Albert C Ludolph

..The field of amyotrophic lateral sclerosis (ALS) has seen a number of remarkable advances during recent years that will be summarized in this review...

..Our aim was to prove whether the panel is a reliable indicating device for the diagnosis of ischemic stroke in a time window of 6 h to fasten the pre- and intrahospital pathway to fibrinolysis...

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..Cardiovascular magnetic resonance (CMR) has the potential to detect cardiac involvement by depicting early scar formation that may appear before onset of wall motion abnormalities...

..We review treatment development in motor neuron disease and discuss the strengths and limitations of past as well as upcoming clinical trials...

..A second international conference to improve the guidelines was held in 2009. These second and improved guidelines are dedicated to the memory of Sean F. Scott...

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..Our data raises the question whether the skin may function as a biomarker for specific aspects of disease pathology in ALS...

..However, the present study suggest that dopamine substitution is much less effective in improving oculomotor performance than it is in restoring skeletomotor abilities...

..We aimed to test whether soluble fragments of beta-amyloid precursor protein (sAPPα and sAPPß) correlated with clinical subtypes of ALS and were of prognostic value...

..The objective of the present study was to optimize technical approaches for this preservation of quantitative and directional information during spatial normalization in data analyses at the group level...

..We aimed to identify a characteristic CSF protein pattern that could provide new candidate biomarkers of disease progression in ALS...

..It has recently been shown that the underlying DNA repair defect plays a central role in the aging process. In addition to skin symptoms, various premature neurological abnormalities have been reported...

..Future studies should investigate if the involvement of the amygdala is due to TS or rather caused by the genetically-linked most frequent comorbidity ADHD...

..In a recently proposed classification, LMND were subdivided according to the clinical disease pattern and time course. This study was performed to investigate the clinical practicability of the classification...

..In summary, substantial neuroanatomical information can be gained for deep subcortical gray matter structures such as the thalamus with an improved detection and directional differentiation of voxel-specific tensors...

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..The differences in pursuit tracking between PD and MSA were large enough to warrant their use as ancillary diagnostic criteria for the distinction between these disorders...

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..For potential future developments in MRI diagnostics in MND, a (perhaps multiparametric) ROI-based approach should include CST and the CC motor segment...

..It may be possible to select sensitive items to create a simplified version of the UHDRS, which would be more efficient and more sensitive for the assessment of disease progression in clinical trials and natural history studies...

..Extensive elevation of routine parameters is not characteristic and should encourage a re-evaluation of the clinical diagnosis...

..Deep brain stimulation (DBS) is increasingly used for the treatment of parkinsonian motor symptoms, but the excellent efficacy of DBS contrasts with a growing number of reports that the treatment may result in behavioural complications...

..Future advanced MRI-based investigations might help to provide a fingerprint-identification of MNDs...

..Our findings of disproportional amygdalar atrophy only in cHSP substantiate the association of morphologically assessable cerebral degeneration with cognitive impairment in cHSP...

..In conclusion, this preliminary data show that changes in CSF tau and Abeta1-42 may indicate different protein processing in PSP patients and might, therefore, be relevant in the differentiation of PSP subtypes...

..Further evaluation of the candidate proteins identified here is necessary...

..It could be demonstrated that the topography of cerebral volume changes differed markedly in pHSP or cHSP at group level. Corpus callosum thinning seems to be a general feature of cHSP...

..In this workshop, clinical and preclinical researchers established in the field of ALS/MND met in Holland in March 2006 in order to establish guidelines for the community for drug testing in mouse models...

..in order to obtain detailed information on disease-associated changes in the integrity of cerebral white matter (WM), complementary image analysis (CIA) was applied to patients with amyotrophic lateral sclerosis (ALS) and controls...

..Cortical networks underlying motor imagery are functionally close to motor performance networks and can be activated by patients with severe motor disabilities...

..The reduction of activity in extrastriate visual areas might be similarly interpreted. The increased brain response in the right supramarginal area of ALS patients might represent an altered sensitivity to social-emotional cues...

..CSF NfH(SMI35) may therefore be of some value for the laboratory-supported differential diagnosis of atypical parkinsonian syndromes...

..Information on anatomical connectivity in the brain by measurements of the diffusion of water in white matter tracts lead to quantification of local tract directionality and integrity...

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..In this review, an updated overview is given on CSF biomarkers related to the pathomechanisms supposed to be participating in the complex disease process of ALS...

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..Our study supports a role for dynein dysfunction in the pathogenesis of neurodegenerative disorders of the basal ganglia, such as Perry syndrome and HD...

..We conclude impaired selective attention reflects a subtle variant of frontotemporal dementia frequently observed in ALS patients at a relatively early stage of the disease...

