bluuebunny/crossref_metadata_2025 huggingface dataset:
Pick’s disease with Pick bodies combined with progressive supranuclear palsy without tuft‐shaped astrocytes: A clinical, neuroradiologic and pathological study of an autopsied case
Authors: Wang Lu‐Ning , Zhu Ming‐Wei , Feng Ya‐Qing , Wang Jian‐Hua
Published: May 2006

We report clinical, neuroradiologic features, and neuropathologic findings of a 76‐year‐old man with coexistent Pick’s disease and progressive supranuclear palsy. The patient presented with loss of recent memory, abnormal behavior and change in personality at the age of 60. The symptoms were progressive. Three years later, repetitive or compulsive behavior became prominent. About 9 years after onset, he had difficulty moving and became bed‐ridden because of a fracture of his left leg. His condition gradually deteriorated and he developed mutism and became vegetative. The patient died from pneumonia 16 years after the onset of symptoms. Serial MRI scans showed progressive cortex atrophy, especially in the bilateral frontal and temporal lobes. Macroscopic inspection showed severe atrophy of the whole brain, including cerebrum, brainstem and cerebellum. Microscopic observations showed extensive superficial spongiosis and severe neuronal loss with gliosis in the second and third cortical layers in the frontal, temporal and parietal cortex. There were Pick cells and argyrophilic Pick bodies, which were tau‐ and ubiquitin‐positive in neurons of layers II–III of the above‐mentioned cortex. Numerous argyrophilic Pick bodies were observed in the hippocampus, especially in the dentate fascia. In addition, moderate to severe loss of neurons was found with gliosis and a lot of Gallyas/tau‐positive globus neurofibrillary tangles in the caudate nucleus, globus pallidus, thalamus, substantia nigra, locus coeruleus and dentate nucleus. Numerous thorned‐astrocytes and coiled bodies but no‐tuft shaped astrocytes were noted in the basal ganglion, brainstem and cerebellar white matter. In conclusion, these histopathological features were compatible with classical Pick’s disease and coexistence with progressive supranuclear palsy without tuft‐shaped astrocytes.
Determination of genomic damage in neuroblastic tumors by arbitrarily primed PCR: MYCN amplification as a marker for genomic instability in neuroblastomas
Authors: Muñoz Jorge , Vendrell Elisenda , Aiza Gemma , Nistal Manuel , Pestaña Angel , Peinado Miguel Angel , Castresana Javier S.
Published: May 2006

The aim of this study is to establish an estimation of the global genomic alteration in neuroblastic tumors (ganglioneuromas, ganglioneuroblastomas and neuroblastomas) and correlate them with different clinical parameters (age, sex, diagnosis, Shimada index, proliferation index, tumor location, and 1p and v‐myc avian myelocitomatosis viral‐related (MYCN) status) in order to find new molecular and/or prognostic markers for neuroblastoma. To assess the genomic damage in neuroblastic tumors, we used an arbitrarily primed PCR approach, a technique based on the reproducibility of band profiles obtained by a PCR with a low annealing temperature in its first cycles. Genomic damage was assessed by comparing band profiles of tumors and normal paired samples. Gains and losses in the intensity of the bands were computerized and referred to the total number of bands analyzed. We found a higher genomic damage fraction (GDF) in the female’s group (U‐Mann–Whitney, P = 0.025), but we could not find any association between GDF and tumor location, proliferation index, diagnosis or age of the patient. There was no relationship between 1p status and GDF, but tumors with MYCN amplification had a slightly higher GDF. MYCN amplification might in some way contribute to genomic instability of neuroblastomas.
Pineal parenchymal tumor of intermediate differentiation with cytologic pleomorphism
Authors: Sasaki Atsushi , Horiguchi Keishi , Nakazato Yoichi
Published: May 2006

We report a case of pineal parenchymal tumor in a 33‐year‐old man incidentally detected by radiological examination. The MRI showed an unhomogeneously enhanced, small tumor (approximately 1 cm in size) in the pineal region. A tumor specimen was obtained at endoscopic biopsy. Routine histology showed a highly cellular tumor characterized by a predominance of small cells showing high nuclear : cytoplasmic ratio and moderate nuclear atypia, pleomorphism including giant cells and an absence of pineocytomatous rosettes. Mitotic figures were rare (approximately 1 per 10 high‐power fields). Tumor necrosis was not evident. Immunohistochemically, the neoplastic cells showed positivity for neural markers (neurofilament protein, synaptophysin) and pinealocyte‐associated antibodies (PP1, PP5, PP6), but not for glial fibrillary acidic protein or S‐100. The MIB‐1 labeling index was relatively high (6.3%). Ultrastructurally, there was some evidence of pinealocytic differentiation, such as vesicle‐crowned rodlets (synaptic ribbons) and paired twisted filaments in neoplastic cells. Thus, the tumor was confirmed as a pineal parenchymal tumor of intermediate differentiation by histology, immunohistochemistry and electron microscopy. This case indicates that marked cytologic pleomorphism can occur in pineal parenchymal tumors of intermediate differentiation.
TrkA expression is associated with an elevated level of apoptosis in classic medulloblastomas
Authors: Ohta Takashi , Watanabe Takao , Katayama Yoichi , Kurihara Jun , Yoshino Atsuo , Nishimoto Hiroshi , Kishimoto Hiroshi
Published: May 2006

