bluuebunny/crossref_metadata_2025 huggingface dataset:
Efficacy of Humalog® injections before an afternoon meal and their acceptance by children and adolescents with Type 1 diabetes
Authors: Martin D. , Licha‐Müntz G. , Grasset E. , Grenèche M. O. , Nouet D. , François L. , Legrand C. , Polak M. , Augendre‐Ferrante B. , Tubiana‐Rufi N. , Robert J. J.
Published: March 2003

Aims To evaluate the acceptability and efficacy of an injection of insulin lispro, before an afternoon meal.

Methods The subjects, 43 patients with Type 1 diabetes, 16 boys and 27 girls, aged 12.4 ± 2.4 years, were randomly assigned to the treatment (n = 20) or the untreated control group (n = 23). The treatment was an injection of insulin lispro immediately before the afternoon meal. The control group had no injection. The treatment and the control group consumed identical types of meals for 2 months. The mean before‐dinner blood glucose was measured during the last 2 weeks of the study.

Results Injection of insulin lispro resulted in a significant reduction in the before‐dinner blood glucose compared with the untreated control group (10.4 ± 3.8 mmol/l vs. 14.7 ± 3.9 mmol/l, respectively). The number of days on which the blood glucose was > 10 mmol/l was reduced by half in the insulin lispro group. The difference in HbA1c between baseline and endpoint differed slightly but significantly between the two groups, in boys. Treated patients ate the meal less frequently (11.4 ± 3.0 times per 15 days) than the control patients (14.4 ± 0.6 times per 15 days) and injected themselves with insulin 8.9 ± 3.6 times per 15 days. The HbA1c increased significantly with the number of meals taken without injection. There was no statistically significant difference in the frequency of hypoglycaemia or changes in weight between the two groups.

Conclusions We conclude that an injection of insulin lispro before the afternoon meal can effectively lower the before‐dinner blood glucose, and in boys also lowers the HbA1c. Patients were satisfied with the lower blood glucose before dinner, and did not find the insulin lispro injection difficult. However, compliance with the protocol procedures decreased during a subsequent 6‐month period.

Diabet. Med. 19, 1026–1031 (2002)
The nature and diversity of neotropical savanna vegetation with particular reference to the Brazilian cerrados
Authors: Furley Peter A.
Published: March 2003

1. The South and Middle American tropics contain the world's largest area of moist savanna. Despite an apparent uniformity in appearance, floristic groupings can be detected and four zones are provisionally outlined with a number of character‐ istic plants.2. Although current knowledge of their bio‐ geography and palaeoecology is scattered and incomplete, it is evident that there have been significant changes throughout the neotropics since the glacial maximum.3. The largest and most significant savanna formations are the Brazilian cerrados, which occupy nearly 23% of that country, an area equivalent to that of western Europe. The cerrados range from savanna woodlands (cerradão), through progres‐ sively less arboreal formations (cerrado), to nearly treeless grasslands (campo). A number of adjoining vegetation formations are closely related, including evergreen and deciduous forests, scrub woodlands and wetlands.4. Until the last decade, the biological diversity of the neotropical savannas was thought to be limited since, structurally, they appeared to form a relatively simple mosaic of repeated physiognomic units. New evidence reveals a rich diversity of tree, shrub and herbaceous plants in a continuously changing spatial pattern. Such diversity reflects the great area of the biome, together with local differences in topography and drainage, soil conditions and fire frequency.5. Research on the dynamic processes affecting savannas is of increasing importance and urgency given the potential value of the area that is still relatively undisturbed (about 50% of the original, but with less than 2% having federal reserve status) and the pace of current conversion to different forms of land use.
Taxonomic level, trophic biology and the regulation of local abundance
Authors: Kaspari Michael
Published: March 2003

Taxocenes — monophyletic ecological assemblages — are a key focus of macroecology. Abundance (individuals per area) is a basic property of taxocenes but has received less attention than diversity, although the two are probably related. Abundance reflects a taxocene’s ability to harvest and sequester available energy and divide it among individuals. This paper explores how two properties of all taxocenes — trophic makeup and taxonomic level (e.g. genus, tribe, subfamily, family … ) — may contribute to patterns of local abundance at geographical scales.

Forty‐nine ground ant taxocenes, in habitats ranging from New World deserts to rain forests, were surveyed along a three‐orders of magnitude productivity gradient using transects of 30 1‐m2 quadrats at each site. Abundance — the number of nests per transect — varied over two orders of magnitude.

Over 80% of the genera collected were omnivores. However, herbivore, omnivore, and predator taxa were added to ant taxocenes in roughly 1 order of magnitude steps up the productivity gradient. Specialist detritivores were added last.

Net primary productivity and mean monthly temperature both consistently entered regression models predicting abundance. However, while productivity was the dominant predictor of abundance for higher taxa (families, subfamilies), temperature was the dominant predictor of abundance for lower taxa (tribes, genera). The answer to the question ‘What regulates the abundance of a taxocene?’ is thus sensitive to the taxonomic level of analysis.

