bluuebunny/crossref_metadata_2025 huggingface dataset:
Restricted T cell receptor delta chain genes repertoire in peripheral blood of multiple sclerosis patients
Authors: Nowak J.S. , Michalowska‐Wender G. , Januszkiewicz D. , Przybylski G. , Wender M.
Published: January 2011

Multiple sclerosis (MS) is a chronic inflammatory disease characterized by focal demyelination of central nervous system (CNS). Susceptibility to MS is thought to be affected by multiple genes including HLA and T cell receptor (TCR) genes. In view of the recent evidence, that in addition to α/β T lymphocytes also γ/δ T cells may have autoreactive potential, TCR delta repertoire in peripheral blood of MS patients has been studied. TCR delta repertoire, as assessed by Vδ‐Jδ rearrangements, has been analysed in 13 MS cases and in 30 healthy individuals by seminested PCR technique. Oligonucleotide primers specific for six Vδ regions and for Jδ1 gene were used for amplification of Vδ‐Jδ junctional region responsible for the diversity of γ/δ TCR. In the majority of MS patients PAGE analysis of Vδ1‐Jδ1, Vδ3‐Jδ1 and Vδ5‐Jδ1 rearrangements showed single‐band or two‐band pattern. The most striking result has been observed in Vδ5‐Jδ1 rearrangement, where in nine cases studied single band and in four patients two bands have been found. In all but one MS cases multi‐band pattern of Vδ2‐Jδ1 rearrangement was obtained. None of the 13 MS patients showed single‐band rearrangement pattern of Vδ4‐Jδ1 and Vδ6‐Jδl. Contrary to the MS group almost all healthy individuals produced smear‐like or multi‐band pattern of Vδ1‐Vδ5 to Jδ1 rearrangements. On the basis of the banding pattern produced by Vδ‐Jδ rearrangement in MS, it can be suggested that T lymphocytes had undergone clonal expansion in vivo, most likely due to stimulation by antigen related to CNS. In particular a very consistent single‐band pattern of Vδ5‐Jδ1 rearrangement observed in almost all MS patients studied, argues very strongly for a significant role of γ/δ T cells with Vδ5 rearrangement in the pathogenesis of MS. However, it cannot be excluded that the observed patterns of TCR δ gene rearrangement in MS patients may represent secondary changes to CNS damage.
Autosomal dominant cerebellar ataxia type I in Morocco: presence of the SCA1 and SCA3/MJD mutations
Authors: Benomar A. , Meggouh F. , Didierjean O. , Kissani N. , Dürr A. , Sefiani A. , Chkili T. , Agid Y. , Yahyaoui M. , Brice A.
Published: January 2011

We searched for CAG repeat expansions at the SCA1 and SCA3/MJD loci in nine families, including 15 examined patients, with autosomal dominant cerebellar ataxia type I from Morocco. Expansion of the CAG repeat was found in one family at the SCA1 and two at the SCA3/MJD locus, demonstrating the existence of genetic heterogeneity among ADCA type I families in Morocco. Instability during transmission was observed at both loci as in other unstable mutations. The phenotypes of the SCA1 and SCA3/MJD patients were similar.
Pattern of memory impairment in two patients with septohippocampal disconnection
Authors: Almonti S. , Silveri M.C. , Gainotti G. , Macchi G.
Published: January 2011

We describe two patients with lesions at different levels of the septo‐hippocampal system. Both of them were selectively affected by anterograde amnesia, with a less severe retrograde amnesia. These data confirm that the integrity of the septohippocampal system is indispensable for the maintenance of normal memory and learning mechanisms and that the lesions along this fibre pathway, causing a functional and anatomic disconnection between the septal area (basal forebrain) and the medial temporal lobe structures, interfere with normal explicit memory functions.
Event‐related brain potentials in paroxysmal kinesigenic choreoathetosis
Authors: Münte T.F. , Johannes S.
Published: January 2011

Event‐related brain potentials (ERPs) were recorded in a 30 year old female patient with familial kinesigenic choreoathetosis and a group of control subjects. In line with previous observations the patient exhibited an enhanced amplitude of the early component of the contingent negative variation of the ERP in a two stimulus paradigm. Moreover, a greatly enhanced P3 amplitude was found in auditory and visual classification (oddball) paradigms. These results suggest a general upregulation of widespread subcortical projection systems leading to both, abnormal movements and abnormally high amplitudes of ERPs.
The effect of repeated thyrotropin‐releasing hormone administration on regional cerebral blood flow in humans
Authors: Nagayama M. , Takahashi W. , Takagi S. , Shinohara Y.
Published: January 2011

Recently, regional cerebral blood flow (rCBF) was found to increase slightly in the thalamus after a single administration of thyrotropin‐releasing hormone (TRH) to healthy adults. Regarding the effect of prolonged TRH administration on rCBF, some scintigraphic improvements have been observed recently. However, no study has investigated the effect quantitatively, except for a preliminary study by positron emission tomography. Therefore, we examined the effect of repeated administration of TRH on rCBF in humans quantitatively. Eight patients with spinocerebellar degeneration (SCD) were given TRH intravenously at a daily dose of 2 mg for 14 days and rCBF was measured by the 133Xe intravenous injection method before and after repeated TRH administration. TRH caused a significant (p < 0.01) increase of 12% in the gray matter flow (fast flow, F|), especially at the parietal and occipital lobes, and also caused a significant (p < 0.05) increase of 8% in the initial slope index (ISI), especially at the parietal lobe. Among seven patients who improved clinically after TRH administration, F1 values were increased in all of them and ISI values were increased in six. We conclude that repeated TRH administration increases rCBF in humans. These results might warrant clinical investigation of a possible therapeutic role of TRH in patients with cerebral ischemia.
Sensory neuropathy and anti‐Hu antibodies in a patient with seminoma
Authors: Gabriel C.M. , Gregson N.A. , Hughes R.A.C.
Published: January 2011