Medulloblastomas represent the most common central nervous system malignancies in children. Despite intensive modality treatment with craniospinal irradiation and multiple drug chemotherapy, their prognosis remains dismal. In the present study, we examined the potential roles of cellular differentiation, proliferation and apoptosis in 21 pediatric patients with newly diagnosed classic medulloblastomas treated by conventional radiation therapy and adjuvant chemotherapy. The expression of glial fibrillary acidic protein, S‐100, synaptophysin, TrkA and TrkC, and the proliferation index of MIB‐1 were evaluated by immunohistochemistry and the apoptotic index was determined using terminal deoxytransferase‐mediated deoxyuridine‐5′‐triphosphate nick‐end labeling assay. The prognostic value of these biological markers was also assessed. Immunoreactive glial fibrillary acidic protein, S‐100, synaptophysin, TrkA and TrkC were observed in seven (33%), four (19%), 12 (57%), 14 (67%) and 11 (52%) of the 21 cases, respectively. TrkA expression was positively correlated with the MIB‐1 staining index (P = 0.0228) and the apoptotic index (P = 0.0058). None of the immunohistochemical markers was found to be of value in predicting the prognosis. Although the present small sample size does not provide sufficient power to discount biological variables as prognostic markers, it was the well‐established clinical prognostic factors, i.e. tumor stage and extent of surgery, that stood out as the most important predictors of survival. The close association between apoptosis and TrkA expression is consistent with in vitro data demonstrating the capacity of the NGF/TrkA signaling pathway to increase medulloblastoma apoptotic cell death, suggesting that this pathway may yield alternative therapeutic targets for novel therapies.
Effects of cotransfection of antisense‐EGFR and wild‐type PTEN cDNA on human glioblastoma cells
Authors: Tian Xin‐Xia , Zhang Yun‐Gang , Du Juan , Fang Wei‐Gang , Ng Ho‐Keung , Zheng Jie
Published: May 2006

The main molecular genetic changes identified in glioblastomas are overexpression/amplification of the epidermal growth factor receptor (EGFR) gene and mutation/deletion of the tumor suppressor PTEN gene. These two genetic changes both play important roles in glial tumorigenesis and progression. In this study, we demonstrated that wild‐type PTEN transfection inhibited the growth and transforming ability of U87MG cells by 69.3% and 73.5%, respectively. On the other hand, antisense‐EGFR transfection inhibited the growth and transforming phenotype of these cells by 50.3% and 46.8%, respectively. However, cotransfection of U87MG cells with wild‐type PTEN and antisense EGFR constructs could inhibit the cellular growth by 91.7%. The transforming phenotype of these cells was completely inhibited. In addition, these cotransfected cells showed a differentiated form and expressed much lower telomerase activity than cells transfected with wild‐type PTEN or antisense‐EGFR alone. In summary, these results suggest that cotransfection is a better approach to suppress glioma cell growth than wild‐type PTEN transfer or antisense‐EGFR transfection alone. This approach may prove useful as an adjunct therapy in the treatment of glioblastomas.
Increased neuronal expression of alpha B‐crystallin in human olivary hypertrophy
Authors: Fukushima Kazuko , Mizuno Yuji , Takatama Masamitsu , Okamoto Koichi
Published: May 2006

We studied morphologic changes in olivary hypertrophy from dentato‐olivary tract lesions by immunohistologic methods with antialpha B‐crystallin and antiheat shock protein 27 (HSP 27). The majority of central chromatolysis‐like enlarged neurons, which are frequently seen in the early stages of olivary hypertrophy on ipsilateral lesions, showed a marked expression of alpha B‐crystallin; however, HSP 27 did not show increased expression in those neurons. In the later stages of olivary hypertrophy, increased expressions of alpha B‐crystallin varied in the remaining neurons and the expression of HSP 27 increased in hypertrophied astrocytes, although the expression of alpha B‐crystallin in hypertrophic astrocytes was not prominent. The accumulation of alpha B‐crystallin and HSP 27 may represent responses to pathologic conditions.
Clinico‐neuropathological study of a Chinese case of familial adult Leigh syndrome
Authors: Piao Yue‐Shan , Tang Guo‐Cai , Yang Hong , Lu De‐Hong
Published: May 2006

Leigh syndrome is a mitochondrial disease of infancy and early childhood and is rare in adults. We report an autopsy case of adult Leigh syndrome of 15 years duration in a 32‐year‐old man with a familial history of the disease. His initial symptom was clumsiness followed by dullness, and dysphasia and dysarthria appeared in the last 3 months. His brother had similar symptoms and died at the age of 27 years. His sister is also demented. Cranial MRI revealed abnormal signals in the bilateral putamen and tegmentum of the brainstem. Neuropathologically, there were symmetrical, well‐demarcated necrotizing lesions with proliferation of capillaries in the putamen, caudate nucleus and thalamus, as well as in the periaqueductal gray matter of the midbrain and tegmentum of the pons. It seems that the lesions in the putamen were more severe and older than those of the brainstem, the latter having numerous macrophage infiltrations. Neuronal loss and gliosis were also observed in the substantia nigra and cerebellar cortex. This is the first autopsy‐confirmed familial adult Leigh syndrome in China. The clinicopathological features are presented together with a literature review.
Permeability of injured blood brain barrier for exogenous bFGF and protection mechanism of bFGF in rat brain ischemia
Authors: Liu Ying , Lu Jin‐Biao , Ye Zhu‐Rong
Published: May 2006

