bluuebunny/crossref_metadata_2025 huggingface dataset:
Distinct Functional Effects of Human 5-HT4 Receptor Isoforms on β-Amyloid Secretion
Authors: Robert Sylvain J. , Lezoualc’h Frank
Published: March 2008

Background: The serotonin 5-HT4 receptor is of potential interest for the treatment of Alzheimer’s disease because it increases memory and learning and influences amyloid precursor protein (APP) processing. Several 5-HT4 receptor isoforms have been cloned in humans. They all belong to the G-protein-coupled receptor superfamily and differ in composition and length of their amino acid intracellular C-terminal sequence. At present, it is still unknown whether 5-HT4 receptor isoforms may have distinct effects on APP processing. Objective: In this study, we investigated the effect of an ultrashort isoform of the human 5-HT4 receptor (2 amino acids after the splicing site), the 5-HT4(d) receptor isoform (h5-HT4(d)), on APP processing in Chinese hamster ovary cells (CHO cells). Methods: Non-amyloidogenic soluble sAPPα was determined by immunoblot and β-amyloid peptides (Aβ1–40 and Aβ1–42) were assayed by ELISA in CHO cells stably expressing h5-HT4(d) and transiently transfected with human APP695. Results: We show here that activation of h5-HT4(d) strongly stimulates the release of sAPPα and concomitantly decreases extracellular Aβ peptides. These data contrast with our previous findings showing that the h5-HT4(e/g) receptor isoform, which has 20 amino acids after the splicing site, did not influence Aβ secretion. Conclusion: We conclude that C-terminal tails of 5-HT4 receptor isoforms may influence their functional effect on Aβ secretion and that the pathways regulating either β- or γ-secretase may be differentially controlled by 5-HT4 receptor isoforms.
Apolipoprotein E ε4 Allele Is Associated with Increased Atrophy in Progressive Mild Cognitive Impairment: A Voxel-Based Morphometric Study
Authors: Hämäläinen A. , Grau-Olivares M. , Tervo S. , Niskanen E. , Pennanen C. , Huuskonen J. , Kivipelto M. , Hänninen T. , Tapiola M. , Vanhanen M. , Hallikainen M. , Helkala E.-L. , Nissinen A. , Vanninen R.L. , Soininen H.
Published: March 2008

Background: The apolipoprotein E (APOE) Ε4 allele is a risk factor for Alzheimer’s disease. Earlier studies have shown differences in brain structure according to the APOE Ε4 status. Objective: To assess possible differences in brain structure according to the APOE Ε4 status in mild cognitive impairment (MCI) subjects in relation to conversion to dementia. Methods: In a follow-up study of 56 MCI subjects, 13 MCI subjects progressed to dementia (PMCI) during a mean follow-up time of 31 months. Brain structure differences in both stable MCI (SMCI) and PMCI Ε4 carriers and noncarriers in the baseline MRI scan were assessed with voxel-based morphometry. Results: The SMCI Ε4 carriers had atrophy in the amygdala and hippocampus compared to the SMCI noncarriers. The PMCI Ε4 carriers revealed atrophy of the left inferior frontal gyrus and parietal cortex compared to the PMCI noncarriers. Conclusion: The rate of brain atrophy in certain brain areas may be increased in Ε4-positive MCI subjects progressing to dementia.
Neuroinflammation in Plaque and Vascular β-Amyloid Disorders: Clinical and Therapeutic Implications
Authors: Eikelenboom Piet , Veerhuis Rob , Familian Atoosa , Hoozemans Jeroen J.M. , van Gool Willem A. , Rozemuller Annemieke J.M.
Published: March 2008

Background: The cerebral β-amyloid (Aβ) disorders show a great variability in the distribution of parenchymal and vascular amyloid deposits. Objective: To study the relationship between amyloid deposition and inflammatory responses in three distinct subtypes of cerebral Aβ disorders. Methods: The distribution of inflammatory proteins and cells in vascular and plaque amyloid deposits was evaluated in postmortem brain tissue using immunohistochemistry. The effects of a mixture of Aβ peptides and inflammation-related Aβ-associated proteins were studied in postmortem obtained human microglia cell cultures. Results: The chronic inflammatory response is associated with amyloid plaques (but not with amyloid in the walls of larger vessels) in Alzheimer’s disease (AD), with amyloid in cerebral arteries in hereditary cerebral hemorrhage with amyloidosis-Dutch type and with amyloid microangiopathy in the vascular variant of AD. Aβ1–42 fibrils complexed with complement factor C1q and serum amyloid P component (the relevant amyloid-associated proteins) stimulate the production of proinflammatory cytokines in human microglia cell cultures and this production is attenuated by minocycline. Conclusion: The pattern of the chronic inflammatory response associated with fibrillar Aβ is strikingly different in the three studied types of Aβ disorders. The site of the fibrillar Aβ-induced chronic inflammatory response is closely related to clinical symptoms. Minocycline is a drug of interest to inhibit microglia-mediated neuroinflammatory response in Aβ brain disorders.
ACAT as a Drug Target for Alzheimer’s Disease
Authors: Huttunen Henri J. , Kovacs Dora M.
Published: March 2008

