water balance regulation to strategies that improve the clinical management of hyponatremia. A physiology-based approach to the patient with hyponatremia is presented, first addressing the possibility of acute hyponatremia, and then asking if and if so why vasopressin is secreted non-osmotically. Additional diagnostic recommendations are not to rely too heavily of the assessment of the extracellular fluid volume, to regard the syndrome of inappropriate antidiuresis as a diagnosis of exclusion, and to rationally investigate the pathophysiology of hyponatremia rather than to rely on isolated laboratory values with arbitrary cutoff values. The features of the major hyponatremic disorders are discussed, including diuretic-induced hyponatremia, adrenal and pituitary insufficiency, the syndrome of inappropriate antidiuresis, cerebral salt wasting, and exercise-associated hyponatremia. The treatment of hyponatremia is reviewed from simple saline solutions to the recently introduced vasopressin receptor antagonists, including their promises and limitations. Given the persistently high rates of hospital-acquired hyponatremia, the importance of improving the management of hyponatremia seems both necessary and achievable.
Gonadal Steroids Regulate Neural Plasticity in the Sexually Dimorphic Nucleus of the Preoptic Area of Adult Male and Female Rats
Authors: Dugger Brittany N. , Morris John A. , Jordan Cynthia L. , Breedlove S. Marc
Published: March 2008

Background: The densely staining sexually dimorphic nucleus of the preoptic area (SDNPOA) is profoundly affected by gonadal steroids during perinatal development. Methods: We tested whether the SDNPOA in rats also remains responsive to gonadal hormones in adulthood. Results: Castration of 60-day-old male rats led to a reduction of soma size in SDNPOA neurons 28 days later, but not 14 days later. In contrast, the SDNPOA volume in males was unaffected by adult castration, increasing somewhat between 60 and 88 days of age in both castrated males and sham controls. For female rats ovariectomized at 60 days of age, testosterone treatment resulted in a significantly larger SDNPOA soma size after either 14 or 28 days of treatment, compared to blank-treated controls. Testosterone-treated ovariectomized females also had a modestly larger SDNPOA volume after 28 days, but not 14 days, of treatment compared to blank-treated ovariectomized animals. A second experiment revealed that these effects in females were due to ovariectomy: both SDNPOA soma size and regional volume shrank in females that were ovariectomized compared to females subjected to sham surgery. The effects of ovariectomy were blocked by testosterone treatment. The cessation of testosterone treatment in females returned SDNPOA soma size and regional volume to that of control-treated ovariectomized females. Conclusion: These results indicate considerable plasticity of the adult rat SDNPOA, and that (1) circulating androgens are required to maintain soma size, but not regional volume in males, and (2) ovarian steroids maintain both soma size and regional volume of the SDNPOA in females, an effect that can be mimicked with testosterone treatment and is fully reversible in adulthood.
Comparison of Tumor-Associated Surface Antigens on cells from Medulloblastomas and from Other Neoplasms of the Human Nervous System
Authors: Sato Kiyoshi , Raimondi Anthony J. , Dray Sheldon , Molinaro Giuseppe A.
Published: March 2008

We used a new immunocytoadhesion method to test 15 different tumors of the human nervous system to see whether medulloblastomas share tumor-associated surface antigens (TSA) with other tumors. Antisera against medulloblastoma cells were raised in rabbits. One antiserum was adsorbed extensively with tonsil and adenoid cells and with cerebral tissues. Freshly isolated cells from 15 different tumors were first sensitized with the adsorbed antiserum and then rosetted with erythrocytes coated with purified antibody to rabbit immunoglobulin. We found that the antimedulloblastoma antiserum detected TSA on the immunizing medulloblastoma cells and on cells from 2 other medulloblastomas, but apparently not on cells from other tumors (astrocytomas, glioblastomas, neurinoma, meningiomas and craniopharyngioma). In addition, the antiserum probably detected the medulloblastoma TSA on some other tumors (oligodendroglioma, ependymoma and malignant glioma of mixed type).
5-HydroxyindoIeacetic Acid (5-HIAA) Levels in the Cerebrospinal Fluid of Hydrocephalic Children before and after Ventricular Shunting Procedure
Authors: Massarotti M. , Migliore A. , Roccella P. , Tegos S. , Toffano G.
Published: March 2008