The study aims to explore the protection mechanism of exogenous basic fibroblast growth factor (exo‐bFGF) in brain ischemia. The first part of experiment was to determine the optimal time window for the permeation of exo‐bFGF through damaged blood–brain barrier in rats with permanently occluded middle cerebral arteries. 125I labeled bFGF was administered to the rats through the caudal vein. The level of γ‐rays of 125I‐bFGF in the ischemic brain were found to increase at 2 h and a high level was maintained for 14 days. The morphology of the basement membrane of capillaries was observed using anti‐blood–brain barrier basement membrane glycoprotein immunohistochemistry. The normal continuous linear or ribbon‐like immunostain of the basement membrane became granular at 0.5 h, gradually faint and finally negative. The newly formed capillaries at the edge of the infarct still showed a negative stain after 14 days. The result suggested the optimal time window of exo‐bFGF began 2 h after insult. The second part of experiment was to observe the dynamic expression of early growth response protein (Egr‐1), endogenous basic fibroblast growth factor (endo‐bFGF) and bFGF receptor (bFGFR) using immunohistochemistry after exo‐bFGF is administered to brain. Egr‐1 was more significantly enhanced in the exo‐bFGF‐used group than in the control group. Endo‐bFGF increased gradually, reaching its peak at 7 days in the control group, while in experiment group, the endo‐bFGF expression showed its first peak at 6 h, indicating that exo‐bFGF could induce earlier and stronger expression of endo‐bFGF. The bFGFR‐group presented an early expression, reaching its maximal level at 3 h, and declining at 6 h. There were no difference in expression of bFGFR between the two groups. The infarct areas reduced from 17% to 24% in the different time intervals. The results suggested that in exo‐bFGF enhanced Egr‐1 protein. Egr‐1 in turn might play an important role in up‐regulating the expression of endo‐bFGF which overlapped with the expression of bFGFR to ensure the combination of ligand and receptor to protect against brain ischemia.
Glottic scar bands following intubation
Authors: WAY CAROLYN , HARTLEY BENJAMIN E.J. , GLAISYER HILARY
Published: May 2006

We present two uncommon cases of abnormalities of the pediatric airway, which may present in the first instance to the anesthetist. Glottic scar bands are a result of intubation trauma and are a treatable cause of voice abnormalities and sometimes respiratory distress.
Predicting which children benefit most from parental presence during induction of anesthesia
Authors: KAIN ZEEV N. , MAYES LINDA C. , CALDWELL‐ANDREWS ALISON A. , SAADAT HALEH , MCCLAIN BRENDA , WANG SHU‐MING
Published: May 2006

Background:  The purpose of this large‐scale prospective cohort study (n = 426) was to identify child and parent characteristics that are associated with low anxiety and good compliance during induction of anesthesia when parents are present.

Methods:  Outcome variables included child's anxiety and child's compliance during induction of anesthesia. Predictor variables included demographics, temperament, trait (baseline) anxiety, coping style, and locus of control.

Results:  Results of a linear regression model (overall proportion of variance accounted for equals 39.5%) showed that significant predictors of anxiety during induction of anesthesia while parents are present included: the child's age (ΔR2 = 0.315, P = 0.0001), behavior during previous medical visits (ΔR2 = 0.025, P = 0.001), child's activity level (ΔR2 = 0.016, P = 0.007), parent's state (contextual) anxiety (ΔR2 = 0.022, P = 0.001) and parent's locus of control (ΔR2 = 0.009, P = 0.036). A linear regression model that was constructed with compliance of the child as the outcome revealed similar findings.

Conclusions:  Children who benefit from parental presence are older, had lower levels of activity in their temperament, and had parents who were calmer and who valued preparation and coping skills for medical situations. The practical implications of these findings are discussed.
Anesthesia management of familial dysautonomia
Authors: NGAI JENNIE , KREYNIN ILYA , KIM JUNG T. , AXELROD FELICIA B.
Published: May 2006

Familial dysautonomia (FD) is an autosomal recessive inherited disorder, predominantly affecting the Ashkenazi Jewish population that is characterized by sensory and autonomic neuropathy. The protean manifestations and perturbations result in high morbidity and mortality. However, as a result of supportive measures and centralized care, survival has improved. As surgical options are increasing to symptomatically treat FD, anesthesiologists need to be familiar with this disorder. Because the Dysautonomia Center at NYU Medical Center is a referral center for FD patients, we have attained considerable anesthetic experience with FD. This article reviews clinical features of FD that could potentially affect anesthetic management and outlines our present practices.
Taxonomic subdivisions within the fossorial skink subfamily Acontinae (Squamata: Scincidae) reconsidered: a multilocus perspective
Authors: Daniels Savel R. , Heideman Neil J. L. , Hendricks Martin G. J. , Crandall Keith A.
Published: May 2006

