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Early Feature (early + feature)
Selected AbstractsClinical application of measurement of hippocampal atrophy in degenerative dementiasHIPPOCAMPUS, Issue 6 2009Josephine Barnes Abstract Hippocampal atrophy is a characteristic and early feature of Alzheimer's disease. Volumetry of the hippocampus using T1-weighted magnetic resonance imaging (MRI) has been used not only to assess hippocampal involvement in different neurodegenerative diseases as a potential diagnostic biomarker, but also to understand the natural history of diseases, and to track changes in volume over time. Assessing change in structure circumvents issues surrounding interindividual variability and allows assessment of disease progression. Disease-modifying effects of putative therapies are important to assess in clinical trials and are difficult using clinical scales. As a result, there is increasing use of serial MRI in trials to detect potential slowing of atrophy rates as an outcome measure. Automated and yet reliable methods of quantifying such change in the hippocampus would therefore be very valuable. Algorithms capable of measuring such changes automatically have been developed and may be applicable to predict decline to a diagnosis of dementia in the future. This article details the progress in using MRI to understand hippocampal changes in the degenerative dementias and also describes attempts to automate hippocampal segmentation in these diseases. © 2009 Wiley-Liss, Inc. [source] Olfaction in neurodegenerative disorderMOVEMENT DISORDERS, Issue 4 2003Christopher Hawkes MD Abstract There has been an increase of interest in olfactory dysfunction since it was realised that anosmia was a common feature of idiopathic Parkinson's disease (PD) and Alzheimer-type dementia (AD). It is an intriguing possibility that the first sign of a disorder hitherto regarded as one of movement or cognition may be that of disturbed smell sense. In this review of PD, parkinsonian syndromes, essential tremor, AD, motor neurone disease (MND) and Huntington's chorea (HC) the following observations are made: 1) olfactory dysfunction is frequent and often severe in PD and AD; 2) normal smell identification in PD is rare and should prompt review of diagnosis unless the patient is female with tremor-dominant disease; 3) anosmia in suspected progressive supranuclear palsy and corticobasal degeneration is atypical and should likewise provoke diagnostic review; 4) hyposmia is an early feature of PD and AD and may precede motor and cognitive signs respectively; 5) subjects with anosmia and one ApoE-4 allele have an approximate 5-fold increased risk of later AD; 6) impaired smell sense is seen in some patients at 50% risk of parkinsonism; 7) smell testing in HC and MND where abnormality may be found, is not likely to be of clinical value; and 8) biopsy of olfactory nasal neurons shows non-specific changes in PD and AD and at present will ot aid diagnosis. © 2003 Movement Disorder Society [source] Ichthyotic-Appearing Skin Changes Associated with Childhood Morphea, Systemic Sclerosis, and Systemic Lupus Erythematosus/Scleroderma OverlapPEDIATRIC DERMATOLOGY, Issue 2 2010Christy M. Williams M.D. We report three children with ichthyotic-appearing skin changes that may be an early feature reflecting underlying dermal sclerosis. Detection of these subtle skin changes may be predictive of more widespread cutaneous involvement, ultimately prompting earlier treatment. [source] A comparative study of cardiac dysautonomia in autosomal dominant spinocerebellar ataxias and idiopathic sporadic ataxiasACTA NEUROLOGICA SCANDINAVICA, Issue 3 2009M. Netravathi Objectives,,, Comparative evaluation of cardiac dysautonomia in spinocerebellar ataxias (SCA) and idiopathic sporadic ataxias (IA) not fulfilling the criteria of multiple system atrophy. Material and methods,,, Cardiac autonomic functions were evaluated in 14 SCA (SCA1 = 6, SCA2 = 5 and SCA3 = 3) and 10 IA patients, comparable for age, age at onset, duration and severity of illness. The results were categorized as early, definitive, or severe autonomic involvement (EI, DI and SI respectively) based on the degree of abnormalities on tests of parasympathetic and sympathetic pathways. Results,,, Cardiac autonomic dysfunction was present in all (EI = 25.0%, DI = 41.7% and SI = 33.3%), parasympathetic dysfunction being an early feature. SI was most often present in SCA3 (100%), followed by those with SCA1 (66.7%), and SCA2 (20%) and none in IA. Conclusions,,, Cardiac dysautonomia was common in both SCA and IA, although the severity was greater in SCA. Among SCAs, the severity was greatest in SCA3, followed by SCA2 and least in SCA1. [source] Restless legs syndrome: an early manifestation of Huntington's disease?ACTA NEUROLOGICA SCANDINAVICA, Issue 4 2009E. Savva Background,,, Far from being uniform, Huntington's disease (HD)'s phenotype encompasses a large variety of motor and non-motor symptoms which occur in various combinations in individual patients. Aim,,, To describe an unusual association between restless legs syndrome (RLS) and HD. Methods and results,,, We report a patient with an atypical presentation of genetically demonstrated HD, who developed typical RLS a few years prior to and during the course of HD. Common causes of RLS were excluded and the polysomnography confirmed frequent and severe periodic limb movements during sleep and while awake. Conclusions,,, We propose RLS as an uncommon but early feature of HD in some patients, and suggest adding HD to the already long list of RLS secondary to neurodegenerative conditions. [source] Angiokeratoma: a cutaneous marker of Fabry's diseaseCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2010L. M. J. Albano Summary The initial symptoms of Fabry's disease (FD) may seem harmless and may delay its diagnosis. A survey and screening for FD were performed on men with biopsy-proven angiokeratoma and some of their relatives (n = 29). Three patients were identified. Dermatologists should be aware of this prominent early feature and investigate unexplained cutaneous vascular lesions to detect FD. [source] Early homing of adult mesenchymal stem cells in normal and infarcted isolated beating heartsJOURNAL OF CELLULAR AND MOLECULAR MEDICINE, Issue 2 2008Claudia Penna Abstract Little is known on the early homing features of transplanted mesenchymal stem cells (MSCs). We used the isolated rat heart model to study the homing of MSCs injected in the ventricular wall of a beating heart. In this model all types of cells and matrix elements with their interactions are represented, while external interferences by endothelial/neutrophil interaction and neurohormonal factors are excluded. We studied the morphology and marker expression of MSCs implanted in normal hearts and in the border-zone of infarcted myocardium. Early morphological adaptation of MSC homing differs between normal and infarcted hearts over the first 6 hrs after transplantation. In normal hearts, MSCs migrate very early through the interstitial milieu and begin to show morphological changes. Yet, in infarcted hearts MSCs remain in the site of injection forming clusters of round-shaped cells in the border-zone of the infarcted area. Both in normal and infarcted hearts, immuno-histochemistry and confocal imaging showed that, besides the proliferative marker proliferating cell nuclear agent (PCNA), some transplanted cells early express myoblastic maker GATA-4, and some of them show a VWF immunopositivity. Moreover, a few hours after injection connexin-43 is well evident between cardiomy-ocytes and injected cells. This study indicates for the first time that the isolated beating heart is a good model to study early features of MSC homing without external interferences. The results show (i) that MSCs start to change marker expression few hours after injection into a beating heart and (ii) that infarcted myocardium influences transplanted MSC morphology and mobility within the heart. [source] | ||||||||||||||||||||||