A man with subacute sensory ncuronopathy (SSN) had a stage 1 seminoma. His serum and cerebrospinal fluid (CSF) contained anti‐Hu antibodies (type 1 anti‐neuronal nuclear antibodies, ANNA‐1). Following orchidectomy, radiotherapy, prednisolone, plasma exchange and intravenous immunoglobulin the antineuronal antibody titre fell and the neuropathy improved.
Epidemiology of Creutzfeldt‐Jakob disease—past and present uncertainties
Authors: Alpérovitch A.
Published: January 2011

Annual incidence rate of Creutzfeldt‐Jakob disease (CJD) is approximately equal to one per million. Because of misdiagnosis, especially in older individuals, and censoring by competitive cause of death, the true incidence of the disease might be underestimated. Incidence rates of CJD increased in all countries during the last 20 years; this increase is likely due to greater interest in this disease. Foci have been described in Lybian Jews in Israel and Slovakia; in these foci the codon 200 mutation of the PRNP gene was found in all CJD cases. Up to now, no CJD focus which might be explained by environmental exposure has ever been documented. Overall, about 85% of CJD cases are sporadic and 15% are associated with different mutations of the PRNP gene. More than half of CJD cases with PRNP mutation have no known family history of CJD. About 100 iatrogenic CJD cases have been published; most have been related to human growth hormone treatment. The causes of sporadic CJD are unknown. Except the codon 129 polymorphism of the PRNP gene, there are no well established risk factors for sporadic CJD. The recent occurence of a new variant of CJD which might be linked with bovine spongiform encephalopathy raises new issues about the role of environmental exposures in sporadic CJD.
Visuo‐spatial dysgnosia and Balint's syndrome as major symptoms of probable Alzheimer's disease
Authors: Davous P. , Panisset M. , De Agostini M. , Boiler F.
Published: January 2011

We describe three patients, respectively 57‐, 54‐ and 55‐years‐old at onset of the disease, who developed over many years a progressive cortical visual dysfunction without overt dementia. At onset, the visual symptoms were mainly a visuo‐spatial dysgnosia, but they progressively worsened to a near complete Balint syndrome in the three cases. In all of them, memory and language were only mildly affected during the first years of evolution, but a constructive and/or dressing apraxia and an optic ataxia in the left visual hemifield suggested in two cases a predominant dysfunction in the right hemisphere. Imaging of the brain by CT scan and MRI showed no focal atrophy, but the metabolic SPECT was early abnormal in all three cases showing a right posterior metabolic defect in two cases and a bilateral posterior hypometabolism in one case. Follow‐up of the patients showed that they all developed language and memory disturbances leading to severe dementia after 5–8 years of evolution. It is suggested that these patients are affected by a visual form of Alzheimer's disease which is probably the most frequent cause of progressive Balint‐Holmes syndrome.
Pure motor hemiparesis due to pontine infarction as the presenting sign of neurosyphilis
Authors: Primavera A. , Solaro C , Cocito L.
Published: January 2011

A 50‐year‐old man with minimal risk factors for cerebrovascular disease had a pure motor hemiparesis due to a pontine infarction. Syphilis serology and CSF findings indicated a previously unrecognized meningovascular syphilis as the cause of the stroke. At variance with the majority of recently reported cases, no prodromal syndrome preceded the cerebrovascular event, and there was no HIV coinfection. Treatment with erythromycin and minocycline was followed by clinical improvement and normalization of main CSF parameters including VDRL; oligoclonal bands and local IgG synthesis, however, were still detectable in post‐treatment CSF.
Magnetic resonance imaging in a case of migraine with ophthalmoplegia
Authors: Aers I. , Van Zandijcke M. , Dehaene I. , Casselman J.
Published: January 2011

We present the case of a migrainous girl who suffered an episode of migraine‐like headache associated with ophthalmoplegia. MRI showed reversible enhancement and thickening of the cisternal portion of the oculomotor nerve. This finding suggests a mechanism different from diabetic ischaemic neuropathy, as similar MRI abnormalities were rarely reported in diabetic ophthalmoplegia. The MRI findings are most in favour of an inflammatory cause.
Isolated palilalia: a case report
Authors: Gorno M. L. , Miozzo A. , Mattioli F. , Cappa S. F.
Published: January 2011

We report the case of a 74‐year‐old patient, whose spontaneous speech was characterized by the compulsive repetition of parts of words, words and sentences with increasing rate and decreasing loudness. On a standardized language examination, his performance was within normal limits, besides the presence of palilalia; neurological examination showed a slight limitation of vertical gaze and a reduction of blinking. A small right capsulo‐thalamic ischemic lesion was present on magnetic resonance imaging. This case provides further evidence that palilalia can be observed as an isolated impairment of speech production following subcortical lesions.
Handbook of Multiple Schlerosis
Authors: Jellinger K. A.
Published: January 2011