Despite recent molecular systematic studies on the fossorial southern African skink subfamily Acontinae, evolutionary relationships among the three genera remain unresolved and disputed. Among these, the most recent study suggests that both Typhlosaurus and Acontias are paraphyletic, contrasting earlier results that suggest the presence of two divergent clades within Acontias. Here we further investigate the evolutionary relationships in the limbless fossorial southern African subfamily Acontinae with partial sequenced data derived from four mitochondrial loci (16S rRNA, 12S rRNA, cytochrome oxidase I and cytochrome b), as well as two nuclear protein coding loci (c‐mos and RAG‐1), in an attempt to clarify evolutionary relationships. Phylogenetic results derived from combined data analyses (comprising all six loci and totalling ∼3.1 kb) using maximum parsimony, maximum likelihood and Bayesian inferences converged on the same topology. The resulting phylogeny showed Typhlosaurus as monophyletic, while the monotypic genus Acontophiops was nested intermediate to two reciprocally monophyletic Acontias clades. These two Acontias clades can be distinguished on the basis of a number of morphological, morphometric and biogeographical characters, underscoring the presence of two distinct groups. In the present study, we propose the following taxonomic changes based on the multilocus phylogeny. We retain the genus name Acontias for the medium‐ and large‐bodied skinks in clade 2 comprising all taxa in the Acontias meleagris complex as well as Acontias plumbeus, Acontias gracilicauda gracilicauda, Acontias breviceps, Acontias percivali percivali and Acontias percivali occidentalis. We designate a new genus Microacontias gen. nov. for the reciprocally monophyletic taxa in clade 1 comprised of all the small‐bodied taxa that include Microacontias litoralis, Microacontias lineatus lineatus, Microacontias lineatus grayi and Microacontias lineatus tristis. We examine the evolution of characters used in the taxonomy of the Acontinae and suggest that symplesiomorphic morphological characters among fossorial taxa have been an impediment to understanding the evolution of this subfamily. This study underscores the importance of the application of multiple molecular markers (both nuclear and mitochondrial) in determining the taxonomic diversity among fossorial skinks and emphasizes the application of phylogenetics in defining synapomorphic (shared derived) features.
Determination of optimal cream formulation from long‐term stability investigation using a surface response modelling
Authors: Moulai Mostefa N. , Hadj Sadok A. , Sabri N. , Hadji A.
Published: May 2006

Synopsis

The aim of this work was to apply an experimental design to formulate a stable depilatory cream at ambient temperature. A preliminary study was performed to optimize the most accurate operating parameters, which are then used for the determination of the rheological properties. Long‐term stability of the emulsion formulations was investigated to obtain the optimal region of each factor. An experimental design using response surface modelling was then applied and emulsion stability was estimated by introducing new characteristic parameters correlated with the experimental results. An optimal region characterized with high stability was found and further explored to verify the effectiveness and tolerance of depilation. As part of the optimized process, the main effects of the formulation ingredients were also investigated.
Validation of a HPLC method for simultaneous determination of five sunscreens in lotion preparation
Authors: Kedor‐Hackmann E. R. M. , De Lourdes Pérez González M. L. , Singh A. K. , Santoro M. I. R. M.
Published: May 2006

Synopsis

The aim of this research was to develop and validate a high‐performance liquid chromatographic (HPLC) method for simultaneous determination of five sunscreens, namely benzophenone‐3 (B‐3), butyl methoxydibenzoylmethane (BM), octyl methoxycinnamate (OM), octyl salicylate (OS) and homosalate (HS). The separation and quantitative determination was made by HPLC at 40 ±1°C with a gradient elution from 10% to 100% mobile phase B in mobile phase A. The gradient liquid chromatographic system constituted of mobile phase A [acetonitrile : water (10 : 90 v/v)] and mobile phase B [acetonitrile : water (90 : 10 v/v)], at a flow rate of 1.0 mL min−1 and ultraviolet detection at 310 nm. The separation was obtained with two Waters® reversed phase columns: Novapack® C‐18 and Symmetry® C‐18 connected in series. All sunscreens were efficiently separated within 17 min. The coefficient of correlation and average recovery for B‐3, BM, OM, OS and HS were 0.9798 and 98.5%, 0.9672 and 98.8%, 0.9922 and 99.1%, 0.9961 and 98.9% and 0.9909 and 99.4% respectively. The relative standard deviations obtained were between 1.07% and 2.44%. The excipients did not interfere in the analysis. The results showed that the proposed method could be used for rapid and simultaneous determination of B‐3, BM, OM, OS and HS in sunscreen lotions with precision, accuracy and specificity.
An examination of non‐invasive imaging techniques in he analysis and review of cellulite
Authors: Callaghan T. , Wilhelm K. P.
Published: May 2006

Presented in part at the 23rd IFSCC Congress, Orlando, Florida, October 2004.
Cosmeceutical properties of levan produced by Zymomonas mobilis
Authors: Kim K. H. , Chung C. B. , Kim Y. H. , Kim K. S. , Han C. S. , Kim C. H.
Published: May 2006

Levan, a polysaccharide that can be produced by both plants and micro‐organisms, is a sugar polymer composed of fructose, with‐2,6 linkages. Here, we have attempted to assess the possible use of levan produced by Zymomonas mobilis as a cosmeceutical ingredient. In service of this goal, we assessed a host of levan's properties, including its moisturizing effects, cell cytotoxicity, cell proliferation effects and anti‐inflammation effects. Levan exhibited a moisturizing effect that was almost exactly the same as that evidenced by hyaluronic acid, as well as a similar cell proliferation effect in human fibroblast and keratinocyte cell lines. Moreover, in our cell proliferation test, which was conducted using bio‐artificial skin constructed via 3‐dimensional (3‐D) culture after the induction of primary skin inflammation with 0.05% sodium lauryl sulphate (SLS), cell viability in the presence of levan (0.01 and 0.05 mg mL–1) was determined to be higher than cell viability in the absence of levan. In our anti‐inflammation test, which was also conducted using 3‐D artificial skin, and which involved the measurement of a quantity of secreted interleukin‐1 (IL‐1), a pre‐inflammatory mediator induced by SLS, we determined that the quantity of IL‐1 in the 3‐D artificial skin treated with 0.01 and 0.05 mg mL–1 of levan was less than that registered in a skin sample that had been treated only with SLS. In this study, we determined that levan exerted an anti‐inflammatory effect against inflammatory reactions to skin irritants, and also that levan exerted a cell‐proliferative effect in bio‐artificial skin, thereby indicating its potential applicability as a cosmeceutical agent.
Cellulite: nature and aetiopathogenesis
Authors: Terranova F. , Berardesca E. , Maibach H.
Published: May 2006