These data support the following scenario. Lower taxa are abiotic specialists given the insufficient number of genomes and generations required for the exploration of the entire abiotic envelope. Higher taxa, in contrast, consist of suites of abiotic specialists arrayed along the entire productivity gradient, with access to productivity everywhere the taxon occurs. If this scenario is true, individual species may respond to global changes in temperature; the higher taxa they belong to may most respond to global changes in productivity.
Vegetation regeneration on blanket mire after mechanized peat‐cutting
Authors: Cooper A. , Mccann T. P. , Hamill B.
Published: March 2003

Blanket mire in Northern Ireland is an ecologically threatened habitat in which land use for hand peat‐cutting, forestry and agriculture has had a major influence. A recent land use change is the introduction of tractor‐powered peat‐harvesting. In this paper, the effect of machine peat‐cutting on ombrotrophic blanket mire vegetation is assessed from a regional sample of cut and uncut plots.

Principal components analysis identified water‐table depth and grazing intensity as major factors influencing the species composition of uncut mire. A key variable affecting the composition of machine‐cut mire across the drainage gradient was the number of times cut, with multiple annual cutting causing progressive decreases in acrotelm depth, catotelm bulk density and plant cover. Ericaceous species and Sphagnum spp. were particularly sensitive to cutting, with Eriophorum angustifolium and Campylopus introflexus characteristic of multiple‐cut sites.

Redundancy analysis, with number of times cut partialled out, showed that recovery time accounted for a significant amount of variance in vegetation composition. Species that significantly increased in abundance with recovery time were Sphagnum spp., Odontoschisma sphagni

, Erica tetralix and Drosera rotundifolia.

Sites cut frequently, or which were grazed, recovered more slowly. Recovery from cutting was partly dependent on the post‐cutting structure of the mire surface and the species that survive cutting. The rate of recovery on sites cut once, then abandoned, is relatively rapid compared with multiple‐cut sites where species colonization is constrained by bare compacted peat.
Evidence for non‐uniform dispersal in the biological invasions of two naturalized North American bird species
Authors: Gammon David E. , Maurer Brian A.
Published: March 2003

Most previous attempts to model the geographical range expansion of an invading species assume random dispersal of organisms through a homogeneous environment. These models result in a series of uniformly increasing circles radiating out from the centre of origin over time. Although these models often give reasonable fits to available data, they do not typically include mechanisms of dispersal. Alternatively, models that include assumptions of non‐random dispersal and a heterogeneous environment inevitably result in an anisotropic or jagged invasion front. This front will include propagules of pioneer individuals for the expanding species. Existing data from biological invasions reveal that the spatial structure of an invading species usually exhibits these propagules. Using population data gathered from the past century, we investigated the propagules of two North American invading bird species: the European starling (Sturnus vulgaris Linnaeus), and the house finch (Carpodacus mexicanus Müller), and found a correlation between propagule location and habitat quality. These results suggest that dispersing individuals seek out favourable habitat and remain there, thus introducing a possible mechanism for explaining non‐uniform dispersal during invasions. When combined with results from other studies, our results suggest that propagules provide starting points for future population expansion of an invading species.
Efficient SNP‐based tests of association for quantitative phenotypes using pooled DNA
Authors: Bader Joel S. , Bansal Aruna , Sham Pak
Published: March 2003

Introduction  Genetic factors underlying complex diseases are difficult to identify: many polymorphisms may contribute, each having a small effect and low penetrance. These factors may be identified by association studies of large populations, an alternative to family‐based linkage studies. Allele frequency measurements of pooled DNA selected from population‐level DNA repositories can reduce the costs of these studies. We provide guidance for selecting unrelated individuals for pooling and for comparing the power of studies based on pooled measurements to the power of individual genotyping, particularly for studies using single‐nucleotide polymorphism (SNP) markers.

Materials and methods  We used exact numerical calculations to set pooling criteria that maximized the power to detect association as a function of marker frequency, inheritance mode, and additive variance. Analytical approximations are also provided.

Results and discussion  Power estimates are provided for two pooled DNA designs: the classification of individuals as affected or unaffected, analogous to a case‐control design, and the optimized selection of individuals with extreme phenotypic values. Optimized selection is approximately fourfold more efficient than affected/unaffected classification. The optimal design for most markers is to pool the top and bottom 27% of individuals. Neglecting experimental measurement error, this design requires a population only 1.24‐fold larger than that required for individual genotyping. When measurement error is included, the pooled DNA association test serves better as a pre‐screen to identify candidate markers which then proceed to individual genotyping. This strategy can still provide a 100‐fold savings over individual genotyping.
The histamine 1 and histamine 2 receptor genes—candidates for schizophrenia and clozapine drug response
Authors: Mancama D. , Arranz M. J. , Munro J. , Makoff A. , Kerwin R.
Published: March 2003

Introduction There is growing evidence to suggest the involvement of histaminergic pathways in the pathophysiology of schizophrenia. Overactive histamine activity is thought to contribute significantly to deficit symptoms such as apathy and social withdrawal associated with the disorder, while the efficacy of certain atypical antipsychotics may include their action at histamine receptors. By selecting the histamine 1 (H1) and histamine 2 (H2) receptor genes as candidates, the aim of this study was to investigate the possible involvement of these receptors both in schizophrenia and in the clinical response of patients undergoing clozapine treatment.

Method Using single strand conformation polymorphism (SSCP) analysis we screened the complete coding region of each receptor gene for mutations in 50 schizophrenics and 50 unaffected individuals. Automated sequencing was used to confirm the presence and nature of each detected polymorphism.