21 cases of hypertensive hydrocephalus were investigated for lumbar 5-HIAA before and after operation. Seven groups (posterior fossa tumors) of patients aged between 2 and 12 years were tested after tumor removal; the others (nontumorous hydrocephalus) after ventricular shunting. This last group included 8 patients with noncommunicating hydrocephalus aged between 15 days and 12 years and 6 with communicating hydrocephalus aged between 6 months and 9 years. At the time of the second determination all patients presented an improvement of clinical signs of intracranial hypertension. A correlative analysis of 5-HIAA values before and after operation is made. In tumor cases, preoperative levels were higher than controls, and a significant decrease was found after operation in comparison with preoperative levels. In nontumor cases, a significant decrease was also observed in noncommunicating hydrocephalus, confirming clinical usefulness of the results for controlling the degree of the intracranial hypertension and, in consequence, shunt performance. In communicating cases the results were disappointing, because almost always postoperative levels did not differ from the preoperative ones. Possible interpretations of the data are analyzed.
Control of Intracranial Pressure during Pediatric Neurosurgery Anesthesia
Authors: Cunitz G. , Soerensen N.
Published: March 2008

Some of the important points of view of anesthesia in pediatric neurosurgery are: adequate alveolar exchange of oxygen and carbondioxide by means of artificial ventilation; stable cardiovascular function; fluid balance; monitoring, and attempts to keep intracranial pressure low. This last point is achieved by various anesthesiological procedures, e.g., hyperventilation, positive-negative pressure ventilation, rapid induction of anesthesia and choice of suitable anesthetics. Enflurane (Ethrane®) increases intracranial pressure less than halothane. The new intravenous anesthetic etomidate lowers intracranial pressure in a favorable manner quite similar to thiopental (Pentothal-Sodium®).
Head Bobbing in a Patient with a Cyst of the Third Ventricle
Authors: Jensen Hans-Peter , Pendl Gerhard , Goerke Wolf
Published: March 2008

The case of a 7 year-old boy with ‘bobble head doll syndrome’ associated with a cyst in the third ventricle is reported. This movement disorder appears in early childhood and consists mostly in a rhythmic to-and-fro bobbing of the head and eventually of the trunk. As in three previously described cases, this case showed only slight decrease of the ‘tic’ after partial removal of the cyst by transcortical exploration of the third ventricle. In an inquiry and a survey of the literature, 17 cases with bobble head doll syndrome were collected. The average age of the described cases was 7 years at time of diagnosis. In 13 cases a slow-growing cyst or mass in the anterior part of the third ventricle of close to this region was found. There was no case observed with an acute lesion, in 3 cases it was thought an aqueduct stenosis should be the cause, and in 1 case the cause was unknown.
Myeloschisis in Early Human Embryos
Authors: Osaka Kunihiko , Matsumoto Satoshi , Tanimura Takashi
Published: March 2008

Four early human embryos with open myeloschisis are reported. These are a thoracolumbar myeloschisis in Carnegie developmental stage 12, a cervical myeloschisis in stage 13, and two lumbosacral myeloschisis in stage 14. All of them are the smallest human embryos with this type of malformation ever reported. In these embryos, the neuroectodermal junction is smooth and there is no microscopic evidence that the neural tube is forced open after its proper closure. The presence of the lesion in such early embryos, especially in one of stage 12, implies that the lesion evolved from the neural plate which never closed. Cellular polarity and the limiting membrane in the lesion are generally well preserved in spite of apparent overgrowth of the neural tissue. These findings do not support the hypothesis of Lemγre et al. that the defective external limiting membrane predisposes to a loss of cellular polarity and resultant neural overgrowth.
On the Pathology of Experimental Hydrocephalus Induced by Artificial Increase in Endoventricular CSF Pulse Pressure
Authors: Di Rocco Concezio , Di Trapani Girolamo , Pettorossi Enrico , Caldarelli Massimo
Published: March 2008