Book reviewed in this article:

Neurological Disease and Therapy Series no. 43. Second edition, revised and expanded. Edited by S. D. Cook.
A model of neurological disorder: a basis for logical and structured thinking and reasoning in neurology
Authors: Ekstedt J. , Forsgren L.
Published: January 2011

A model of neurological disorder in graphical form, based upon a model proposed by the World Health Organisation, is presented. This model is intended to be a basis for a logical and structured thinking and reasoning in neurology, not least in diagnosis. The model is a chain of basic elements in which each element causes the following one. The chain can be divided in two parts. The first part is: Etiology → Pathology → Manifestation. Pathology is described by the properties of the lesion in the nervous system. Manifestation is the symptoms and signs, that can be summarised as Semiology, in fact, synonymous with Impairment. The second part of the model is then: Manifestation (= Impairment) → Disability → Handicap. The terms disease and disorder are not contained within the chain of basic elements. The manifestation gives the patient an awareness of disorder and the condition may be classified as syndrome or disease. Neither the awareness of disorder nor the classification influences the chain of basic elements.
A prospective radiologic and neurologic follow‐up study of 61 HIV‐1 ‐infected subjects: early beginning and slow progression of brain atrophy
Authors: Raininko R. , Elovaara I. , Poutiainen E. , Virta A. , Valanne L. , Haltia M. , Lähdevirta J.
Published: January 2011

The course of the organic brain disease caused by human immunodeficency virus (HIV‐1) was evaluated in a follow‐up study. The primary material included 200 consecutive HIV‐1 infected persons. Sixty‐one subjects, in whom other brain‐affecting factors were excluded, consented to the follow‐up. They underwent 278 radiologic examinations: computed tomography, magnetic resonance imaging, or a combination of both (mean 4.6 examinations/subject). Clinical neurologic status and, in 40 subjects, cognitive performance were repeatedly evaluated. Sixteen subjects were followed up until death and 11 of them were autopsied. Median follow‐up time was 27 mo (range 2.5–66 mo). The most common radiologic finding was atrophy, found in 19 subjects at study entry and developing in 10 subjects during the study. Twenty‐four subjects (39%) showed the development and/or progression of atrophy. Atrophic changes progressed most rapidly in acquired immunodeficiency syndrome (AIDS), but mild developing/progressive atrophy was found even in 33% of asymptomatic or neurologically intact subjects. Cognitive and radiologic worsening were simultaneous in 6/7 subjects with declining neuropsychologic test performance. Signal intensity changes including HIV‐1 leukoencephalopathy appeared in AIDS patients with clear cognitive decline.
Neuropsychological functions in systemic lupus erythematosus: a comparison with chronic whiplash patients
Authors: Waterloo K. , Omdal R. , Mellgren S. I. , Husby G.
Published: January 2011

A comprehensive battery of neuropsychological tests sampling a wide range of cognitive functions was administrated to 36 patients with systemic lupus erythematosus (SLE), and a control group consisting of 31 patients with persistent symptoms after whiplash injury. Our results demonstrated significant group differences and suggest that cognitive dysfunction is common in SLE and that there are significant abnormalities in the SLE group compared to chronic illness of non‐immunological nature. Considerable variability occurred in the neuropsychological profiles for SLE patients. No significant association was found between cognitive dysfunction and use of corticosteroids, except for the two neuropsychological tests Digit span and Seashore rhythm test. Associations were not found between cognitive dysfunction and depression either, except for the Seashore rhythm test. These findings indicate that cognitive dysfunction in SLE reflects CNS involvement, rather than coexisting emotional disturbance. No significant cognitive impairment was found in the whiplash group. However, our results indicate depressed mood among the whiplash group.
Late onset myasthenia gravis
Authors: Aarli J. A.
Published: January 2011

Late onset myasthenia gravis (MG) is defined as the onset of the disease after the age of 50 in a patient with no clinical or paraclinical evidence of a thymoma. Myasthenia gravis has a bimodal appearance for both sexes with a peak age of onset located in the early onset group and another peak for late onset of MG. Early onset of MG in men is approximately 10 years later than in females. In late onset MG, the thymus is smaller than in early onset and much of the lymphoid tissue has been substituted with fat. There is no morphological evidence that the thymus atrophy seen in late onset MG is any different from the thymus involution which is age‐related. The AChR antibody concentration is lower in cases with late onset MG than it is in early onset and in thymoma‐associated MG. Sera from late onset MG patients can be divided in 2 groups; those with and those without striated muscle (MGT 30) antibodies. Their presence is not an effect of age since they are not detected among old healthy individuals. Nor is it an effect of long MG duration, since there is no relation between titre and duration. Non‐thymoma MG patients who demonstrate an immune response to titin identical to that observed in thymoma, have a worse prognosis and seem to do less well after thymectomy than late onset MG patients without muscle antiboides.
Alois Alzheimer and Gaetano Perusini: should man divide what fate united?
Authors: Macchi G. , Brahe C. , Pomponi M.
Published: January 2011