Accumulation of β-amyloid peptide (Aβ) in the brain regions responsible for memory and cognitive functions is a neuropathological hallmark of Alzheimer’s disease. Cholesterol may be involved in many aspects of Aβmetabolism. It affects generation, aggregation and clearance of Aβin the brain. Not only the amount but also the distribution of cholesterol within cells appears to modulate Aβbiogenesis. ACAT is an enzyme that regulates subcellular cholesterol distribution by converting membrane cholesterol to cholesteryl esters for storage and transport. We have used various cell- and animal based models to show that inhibition of ACAT strongly reduces Aβgeneration and protects from amyloid pathology. Here, we discuss data supporting ACAT inhibition as a strategy to treat Alzheimer’s disease.
In vivo and Postmortem Clinicoanatomical Correlations in Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17
Authors: Ghetti Bernardino , Spina Salvatore , Murrell Jill R. , Huey Edward D. , Pietrini Pietro , Sweeney Brian , Wassermann Eric M. , Keohane Catherine , Farlow Martin R. , Grafman Jordan
Published: March 2008

Background: Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is associated with mutations in the Microtubule-Associated Protein Tau(MAPT) gene or the Progranulin(PGRN) gene. MAPT mutations lead to widespread deposition of hyperphosphorylated tau protein (FTDP-17T). PGRN mutations are associated with ubiquitin- and TDP-43-positive inclusions in the frontotemporal cortex, striatum and hippocampus (FTDP-17U). Despite the differences, FTDP-17T and FTDP-17U share a largely overlapping clinical phenotype. Objective: To determine whether neuroimaging studies may allow an in vivo early differentiation between FTDP-17T and FTDP-17U. Methods: We studied 25 individuals affected with FTDP-17T associated with either the exon 10+3 (24 subjects) or the G335S (1 subject) MAPT mutation, as well as 3 FTDP-17U individuals, who were carriers of the A9D, IVS6-2A>G or R493X PGRN mutation. Neuroimaging studies, obtained along the course of the disease, were compared to the neuropathologic findings. Results: FTDP-17T cases were associated with symmetric frontotemporal atrophy. Behavioral changes constituted the predominant clinical presentation. Conversely, an asymmetric degenerative process was seen in all 3 PGRN cases, who presented with either corticobasal syndrome (A9D) or frontotemporal dementia and language deterioration (IVS6-2A>G and R493X). Conclusion: Neuroimaging data, in the early disease stage of FTDP-17, may offer the possibility of an early differentiation of FTDP-17T and FTDP-17U phenotypes, independent of the genetic analysis.
Lithium, a Potential Protective Drug in Alzheimer’s Disease
Authors: Engel T. , Goñi-Oliver P. , Gómez de Barreda E. , Lucas J.J. , Hernández F. , Avila J.
Published: March 2008

Alzheimer’s disease is characterized by the presence of two histopathological aberrant structures, the senile plaques and the neurofibrillary tangles. The main component of these tangles is the cytoskeletal protein tau in hyperphosphorylated form. Since a main tau kinase is glycogen synthase kinase 3 (GSK-3), the use of specific GSK-3 inhibitors, like lithium, could be a potential therapy in Alzheimer’s disease. In this short article, we have done a review on tau phosphorylation in Alzheimer’s disease and other tauopathies, and on the inhibition of kinases like GSK-3, involved in tau modification.
Seeding Neuritic Plaques from the Distance: A Possible Role for Brainstem Neurons in the Development of Alzheimer’s Disease Pathology
Authors: Muresan Zoia , Muresan Virgil
Published: March 2008

Background and Objective: Our goal is to obtain insight into the causes of the pathological lesions in Alzheimer’s disease (AD). It is thought that the β-amyloid (Aβ) deposits within the cerebral cortex and hippocampus of AD brains are initiated by a ‘bad seed’ of oligomeric Aβ. The origin of this seed is unknown. Here, we focused on the events that might trigger the formation of neuritic plaques, aiming to explain how these plaques form in cortical and hippocampal regions. Methods and Results: Using immunocytochemical and biochemical methods, we showed that brainstem-derived, neuronal cells (CAD) – but not cortical or hippocampal neurons – show large amounts of Aβ accumulated at the terminals of their processes. This is similar to what is believed to occur in brain neurons, in the early phases of AD. CAD cells that contain Aβ accumulations also concentrate β-secretase at process terminals. We show that, while the anterograde transport of small vesicles is not significantly affected, the mitochondrial transport is perturbed in CAD cells that contain Aβ accumulations. We further show that intracellular, neuritic Aβ accumulations may become extracellular upon neurite degeneration, thus providing the initial ‘bad seed’ of Aβ oligomers that triggers further aggregation of extracellular proteins. Conclusion: We propose that brainstem neurons, known to send projections throughout the brain, could provide the ‘bad seed’ of Aβ that nucleates plaques in the cerebral cortex and hippocampus of AD brains.
The Long-Term Effects of Oophorectomy on Cognitive and Motor Aging Are Age Dependent
Authors: Rocca Walter A. , Grossardt Brandon R. , Maraganore Demetrius M.
Published: March 2008

Background: The evidence for a neuroprotective effect of estrogen in women remains controversial. Objective: We studied the long-term risk of parkinsonism and of cognitive impairment or dementia in women who underwent oophorectomy before menopause. Methods: We conducted a historical cohort study among all women residing in Olmsted County, Minn., USA, who underwent unilateral or bilateral oophorectomy before the onset of menopause for a noncancer indication from 1950 through 1987. Each member of the oophorectomy cohort was matched by age to a referent woman from the same population who had not undergone oophorectomy. In total, we studied 1,252 women with unilateral oophorectomy, 1,075 women with bilateral oophorectomy, and 2,368 referent women. Women were followed for a median of 25–30 years. Parkinsonism was assessed using screening and examination, through a medical records- linkage system, and through death certificates. Cognitive status was assessed using a structured questionnaire via a direct or proxy telephone interview. Results: The risk of parkinsonism and of cognitive impairment or dementia increased following oophorectomy. In particular, we observed significant linear trends of increasing risk for either outcome with younger age at oophorectomy. Conclusion: Our findings, combined with previous laboratory and epidemiologic findings, suggest that estrogen may have an age-dependent neuroprotective effect.
Assessing Change in Cognitive Function in Dementia: The Relative Utilities of the Alzheimer’s Disease Assessment Scale – Cognitive Subscale and the Cognitive Drug Research System
Authors: Wesnes Keith A.
Published: March 2008