In lambs, acute and chronic communicating hydrocephalus has been created by merely increasing the amplitude of the intraventricular cerebrospinal fluid (CSF) pulsations, leaving the mean CSF pressure unchanged. The pathologic changes occurring in this type of hydrocephalus do not differ qualitatively from those observed in obstructive hydrocephalus. Beside demonstrating a direct role of ventricular pulsations in the ventricular dilation, the observed findings indicate that an increase in mean CSF pressure is not a necessary condition for the occurrence of the hydrocephalus. Consequently, the hypothesis of abnormal high intraventricular CSF pressure oscillations at the basis of the ventricular dilation of normal pressure hydrocephalic patients, receives a significant support. Further support is provided by considering that the recorded experimental pathological lesions are quite similar to those observed in normal pressure hydrocephalic patients with patent subarachnoid CSF pathways.
Analysis of Therapeutic Modalities for Head-Injured Children
Authors: James Hector E.
Published: March 2008

The various stages and therapeutic modalities in the management of children with moderate and severe head injuries is reviewed. Emphasis on aggressive management upon arrival to the emergency room, and continuing until brain swelling has definitely resolved, is stressed. Management includes prompt endotracheal intubation and hyperventilation, arterial and venous lines and bladder catheter insertion. Computed tomography to assess underlying pathology, intracranial pressure monitoring for appropriate therapy should follow. In this way mannitol, other diuretics, hypothermia and barbiturate coma can be decided on, and the response assessed. The role of high dose steroids is discussed.
Klippel-Feil Syndrome, Iniencephalus, Anencephalus, Hindbrain Hernia and Mirror Movements
Authors: Gardner James
Published: March 2008

Bony anomalies encountered in the ‘no neck’ form of Klippel-Feil syndrome (KFS) are a wide, short, fused, bifid, retroflexed spinal canal; craniolacunia, cranium bifidum, and acrania. The only symptom may be mirror movement (MM). The CNS anomalies are hindbrain hernia, hydrocephalus, hydromyelia, syringomyelia, meningocele, myelocele, encephalocele, and anencephalus. In severe KFS, i.e. iniencephalus (IN) and in anencephalus (AN), the inion is in contact with the back. In both there is hindbrain hernia and the left thorax may contain the stomach tethered to an anterior spina bifida. KFS results from distortion of somites by an overdistended neural tube. A neural tube that fails to close cannot overdistend.
Congenital Midline Porencephaly
Authors: Yokota Akira , Matsukado Yasuhiko
Published: March 2008

5 cases of dysgenetic hydrocephaly associated with scalp anomalies are described. This brain-scalp anomaly complex is characterized by congenital hydrocephalus, alopecia or cephalocele in the parietal midline and symmetrical defect of the bilateral posterior cerebral mantle along the midline. Midline septal structures of the cranial cavity and tentorium cerebelli were also defective and replaced by a huge dorsal CSF cavity. Histological features of the covering membrane of the dorsal cyst indicate that this new brain anomaly is the consequence of a porencephalic process and we postulate the ‘midline porencephaly’. Cerebral angiography is the most valuable method for the diagnosis of this anomaly and its venous phase reveals dysgenesis of the galenic drainage as well as of the dural sinuses.
The Physiopathogenetic Basis for the Angiographic Diagnosis of Bacterial Infections of the Brain and Its Coverings in Children
Authors: Raimondi Anthony J. , Di Rocco Concezio
Published: March 2008