Three points of interest lie in considering how Alzheimer, and more significantly Perusini, struggled to throw light on the cause of this devastating disease. There is a stimulating possibility that Perusini believed presenile forms of AD described the same disease as senile forms. If so, this would anticipate current opinion, and reveal that Perusini dissented from Kraepelin. In addition, Perusini may have understood the pathological relationship between neuritic plaques and vascular changes, once more foreseeing the modern view of Alzheimer's disease. Finally, Perusini and Alzheimer disagreed with Jung's view concerning the relationship between neuropathology and clinical psychiatry. This point highlights the major changes occurring at that time in moving from classical neurology to the psychoanalytic era. In his last work (1911) Alzheimer quoted his Italian disciple many times, even speaking of “Perusini's cases” (Perusinischen Fälle). This article is an attempt to change the eponym of Alzheimer's disease into the Alzheimer–Perusini disease. This is a brief history of a master and his disciple, whose scientific lives were, by events, divided.
Clinical, genetic and electrophysiologic correlation in hereditary neuropathy with liability to pressure palsies with involvement of PMP22 gene at chromosome 17p11.2
Authors: Cruz‐Martínez A. , Bort S. , Arpa J. , Duarte J. , Palau F.
Published: January 2011

Clinical and electrophysiological studies were performed in affected and unaffected individuals from five unrelated families segregating hereditary neuropathy with liability to pressure palsies (HNPP) as an autosomal dominant trait. A molecular lesion at the HNPP locus in chromosome 17p11.2 was previously confirmed in all families. In four families the HNPP 1.5Mb was demonstrated. In the fifth family the mutation was a point mutation involving the 5′ donor splicing site of the first intron of PMP22 gene. Clinical variability between and within families was observed. Susceptibility to minimal traumas was also variable. We mention certain peculiarities such as painless brachial plexus neuropathy, conduction block lasting more than 9 years, slimmer's paralysis as the unique clinical manifestation of the disease, and diagnostic problems in asymptomatic individuals. There is a genetic and electrophysiological correlation in affected individuals with HNPP.
Sneddon's syndrome: a clinical and neuropsychological study on 21 patients
Authors: Lorusso S. , Gallassi R. , Arcangeli F.
Published: January 2011

We systematically investigated the cognitive performances of a large series of patients with Sneddon's syndrome (SS) and their relation to clinical or instrumental parameters. We evaluated twenty‐one patients with idiopathic generalized livedo reticularis and neurological symptoms and signs consistent with cerebrovascular disease, comparing their cognitive performances with those of the same number of matched normal controls. The patients underwent dermatological evaluation with skin biopsy, biochemical tests including anticardiolipin antibodies, neurological and neuroimaging investigations. Only three patients were classifiable as demented, but all the others showed multiple or few cognitive disorders (nine patients for each group, respectively). Both patients and controls were examined by neuropsychological tests investigating mental deterioration, executive functions, memory and instrumental abilities. The group with few cognitive disorders performed significantly worse with respect to the controls in copy design, verbal fluency, immediate visual memory and forward and reverse digit span. In the whole group of SS patients, cognitive involvement was correlated with neurological focal signs and neuroradiological focal alterations. These data suggest a constant cognitive involvement in SS, to varying degrees, which is more often mild and subclinical. The prominent visual–constructive, attentional and working memory dysfunctions which were detected in our series point to a subcortical pattern.
Is juvenile spinal muscular atrophy of the distal upper limbs due to cervical flexion myelopathy? A case report
Authors: Siebner H. R. , Keinath S. , Bischoff C. , Conrad B.
Published: January 2011

A 22‐year‐old woman developed a slowly progressive symmetric weakness and muscular atrophy of distal upper limbs at the age of 17. Radiography during anteflexion and retroflexion showed a hypermobile cervical spine with a maximum at the C5/6 disc level. Cervical myelography and postmyelographic computed tomography (CT) of the lower cervical spine demonstrated a remarkable anterior shift of the dural sac during anteflexion resulting in anteroposterior compression of the lower spinal cord. Postmyelographic CT and magnetic resonance imaging (MRI) revealed atrophy of the lower spinal cord with bilateral cystic lesions. We suppose that repetitive straining and compression of the lower cervical cord during neck flexion of the hypermobile cervical spine caused selective necrosis of anterior horn cells with secondary cystic transformation. Mechanically induced flexion myelopathy should be considered in all young patients presenting with muscular atrophy of the distal upper limb. Functional CT myelography or dynamic MRI of the cervical spine are appropriate to demonstrate lower spinal cord compression during flexion.
Reflex sympathetic dystrophy in a patient with peripheral sympathetic denervation
Authors: Kimber J. R. , Smith G. D. P. , Mathias C.J.
Published: January 2011

Reflex sympathetic dystrophy (RSD) includes persistent pain in an extremity, associated with features of vasomotor dysfunction and, later, dystrophic changes. Many theories have been proposed to explain these clinical features and include sympathetic dysfunction. We report a unique case of RSD following an upper limb fracture in a patient with established severe peripheral sympathetic denervation as part of idiopathic chronic autonomic failure. This case provides evidence that the clinical features of RSD were not mediated via increased sympathetic nerve activity in the affected limb. Locally released neuropeptides or autocoids therefore may be involved in the pathogenesis of RSD. The term sympathetic in RSD may be inappropriate and require revision.
Sleep related breathing disturbances and nocturnal akinesia in Parkinson's disease patients: a cross section study
Authors: Schwalen S. , Leclaire J. , Rechlin M. , Jörg J.
Published: January 2011