This paper considers the suitability of the Alzheimer’s Disease Assessment Scale – Cognitive Subscale (ADAS-cog) as the gold standard in registration trials of treatments for Alzheimer’s disease. Working groups have recommended replacing the ADAS-cog if suitable automated alternatives can be found. This paper makes the case for the Cognitive Drug Research (CDR) computerised cognitive assessment system, as an example of a suitable instrument to replace the ADAS-cog. The CDR system has been widely used in dementia work for 20 years and shows good correlations to the ADAS-cog, while additionally assessing the domains of attention, working memory, information processing and retrieval speed of information held in memory. The utility of the system in evaluating and differentiating the major dementias will be described, as well as its ability to track deterioration over time. Its validation as a core measure of cognitive dysfunction in the dementias will be described, as will work showing that various CDR measures relate closely to activities of daily living. The sensitivity of the CDR system to anticholinesterases will be described in Alzheimer’s disease, dementia with Lewy bodies and Parkinson’s dementia. Finally, the CDR system has a large normative database which allows treatment effects in dementia to be put into an unambiguous clinical perspective.
Disease Severity, Quality of Life and Health Care in Plaque-Type Psoriasis: A Multicenter Cross-Sectional Study in Germany
Authors: Augustin M. , Krüger K. , Radtke M.A. , Schwippl I. , Reich K.
Published: March 2008

Background: Plaque-type psoriasis produces significant morbidity, has negative effects on patients’ health-related quality of life (HRQoL), and represents an economic burden. Objectives: The assessment of disease severity, HRQoL and health care in plaque-type psoriasis in everyday German medical practice. Methods: Details of patients with plaque-type psoriasis were recorded by 48 dermatologists in Germany. During the visit, demographic data, medical history, previous and current treatments, occupational impairment, the current state of the disease (measured by the Psoriasis Area and Severity Index; PASI), overall lesion severity, and HRQoL were evaluated. Results: In total, 1,511 plaque-type psoriasis patients were included. The average PASI score was 12.0. The average Dermatology Life Quality Index score was 8.6. Among the patients with the severest psoriasis (PASI >20), only 45.4% had ever been prescribed systemic treatments. Conclusions: Psoriasis patients have a reduced HRQoL and are not sufficiently treated in practice. A more widespread use of systemic treatment and the definition of treatment goals are essential to improve the standard of care for psoriasis patients.
Testosterone and Blood Pressure Regulation
Authors: Kienitz Tina , Quinkler Marcus
Published: March 2008

Background: There is substantial evidence that androgens may play a role in determining sex-specific blood pressure. Men are at higher risk for developing coronary heart disease or hypertension compared to premenopausal women. However, effects of androgens on the renal and cardiovascular system are complex. This review provides a critical overview of testosterone actions. Methods: We searched Pubmed library for experimental, animal and clinical studies, using the keywords ‘blood pressure’, ‘hypertension’, ‘testosterone’ and ‘androgens’. Results: While acute administration of testosterone seems to decrease vascular tone, the long-term net effect of androgens appears to be vasoconstriction via upregulation of thromboxane A2 expression, norepinephrine synthesis, angiotensin II expression, and endothelin-1 action. Furthermore, androgens cause cardiac hypertrophy, promote atherosclerosis, vascular remodelling and stimulate renal prohypertensive processes involving the renin-angiotensin-aldosterone system. Androgens seem to promote oxidative stress in the kidney and may also play a role in the differentiation of brain areas involved in blood pressure regulation. Conclusion: The effects of sex steroids on different parts of the renal-vascular system are complex and often contradictory. In sum, net effects of androgen action seem to be vasoconstriction, atherosclerosis and stimulation of the renin-angiotensin-aldosterone system. Therefore, androgens may determine blood pressure and the prevalence of cardiovascular disease.
Voxel-Based Analysis of Gray Matter and CSF Space in Idiopathic Normal Pressure Hydrocephalus
Authors: Ishii Kazunari , Kawaguchi Tetsuro , Shimada Kenichi , Ohkawa Shingo , Miyamoto Naokazu , Kanda Tomonori , Uemura Takafumi , Yoshikawa Toshiki , Mori Etsuro
Published: March 2008

Aims: To investigate regional morphologic changes in idiopathic normal pressure hydrocephalus (INPH) based on diagnosis with INPH Guidelines using voxel-based morphometry. Method: Three-dimensional magnetic resonance imaging was performed in 34 INPH patients, who met probable INPH criteria, probable 34 Alzheimer disease patients, and 34 normal control subjects. Results: Statistical parametric mapping was used to conduct voxel-based morphometry analysis of the morphologic data and revealed enlarged ventricles and sylvian fissures and stenotic sulci of high convexity, especially in the precuneus in the INPH group, with decreased gray matter density in the insula, caudate and thalamus. Conclusion: In INPH, morphologic change occurs in the frontoparietal high convexity with ventricular dilatations, dilated sylvian fissures and tight sulci in the medial parietal lobes.
Heparin Cryoprecipitation Reduces Plasma Levels of Non-Traditional Risk Factors for Atherosclerosis in vitro
Authors: Meilin E. , Sela S. , Kristal B.
Published: March 2008