Only a limited number of studies on cellulite have been published in the international literature and many of them reach somewhat antithetical conclusions. Consequently, it is not yet possible to reconcile the extreme differences of opinion which have lingered on for years concerning the nature of this disorder, as well as its origin and even the most basic aspects of its histopathological classification. It does not even have a recognized name: in fact, the term ‘cellulitis’ is used in scientific English to indicate a spreading gangrenous infection of the subcutaneous cellular tissue. The other terms used from time to time [panniculitis, lipodystrophy, edematofibrosclerotic panniculitis (EFP), liposclerosis, lipoedema, etc.] have quite different morphological and pathogenetic connotations in general. Over the last few decades, three major conflicting theories have emerged in relation to the ethiopathogenesis of cellulite. These indicate, respectively, the following causes:

1. Oedema caused by excessive hydrophilia of the intercellular matrix.

2. A homeostatic alteration on a regional microcirculatory level; this pathogenetic theory is summarized in a synthetic and self‐explanatory denomination: EFP.

3. A peculiar anatomical conformation of the subcutaneous tissue of women, different from male morphology.

These theories must all now be updated in the light of recent advances on the sophisticated and composite physiopathology of the adipose organ – which acts not only as a control device which regulates the systematic equilibrium of energy and modulates the food intake and the metabolism of other tissue substrate through a multiple glandular secretion of hormones and parahormones.
A new covalently bound ceramide from human stratum corneum –ω‐hydroxyacylphytosphingosine
Authors: Hill J. , Paslin D. , Wertz P. W.
Published: May 2006

Synopsis

The outermost layer of the skin, the stratum corneum, consists of non‐viable keratin‐filled cells, or corneocytes, embedded in a matrix of lipids. The boundary of the cells consists of cross‐linked proteins with covalently bound lipids on the outer surface. The spaces between cells are filled with a mixture of ceramides, cholesterol and fatty acids. The stratum corneum provides a protective barrier against water loss through the skin and limits the penetration of potentially harmful substances from the environment. Among the covalently bound lipids on the corneocyte surface are ω‐hydroxyacylsphingosine and ω‐hydroxyacyl‐6‐hydroxysphingosine. The previously suspected presence of ω‐hydroxyacylphytosphingosine is confirmed in this report through its specific isolation and characterization based on chromatographic behaviour and proton magnetic resonance spectral data.
Survival and mortality of myotonic dystrophy type 1 (Steinert's disease) in the population of Belgrade
Authors: Mladenovic J. , Pekmezovic T. , Todorovic S. , Rakocevic‐Stojanovic V. , Savic D. , Romac S. , Apostolski S.
Published: May 2006

The purpose of this investigation was to determine survival and mortality in patients with myotonic dystrophy type 1 (DM1) in the Belgrade population within the period from 1983 to 2002. Data of a number of diagnosed DM1 patients with their demographic, clinical and genetic characteristics were gathered from hospital records in all neurologic institutions in Belgrade for the period 1983–2002. Death certificates were reviewed to determine the cause of death. Survival analysis by life table method and Cox proportional hazard model was performed. Within the observed period, in the population of Belgrade, 15 fatal outcomes among 101 patients with DM1 were registered. Average DM1 mortality rate was 0.5/1 000 000 (95% CI 0.3–0.8), and standardized mortality ratio (SMR) was 5.3. A significant inverse correlation was found between age at onset of DM1 and CTG repeats (P = 0.023). The cumulative probability of 15‐year survival for DM1 patients in Belgrade was 49 ± 5% (48 ± 2% for males and 50 ± 7% for females). Younger age at onset was a significant unfavorable prognostic factor (hazard ratio = 4.2;P = 0.012).
The significance of intact sympathetic skin responses in carpal tunnel syndrome
Authors: Argyriou A. A. , Polychronopoulos P. , Moutopulou E. , Aplada M. , Chroni E.
Published: May 2006

The current study aimed to investigate the impact of carpal tunnel syndrome (CTS) on sympathetic skin response (SSR) recorded from the median and ulnar territory. Thirty patients were studied and idiopathic CTS was documented in a total of 46 hands. These were classified, according to electrophysiological criteria, into two groups; a group of 31 hands with severe CTS and a group of 15 hands with mild/moderate CTS, and were compared with a group of 30 hands of age‐matched controls. SSR was recorded simultaneously from the median and ulnar side of the palm following electrical stimulation at the wrist, in a mid‐point between median and ulnar nerve. Latency, amplitude, habituation and the median‐to‐ulnar ratio were estimated. In all controls clear recordings of SSR were obtained. In the patient groups, absence of SSR was never observed either in mild/moderate or in the severe CTS hands. The mean SSR latency and amplitude values recorded from both the median and ulnar nerves did not significantly differ between mild/moderate or severe CTS hands and controls. Likewise, the median‐to‐ulnar ratio and habituation of SSR latencies and amplitudes did not significantly differ between groups. SSR does not seem to be a sensitive method for evidence of autonomic involvement in CTS, even in patients manifesting sudomotor or other autonomic symptoms. In the present setting, SSR appeared to be independent of somatic afferent function and the corresponding sensory action potentials.
No association of C‐1019G promoter polymorphism of 5‐HT1A receptor gene with migraine
Authors: Yang X. S. , Xu X. P. , Yang Q. D.
Published: May 2006