We studied the occurrence of sleep related breathing disturbances (SRBD) and nocturnal akinesia in patients with Parkinson's Disease (PD) and evaluated the likelihood that PD symptoms consistently influence these sleep disorders. Ninety‐two PD patients (mean age 69 years; mean Webster rating score 13) were studied. An ambulatory sleep disorder screening device (MESAM4) was used to measure oxygen saturation, heart rate, snoring sounds and body position during sleep. Sleep related breathing disturbance (SRBD) was diagnosed if typical cyclic oxygen desaturations > 4% more than 10 times/h were recorded. A nocturnal akinesia was diagnosed if there were < 0.3 changes of body positions/hour or a maximal time in one position of more than 4.3 h. There was evidence of SRBD in 17% of PD patients. SRBD correlated positively with high rigidity and bradykinesia scores as well as high Webster rating scale scores and age of men. Thirty percent of PD patients had an objective nocturnal akinesia. This was also combined with a high rigidity score. No relation between SRBD or Nocturnal akinesia, l‐Dopa intake, duration of the disease or onset of the disease was found.
Incidence and prevalence of motor neuron disease in Coastal and Mountainous Regions, Croatia, 1984–1993. A preliminary survey
Authors: Zivadinov R. , Jurjevic A. , Willheim K. , Biasutti E. , Cazzato G. , Zorzon M.
Published: January 2011

In the period 1984–1993, we carried out an incidence and prevalence study of motor neuron disease in Coastal and Mountainous Regions, Croatia. This was the first epidemiological study on motor neuron disease in Croatia. A total of 22 incident cases were identified. The crude annual incidence rate was 0.68 (95% confidence interval 0.43‐1.03) per 100,000 person‐years; 0.95 (95% confidence interval 0.60‐1.43) per 100,000 person‐years for men and 0.42 (95% confidence interval 0.26‐0.63) per 100,000 person‐years for women. The rate adjusted to the European population was 0.63 (95% confidence interval 0.39‐0.95) per 100,000 person‐years. The age‐adjusted incidence in our population is similar to the rates, standardized to the European population, observed in Italy, but lower than recent rates in the US and northern European studies. On 31st December 1993, there were 18 prevalent cases. The prevalence adjusted to the European population was 5.24 (95% confidence interval 3.10‐8.28) per 100,000 population.
Long‐term follow‐up by video of cervical dystonia treated with botulinum toxin
Authors: Jamieson D. R. S. , Mann A.C.
Published: January 2011

We have analysed video recordings of 21 patients with cervical dystonia treated with botulinum toxin. Fourteen patients have a record both of their response shortly after injections were commenced and between four years five months and six years seven months later. Our analysis shows that the long term outcome is often better than the initial response. We suggest that chronic treatment with botulinum toxin allows different muscles to those initially injected to be identified as contributors to the dystonia. Subsequent injection of these muscles leads to further improvement. It implies that cervical dystonia is a more widespread disorder of motor control, rather than simply limited to a few muscles.
Limbic encephalitis, specifically depicted by PET
Authors: Bernsen R. A. , de Jong B. M.
Published: January 2011

A 44‐year‐old woman suffered from limbic encephalitis, possibly caused by herpes simplex virus. Neither CT‐scanning nor MR‐Imaging showed a persistent lesion. Positron emission tomography, however, indicated a metabolic deficit in circumscript limbic structures, namely gyrus cinguli and temporal cortices. This distribution supported the clinical diagnosis. Moreover, it suggested a close relation of the gyrus cinguli with the organization of limbic functions in the brain. It might support suggestions made in the literature that the gyrus cinguli is more frequently affected by HSV encephalitis. Further PET‐studies in cases of limbic encephalitis would be of interest.
Semantic category and initial letter word fluency in left‐brain‐damaged patients
Authors: Basso A. , Burgio F. , Prandoni P.
Published: January 2011

Fluency deficits have been found in different neuropsychological populations, and dissociation of letter initial and category fluency has been demonstrated in right‐brain‐damaged and Alzheimer patients. Studies on normal controls also indicate that letter initial and category fluency are supported by different brain regions. We studied letter initial and category fluency in 79 left‐brain‐damaged patients, 18 non‐aphasic and 61 aphasic. Our results indicate that brain‐damaged patients are significantly impaired in both fluency tasks compared to normal controls, and a difference between letter initial and category fluency emerges from our data. Firstly, only letter initial fluency is more impaired in aphasic than in non‐aphasic patients; secondly, many aphasics have normal category fluency and pathological letter initial fluency but the reverse is seldom true and thirdly, only category fluency scores correlate with confrontation‐naming scores. It is suggested that strategies to recover words based on a semantic or a phonological prompt are different. A semantic strategy is coherent with the organizational structure of the semantic system and corresponds to the normal way in which we recover words whereas a phonological strategy is quite unusual and it is not used for actual communication.
Cytokine profile in interferon‐β treated multiple sclerosis patients: reduction of interleukin‐10 mRNA expressing cells in peripheral blood
Authors: Andersson M. , Khademi M. , Wallström E. , Olsson T.
Published: January 2011