Aims: To show that heparin cryoprecipitation (HCP), an in vitro method of plasma purification, reduces the levels of in vivo modified proteins and non-traditional risk factors from plasma of atherosclerotic hemodialysis (HD) patients. Methods: HCP was applied to plasma obtained from HD patients and controls, forming a precipitate – cryogel. Levels of fibrinogen, albumin, CRP, TNF-α, IL-6, advanced oxidation protein products, carbonylated fibrinogen and carbonylated albumin were determined in plasma before and after applying HCP and in the cryogel. Results: Treatment of HD plasma with HCP, beyond the significant reduction of the increased levels of all the above-mentioned molecules, reduced fibrinogen, TNF-α, carbonylated fibrinogen and carbonylated albumin to control levels which were simultaneously found in the cryogel. Conclusions: HCP applied to plasma enables the simultaneous precipitation of modified molecules and circulating non-traditional risk factors for atherosclerosis. This study may serve as a base for the future development of a clinical purification technique.
Neuroanatomical Correlates of Unawareness of Memory Deficits in Early Alzheimer’s Disease
Authors: Hanyu Haruo , Sato Tomohiko , Akai Tomotaka , Shimizu Soichiro , Hirao Kentaro , Kanetaka Hidekazu , Iwamoto Toshihiko , Koizumi Kiyoshi
Published: March 2008

Background/Aims: To investigate neuroanatomical substrates of unawareness of memory deficits in patients with early Alzheimer’s disease (AD). Methods: We compared regional perfusion deficits between AD patients with awareness (n = 19) and unawareness (n = 19). SPECT data were analyzed by statistical brain imaging method. Results: Statistical maps demonstrated a more extensive and severe reduction in perfusion in the unaware group than in the aware group. Quantitative analysis demonstrated a significant difference between the groups in the right subcallosal, anterior cingulate and cingulate gyri and left orbital, subcallosal, and anterior cingulate gyri. Conclusion: Functional damage to the inferior, medial and orbital frontal lobes as well as the anterior cingulate gyri may be associated with the lack of awareness in patients with early AD.
Progesterone, Estrogen and Pregnancy Do Not Decrease Colon Myoelectric Activity in Rats: An in vivo Study
Authors: Speranzini L.B.M. , Lopasso P.P. , Laudanna A.A.
Published: March 2008

Background: Progesterone, estrogen and the hormonal complex of pregnancy have been responsible for some degree of colon hypomotility in human pregnancy. Objective: To find out if estrogen, progesterone and the hormonal complex of pregnancy decrease colon myoelectric activity. Methods: The study was performed in 37 healthy female rats in which electrodes were implanted on the serosa of the proximal ascendent, distal ascendent, transverse, and descendent colon. We analyzed the records of colon myoelectric activity in vivo in five groups: control, ovariectomized, ovariectomized and treated with estrogen, ovariectomized and treated with progesterone, and pregnant rats. Results: We found a great variation in myoelectric activity in all groups studied. The mean of electric activity did not show statistical difference among the five groups, but pregnant rats had a statistically significant higher duration of maximum electric activity in all distances from the cecocolon junction. Conclusion: Pregnant rats had a statistically higher duration of maximum electric activity. If we could transpose these results to humans, this increase in duration of colon myoelectric activity could explain, in part, the slight constipation that some pregnant women have.
Vanadate-Induced Suicidal Erythrocyte Death
Authors: Föller Michael , Sopjani Mentor , Mahmud Hasan , Lang Florian
Published: March 2008

Vanadium, a trace element, as vanadate (VO43–) is known to interfere with a wide variety of enzymes including Ca2+ ATPase and Na+/K+ ATPase. VO43– is excreted mainly via the kidney. In renal insufficiency, the impaired VO43– excretion leads to VO43– accumulation in blood.The present study explored the effect of VO43– on eryptosis, the suicidal death of erythrocytes. Eryptosis is characterized by cell shrinkage and phosphatidylserine exposure at the erythrocyte surface. Eryptotic cells are phagocytosed and thus rapidly cleared from circulating blood. Stimulators of eryptosis include an increase of the cytosolic Ca2+ concentration. Erythrocyte Ca2+ activity was estimated from Fluo-3 fluorescence, phosphatidylserine exposure from annexin V-binding, and erythrocyte volume from forward scatter in FACS analysis. Exposure of erythrocytes to VO43– increased cytosolic Ca2+ concentration, enhanced the percentage of annexin V-binding erythrocytes, decreased erythrocyte forward scatter, and lowered the intracellular ATP concentration. In conclusion, VO43– induces eryptosis at least partially through increase of cytosolic Ca2+ concentration, an effect presumably contributing to the development of anemia in chronic renal failure.
Antibody Response to Influenza Immunization in Kidney Transplant Recipients Receiving either Azathioprine or Mycophenolate: A Controlled Trial
Authors: Keshtkar-Jahromi Maryam , Argani Hassan , Rahnavardi Mohammad , Mirchi Elham , Atabak Shahnaz , Tara Seied Ahmad , Gachkar Latif , Noori-Froothghe Azam , Mokhtari-Azad Talat
Published: March 2008