Results We report here the identification of five previously undescribed H1 receptor polymorphisms (Lys19Asn, Asp349Glu, 1068‐A/G, Phe358Δ and Leu449Ser) and a single novel H2 receptor polymorphism (543‐G/A). Initial analysis of these polymorphisms in case vs. control association studies revealed a significant increase in distribution of one, the H1Ser449 allele, in our control sample (13/97) compared with the group of schizophrenics (0/90), prompting further analysis of this polymorphism in an extended patient sample population. Overall we observed a trend towards increased frequency of this allele amongst our controls compared with the corresponding group of schizophrenics, though the significance was estimated to be marginal (χ2 = 3.94, P = 0.047). Similarly, no conclusive evidence could be found to suggest the involvement of any other H1 and H2 receptor variants in schizophrenia or clozapine response. Further investigation of the five polymorphic H1 loci by haplotype analysis indicated a lack of association between these loci and both schizophrenia and clozapine response.

Conclusions These results suggest that histamine H1 and H2 receptor variants do not bear significant influence on the susceptibility of individuals to schizophrenia, nor do they appear to influence clinical response to clozapine treatment.
Power of the TDT to detect association: effect of linkage disequilibrium between genetic markers and an unobserved disease mutation
Authors: Lewis Cathryn M. , Fisher Sheila A.
Published: March 2003

The transmission disequilibrium test (TDT) is widely used to test for association in families. Many previous papers have shown sample size calculations where the true disease mutation is tested. When a neighbouring marker is used in place of the mutation, the power to detect association depends on the linkage disequilibrium between the loci. A difference in frequency between the mutation and the tested allele, or a reduction in linkage disequilibrium will substantially increase the required sample size. If a study design includes reasonable assumptions regarding linkage disequilibrium, using the TDT with anonymous nonfunctional markers may not be feasible.
Precise mapping of the fragile siteFRA12Aon chromosome 12q13.1
Authors: Winnepenninckx Birgitta , Reyniers Edwin , Bossuyt Paul , Smits Arie , Wauters Jan , Kooy R. Frank
Published: March 2003

Introduction A causative relationship has been reported between fragile site expression and disease forFRA12A, a rare, folate‐sensitive fragile site on chromosome 12q13.1.FRA12Aexpression has been described in a number of patients with mental retardation, sometimes in combination with clinical abnormalities. In correspondence to the molecular mechanism of previously cloned, rare, fragile sites, it may be expected thatFRA12Ais caused by repeat expansion, affecting the expression of genes in the region. To identify the repeat and the associated gene, this paper reports the precise mapping ofFRA12Aon chromosome 12q12–13.

Methods Fluorescencein situhybridization (FISH) techniques were used to mapYACandPACclones in the neighbourhood ofFRA12A.PACDNA pools andPACfilters were screened to find additionalPACclones spanning the candidate region. Markers in the region were obtained via web searches and used to construct bothPACandYACcontigs.

Results and Discussion A singleYACclone that overspans the fragile site was identified and a completeYACandPACcontig for theFRA12Aregion was constructed. The region contains several candidate genes, including a calcium ion channel (CACNLB3), a GTP‐binding factor (ARF3), a gene involved in brain development (INT1) and two other genes involved in developmental processes (WNT10BandALR). TheFXR1gene, a homologue of theFMR1gene, that is associated with fragile X syndrome and that maps to chromosome 12q12–13, was ruled out as a possible candidate gene for theFRA12Asite.
How behavioural studies contribute to the species problem: a piscine perspective
Authors: Barlow George W
Published: March 2003

During the ascendency of ethology, the study of animal behaviour contributed to taxonomic and phyletic issues, but the approach faded in parallel with the waning of systematic studies in general. Systematics and particularly phylogeny has experienced a resurgence with the advent of molecular tools. The understanding of behavioural evolution has benefited from molecular phylogenies. Behaviour's reciprocal contribution, however, currently lies chiefly in resolving the validity of species designation of allopatric species and in potentially helping in the overall classification of species. I review and evaluate the experimental methods favoured in seeking behavioural isolating mechanisms, and the challenge to the species concept when gene trees and species trees disagree. The dynamic situation in the Great Lakes of Africa offers under‐exploited opportunities to test whether behavioural isolating mechanisms evolve in the absence of secondary contact. Given that behaviour is more immediately responsive to changes in the physical and biological environment, behaviour may be the single most salient adaptive feature in speciation events. The species concept is viewed as a fuzzy but nonetheless useful set.
The relationship between fishing methods, fisheries management and the estimation of maximum sustainable yield
Authors: Maunder Mark N
Published: March 2003

The allocation of effort among fishing gears is as important as controlling effort with respect to both sustainable yield and ecosystem management. Differences in age‐specific vulnerability to the fishing method can modify the maximum sustainable yield (MSY) that is obtainable from a fish stock. Different gears or methods are more or less selective for the species targeted, and MSY is rarely, if ever, attainable simultaneously for all species. The different fishing methods capture different types of nontarget species. Some methods will often be more profitable than others, and different user groups will prefer different methods. In many fisheries, it is unlikely that fishing can be limited to a single gear or method, so compromises among them will be required. Global MSY is discussed as a possible reference point for fisheries management. The yellowfin tuna fishery in the eastern Pacific Ocean (EPO) shows all the above characteristics and is used to illustrate effort allocation among fishing methods.
Prevalence of high IgM anticardiolipins in patients with ischemic stroke
Authors: Szczepański M. , Cieślak E. , Gardas A. , Bauer A. , Majkowski J.
Published: March 2003