It is well known that migraine has a strong genetic component, although the type and number of genes involved remains unclear. There is evidence to suggest that serotonin‐related genes may be involved in the pathogenesis of migraine. To investigate whether the 5‐HT1A receptor gene contributes to the risk of migraine we performed an association study of C‐1019G promoter polymorphism of the gene in 102 migraineurs and 93 controls. Subjects were of Han Chinese origin. No significant differences in allele (P = 0.82) or genotype frequencies (P = 0.71) were seen in migraineurs compared with the controls. When migraine with aura, without aura, with family history, without family history were analyzed separately, the frequencies did not vary significantly. Our results suggest that C‐1019G in 5‐HT1A is not a major genetic risk factor for migraine.
Topography of α‐internexin‐positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease
Authors: Armstrong R. A. , Cairns N. J.
Published: May 2006

Abnormal neuronal intermediate filament (IF) inclusions immunopositive for the type IV IF α‐internexin have been identified as the pathological hallmark of neuronal intermediate filament inclusion disease (NIFID). We studied the topography of these inclusions in the frontal and temporal lobe in 68 areas from 10 cases of NIFID. In the cerebral cortex, CA sectors of the hippocampus, and dentate gyrus granule cell layer, the inclusions were distributed mainly in regularly distributed clusters, 50–800 μm in diameter. In seven cortical areas, there was a more complex pattern in which the clusters of inclusions were aggregated into larger superclusters. In 11 cortical areas, the size of the clusters approximated to those of the cells of origin of the cortico‐cortical pathways but in the majority of the remaining areas, cluster size was smaller than 400 μm. The topography of the lesions suggests that there is degeneration of the cortico‐cortical projections in NIFID with the formation of α‐internexin‐positive aggregates within vertical columns of cells. Initially, only a subset of cells within a vertical column develops inclusions but as the disease progresses, the whole of the column becomes affected. The corticostriate projection appears to have little effect on the cortical topography of the inclusions.
Neurobrucellosis as an exceptional cause of transient ischemic attacks
Authors: Bingöl A. , Togay‐Işıkay C.
Published: May 2006

We report a series of four cases presented with transient ischemic attacks (TIA) or ischemic stroke as the predominant manifestation of neurobrucellosis (NB). Three of the patients were 20–28 years of age, and one patient was 53 years old. They all used to consume unpasteurized milk or its products. Two patients had systemic brucellosis in the past and received antibiotic treatment. Other causes of TIA including cardiac embolism, hypercoagulability, vascular malformations, systemic vasculitis, and infective endocarditis were excluded. NB was diagnosed with serological tests or cultures for Brucella in the cerebrospinal fluid. None of the patients had any further TIA after the initiation of specific treatment. NB should always be sought in young patients with TIA or ischemic stroke, especially if they have no risk factors for stroke and live in an endemic area for brucellosis, even if they do not have other systemic signs of brucellosis.
Common carotid arterial stiffness and the risk of ischaemic stroke
Authors: Tsivgoulis G. , Vemmos K. , Papamichael C. , Spengos K. , Daffertshofer M. , Cimboneriu A. , Zis V. , Lekakis J. , Zakopoulos N. , Mavrikakis M.
Published: May 2006

In the present case–control study we aimed to investigate the association of common carotid arterial (CCA) stiffness with ischaemic stroke (IS) and to determine whether this relationship was independent of conventional risk factors including CCA intima‐media thickness (CCA‐IMT). CCA distensibility, defined as the change of CCA‐diameter during the cardiac cycle, and CCA‐IMT were evaluated by means of high‐resolution B‐mode carotid ultrasound examination in consecutive, first‐ever IS patients (n = 193) and in age‐ and sex‐matched control subjects (n = 106). The CCA distensibility (inverse of CCA stiffness) was significantly (P = 0.007) lower in IS (0.353 mm, 95% CI: 0.326–0.379) than in control subjects (0.415 mm, 95% CI: 0.378–0.451) even after adjusting for blood pressure values, diastolic CCA‐diameter and height. The multivariate logistic regression procedure selected CCA‐IMT and CCA distensibility as the only independent predictor variables of IS. Each 1 SD increase in the CCA‐IMT and each 1 SD decrease in the CCA distensibility independently increased the likelihood of IS by 167.0% (OR: 2.67, 95% CI: 1.80–3.96, P < 0.001) and 59.0% (OR: 1.59, 95% CI: 1.22–2.07, P = 0.001) respectively. Increased CCA stiffness is associated with IS independent of conventional risk factors and CCA‐IMT. The causal interrelationship between the elastic properties of the CCA and the risk of stroke deserves further investigation by longitudinal studies.
Changes in Mini Mental State Examination score after stroke: lacunar infarction predicts cognitive decline
Authors: Appelros P. , Andersson Å. G.
Published: May 2006