Aims: We aimed to assess humoral immune response to the influenza vaccine in adult kidney transplant recipients (KTRs) subjected to two immunosuppressive regimens containing either mycophenolate mofetil (MMF) or azathioprine (Aza). Methods: 40 eligible KTRs (24 treated with Aza [KTRs-Aza] and 16 treated with MMF [KTRs-MMF]) and 40 matched healthy controls (HCs) were administered the trivalent 2006–2007 anti-influenza vaccine. Antibody (Ab) titers were measured before (pre-vacc) and 1 month after (post-vacc) vaccination. The proportion of protective Ab titers (i.e. ≧1:40), the serological response (i.e. ≧4-fold rise in titers) rates, and the magnitudes of change in titers were evaluated. Results: KTRs and HCs were similar in serologic responses, magnitudes of change in Ab titers, and proportions of acquired protective titers against all antigens. Whereas KTRs-MMF and KTRs-Aza were identical in magnitude of rise in titers as well as in serologic responses, KTRs-MMF did poorer in developing post-vacc-protective titers against A/H1N1 (p < 0.05). The function of the transplanted kidney has not deteriorated after vaccination. Conclusions: Anti-influenza vaccination was safe in KTRs and evoked Ab responses comparable to those of HCs. KTRs-MMF and KTRs-Aza responded almost equally to the vaccine. Annual anti-influenza vaccination can be recommended to all stable KTRs.
p75NTR and TrkC Neurotrophin Receptors Demonstrate a Different Immunoreactivity Profile in Comparison to TrkA and TrkB Receptors in Human Normal Pituitary Gland and Adenomas
Authors: Assimakopoulou Martha , Zolota Vassiliki , Chondrogianni Christina , Gatzounis George , Varakis John
Published: March 2008

Background/Aims: Recent knowledge indicates that neurotrophins play a significant role in neuroendocrine systems through their specific receptors TrkA, TrkB, TrkC and low-affinity p75NTR receptor. TrkA and TrkB receptors have been previously detected in numerous endocrine cells in human anterior pituitary and adenomas. In the present study, the localization of p75NTR and TrkC along with TrkA and TrkB receptors was investigated. Methods: Semi-serial paraffin-embedded sections of 5 human normal pituitaries and 30 adenomas were immunostained using specific antibodies. Results: Expression of p75NTR receptor was demonstrated in the intricate capillary and reticulin network in the anterior pituitary and in the pericapillary tissue and pituicytes in the posterior lobe. p75NTR immunoreactivity was absent from all adenomas. In normal anterior pituitary, a few scattered cells showed weak TrkC immunoreactivity in contrast to a high percentage of endocrine cells distributed throughout the pars distalis and pars intermedia which exhibited strong TrkA and/or intermediate TrkB immunoreactivity. Double immunohistochemistry demonstrated TrkA immunoreactivity in more than 80% of lactotropes and 70% of corticotropes and to a lesser extent in other cell types. Furthermore, in the majority of adenomas, independently of type, sex and age, a high percentage of TrkA- and/or TrkB-positive cells was detected. Interestingly, TrkC expression appeared to be increased in some adenomas compared to normal pituitary. Endothelial cells and perivascular connective tissue were always TrkB-immunostained. Conclusion: The above findings support a potential role of all neurotrophins, through their different receptors, in pituitary functions.
Occlusive Arterial Disease of the Child and Young Adult
Authors: Chiofalo N. , Madsen J. , Fuentes A. , Basauri L. , Castro M.
Published: March 2008

26 patients suffering from acute arterial disease, predominant in childhood and young adults, have been studied. Convulsive symptom and electroencephalographic (EEG) findings are especially analyzed. The series are grouped following the Hilal classification, based on angiographical aspects. The incidence and etiological facts as well as the clinical and the angiographical findings, are summarized. The EEG analysis shows the high incidence of localized or lateralized abnormalities (23 out of 26), closely related with the clinical signs. However, there is no close relationship between these findings and the conscious state of the stage of the disease. An attempt is made to correlate the added diffuse and/or paroxysmal activities with the brain damage suspected by the angiographical signs. Seizures are present in 10 cases, 9 of them as initial symptoms predominantly of the focal type. There seems to be no relationship between the incidence and type of fits and the angiographical findings. Follow-up study shows that seizures do not modify the immediate course of the disease nor the persistence of this sequela in 9 of the controlled patients. The histopathological findings of cerebral vessel involvement are reported.
Intracranial Supratentorial Cysts in Children Excluding Tumor and Parasitic Cysts
Authors: Choux Maurice , Raybaud Charles , Pinsard Nicole , Hassoun Jacques , Gambarelli Danièle
Published: March 2008

The intracranial, liquid-containing cysts in children (excluding tumor and parasitic cysts) are relatively frequent in neurosurgical practice. They raise several problems about their nosology, etiology, clinical and radiological diagnosis, and treatment which are analyzed in a series of 36 cases of supratentorial cysts. The most frequent clinical feature is increased head circumference (22 cases) followed by epileptic fits (18 cases), as well as mental and motor retardation (19 cases). Fun-di were found abnormal in only one case out of three. Electroencephalogram was abnormal in almost every case, showing either spikes, spikes and waves or localized slow waves, or an asymmetric depression of the electrical activity. Radiological investigation is essential for diagnosis. Plain radiographs of the skull may show an asymmetry (11 cases). Carotid angiogram and pneumoencephalography give the diagnosis of the lesion without accuracy as to the histology. Computerized axial tomography shows the position of the liquid cavity within the head. The surgical approach (simple shunting [6], direct approach [19], or both [7]) should be carefully considered according to anatomical variety and age of the child. An anatomical classification is proposed, based on the radiological, surgical and pathological findings. Three types of cysts are defined: external cysts (cortical or extracortical) which may or may not be communicating with the ventrioles or the subarachnoid space; internal cysts which again may or may not be communicating; and the corticoventricular cysts. The ultrastructural study represents further progress in the attempt to define the exact anatomical type.
Miliary Fungal Granulomata Confined to the Brain: Mechanism of Meningitis
Authors: Averback P. , Wiglesworth F.W.
Published: March 2008