Lupus anticoagulant (LA), IgG and IgM isotypes of anticardiolipins (aCL), lipoprotein (a), and the resistance to activated protein C were determined in patients with ischemic stroke. The raised concentration of the aCL‐IgM isotype was noted in 42% of patients with this type of stroke, and it was in contrast with an 8% frequency of an increased level of aCL‐IgG isotype in these cases. The high level of lipoprotein (a) was found with similar frequency in stroke patients and in age‐matched control subjects. It is concluded that the elevated concentration of IgM isotype of anticardiolipin antibodies can be regarded as significant in the ethiological work‐up in elderly stroke patients.
Myoclonic astatic attacks in patients suffering from Parkinson's disease
Authors: Henneberg Alexandra E. , Schöll Irma , Kogan Emanuel
Published: March 2003

Myoclonic astatic seizures are well known in children's generalized epilepsy. Some of our patients suffering from Parkinson's disease complained about sudden loss of postural reflexes together with dizziness or short loss of consciousness. When we looked into their electroencephalograms (EEG's), we found polyspikes and polyspike‐wave complexes that were seen especially after provocation by hyperventilation or photic stimulation. An additional therapy with sodium valproate diminished the myoclonic astatic attacks as well as the alterations in the EEG in all of the patients. None of them experienced a deterioration in their Parkinson's disease.
Selective proper name anomia in a patient with asymmetric cortical degeneration
Authors: Fadda Lucia , Turriziani Patrizia , Carlesimo Giovanni A. , Nocentini Ugo , Caltagirone Carlo
Published: March 2003

All cases of selective anomic deficit for proper names described until now are a consequence of focal cerebral lesions. In the present paper, we report the case of a patient, AF, with a deficient access to proper names of persons and normal access to common names probably subsumed by a degenerative process of the left cerebral hemisphere. MRI evidenced an atrophy of the left temporal lobe and SPECT highlighted hypoperfusion of the left hemisphere. Neuropsychological examination documented a deficient production of proper names belonging to famous personalities both on visual presentation and verbal definition. Moreover, on verbal fluency tasks, AF was poor for the categories of famous peoples and some commercial articles. Finally, AF was errorless in a proper name comprehension task.
Visual hallucinations in a case of reversible hypertension‐induced brain oedema
Authors: Tallaksen Chantal M.E. , Kerty Emilia , Bakke Søren
Published: March 2003

Visual hallucinations are commonly associated with seizures drug effects psychiatric disorders or visual loss as ‘release’ phenomena. We report the case of a previously healthy 65‐year‐old woman who was admitted to hospital with intermittent headache episodes accompanied by complex visual hallucinations. During these episodes the patient's blood pressure was 220/120 mm Hg. In between symptomatic episodes she had no complaints and felt healthy. The neurological and ophthalmological examinations were normal but cerebral magnetic resonance imaging (MRI) showed multiple white matter abnormalities in the parieto‐occipital regions. Rapid reversal of the symptoms and imaging abnormalities occurred concurrently with lowering of blood pressure. The history and the findings were similar to those recently described in the clinicoradiological ‘posterior leukoencephalopathy’ syndrome. Different pathogenic mechanisms are discussed.
CTG‐repeat length in distal and proximal leg muscles of symptomatic and non‐symptomatic patients with myotonic dystrophy: relation to muscle strength and degree of histopathological abnormalities
Authors: Hedberg Birgitta , Anvret Maria , Ansved Tor
Published: March 2003

Myotonic dystrophy (DM) is an autosomal dominant, multisystemic disorder with a variable phenotypic expression including muscle weakness and myotonia. The muscle wasting is most marked in distal limbs and in facial and neck muscles, although proximal limb muscles become affected as the disease progresses. The CTG‐trinucleotide‐repeat expansion associated with myotonic dystrophy is usually larger in muscle tissue than in leukocytes. It is unclear whether the repeat length itself bears any relation to the differences in the degree of weakness and atrophy between different muscles. We therefore analysed CTG‐repeat lengths in blood and in proximal (m. vastus lateralis) and distal (m. tibialis anterior) muscles of patients with DM (n= 4) and non‐symptomatic carriers of the mutant DM allele (n= 2) using conventional Southern blot hybridization. Muscle strength and histopathological abnormalities were evaluated for each muscle. In patients with clinical symptoms, the degree of paresis and morphological abnormalities was markedly more pronounced in m. tibialis anterior than in m. vastus lateralis. In these individuals, the CTG‐repeat length was larger in muscles than in leukocytes, whereas in the two non‐symptomatic carriers no difference could be detected. Furthermore, there was no clear difference in the repeat length between the two muscles in any of the patients. In conclusion, the selective muscular weakness and atrophy in DM do not seem to be related to differences in CTG‐repeat length between different muscles.
Contrast enhanced pulsed Doppler and colourcoded Duplex studies of the cranial vasculature
Authors: Haggag Kathrine J. , Russell David , Brucher Rainer , Dahl Arve , Jakobsen Jarl , Bakke Søren , Muan Berit
Published: March 2003