Most of the small (50–60 µm) vessels nourish exclusively the cerebral cortex, whereas larger caliber (80–150 µm) arteries penetrate the entirety of the cortical layer to enter the underlying white matter. A single layer of nonfenestrated endothelial cells, surrounded by a continuous layer of basement membrane (which in places splits to envelope a pericyte), and perivascular glial cells attached to the outer surface of this basement membrane, with no pericapillary space, represent the anatomical structure of the blood-brain barrier (BBB). Concentrated electrolyte solutions and increases in mean arterial pressure may reopen the BBB. Water-soluble contrast media used in cerebral angiography are hypertonic solutions of iodinated salts. In cerebritis, one observes a very typical angiographic picture: ‘laminar’ staining of the gyri. The intervening sulci appear as negative images. In brain abscess, one may note angiographic evidence of a space-occupying lesion, a capsular stain in the granulation tissue surrounding the abscess, ‘halo’ formation and ‘pooling’ of contrast media in the sulci, and ‘laminar’ staining of the gyri.
What can be Expected from the Surgical Treatment of Craniopharyngiomas in Children
Authors: Rougerie J.
Published: March 2008

92 cases of craniopharyngiomas have been operated, 63 with a follow-up extending from 30 months to 20 years. The possibilities of the surgical treatment are mainly discussed on the basis of the anatomical location of the tumors. Simple intrasellar craniopharyngiomas can be operated by a transphenoidal approach with, sometimes, spectacular improvement (case of spontaneous normal pregnancy). The tumors most frequently seen are those with predominantly prechiasmatic expansion. A subfrontal approach is always required, aiming at radical excision. Microscopy can offer interesting possibilities for selective surgery. However, if the vital prognosis is excellent and recurrences rare, functional results remain very poor. In retrochiasmatic tumors involving the posterior hypothalamus, the principle of radical excision must be seriously discussed and sometimes challenged. The various combined approaches are described with their own limits and risks. Other therapeutic techniques are suggested. In many cases, selective neurosurgical dissection may raise some hope for better improvement of functional results.
Lipoma of the Corpus callosum: Report of a Case and Review of the Literature
Authors: Suemitsu Tatsunori , Nakajima Shun-Ichi , Kuwajima Katsuko , Nihei Kenji , Kamoshita Shigehiko
Published: March 2008

A case of lipoma of the corpus callosum associated with subcutaneous lipoma, whose symptoms were headache and trembling of the extremities, was reported. Roentgenograms showed characteristic findings and computerized tomography revealed a mass of very low density. The literature was reviewed and 15 cases not included in previous compilations were found. Clinical and radiological findings in 89 cases including the present case were analyzed.
Acute Vestibular Dysfunction in Childhood
Authors: Curless Richard G.
Published: March 2008

Acute, vestibular symptoms in childhood are primarily caused by acute labyrinthitis (end organ dysfunction) or central nervous system diseases such as neoplasia involving the pons or cerebellar nuclei or infection manifested by pontine encephalitis. The evaluation, treatment, and prognosis of end organ versus central disease is so different that the ability to make a bedside, presumptive diagnosis provides an important advance. Both patients presented here had a central vestibulopathy. By paying particular attention to the character of the nystagmus and past pointing, the Romberg test, and the details of the vertiginous history, central disease was the presumptive diagnosis prior to invasive procedures. The neurophthalmological clues are reviewed to demonstrate their value in making more accurate diagnoses in children with acute vestibular dysfunction.
Sacral Epidermoid Cyst Communicating with the Spinal CSF Canal
Authors: Nakamura Saburo , Wakamatsu Kenichi , Tsubokawa Takashi , Moriyasu Nobuo
Published: March 2008

A 6-year-old girl was found to show a presacral epidermoid cyst which had a communication with the spinal canal. The patient was admitted as a case of meningitis, but squamous cells and cholesterin crystals were observed in the cerebrospinal fluid (CSF), and the CSF cholesterol level was very high. Diagnosis was established by myelography, and a cyst measuring 4.5 × 6.0 × 4.0 cm wide was totally excised surgically. A comparative discussion of this case and other reported cases of dermoid and epidermoid cysts in the central nervous system is given.
Extradural Masses at the Foramen magnum in Children
Authors: Amacher Loren , Durward Quentin , Shillito, Jr. John
Published: March 2008