An unusual case of multiple giant cell granulomata due to Candida albicans confined to the brain of a premature neonate is reported, with evidence to suggest that this fungal meningitis is preceded by a parenchymal focus.
Pre- and Postoperative Cerebrospinal Fluid Absorption Studies in Patients with Myelomeningocele Shunted for Hydrocephalus
Authors: Portnoy Harold D. , Croissant Paul D.
Published: March 2008

Nine infants with myelomeningocele and hydrocephalus had cerebrospinal fluid formation and absorption studies before insertion of a ventriculoperi-toneal shunt. Six were also tested postoperatively. The preshunt formation rate varied from 0.21–0.42 ml · min–1 and the cerebrospinal fluid absorption rate or CSF conductance (Ccsf) varied from 0.008 to 0.031 ml · min–1 · mm Hg–1. The postoperative formation rate varied from 0.21 to 0.39 ml · min–1. The postoperative CSF conductance (Ct) was calculated as the sum of Ccsf and the shunt conductance (Cs). The relationship between the Ccsf and Cs curves was determined by matching the sum of these curves to the postoperative data points using the method of least-squares. The data suggests that the shunt may work directly or indirectly to establish a new resting pressure; the latter by stabilizing the sagittal sinus pressure which preoperatively increased concomitantly with the CSF pressure and thus enhanced an already-existing absorption deficit.
Cricopharyngeal Achalasia Associated with Arnold-Chiari Malformation in Childhood
Authors: Gendell Howard M. , McCallum Jack E. , Reigel Donald H.
Published: March 2008

Six patients with myelomeningocele and the Arnold-Chiari malformation developed cricopharyngeal achalasia and lower cranial nerve deficits. Diagnosis is established by cine-esophagram. Distortion of the brain stem or cranial nerves secondary to the Arnold-Chiari malformation may produce the autonomic imbalance necessary for cricopharyngeal achalasia. Treatment is supportive and includes verification of cerebral spinal fluid shunt function. Suboccipital craniectomy may reverse progressive lower cranial nerve deficits and reduce cricopharyngeus spasm. Cricopharyngeal myotomy may be considered when the cranial nerve deficits and cricopharyngeal achalasia are fixed, irreversible, and continue to cause disability.
Recurrent Headaches in 100 Children
Authors: Millichap Gordon
Published: March 2008

The causes of chronic, recurrent headaches, the electroencephalographic findings and the response to phenytoin (Dilantin) and other medications have been evaluated in 100 children. A history of head injury was reported in 41% and convulsions had occurred in 15%. Electroencephalographic dysrhythmias were severe in 18 and moderate in 27%. Migraine was diagnosed in 42% and tension headaches in 18%; psychogenic factors complicated learning disabilities and minimal brain dysfunction in 21%. Phenytoin controlled migraine in 77% and headaches diagnosed as seizure equivalents in 40%; the response was unrelated to the degree of electroencephalographic abnormality. An abnormal electroencephalogram and response to phenytoin are insufficient criteria for a diagnosis of epilepsy in children with recurrent headaches.
Interhemispheric Subdural Empyema: Neuroradiological Diagnosis
Authors: Rao Krishna C.V.G. , Williams Powell , Brennan Thomas G. , Kosnik Edward
Published: March 2008

A case of subdural empyema situated in the interhemispheric subdural space is described. CT scan is useful in the diagnosis and follow-up of intracranial inflammatory pathology. Selective angiography was helpful in the compartmental localization of the abscess in the interhemispheric subdural space, adjacent to the falx and tentorium by virtue of the hypertrophied tentorial meningeal arteries and vascular displacement. CT scans are also useful in follow-up of cerebral abscess after micropaque barium has been injected into the cavity as in this patient.
Intraventricular Chloramphenicol
Authors: Salmon James H.
Published: March 2008

Very high intraventricular chloramphenicol levels can be obtained if the standard systemic dose is supplemented with a small intraventricular dose. Chloramphenicol sodium succinate can be hydrolyzed to the microbiologically active chloramphenicol in the ventricular fluid. Daily injections are ordinarily adequate to maintain a high concentration of antibiotic. Initial dosage should vary with ventricular volume. The brain does not tolerate repeated needle puncture and the use of a ventriculostomy reservoir is recommended.
Anterior Cervical Fusion after Traumatic Dislocation of the Cervical Spine in Childhood and Adolescence
Authors: Wickboldt Jürgen , Sörensen Niels
Published: March 2008

5 patients aged 11–16 years with traumatic lesions of the cervical spine are reported. 4 patients were treated by operative ventral fusion with good results. Regarding the preoperative history of these 4 patients and the history of another 1 treated by conservative means, the authors tend to relatively early operative ventral fusion.
External Drainage for Ventricular Infection following Cerebrospinal Fluid Shunts
Authors: Scarff Timothy B. , Nelson Paul B. , Reigel Donald H.
Published: March 2008

57 children with shunt-related ventricular infection were treated with external ventricular drainage and a combination of systemic and intraventricular antibiotics. For persistent infection or inadequate drainage volume the external ventricular drain was promptly changed. Cerebrospinal fluid cultures of 54 (95°/o) of the children became sterile within an average of 5.1 days. As soon as three consecutive 48-hour cultures of the cerebrospinal fluid were negative, the external ventricular drainage was removed and a new ventriculoperitoneal shunt was inserted. Provided that three consecutive 48-hour cultures of the cerebrospinal fluid were negative at the time of reinstitution of ventriculoperitoneal shunting, low cerebrospinal fluid glucose readings were not associated with any greater risk of recurrent infection than following primary ventriculoperitoneal shunting.
Normotopic plus Heterotopic Atypical Teratomas
Authors: Camins Martin B. , Takeuchi Jim
Published: March 2008