Introduction: The aim of this study was to assess the effect of Albunex, a vascular contrast agent based on albumin‐coated air microbubbles, on pulsed Doppler and colour‐coded duplex sonography of the cranial vasculature. Methods: Twenty healthy male volunteers received intravenous injections of contrast in single doses ranging from 0.08 to 0.30 ml/kg. Pulsed wave Doppler sonography examination and colour‐coded duplex sonography were carried out in the right internal carotid artery (ICA) and middle cerebral artery (MCA) before and after i.v. contrast. The relative intensity increase of the Doppler signal was measured in decibels. Results: Transpulmonary passage of contrast occurred in sufficient amounts to enhance the intensity of the Doppler signal significantly, but the duration of this effect was short. Contrast enhancement also improved visualization of both the ICA and MCA in all subjects. For the transcranial examinations, this resulted in visualization of a greater length of the middle cerebral arteries and additional vessels in the Circle of Willis. Conclusions: These results confirm that contrast enhancement can significantly improve the quality of Doppler examination and colour‐coded duplex sonography of both the intracranial and extracranial vessels. However, the use of Albunex in neurosonology will be of limited value due to its relatively short duration.
Bilateral symmetrical enhancing brainstem lesions: an unusual presentation of primary CNS lymphoma
Authors: Stankoff Bruno , Calvez Vincent , Suarez Sandra , Bossi Philippe , Rosenblum Ouriel , Conquy Leslie , Turell Enrique , Dubard Thierry , Coutellier Anne , Baril Laurence , Bricaire François , Lacomblez Lucette , Lubetzki Catherine
Published: March 2003

Cerebrospinal fluid (CSF) and plasma HIV‐1 RNA levels were prospectively measured by the Roche Amplicor Monitor polymerase chain reaction assay in 30 HIV‐1 infected patients without central nervous system opportunistic infections. All participants completed a global neuropsychological battery consisting of Mattis Dementia Rating Scale. Additional tests were use to better characterize the type of cognitive changes with a specific reference to frontal lobe function. The neuropsychological evaluation confirmed the subcortical pattern of cognitive dysfunction. CSF and plasma HIV‐1 RNA levels were significantly correlated. No correlation was detected with either blood or CSF RNA levels and the global cognitive status, but when stratified in three cognitive subgroups, higher CSF HIV‐1 RNA levels were observed in the more cognitively impaired subjects. Our results provide further evidence that plasma and CSF HIV‐1 RNA level cannot be used as a reliable diagnostic marker for HIV‐1 associated cognitive disorders. Only longitudinal studies will determine whether a high CSF HIV‐1 level could be a risk factor for HIV‐1 dementia.
Muscular Dystrophy in Children. A Guide for Families
Authors: Jellinger K. A.
Published: March 2003

Irwin M. Siegel. Demos Medical Publishing, New York, NY, 1999. 130 pp., ISBN 1–888799–33–1, US$ 19.95 (paperback).
Prediction of angiographic carotid artery stenosis indexes by colour Doppler‐assisted duplex imaging. A critical appraisal of the parameters used
Authors: Bönig L. , Weder B. , Schött D. , Keel A. , Nguyen T. , Zaunbauer W.
Published: March 2003

The aim of our study was to establish colour Doppler‐assisted duplex imaging (CDDI)‐criteria to predict an angiographic internal carotid artery (ICA) stenosis of at least 70%, according to the North American Symptomatic Carotid Endarterectomy Trial (NASCET) and European Carotid Surgery Trialists (ECST) methods of measurement. In the following, we describe the findings in 79 patients who were screened for carotid endarterectomy by CDDI and further evaluated by digital subtraction angiography (DSA). In 158 carotid arteries, 107 stenoses of > 30% and nine occlusions were found. Receiver operator characteristic graphs were constructed in order to calculate sensitivities and specificities of the assessment by CDDI in the prediction of high‐grade stenoses determined by angiography. Optimal cut‐off points were defined by highest accuracy which reflects the combination of high sensitivity and specificity. The critical index of a high‐grade ICA stenosis according to the ECST method could be predicted with an accuracy greater than 90% by a systolic peak velocitiy of 1.25 m/s or an area reduction by CDDI of 70%. Corresponding values, 1.6 m/s and 80% area reduction, predicted the stenosis indexes according to the NASCET method less reliably, with accuracies of between 80% and 90%. Flow velocity criterion was slightly less accurate than the area reduction criterion by CDDI. Finally, double‐blind evaluation performed by two readers per examination modality showed that the measurement of area reduction in CDDI is at least as reliable as stenosis indexes according to ECST and NASCET methods.
Olivopontocerebellar atrophy: paraneoplastic syndrome of brain tumour?
Authors: Silveri M. C. , Misciagna S. , Laudisio A. , Colosimo C.
Published: March 2003

We describe a patient who, three years after the onset of an olivopontocerebellar atrophy, developed a right cerebral tumour. The cerebellar symptomatology also included, as in other cerebellar patients previously described, a peripheral dysgraphia. Because this deficit of writing is generally reported in patients with right cerebral lesion, the authors hypothesized that functional alterations of supratentorial structures preceding the tumour by years may be able to damage the neural substrates connecting cerebral and cerebellar structures and to produce cerebellar atrophy.
Malignant pheochromocytoma with progressive paraparesis in  von Hippel–Lindau disease
Authors: Mössner R. , Keidel M.
Published: March 2003