This report describes the clinical features, treatment, and sequelae in 3 children with extradural masses in the region of the foramen magnum. Such lesions are rare, their nature diverse, and their treatment hazardous. The common presenting feature was neck stiffness.
31 Primary Orbital Mass Lesions in Infancy and Childhood
Authors: Nicole Sandro , Palma Lucio , Giuffrè Renato , Fortuna Aldo
Published: March 2008

A series of 31 primary orbital mass lesions in patients up to 15 years of age is presented (malignancies: 5 sarcomas, 1 retinoblastoma; benign lesions: 7 optic nerve gliomas, 4 pseudotumors, 4 angiomas, 3 fibrous dysplasias, 2 dermoids, 1 hemiangio-blastoma, 1 osteoma, 1 neurofibroma, 1 eosinophilic granuloma, 1 echinococcus). All the patients were operated upon using the subfrontal-extradural approach. The pathological, clinical and roentgenological features, and the long-term follow-up are analyzed. In the author’s experience, computerized tomography scan (CT scan) is useful in planning the surgical approach more than in the pathological differential diagnosis. In orbital pseudotumors, without visual function impairment, CT scan is also useful in checking the results of conservative steroid therapy. The author’s stress the importance of performing radical surgery, either along with (in benign lesions, including optic nerve gliomas) or followed by radiotherapy (in malignancies).
Combined Treatment of Pediatric Medulloblastoma
Authors: Gerosa Massimo , DiStefano Enzo , Carli Modesto , Iraci Giorgio
Published: March 2008

29 out of 34 consecutive patients of pediatric age, operated upon for posterior fossa medulloblastoma, were divided into 2 groups according to the chemotherapeutic treatment (mtrathecal methotrexate or intravenous cyclophosphamide) received at random after surgery and radiation treatment. The modalities of irradiation and chemotherapy are described. 9 patients have not yet shown a local recurrence and are alive at various intervals after surgery. Only 1 patient with local recurrence is still alive 31 months after the primary operation. The mean actuarial survival of the whole series of patients is about 38 months. Differences between the two groups, concerning either the survival rate till local recurrence, or the general and neurologic conditions of survival, are not statistically significant. Hematologic toxicity was more pronounced in the group treated with cyclophosphamide, whereas late neurologic sequelae were a more prominent feature of the in-trathecal methotrexate trial.
Intracranial Aneurysms of Childhood
Authors: Gerosa Massimo , Licata Claudio , Fiore Daniele L. , Iraci Giorgio
Published: March 2008

15 cases of cerebral aneurysms in children between 8 and 15 years of age, all operated upon in a 20-year span (1956–1976), are reported. 7 of them were the carriers of large aneurysms (3 ‘giant’). Surgical mortality was limited to 2 patients with aneurysms of the middle cerebral artery and large intracerebral hematomas. All other patients are in satisfactory conditions, with a follow-up ranging from 2 to 22 years. A direct approach to the aneurysm was used in all but 3 cases. Certain features of cerebral aneurysms in children are discussed pertinently to this series.
Supratentorial Hemangioblastoma in Childhood
Authors: Tomaccini D. , Cordelli F. , Luzi P. , Scarfò G.B.
Published: March 2008

Only 2 cases of supratentorial hemangioblastoma in children are recorded. We report a third: an occipital cerebral hemangioblastoma in a 9-year-old girl. The tumor had a solid subpial portion continuous with a cystic intraparenchymal portion. It had no dural insertion, although it was in contact with the falx. A year after complete removal there is no sign of recurrence.
Parasellar Tumors in Children
Authors: Richmond Isabelle L. , Wilson Charles B.
Published: March 2008

This paper reviews the clinical presentation, neuro-ophthalmologic, endocrinologic, and radiographic findings of 74 children (0–20 years) harboring parasellar lesions who were treated on our service between 1968 and 1977. Our data support the conclusion that earlier diagnosis is occurring, although there is still a significant lag between onset of symptoms and treatment, especially in histologically benign lesions. The differential diagnosis is facilitated by recognition of key features of four major tumor types: craniopharyngioma, parasellar glioma, germinoma, and pituitary adenoma.
Specific Type of Head Injury in Children
Authors: Takahashi Hiroshi , Nakazawa Shozo
Published: March 2008