Treatment with interferon‐β reduces relapse rate, slows progression of neurological disability and reduces the number of active brain lesions observed with magnetic resonance imaging in relapsing‐remitting multiple sclerosis. Interferon‐β has antiviral properties, but in addition it affects the expression of several immunoregulatory genes, including genes for cytokines such as interferon‐γ and interleukin‐10. Cytokines are believed to be central in the pathologic process in multiple sclerosis, by regulating autoreactive T‐ and B‐cell responses. In this study we have determined effects of interferon‐β on the frequency of cells in peripheral blood and cerebrospinal fluid expressing mRNA for interferon‐γ, tumor necrosis factor‐α, interleukin‐4 and interleukin‐10 in a group of multiple sclerosis patients. All patients were treated for two months or more, since the beneficial effect of interferon‐β is not apparent until after several months of treatment. We detected a significant reduction of interleukin‐10 mRNA expressing cells in the peripheral blood during interferon‐β treatment compared with pretreatment values (10 vs 33 cells/105; p = 0.028) while the other investigated cytokines were not significantly affected. We conclude that there is a long term effect of interferon‐β on cytokine expression in multiple sclerosis patients. Its relation to the therapeutic effect is as yet not clear.
The molecular diagnosis of spinocerebellar ataxia type 1 in patients with ataxia
Authors: Koefoed P. , Nielsen J. E. , Hasholt L. , Jensen P. K. A. , Fenger K. , Sørensen S. A.
Published: January 2011

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disease caused by an expanded CAG trinucleotide repeat in the SCA1 gene on chromosome 6. We screened 40 probands with ataxia for the CAG repeat expansion and found five probands with SCA1 representing five different families. The SCA1 gene was analysed in 50 members of these families, and the CAG repeat expansion was found in all 17 affected persons and in 14 unaffected at‐risk individuals. The range of expansion was 41–53 repeat units, while the range of normal alleles was 22–36 repeat units. We found pronounced inter‐ and intrafamilial phenotypical variation. One of the families had a comparatively mild phenotype which correlates with a CAG repeat length in the low end of the range of expansions and a late age at onset. With few exceptions, normal alleles of the SCA1 gene have one to three CAT interruptions in the middle of the CAG repeat, while all expanded alleles are uninterrupted. We report the hitherto longest normal uninterrupted allele of 22 repeat units and stress the importance of analysis for the presence of CAT interruptions in the diagnosis of SCA1.
The Community Engagement of Immigrants in Host Societies: The Case of Canada
Authors: Kazemipur Abdolmohammad
Published: January 2011

Using the data from the General Social Survey (2003), the community engagement of immigrants in Canada has been examined along 15 different dimensions. The findings indicate that immigrants add to the overall level of community engagement in Canada in the areas of confidence in public institutions ‐‐ such as judiciary, government, police, welfare system, education, and health care ‐‐ and involvement in religious activities. The areas in which immigrants fall behind are those that involve social interactions with the host population (e.g., trust, neighbourliness, social networks, group activities, volunteering, etc.) or engagement with private sector (i.e., confidence in private institutions such as banks and major corporations). Some of these measures of community engagement improve over time, but there is also an alarming trend that some decline with longer stays in Canada. The implications of these findings are discussed.
On Compensation and Return: Can The ‘Continuing Injustice Argument’ for Compensating for Historical Injustices Justify Compensation for Such Injustices or the Return of Property?
Authors: PEREZ NAHSHON
Published: January 2011

abstract

This paper offers a critique of recent attempts, by George Sher and others to justify compensation to be paid to descendants of deceased victims of past wrongs. This recent attempt (the ‘continuing injustice argument’) is important as it endeavours to avoid some well‐known critiques of previous attempts, such as the non‐identity problem. Furthermore, this new attempt is grounded in individual rights, without invoking a more controversial collectivist assumption. The first step in this critique is to differentiate between compensation and restitution. Once this important distinction is clear, an examination of several factors follows: the importance of the passage of time vis‐à‐vis claims for compensation and/or restitution (and especially the passing away of the original victims and wrongdoers), the responsibility of the would be payers, the responsibility of the descendants of the victims, the welfare level of the descendants of the victims, information‐related issues, and several additional factors. The conclusion is that once we take into account the distinction between compensation and restitution, and the additional factors mentioned, the case for compensation and/or restitution under the ‘continuing injustice argument’, is highly limited.
Annual Research Review: Prenatal stress and the origins of psychopathology: an evolutionary perspective
Authors: Glover Vivette
Published: January 2011