Stroke and cognitive impairment are inter‐related. The purpose of this study was to show the natural evolution of cognitive performance during the first year after a stroke, and to show which factors that predict cognitive decline. Subjects were patients with a first‐ever stroke who were treated in a stroke unit. A total of 160 patients were included. At baseline patients were evaluated with regard to stroke type, stroke severity, pre‐stroke dementia and other risk factors. Mini Mental State Examinations (MMSE) were performed after 1 week and after 1 year. Patients had a median increase of 1 point (range −8 to +9) on the MMSE. Thirty‐two pre cent of the patients deteriorated, 13% were unchanged, and 55% improved. Lacunar infarction (LI) and left‐sided stroke were associated with a failure to exhibit improvement. Patients with LI had an average decline of 1.7 points, whilst patients with other stroke types had an average increase of 1.8 points. Most stroke survivors improve cognitively during the first year after the event. The outcome for LI patients is worse, which suggests that LI may serve as a marker for concomitant processes that cause cognitive decline.
EFNS guideline on the management of status epilepticus
Authors: Meierkord H. , Boon P. , Engelsen B. , Göcke K. , Shorvon S. , Tinuper P. , Holtkamp M.
Published: May 2006

The objective of the current paper was to review the literature and discuss the degree of evidence for various treatment strategies for status epilepticus (SE) in adults. We searched MEDLINE and EMBASE for relevant literature from 1966 to January 2005. Furthermore, the Cochrane Central Register of Controlled Trials (CENTRAL) was sought. Recommendations are based on this literature and on our judgement of the relevance of the references to the subject. Recommendations were reached by informative consensus approach. Where there was a lack of evidence but consensus was clear we have stated our opinion as good practice points. The preferred treatment pathway for generalised convulsive status epilepticus (GCSE) is intravenous (i.v.) administration of 4 mg of lorazepam or 10 mg of diazepam directly followed by 15–18 mg/kg of phenytoin or equivalent fosphenytoin. If seizures continue for more than 10 min after first injection another 4 mg of lorazepam or 10 mg of diazepam is recommended. Refractory GCSE is treated by anaesthetic doses of midazolam, propofol or barbiturates; the anaesthetics are titrated against an electroencephalogram burst suppression pattern for at least 24 h. The initial therapy of non‐convulsive SE depends on the type and the cause. In most cases of absence SE, a small i.v. dose of lorazepam or diazepam will terminate the attack. Complex partial SE is initially treated such as GCSE, however, when refractory further non‐anaesthetising substances should be given instead of anaesthetics. In subtle SE i.v. anaesthesia is required.
A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS‐ES Task Force
Authors: Albanese A. , Barnes M. P. , Bhatia K. P. , Fernandez‐Alvarez E. , Filippini G. , Gasser T. , Krauss J. K. , Newton A. , Rektor I. , Savoiardo M. , Valls‐Solè J.
Published: May 2006

To review the literature on primary dystonia and dystonia plus and to provide evidence‐based recommendations. Primary dystonia and dystonia plus are chronic and often disabling conditions with a widespread spectrum mainly in young people. Computerized MEDLINE and EMBASE literature reviews (1966–1967 February 2005) were conducted. The Cochrane Library was searched for relevant citations. Diagnosis and classification of dystonia are highly relevant for providing appropriate management and prognostic information, and genetic counselling. Expert observation is suggested. DYT‐1 gene testing in conjunction with genetic counselling is recommended for patients with primary dystonia with onset before age 30 years and in those with an affected relative with early onset. Positive genetic testing for dystonia (e.g. DYT‐1) is not sufficient to make diagnosis of dystonia. Individuals with myoclonus should be tested for the epsilon‐sarcoglycan gene (DYT‐11). A levodopa trial is warranted in every patient with early onset dystonia without an alternative diagnosis. Brain imaging is not routinely required when there is a confident diagnosis of primary dystonia in adult patients, whereas it is necessary in the paediatric population. Botulinum toxin (BoNT) type A (or type B if there is resistance to type A) can be regarded as first line treatment for primary cranial (excluding oromandibular) or cervical dystonia and can be effective in writing dystonia. Actual evidence is lacking on direct comparison of the clinical efficacy and safety of BoNT‐A vs. BoNT‐B. Pallidal deep brain stimulation (DBS) is considered a good option, particularly for generalized or cervical dystonia, after medication or BoNT have failed to provide adequate improvement. Selective peripheral denervation is a safe procedure that is indicated exclusively in cervical dystonia. Intrathecal baclofen can be indicated in patients where secondary dystonia is combined with spasticity. The absolute and comparative efficacy and tolerability of drugs in dystonia, including anticholinergic and antidopaminergic drugs, is poorly documented and no evidence‐based recommendations can be made to guide prescribing.
Global online marketplace: a cross‐cultural comparison of website quality
Authors: Kim Soyoung , Lee Yuri
Published: May 2006

This study used Loiacono’s WebQualTM scale to compare 278 US and 347 Korean Internet users in terms of their perceptions of retail website quality. The results of the study suggested that the dimensionality of website quality was not consistent across the two samples. This study also examined which dimensions of website quality influenced shoppers’ satisfaction with the site and purchase intention. The findings indicated that the relationship between website quality dimensions and the two dependent variables varied between the two groups.
Gender differences in Egyptian consumers’ green purchase behaviour: the effects of environmental knowledge, concern and attitude
Authors: Mostafa Mohamed M.
Published: May 2006