Pheochromocytomas are a feature of the von Hippel–Lindau disease spectrum, a multisystem disorder of autosomal dominant inheritance. Pheochromocytomas are, however, observed during life with a lower frequency than other features of this disease, such as retinal angiomas, haemangioblastomas of the CNS, and renal carcinomas. We present the highly unusual case of a patient who required an emergency operation for an intradural extramedullary thoracic tumour which was clinically suggestive initially of neurinoma. We present evidence from NMR, histological and isotope scan investigations of this being a pheochromocytoma metastasis and of an additional right‐sided paraganglioma at the same height. A detailed history revealed that this patient had suffered from four other pheochromocytomas and two other paragangliomas, in addition to retinal angiomatosis of von Hippel–Lindau disease. This case is extraordinary due to (i) the unusual site of the metastasis, (ii) the neurological requirement for an emergency operation of pheochromocytoma, (iii) metastasis of pheochromocytoma in von Hippel–Lindau disease (only eight previous cases), and (iv) the number of recurrent pheochromocytomas. It clearly demonstrates the necessity for frequent and life‐long follow‐up in von Hippel–Lindau disease.
Bilateral GPi DBS is useful to reduce abnormal involuntary movements in advanced Parkinson’s disease patients, but its action is related to modality and site of stimulation
Authors: Peppe A. , Pierantozzi M. , Altibrandi M. G. , Giacomini P. , Stefani A. , Bassi A. , Mazzone P. , Bernardi G. , Stanzione P.
Published: March 2003

In Parkinson’s disease (PD) patients, internal globus pallidus (GPi) stimulation has been reported to produce good effects on abnormal involuntary movements (AIM); less improvement has been observed in extrapyramidal symptoms. We assessed the effect of monopolar dorsal (uppermost), ventral (lowest) and bipolar (uppermost vs. lowest) bilateral globus pallidus stimulation by quadripolar electrode on extrapyramidal symptoms and AIM induced by a dose of apomorphine. Six PD patients were studied in OFF therapy condition after surgery without stimulation (STIM OFF) and during stimulation (STIM ON) with the three different modalities. All patients were evaluated by the unified Parkinson’s disease rating scale, section III (UPDRS) and by the AIM. Our results show that all three bilateral GPi stimulation modalities reduce the UPDRS score (between 49.7 and 31.5%), although the bipolar and lowest stimulation are the most effective. Similarly, bipolar and lowest stimulation were also the most effective in reducing the occurrence and intensity of the apomorphine‐induced AIM. On the contrary, uppermost stimulation (UP ON) produced an AIM occurrence similar to that observed in the OFF stimulus condition.

We suggest that bilateral GPi stimulation is an useful procedure to ameliorate extrapyramidal signs of advanced PD patients; however, it produces an antidyskinetic effect only if the ventral or the entire GPi is stimulated. On the contrary, the UP ON, most probably located in the external globus pallidus (GPe), does not modify the AIM occurrence.
Guidance for the preparation of neurological management guidelines by EFNS scientific task forces
Authors: Hughes R. A. C. , Barnes M. P. , Baron J.‐C. , Brainin M.
Published: March 2003

The aim of an EFNS neurological management guideline is to provide guidance for clinical neurologists, other health care professionals and health care providers about important aspects of management of neurological disease. It represents the view of an expert task force appointed by the chairperson of the scientific committee with the agreement of the chairperson of a Scientist Panel. It will be a peer‐reviewed statement of minimum desirable standards for the guidance of practice based on the best available evidence. It is not intended to have legally binding implications in individual cases.
Complex hallucinations following occipital lobe damage
Authors: Beniczky Sándor , Kéri Szabolcs , Vörös Erika , Ungureán Aurélia , Benedek György , Janka Zoltán , Vécsei László
Published: March 2003

Visual hallucinations may accompany many neurological and psychiatric disorders. A common localization principle is that lesions to the early sensory cortices lead to elementary hallucinations, whereas complex perceptual experiences are related to the pathology of higher‐level cortical regions. We report the case of a patient who experienced complex, non‐stereotyped, multimodal (visual and somatosensory) hallucinations following an acute ischaemic vascular lesion in the right medial occipital lobe. This illustrates that the phenomenology of hallucinations not necessarily reflects the exact localization of cerebral pathology. Instead, the damaged area may serve as a focus of an abnormally activated neuronal network.
Impaired parvocellular pathway in dyslexic children
Authors: Farrag A. F. , Khedr E. M. , Abel‐Naser W.
Published: March 2003

Recent studies report that some children with dyslexia have impaired visual processing, specifically in the fast‐processing magnocellular pathway. The objective was to study the effect of varying luminance and temporal and spatial frequency on the latency and amplitude of the visual evoked potentials (VEPs) in normal and dyslexic Egyptian children who speak Arabic (a right‐left reading and writing system). VEPs were recorded in 52 dyslexic and 41 normal children in the fourth grade using a black and white checkerboard pattern with different checkerboard sizes and different rates of stimuli at high‐ and low‐contrast media. The peak of the major positive wave component (P100) of each waveform and the trough of the previous major negative wave component were identified, and the peak‐to‐trough amplitude was measured. The latency and amplitude of VEPs in response to different experimental conditions showed significant shortening of P100 latency under high‐contrast media and under low spatial frequency in children with dyslexia compared with normal readers. Furthermore, dyslexia children showed prolonged P100 latency in response to high spatial frequency stimulation compared with the low spatial frequency (P=0.003) and significantly higher N1–P1 amplitude under high‐contrast media compared with low‐contrast media (P=0.02), whilst no such changes were observed in normal readers. These results are suggestive of deficiency within the parvocellular pathway rather than the magnocellular pathway. As reading apparently places demands primarily on the ability to discriminate fine details, which is to say, on the parvocellular system, we suggested that deficiency in this system, at least in Arabic speaking children, could be a predisposing factor in dyslexia.
Myotonic dystrophy type 2
Authors: Finsterer J.
Published: March 2003