If a mother is stressed or anxious while pregnant her child is more likely to show a range of symptoms such as those of attention deficit hyperactivity disorder, conduct disorder, aggression or anxiety. While there remains some debate about what proportion of these effects are due to the prenatal or the postnatal environment, and the role of genetics, there is good evidence that prenatal stress exposure can increase the risk for later psychopathology. Why should this be? In our evolutionary history it is possible that some increase in these characteristics in some individuals was adaptive in a stressful environment, and that this type of fetal programming prepared the child or group for the environment in which they were going to find themselves. Anxiety may have been associated with increased vigilance, distractible attention with more perception of danger, impulsivity with more exploration, conduct disorder with a willingness to break rules, and aggression with the ability to fight intruders or predators. This adaptation for a future dangerous environment may explain why stress and anxiety, rather than depression, seem to have these programming effects; why there is a dose–response relationship with prenatal stress from moderate to severe and it is not only toxic stress that has consequences; why not all children are affected and why individual children are affected in different ways; and why the outcomes affected can depend on the sex of the offspring. An evolutionary perspective may give a different understanding of children in our society with these symptoms, and suggest new directions for research. For example, there is some evidence that the type of cognitive deficits observed after prenatal stress have specific characteristics; these may be those which were adaptive in a past environment.
Elucidation of mechanism of aminoreductone formation in the Maillard reaction of lactose
Authors: TRANG VU THU , SHIMAMURA TOMOKO , KASHIWAGI TAKEHIRO , UKEDA HIROYUKI , KATSUNO SHINYA
Published: January 2011

The aim of this study was to elucidate the formation mechanism of aminoreductone, an important indicator for estimating the extent of Maillard reaction of lactose and amino compounds. Using the model system of lactose and butylamine, the model solution of lactose and milk proteins, and milks, it was concluded that d‐galactose was liberated at the same time as the generation of aminoreductone. It was shown that the extent of the Maillard reaction and the lactose degradation during heating depended closely on the concentration ratio of amino group/lactose in the sample solution.
Flavonoid modulation of GABAAreceptors
Authors: Hanrahan Jane R. , Chebib Mary , Johnston Graham A. R.
Published: January 2011

There has been a resurgence of interest in synthetic and plant‐derived flavonoids as modulators of γ‐amino butyric acid‐A (GABAA) receptor function influencing inhibition mediated by the major inhibitory neurotransmitter GABA in the brain. Areas of interest include (i) flavonoids that show subtype selectivity in recombinant receptor studiesin vitroconsistent with their behavioural effectsin vivo, (ii) flumazenil‐insensitive modulation of GABAAreceptor function by flavonoids, (iii) the ability of some flavonoids to act as second‐order modulators of first‐order modulation by benzodiazepines and (iv) the identification of the different sites of action of flavonoids on GABAAreceptor complexes. An emerging area of interest is the activation of GABAAreceptors by flavonoids in the absence of GABA. The relatively rigid shape of flavonoids means that they are useful scaffolds for the design of new therapeutic agents. Like steroids, flavonoids have wide‐ranging effects on numerous biological targets. The challenge is to understand the structural determinants of flavonoid effects on particular targets and to develop agents specific for these targets.
Public Health Nurses Tailor Interventions for Families at Risk
Authors: Monsen Karen A. , Radosevich David M. , Kerr Madeleine J. , Fulkerson Jayne A.
Published: January 2011

ABSTRACT
Objectives: To use structured clinical data from public health nurse (PHN) documentation to describe client risk, to describe family home visiting interventions, including tailoring, and to assess the associations between client risk and intervention tailoring.

Design and Sample: Retrospective cohort design. A cohort of 486 family home visiting clients who received at least 3 visits from PHNs in a local Midwest public health agency (2000–2005).

Measures: Omaha System variables documenting assessments, interventions, and outcomes. A risk index was created to identify low‐ and high‐risk clients. Descriptive and inferential methods were used to describe interventions, and to assess intervention tailoring between groups.

Intervention: Routine PHN family home visiting practice.

Results: The risk index meaningfully discriminated between groups. PHNs provided more visits and interventions to clients in the high‐risk group, with variations in problem, category, and target by group, demonstrating that PHNs tailored interventions to address specific client needs.

Conclusions: Standardized terminologies and structured clinical data are useful tools to support PHN practice, and may be useful to advance health care quality research, program evaluation, policy development, and population health outcomes.
A shear‐based assay for assessing plasma ADAMTS13 activity and inhibitors in patients with thrombotic thrombocytopenic purpura
Authors: Han Yue , Xiao Juan , Falls Erica , Zheng X. Long
Published: January 2011

BACKGROUND: Severe deficiency of plasma ADAMTS13 activity is a frequent finding in patients with hereditary and acquired thrombotic thrombocytopenic purpura (TTP). To date, plasma ADAMTS13 activity is determined by cleavage of either predenatured von Willebrand factor (VWF) or small peptides derived from the VWF‐A2 domain. The physiologic relevance of the assay results is uncertain.

STUDY DESIGN AND METHODS: We sought to develop a novel shear‐based assay to assess plasma ADAMTS13 activity and inhibitors. We also compared this assay with a fluorogenic peptide assay.

RESULTS: We found that an incubation of purified plasma VWF with 0.5 to 1.0 µL of citrated plasma under constant vortexing at 2500 rpm for 60 minutes in the presence of 5 mmol/L CaCl2 and 1.7 µmol/L ZnCl2 and low concentration of NaCl resulted in the maximal cleavage of VWF. The cleavage product could be separated by a 2.5% agarose gel and detected by Western blotting. The assay revealed that plasma and recombinant ADAMTS13 are highly sensitive to inhibition by zinc and chloride ions. Under the optimal conditions, the shear‐based assay appeared to be more sensitive than the guanidine‐denaturization assay for determining plasma ADAMTS13 activity.