Among many cases of head injuries in children, 5 cases showing specific symptoms were recorded. All of these 5 cases were under 10 years of age, with no initial loss of consciousness after suffering trivial head injuries and then, after a latent period, manifested transient neurological disorders accompanied with or without convulsion. The neurological disorders lasted for an extensive period of time, but complete recovery was achieved. This clinical appearance might be the combined effects of the organic disorder of a cerebral contusion and a certain functional disorder in the brain. In diagnosis and treatment of head injuries in children, sufficient attention should be paid to such a specific type.
Cerebral Injury in Child Abuse: A Changing Profile
Authors: McClelland Charles Q. , Rekate Harold , Kaufman Benjamin , Persse Linda
Published: March 2008

This report of 21 patients with cerebral injury resultant from child abuse, identifies a broad profile of central nervous sequelae from such injuries. 6 patients were admitted with whiplash shaken infants syndrome, all with severe signs of CS dysfunction. 3 children presented with acute cerebral contusions, 2 with severe spinal cord injuries with resultant paresis and 10 with skull fractures or miscellaneous injuries. Computer tomography has proved to be of great assistance in precise identification of site and size of subdural/intracerebral hematomas in abused children and in identifying posterior fossa lesions and providing longitudinal assessment of sequelae of cerebral trauma. Also the role of the Child Protection Team in management of such acutely injured children is defined.
Evaluation of Complicated Migraine in Childhood
Authors: Ment Laura R. , Duncan Charles C. , Parcells Patrick R. , Collins, Jr. William F.
Published: March 2008

We report on 3 patients with complicated migraine of childhood. Family history was positive for migraine in each case, and all experienced the acute onset of neurologic deficits in association with throbbing hemicranium. Abnormalities of cerebral blood flow determinations, electroencephalograms and cerebral arteriograms in addition to persistent neurologic sequelae were demonstrated. We propose that complicated migtaine of childhood is not an entirely benign symptom complex.
Benign Ependymal Cyst of the Pons
Authors: Rilliet Bénédiet , Berney Jean
Published: March 2008

Clinical presentation, radiological aspects, surgical treatment and histology of a case of an ependyma-lined cyst of the pons are depicted. Slight psychomotor retardation and long-standing neurological signs associated with a marked dolichocephalic skull and right aortic arch suggest a congenital anomaly. Differential diagnosis of cystic lesions in the posterior fossa is discussed.
Interfascicular Nerve Grafting of Peripheral Nerve Lesions in Childhood
Authors: Haase Jens
Published: March 2008

Interfascicular cable grafting with autologous sural nerve was carried out in 43 peripheral nerve lesions among 37 children aged 5-16 years. The follow-up study included neurophysiologically proved regeneration. The results seem superior to those obtained with the conventional technique. The technique is advocated for use in children with nerve gaps of more than 2–2.5 cm.
Experimental Spina bifida and Associated Malformations
Authors: Warkany Josef , O’Toole Barbara A.
Published: March 2008

Seven litters and 17 near-term rats of mothers treated with teratogens during gestation were analyzed for concordance and discordance of congenital malformations of the central nervous system. In animals with spina bifida a firm association with Arnold-Chiari malformation was found but only in fetuses near term. Associations with aqueduct stenosis and hydrocephalus were poor. In general, the findings support the theory that the components of the spina bifida complex develop from a common teratogenic disturbance but independent from each other.
Monoamine Metabolites and Ventricular Fluid Pressure in Children
Authors: Yates C.M. , Minns R.A.
Published: March 2008

Samples of ventricular CSF were obtained from 20 children in whom ventricular fluid pressure was continuously monitored. The concentrations of the monoamine metabolites, 5-hydroxyindolylacetic acid, homovanillic acid and 3-methoxy-4-hydroxyphen-ylglycol, in the CSF were not related to the ventricular fluid pressure. The mean concentration of 3-methoxy-4-hydroxyphenylglycol was higher (p< 0.01) in 9 children aged 1 day to 6 months than in 12 children aged 8 months to 11 years.
Anterior Third Ventricle Meningioma in a Child
Authors: Roberts, Jr. Boswell , Horner Frederick A. , McDonald Joseph V.
Published: March 2008