6 cases of normotopic plus heterotopic atypical teratomas are presented. The difficulty of making an accurate early diagnosis is stressed. Suggestions for interpreting both clinical and radiograph data are presented.
Pyocephalus of the Newborn Child
Authors: Izquierdo J.M. , Sanz F. , Coca J.M. , Vila F. , Dierssen G.
Published: March 2008

3 cases of pyocephalus (ventricular empyema) in newborn children are described and 16 cases from the literature are analyzed. Half of the previously published cases of pyocephalus are secondary to cerebral abscesses. Neuroradiological findings and especially the necroscopic study of a case demonstrated the existence of membranous blockages inside the ventricles which may make the circulation of CSF impossible. Our treatment of choice consists of repeated punctures and evacuations of purulent content and topic as well as parenteral administration of antibiotics. Further CSF derivative measures are almost always necessary. By these means, we believe the high mortality of these cases could be lowered.
Computerized Axial Tomography in the Preoperative Evaluation of Posterior Fossa Tumors in Children
Authors: Rappaport Z.H. , Epstein F.
Published: March 2008

22 cases of pediatric posterior fossa tumors that underwent preoperative evaluation by computerized tomography are presented. 8 of the cases, in the latter part of the series, were successfully operated upon on the basis of computerized tomography alone. In 14 other cases, additional angiographic investigation was carried out. In only 1 case of multiple hemangioblastoma did angiography provide further significant information. On the basis of this experience, it is suggested that computerized tomography is usually sufficient and further contrast studies are unnecessary in the preoperative evaluation of pediatric posterior fossa tumors.
Long-Standing Myelomeningocele Associated with Spinal Subarachnoid Hemorrhage
Authors: Yusefovic T. , Avram J. , Tiberin P.
Published: March 2008

The fate of a giant sacral myelomeningocele in a boy aged 7 years is reported. A striking feature of this case is its unusual conjunction to subarachnoid hemorrhage from vascular hamartoma of its wall. The pathophysiological and clinical implications of this observation are reviewed.
The Central (Spinal) Canal in Congenital Murine Hydrocephalus: Morphological and Physiological Aspects
Authors: Kuwamura Keiichi , McLone David G. , Raimondi Anthony J.
Published: March 2008

Obstruction of cerebrospinal fluid pathways produces changes in the parenchyma of the brain and spinal cord. A murine model of hydrocephalus (hy-3) used to study changes in the central canal of the spinal cord and these changes are correlated with events occurring in the intracranial compartment. The central canal is enlarged and subependymal interstitial edema is produced by cerebrospinal fluid at the same time as the intracranial ventricular system is expanding. Plugging of the obex results in collapse of the central canal. Ferritin tracer studies demonstrate transependymal flow of cerebrospinal fluid throughout the central canal.
Cerebral Arteriovenous Malformations in Children
Authors: Cho So Sing
Published: March 2008

A series of 36 children under the age of 13 with cerebral arteriovenous malformations (AVM) was retrospectively reviewed. It is shown that haemorrhage is by far the commonest initial manifestation of the lesion, followed by seizures. Of the 14 children who have seizures, 10 suffered from haemorrhage within an observation period of 3 months to 7 years. Analysis of the results of the various forms of treatment indicates that total surgical extirpation provides the best safeguard against future haemorrhage. It is suggested that all cerebral AVM in children should be excised whenever possible.
Unusual Subarachnoid Metastasis of an Intracranial Chordoma in Infancy
Authors: Plese Píndaro , Borges J.M. , Nudelman Marisa , Lefèvre A.B. , Sallum J.
Published: March 2008

The authors report a case of large clivus chordoma in a 6½-year-old child with multiple subarachnoid metastatic dissemination over the cerebellar hemispheres and spinal cord.
Lumbar Spinal Stenosis
Authors: Bowen Vaughan , Shannon Robert , Kirkaldy-Willis W.H.
Published: March 2008

(1) In discussing the types of lumbar spinal stenosis, emphasis has been placed on the developmental form. (2) The importance of nerve entrapment in the lateral recess has been stressed. (3) An attempt has been made to demonstrate the relationship of developmental stenosis to other lesions affecting the lumbar spine. (4) Our knowledge of the development of the lumbar spine and its patho-anatomy in children is limited. (5) Attention to minor trauma and to preventive measures during childhood will do much to prevent stenosis in later years.
Experimental Hydrocephalus: Cerebrospinal Fluid Dynamics and Ventricular Distensibility during Early Stages
Authors: Drapkin Allan J. , Sahar Abraham
Published: March 2008

Adult craniectomized cats, rendered hydrocephalic by intracisternal kaolin injection, were repeatedly studied as to parameters pertaining to cerebrospinal fluid (CSF) dynamics and ventricular size. Intraventricular pressure was up to 8-fold of normal (mean 17.1 cm H2O) initially after induction of hydrocephalus and then went gradually down after 1 month. Ventricular volumes attained their maximum volume of approximately 4.5 ml at the very earliest study. The absorptive reserve (excess of absorption over formation rate of CSF) increased with time, thereby theoretically enabling the ‘arrest’ of the hydrocephalic process. Ventricular distensibility also increased with time, thus even the low ventricular pressure maintains the larger ventricular volume.
Surgical Treatment of Spasticity in Cerebral Palsy
Authors: Fasano V.A. , Broggi G. , Barolat-Romana G. , Sguazzi A.
Published: March 2008