This study investigates the influence of three cognitive and attitudinal factors on gender differences in green purchase behaviour. Using a large sample size (n = 1093), a survey has been developed and administered across Egypt. The findings from the multivariate analysis of variance (MANOVA) confirm the influence of consumers’ ecological knowledge, concern and attitude on gender differences in green purchase behaviour. Consistent with previous studies, this study found that women appeared to be less aware of environmental issues compared with men. However, contrary to other studies conducted in the West, men showed more environmental concern and more positive outlook towards green purchase compared with women. The study discusses how the present findings may help policy makers and marketers alike to fine‐tune their environmental and marketing programmes.
Consumer debt in Australia: why banks will not turn their backs on profit
Authors: Griffiths Margaret
Published: May 2006

Five years into the 21st century and consumer debt levels in Australia are still escalating. Simultaneously, there is concern that an increasing number of consumers may be unable to meet their future financial commitments and also mounting alarm at the relative ease with which the majority of consumers can access additional credit facilities. At the same time, credit providers are avidly seeking greater profits by enticing consumers to borrow more and more. Against this background, the issue of corporate social responsibility (CSR) in the Australian consumer credit industry is discussed. The application of strategic stakeholder management theory to the activities of a specific class of lender – banks – is then discussed. The aim is to better understand why the contemporary demands of CSR will not lead banks to undertake a more vigilant approach to consumer lending advocated by consumer groups who, witnessing the impact of the growing level of consumer indebtedness on a number of consumers, are calling for more responsible lending practices. The opportunity to contribute to debate aimed at alleviating the risk of growing consumer indebtedness is highlighted. The paper concludes with an acknowledgement that, without intervention, factors such as competition in the free market for consumer credit, the demands of shareholders for profits, and consumers’ own folly in demanding immediate gratification and readily accepting additional credit as a means of financing their consumption, ensure that consumer debt levels will continue to rise.
Expanded polyglutamines impair synaptic transmission and ubiquitin–proteasome system in Caenorhabditis elegans
Authors: Khan Liakot A. , Bauer Peter O. , Miyazaki Haruko , Lindenberg Katrin S. , Landwehrmeyer Bernhard G. , Nukina Nobuyuki
Published: May 2006

Polyglutamine (polyQ) expansion in many proteins, including huntingtin and ataxin‐3, is pathogenic and responsible for neuronal dysfunction and degeneration. Although at least nine neurodegenerative diseases are caused by expanded polyQ, the pathogenesis of these diseases is still not well understood. In the present study, we used Caenorhabditis elegans to study the molecular mechanism of polyQ‐mediated toxicity. We expressed full‐length and truncated ataxin‐3 with different lengths of polyQ in the nervous system of C. elegans. We show that expanded polyQ interrupts synaptic transmission, and induces swelling and aberrant branching of neuronal processes. Using an ubiquitinated fluorescence reporter construct, we also showed that polyQ aggregates impair the ubiquitin–proteasome system in C. elegans. These results may provide information for further understanding the pathogenesis of polyQ diseases.
Transient glucose and amino acid deprivation induces delayed preconditioning in cultured rat cortical neurons
Authors: Gáspár Tamás , Kis Béla , Snipes James A. , Lenzsér Gábor , Mayanagi Keita , Bari Ferenc , Busija David W.
Published: May 2006

Several studies have demonstrated that glucose deprivation, combined either with anoxia or with the inhibition of oxidative phosphorylation, leads to the development of ischemic tolerance in neurons. The aim of our experiments was to investigate whether similar effects could be achieved by transient energy deprivation without either anoxia or the inhibition of the electron transfer chain. Preconditioning was carried out by incubating primary rat cortical neuronal cultures for 3, 6 or 9 h in a glucose‐ and amino acid‐free balanced salt solution supplemented with B27 in normoxic conditions. After 24 h, neuronal cultures were exposed to oxygen–glucose deprivation, glutamate or hydrogen peroxide. Cell viability was measured 24 h after the lethal insults. Potential mechanisms that can influence free radical production were also examined. Energy deprivation protected neuronal cells against lethal stimuli (e.g. cell survival after oxygen–glucose deprivation was 33.1 ± 0.52% in the untreated group and 80.1 ± 1.27% in the 9‐h energy deprivation group), reduced mitochondrial membrane potential, decreased free radical formation, attenuated the intracellular free calcium surge upon glutamate receptor stimulation, and resulted in an elevated level of GSH. Our findings show that transient energy deprivation induces delayed preconditioning and prevents oxidative injuries and neuronal cell death.
Isolation, characterization and multiplex genotyping of raccoon tetranucleotide microsatellite loci
Authors: CULLINGHAM C. I. , KYLE C. J. , WHITE B. N.
Published: May 2006

We have isolated and characterized 12 polymorphic tetranucleotide microsatellite loci in the raccoon (Procyon lotor). Three multiplex panels comprising the 12 loci were developed and 80 individuals from southeastern and western Ontario were genotyped; allele sizes were assigned without difficulty. One locus isolated was identified as an X‐linked marker. The number of alleles per locus ranged from six to 25 with the average heterozygosity ranging from 0.674 to 0.925. These loci will be used to characterize raccoon population structure across North America, and the data used to further understand the spread of raccoon rabies.