A case report of a meningioma in the anterior part of the third ventricle of a 10-year-old boy is presented. The tumor was successfully removed through a right trans-cortical ventricular approach.
Schilder’s Disease
Authors: Ferrer Isidro , Fábregves Isidro , Fernandez Alvarez Emilio , Vila Jose
Published: March 2008

The cerebral cortex of a patient with Schilder’s disease was studied with the Golgi method. The number of synaptic spines, basal dendrites, branching points, and dendritic intersection points of 5th layer pyramids are similar to those of controls, in spite of almost complete loss of extrinsic fibers. These data illustrate the probable remodeling of intrinsic cortical circuits and neuronal plasticity in the human cerebral cortex.
Early Third Ventriculostomy in Meningomyelocele Infants – Shunt Independence?
Authors: Natelson Stephen E.
Published: March 2008

Percutaneous anterior and posterior third ventriculostomy utilizing a McKinney leukotome has been performed with fluoroscopic control in 9 newborn infants with meningomyelocele and hydrocephalus immediately following their shunt. The procedure was instituted in 1975, and there have been not shunt revisions in such patients since that time. Wider application of this or a similar technique in newborn meningomyelocele infants seems warranted.
Absence of Hydrocephalus despite Disproportionately Increasing Head Size after the Neonatal Period in Preterm Infants with Known Intraventricular Hemorrhage
Authors: Bridgers Samuel L. , Ment Laura R.
Published: March 2008

Of a group of 20 infants weighing less than 1,250 g at birth and surviving with intraventricular hemorrhage (IVH) documented by computed tomography, 6 exhibited excessive head growth between 6 and 14 weeks after birth with no evidence of neurologic deterioration. Repeat computed tomography was normal in all 6 infants. Rapid head growth after the immediate neonatal period can be expected in some infants with a history of lesser degrees of IVH and is not in itself indicative of posthemorrhagic hydrocephalus.
Recurrent Thrombotic Deterioration in the Sturge-Weber Syndrome
Authors: Garcia Juan Carlos , Roach Steve , McLean William T.
Published: March 2008

Hemiparesis, homonymous hemianopia and intellectual impairment are common features of the Sturge-Weber syndrome. While cerebrovascular thromboses have been noted with the syndrome, the pattern of recurrent thrombotic episodes producing an apparently gradual loss of function is not widely appreciated. The most important implication of this concept is the possibility of developing new methods of treatment, such as antiplatelet agents.
Light Microscopy and Ultrastructural Studies of Sturge-Weber Disease
Authors: Di Trapani G. , Di Rocco C. , Abbamondi A.L. , Caldarelli M. , Pocchiari M.
Published: March 2008

Different degrees of cerebral calcifications together with encephalofacial angiomatosis and seizure disorder characterize the Sturge-Weber syndrome. According to the observations reported in the literature, calcium deposits may be found in the wall of cerebral vessels, in the perivascular tissue and rarely within the neurons. Corresponding to the variety of localizations, the interpretation of the phenomenon remains obscure. Most theories postulate the role of a vascular factor and of a mesenchymal factor. Ultramicroscopic studies of the specimens obtained in 2 children with the Sturge-Weber syndrome provided the following findings. A mucopolysaccharidic substance constitutes the substratum for the deposition of calcium. Small amounts of this substance and calcium deposits may be detected within the connective tissue of cerebral vessels precociously; later on, while increasing in size and calcium concentration, they obviously migrate to outside the vessels. Successively, the calcium deposits seem to localize around the blood vessels. In our opinion, these observations stress the role of a primitive vascular factor; consequently, anoxia, necrosis of cerebral tissues, and variation in the calcium ion concentration would act only as secondary factors.
Chronic Cortical Electrode Array for Seizure Investigation
Authors: Levy W.J. , Hahn J.H. , Lueders H. , Lesser R.
Published: March 2008