Since 1971, we have surgically treated spasticity in 109 cases of cerebral palsy with posterior rhizotomy. The best results have been obtained with functional posterior rhizotomy, an original modification of Foerster’s technique, in which the selection of the roots/rootlets to be sectioned is based on functional exploration of the spinal circuits involved in the maintenance of spasticity, using intra-operative electrostimulation on dorsal roots. This method makes selective sections possible, thereby saving a larger number of normal proprioceptive afferents, with consequent reduction in negative side effects. No recurrencies have been observed in our 3-year follow-up. The main indication is Little’s disease.
Hydrocephalus in Children Treated with the One-Piece Shunt
Authors: Villarejo F. , Heredero J. , Amaya C.
Published: March 2008

The results of the surgical treatment of 50 children with hydrocephalus with a new one-piece shunt are presented. The advantages and disadvantages of this shunt are discussed.
Brain Tumor in Newborn Babies
Authors: Takaku Akira , Kodama Namio , Ohara Hiroo , Hori Shigeaki
Published: March 2008

5 cases of brain tumor in newborn babies under 2 months are presented. 4 of them were supratentorial teratoma and originated from the midline, and 1 was a glioma at the cerebellopontine angle. 2 cases died before surgery and 2 cases after surgery. In our 5th case a benign teratoma of 150 g was removed from the third ventricle. He was discharged and enjoyed rather good health for 2 years and 1 month before dying of recurrence of tumor. Internal hydrocephalus associated with the huge tumor was responsible for the sudden deterioration of general condition. Therefore, shunting procedures should be performed as the first aid. However, in our experiences, the conditions at admission were too critical and/or too late to be beneficial. Thus, early diagnosis is absolutely necessary for surgical treatment. A summary and discussion of the data of 103 previously reported cases are included.
Experimental Progressive Hydrocephalus in the Young Animal
Authors: Sahar A.
Published: March 2008

22 puppies with kaolin-induced hydrocephalus were repeatedly studied for 4 months in respect to their head size, ventricular size, pressure and cerebrospinal fluid (CSF) dynamics. The intraventricular pressure was markedly elevated during the first week and dropped towards the end of the first month of the hydrocephalic process. It then escalated and again returned to normal values after 3–4 months. Formation of CSF increased with age until it reached adult values of 0.049 ml/min at the age of 3 months. Absorption rates were very low initially. The absorptive capacity surpassed formation in the puppies which survived to the fourth month of the process. Ventricular size and rate of head enlargement correlated inversely with thickness of the skull. In the surviving puppies (4 out of 22) the hydrocephalic process seemed to have arrested.
Problems of Diagnosis and Treatment in the Dandy-Walker Syndrome
Authors: James Hector E. , Kaiser George , Schut Luis , Bruce Derek A.
Published: March 2008

10 children with Dandy-Walker syndrome are presented to discuss their clinical assessment and therapy. The presence on physical examination of a large posterior fossa was the most reliable clinical finding and was diagnostic in 9 out of the 10 patients. The different diagnostic confirmatory studies are discussed. The most satisfactory treatment in this series was the combination of a shunt from the lateral ventricle and the posterior fossa to the peritoneum.
Ventriculopleural Shunting in the Management of Hydrocephalus
Authors: Venes Joan L. , Shaw Richard K.
Published: March 2008

6 cases of ventriculopleural shunting for control of hydrocephalus are presented. In the older child and adult, drainage of cerebrospinal fluid into the pleural cavity seems to be well tolerated in exceptional circumstances where other shunt procedures, including intracranial shunts, are contraindicated. In the infant and young child, accumulation of fluid in the pleural space is enchanced by a poorly understood immune-related mechanism and may lead to significant pulmonary dysfunction. On the basis of our experience, this procedure appears contraindicated in this age-group.
Computed Tomography of Sturge-Weber Disease
Authors: Maki Yutaka , Semba Akio
Published: March 2008

Findings in computed tomography (CT) with or without contrast infusion were analyzed in 8 patients with Sturge-Weber disease. From the extent of calcification in the CT, Sturge-Weber disease can be classified into two types, localized and diffuse. The extent of calcification or cortical atrophy is predictable, if it can be shown that enhanced areas in contrast infusion CT truly represent leptomeningeal angiomatos¡s. Thus, using enhanced CT, the capacity of prediction as to whether the patient will have a localized or diffuse type of disease will be possible with further observations.
Cerebral Medulloepithelioma
Authors: Scheithauer B.W. , Rubinstein L.J.
Published: March 2008

A cerebral medulloepithelioma, a rare embryonal multipotential central neuroepithelial neoplasm of childhood, is described, bringing to 16 the total number of cases reported in the literature. It is the first example recorded to date which displayed the entire range of differentiation from embryonal to mature cells, of both glial and neuronal lineage (i.e., primitive medullary epithelium, spongioblasts, astrocytes, oligodendroglia, ependymal cells, neuroblasts, and mature ganglion cells). The significance of this tumor as a paradigm of multipotential divergent neuroepithelial differentiation is discussed.
Hydrocephalus and Elevated Intracranial Venous Pressure
Authors: Young Byron
Published: March 2008

An infant with a large arteriovenous malformation and communicating hydrocephalus is described. The author suggests that the hydrocephalus is caused by impaired absorption of cerebrospinal fluid due to increased intracranial venous pressure.
Spontaneous Spinal Epidural Haematoma in a Youngster
Authors: Ventureyra Enrique C.G. , Ghanem Qais , Ivan Leslie P.
Published: March 2008

The case of an 8-year-old boy with spontaneous spinal epidural haematoma and complete post-operative recovery is described. A review of the literature with particular emphasis on the paediatric age-group is given. The clinical presentation and aetiology are discussed.