CONCLUSIONS: Our fluid shear‐based assay may be useful for investigating basic biologic function and regulation of ADAMTS13 metalloprotease. It may also be applicable for assessing plasma ADAMTS13 activity and inhibitors in TTP patients.
Bone regeneration: stem cell therapies and clinical studies in orthopaedics and traumatology
Authors: Gómez‐Barrena Enrique , Rosset Philippe , Müller Ingo , Giordano Rosaria , Bunu Carmen , Layrolle Pierre , Konttinen Yrjö T. , Luyten Frank P.
Published: January 2011

Introduction

Characterization of cells for bone regeneration in human beings

State of the art in bone tissue engineering

Clinical targets for cell therapy in orthopaedics

Selected paediatric bone disorders and cellular therapies

Ethical aspects of EU clinical trials

Future directions and remarks

Regenerative medicine seeks to repair or replace damaged tissues or organs, with the goal to fully restore structure and function without the formation of scar tissue. Cell based therapies are promising new therapeutic approaches in regenerative medicine. By using mesenchymal stem cells, good results have been reported for bone engineering in a number of clinical studies, most of them investigator initiated trials with limited scope with respect to controls and outcome. With the implementation of a new regulatory framework for advanced therapeutic medicinal products, the stage is set to improve both the characterization of the cells and combination products, and pave the way for improved controlled and well‐designed clinical trials. The incorporation of more personalized medicine approaches, including the use of biomarkers to identify the proper patients and the responders to treatment, will be contributing to progress in the field. Both translational and clinical research will move the boundaries in the field of regenerative medicine, and a coordinated effort will provide the clinical breakthroughs, particularly in the many applications of bone engineering.
A study of personality factors and interaction in 4th‐year dental students and their teachers
Authors: Watts Trevor L. P. , Millard Lesley
Published: January 2011

No previous investigation has considered dental student and teaching staff opinions on their relationship with each other. In a day when students are increasingly asked for feedback on the quality of teaching by staff, such investigations are of particular interest. This exploratory study was designed to compare the personality characteristics of a clinical year of dental students with those of the teaching staff they most frequently encountered, and to investigate these factors for possible associations with the quality of perceived teaching‐learning interaction between the 2 groups. A complete 4th year of dental students (n = 87), and those teachers whom they met regularly (n = 80), were asked to participate. Subjects completed a form of the Myers‐Briggs personality questionnaire simplified for use in education, and were asked to assess their relationship with persons in the other group. All the students and 75% of the staff, after follow‐up, returned usable data. There was close similarity between staff and student personality profiles, and perception of working relationships by both groups was largely independent of personality factors and temperament. There were differences in staff perception of their relationship with extrovert and introvert students. Students showed minor differences in their perception of staff relationships with respect to two other personality factors. These findings indicate a substantial similarity between staff and students, and suggest a mature and stable relationship between people in the 2 groups.
Trends in medical education*
Authors: Bligh John G.
Published: January 2011

Undergraduate medical education in the United Kingdom is undergoing major changes. These changes are the result of pressures from three different but interrelated areas. Alterations in the funding and management of higher education and health care, new approaches to curriculum design and advances in clinical practice each have significant impact on how medical education is delivered. Students, teachers, medical schools and hospital and community practice are all affected by these changes.
Dental Education Resources on the Web
Authors: Davis L. G. , Winstanley R. B. , Duffin R. , Griffiths A.
Published: January 2011

New methods of delivering educational material are being developed in order to improve effectiveness and efficiency of learning. They are not designed to replace conventional teaching methods, but to complement them and encourage self‐directed learning among students. In the present technological age, the use of computers in teaching and learning is almost mandatory. This article describes the development of educational aids available on the World Wide Web through the project DERWeb (Dental Education Resources on the World Wide Web) and its expansion as a site for dental information for the whole dental community. Courses and demonstrations to various groups by the team have led to interest and uptake by those who were previously unaware of this form of information dissemination or were “afraid” to use computers. The project is expanding continually and projections for future development are discussed.
The basic science teaching experience in the Nordic countries*
Authors: Fiehn Nils‐Erik
Published: January 2011

The article concerns the dental education at 10 different faculties in Denmark, Iceland, Norway, Sweden, and Finland. The information is based upon a questionnaire to the faculties concerning the definition, the structure and succession of the basic science subjects/courses/themes/topics in the dental curriculum, the time span of the basic science teaching, and the methods of teaching. Furthermore, there is information about integration among the basic science subjects and the coordination/integration between the basic science teaching and the clinical teaching. Finally, the teachers' educational background and the cowork with the medical education is elucidated.

The main findings are: the basic science teaching is structured in different ways in the Nordic countries. In Reykjavik, Bergen, and Aarhus this teaching is subject specific, while in Copenhagen it is mainly subject specific, as it has few integrated courses. In Gothenburg, Stockholm, and Umeå, the basic science teaching consists of a mixture of integrated courses and subject specific teaching. In Helsinki, Oslo and Malmö the basic sciences are theme and topic based, and for the two last‐mentioned institutions, integrated with the clinic, except at the faculty in Malmö. Here the basic science teaching is totally integrated in the clinical teaching throughout the study. Usually, the basic science teaching is placed mainly in the first part of the curriculum; however, in Umeå, Copenhagen, and Oslo, some integration takes place.