Localization of seizure focus if surgical extirpation is contemplated is critical. It is routine to use cortical recording and stimulation for this purpose but the limited time available in the operating room limits what can be learned. We report a flexible electrode array of four 0.5 cm discs which can be banked to larger sizes, and left in place subdurally as well as epidurally. It can be used for stimulation as well as recording.
Closure of Thoracic and Lumbar Dysgraphic Defects Using Bilateral Latissimus Dorsi Myocutaneous Flap Transfer with Extended Gluteal Fasciocutaneous Flaps
Authors: McDevitt N.B. , Gillespie R.P. , Woosley R.E. , Whitt J.J. , Bevin A.G.
Published: March 2008

The latissimus dorsi-gluteal myofasciocutaneous flap has been successfully employed in closure of 6 large thoracolumbar myelomeningoceles in newborn infants. Good wound healing was attained in all cases. The technique of flap mobilization and closure is outlined and possible pitfalls are explained.
Preliminary Experiences with the Use of Three Lasers (CO2, Nd: YAG, Argon) in Some Posterior Fossa Tumors in Childhood
Authors: Fasano V.A. , Lombard G.F. , Ponzio R.M.
Published: March 2008

The authors present 5 cases of posterior fossa tumors in childhood operated on using three laser sources: CO2, Nd: YAG and Argon. Each laser source is used as required during the different steps of the operation: CO2 for cutting and vaporizing, Nd: YAG for shrinking the tumoral mass and deep coagulation and Argon for selective cutting and superficial coagulation of small vessels. The main advantages of multiple laser surgery are: a more radical surgery with less side effects (edema); the possibility of operating near high functional structures with great accuracy, and reduction of blood loss.
Stereotactic Biopsy of Deep Brain Tumors in Infancy and Childhood
Authors: Broggi G. , Franzini A. , Migliavacca F. , Allegranza A.
Published: March 2008

In 17 patients, 2–16 years old, the clinical and neuroradiological data were unable to give clear-cut information regarding the histology of deep brain neoplasms. The lesions were located within the brain stem in two cases, in the pineal region in two cases, within the third ventricle in two cases, in the sellar and parasellar area in three cases, in the basal ganglia and thalamic region in seven cases, and in the fronto-callosal region in one case. In these patients, serial stereotactic biopsies were performed with the guidance of CT scan in order to assess the nature and the real boundaries of the growths. The choice between conservative or surgical treatment was made after the established histological findings became available. The operative technique of stereotactic biopsy is briefly described and the value of this method is stressed in the treatment of brain tumors in children.
Visual-Evoked Responses in Children with Optic Gliomas, With and Without Neurofibromatosis
Authors: Cohen Michael E. , Duffner Patricia K.
Published: March 2008

Gliomas of the optic pathway are considered to be slowly growing, potentially benign neoplasms. Association of this tumor with neurofibromatosis has been well established. We have recently evaluated 7 children with putative evidence of optic glioma, 6 of whom had additional evidence of neurofibromatosis. All children had CT scans and visual-evoked responses. CT scan allows anatomic assessmant of the optic nerve mass while evoked responses offer the possibility of identifying functional abnormalities in the optic pathway. It is felt that both these modalities will add in the early diagnosis of optic gliomas and help to further establish the true natural history of this tumor.
Epidural Hematomas of the Posterior Fossa in Children
Authors: Mori Koreaki , Handa Hajime , Munemitsu Hirofumi , Oda Yoshifumi , Hashimoto Nobuo , Kojima Masayuki
Published: March 2008

The authors report 3 cases of epidural hematoma of the posterior fossa in children. The clinical signs and symptoms are discussed. Onset of symptoms is not necessarily acute. The radiological findings are discussed. Epidural hematoms of the posterior fossa in children have a tendency to liquefy earlier than supratentorial hematomas. We emphasize the value of computed tomography for the diagonosis of epidural hematoma of the posterior fossa. One should keep in mind the possibility of the presence of an epidural hematoma of the posterior fossa in the treatement of head injury in children, even in the case of a minor head injury.