Myotonic dystrophy type 2 (DM2) is a clinically but not genetically heterogeneous, multisystem disorder, that is clinically similar to, but distinct from myotonic dystrophy type 1 (DM1). Initially, different phenotypes of DM2 were described by Ricker (proximal myotonic myopathy, PROMM), Ranum (myotonic dystrophy 2, DM2) and Udd (proximal myotonic dystrophy, PDM). Clinical features these three phenotypes had in common were diffuse, proximal or distal weakness, wasting, myotonia, cataract, cerebral, endocrine and cardiac abnormalities. Initially, the clinical differences between DM1 and PROMM seemed unmistakable, but meanwhile it has become apparent that the clinical differences between these entities are blurring. In 1999, Day et al., Meola et al. and Ricker et al. mapped the mutated gene of all three phenotypes to chromosome 3q. In 2001, the three different phenotypes were found to rely on the same mutation in the ZNF9 gene on chromosome 3q21.3. Although DM2 may be clinically heterogeneous, it is by result of a mutation in a single gene. The mutation responsible for DM2 is a CCTG‐repeat expansion of 75–11 000 repeats in intron 1 of the ZNF9 gene on chromosome 3q21.3. Because of the clinical heterogeneity, the diagnosis of DM2 should rely on DNA analysis alone.
Adult onset pigmentary orthochromatic leukodystrophy with ovarian dysgenesis
Authors: Verghese J. , Weidenheim K. , Malik S. , Rapin I.
Published: March 2003

Pigmentary type of orthochromatic leukodystrophy (POLD) is an adult‐onset leukodystrophy, characterized pathologically by the presence of glial and microglial cytoplasmic pigment inclusions. The complete phenotype, genotype and pathogenetic mechanisms in POLD have not been elucidated. We followed for 18 years a woman with autopsy‐proven POLD, who presented with ‘frontal’ dementia and spasticity. Her further course was marked by progressive mutism, apraxia and seizures. Her sister had died of the same disease after a much more rapidly progressing course. These sisters had primary infertility with pathologic evidence of streak ovaries. Diagnosis was confirmed in both cases by post‐mortem examination. POLD is a rare cause of adult‐onset leukodystrophy presenting with dementia. Ovarian dysgenesis is extremely rare in the absence of demonstrable chromosomal abnormalities and extends the clinical spectrum of POLD.
Effects of vigabatrin on brain GABA+/Cr signals in focus‐distant and focus‐near brain regions monitored by 1H‐NMR spectroscopy
Authors: Mueller S. G. , Weber O. M. , Duc C. O. , Meier D. , Russ W. , Boesiger P. , Wieser H. G.
Published: March 2003

The new antiepileptic drug vigabatrin (VGB) increases gamma‐aminobutyric acid (GABA) in the brain. We compared GABA+/Cr signals measured focus‐near and focus‐distant and correlated it with the degree of response to VGB.

Brain GABA+/Cr signals were measured in 17 epileptic patients in structurally normal appearing tissue by nuclear proton magnetic resonance (1H‐NMR) spectroscopy using a special editing sequence for GABA. In 11 patients the measurements were done in brain areas distant to focus and in six near to focus.

Full‐responders (seizure reduction of ≥50% at the end of the treatment phase) and partial‐responders (seizure reduction of ≥50% at the end of the first month of treatment but ≤50% at end of treatment) had lower GABA+/Cr signals in the hemisphere with the epileptogenic focus and increases of the GABA+/Cr signals with VGB. Non‐responders (seizure reduction of ≤50%) had no side difference in the GABA+/Cr signals before treatment and no increase during treatment. These observations were made in structurally normal appearing tissue near to the focus and distant to the focus.

A side difference in brain GABA+/Cr signal between the epileptogenic and non‐epileptogenic hemisphere before VGB treatment correlates with an improved seizure control under VGB treatment regardless whether the measurement is done focus‐near or focus‐distant.
Features of Migraine Comparison of a Questionnaire Study and a Neurologist-Examined Random Sample
Authors: Nikiforow Rita
Published: March 2003

In a community survey on headache in which a questionnaire was used, the responders were asked to state if their headaches were accompanied by symptoms usually associated with migraine. A random sample of 200 out of the 3,067 who returned the questionnaire were examined by a neurologist and the answers compared with those given in connection with the case history. Migraine features occurred sometimes in a very large percentage of the 2,018 who suffered from headache. When the answers were compared, “warning” and “nausea” occurring sometimes were reported significantly more often in the questionnaire. No difference was found when these features were reported as occurring usually or always. When interviewed by the neurologist, 20% of those with a clinical diagnosis of tension headache reported two of the three features “unilaterality”, “warning” and “nausea”, also. It is concluded that the questionnaire is a satisfactory tool if the aim is to select a population with distinct features of migraine, but that reliability rapidly decreases if efforts are made to include more elusive forms of the condition.
Changes in the 24-Hour Prolactin Pattern in Cluster Headache
Authors: Polleri A. , Nappi G , Murialdo G. , Bono G. , Martignoni E. , Savoldi F.
Published: March 2003

The regulation of prolactin (PRL) secretion periodicity in cluster headache (CH) and in atypical facial pain (AFP) has been studied in nine and seven patients, respectively. The physiological periodicity of the hormone secretion, with its highest levels during night sleep and its lowest during the waking hours, is upset in CH, but not in AFP. A rhythmicity occurs in CH only in the presence of severe pain, which appears to be the synchronizing event. Lithium carbonate treatment does not interfere with the mentioned changes in PRL secretion in CH.
Ergotamine Abuse: Results of Ergotamine Discontinuation, With Special Reference To the Plasma Concentrations
Authors: Ala-Hurula Veli , Myllylä Vilho , Hokkanen Eero
Published: March 2003

Twenty-three patients suffering from continuous headache linked with habitual daily use of ergotamine tartrate were studied. Their headaches were classified clinically, and possible side effects of ergotamine medication, plasma levels of ergotamine, and occurrence of withdrawal symptoms after discontinuation of drug abuse were recorded. Seventeen of the patients were clinically diagnosed as suffering from “ergotamine headache”, and seven of them complained of coldness in the extremities. Plasma ergotamine levels were measured by using a radioimmunoassay. In almost half of the patients the 1 h plasma levels after the daily dose were below the detection limit of the procedure (0.12 ng/ml). The duration and severity of the withdrawal symptoms did not correlate with the doses and plasma levels of ergotamine. In only 4 of the 21 patients who were followed up for 3 to 6 months did headache symptoms not improve after ergotamine withdrawal. The results indicate that even small (0.5-1.0 mg/day) doses of ergotamine tartrate taken regularly may cause continuous headache symptoms and withdrawal symptoms after discontinuation.
Optimal Routes of Administration of Ergotamine Tartrate in Cluster Headache Patients. A Pharmacokinetic Study*
Authors: Ekbom K. , Krabbe A. Æ. , Paalzow G. , Paalzow L. , Tfelt-Hansen P. , Waldenlind E.
Published: March 2003

Bioavailability and rate of absorption of ergotamine were studied in eight cluster headache patients outside attacks. In a cross-over design, approximately 2 mg ergotamine tartrate was administered as effervescent tablets, suppositories, and from an inhalation device, with 0.25 mg intravenously as the reference. Ergotamine in plasma was measured by high performance liquid chromatography with fluorescence detection from 5 to 420 min. For all three routes of administration, a similar low (0.5–4.2%) bioavailability of ergotamine was estimated. Only inhalation of ergotamine resulted in early (at 5 min) peak concentrations of ergotamine in plasma and is therefore most likely to relieve the short-lived attacks of cluster headache. The inhalation route for ergotamine poses problems, however, and we suggest ways of improving the inhalation device.
Treatment of the Acute Migraine Attack Current Status
Authors: Wilkinson Marcia
Published: March 2003

The main treatment of the acute migraine attack remains sleep, sedation, an anti-nauseant and analgesics, and in some patients 1 or 2 mg of ergotamine tartrate. Drugs containing large amounts of caffeine should not be used. Absorption of drugs may be impaired in a migraine attack. Metoclopramide is probably the anti-emetic of choice because it is an effective anti-nauseant and promotes normal gastrointestinal activity. Domperidone has a similar action but is said not to go through the blood-brain harrier, so is less likely to cause extrapyramidal reactions. All drugs, including analgesics such as aspirin and paracetamol, are best given in a soluble or effervescent form. Where vomiting occurs early in the attack, suppositories may be indicated. Ergotamine tartrate is necessary in about one third of attacks and is best given by suppository or by inhalation. Doses higher than 2 mg per attack or 6 mg in one week may cause toxic symptoms, the early signs of which are headache, nausea, vomiting and a feeling of not being very well. The non-drug treatments of an acute attack include pressing on the temporal artery, hot and cold compresses and relaxation.
Ergotamine-Induced Constriction of Cranial Arteriovenous Anastomoses in Dogs Pretreated with Phentolamine and Pizotifen
Authors: Saxena Pramod R. , Koedam Nick A. , Heiligers Jan , Hof Robert P.
Published: March 2003

Previous investigations from our laboratory have shown that ergotamine causes a selective vasoconstriction in the carotid vascular bed of the dog and that the drug constricts arterio-venous anastomoses (AVAs) in cats and pigs. Since ergotamine can act via alpha-adrenergic or D-serotonergic receptors in certain vascular and non-vascular tissues, we have attempted to ascertain here if these receptors mediate the constriction of AVAs. Using radioactive microspheres of 15 μm diameter we found in the dog that about 40% of the carotid arterial blood is shunted to the venous side via AVAs. Ergotamine (2–20 μg.kg-1, i.v.) reduced total carotid blood flow to a larger extent in the AVA part than in the extracerebral part (muscles, ears, skin and fat). The cerebral component of carotid blood did not change. These results, confirming that ergotamine decreases arteriovenous shunting, show that the drug has a more selective action on the AVAs than on the arterioles. Pretreatment with phentolamine (0.5 mg.kg-1), pizotifen (0.5 mg.kg-1) or their combination did not effectively modify the responses to ergotamine. It is concluded that the vasoconstriction of cranial AVAs (and arterioles) by ergotamine does not appear to be primarily mediated by either alpha-adrenergic or D-serotonergic receptors. However, the role of atypical serotonin receptors has yet to be determined.