..Finally, our data suggest that MRI does not only resemble the findings of behavioural tests, but is potentially superior to behavioural studies...

..The L144F mutation, which is the most prevalent mutation in the Balkan countries, and the D90A mutation which is the most frequent SOD1 mutation globally, seem to be the second most common disease-causing mutations in Germany...

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..In recent years, bone marrow (BM)-derived mesenchymal stromal cells (MSC) have become a promising source for neuroregenerative therapies. We evaluated the trophic effects of neuroectodermally converted MSC (mNSC) on neural stem cells (NSC)...

..In ALS, an important underlying mechanism might be the influence of the arterial pCO(2) which may alter the CSF flow...

..Similar symptoms are known in patients with parkinsonian syndromes, but their pathophysiology remains unclear. One hypothesis is a relation between the movement disorder and the skeletal muscle pathology...

..The unique aspect of this case is the muscle weakness and motor findings on electrophysiological testing in what is suggested by the literature to be a pure sensory neuronopathy...

..VBM might be a potential tool to visualize disease progression in future longitudinal studies...

..Moreover, the association of the GVD stages with those of AD-related pathology but not with other neurodegenerative disorders points to a possible role of GVD and the response to chronic stress in the pathogenesis of AD...

..Our findings indicate a general upregulation of MMP-9 in ALS. MMP-9 seems to play a role in both neurodegeneration and skin changes in ALS and could thus be a common factor linking otherwise distant aspects of disease pathology...

..We aimed to evaluate the relevance of MRZR to predict conversion of patients with clinically isolated syndrome (CIS) to MS, and to compare it to oligoclonal bands (OCB) and MRI...

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..The causes underlying idiopathic intracranial hypertension (IIH) are poorly understood...

..Therefore, we conclude that the combination of rasagiline and riluzole is a promising clinical combination for the improvement of current neuroprotective treatment strategies of ALS...

..These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging...

..This novel model is particularly suited to decipher the underlying mechanisms and to develop hopefully novel connective tissue-specific anti-aging strategies...

..Structural and functional imaging techniques were combined to investigate sensory system function in amyotrophic lateral sclerosis (ALS)...

..Here, the technical state-of-the-art and the results of VBM and DTI studies in MND as the current state are reviewed, and future perspectives for further neuroimaging applications are highlighted...

..We review recent advances in CSF biomarkers research in CND and thereby focus on markers associated with neurodegeneration...

..The establishment of age-related reference values in a large cohort of controls will improve the interpretation of future CSF EPO evaluations in neurological diseases...

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..Overall, in terminally ill patients the psychological response to the prognosis is not associated with neurobiological changes (e.g., associated with subclinical deficits in ALS) or with physical decline...

..A better understanding of genetic influences on biomarkers like CSF Aβ42 might help to stratify patients and develop specific treatment strategies...

..The aim of this study was to investigate possible alterations of amygdala volumes in vivo...

..Low concentrations of CSF EPO seem to point towards a rapid progression of disease that may be associated with a poorer prognosis...

..The pathophysiological role of these proteins remains to be clarified by further studies...

..However, in some of these patients ultrastructural connective tissue changes can be detected. An overview of connective tissue disorders associated with dissections of the carotid or vertebral arteries is presented...

..The techniques presented in this study are likely to be of use in obesity-related investigations, as well as in the examination of longitudinal changes in weight during various medical conditions...

..This is likely to add to the understanding of motor neuron disease and may be relevant in terms of future therapeutic approaches...

..Our results demonstrate that hypoxia facilitates alternative splicing of EAAT2 in the APP23 model. This may be a molecular mechanism linking vascular factors to early pathophysiology of Alzheimer's disease...

..There was no association of the A0/A0 genotype with the age and site of disease onset or the presence of dementia. The studied tau genotype may contribute to the multifactorial genetic background of ALS...

..To assess the effect of a single levodopa dose (200 mg levodopa, 50 mg carbidopa=sdLD) on cortical and subcortical motor-circuit activation during bimanual grip force in patients with Parkinson's disease (PD)...

..We conclude that apparently pure HSP may present with MRI features of dysplastic CC. This finding extended the spastin-related phenotype which is distinct from previous reports of thin CC in HSP...

..1. The breakpoints are assigned to sequences flanked by the markers ATA1H06, D18S462, D21S1915, and D21S1898. These critical regions may contain susceptibility loci for FTD associated with ALS...

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..Elevation of the CSF/serum albumin quotient occurred in 20% of the cases most likely representing a non-specific finding in ALS...

..At the thoracic level, KD patients had significant upper cord atrophy as compared with controls and LMND. Marked atrophy of the upper spinal cord seems to be a feature of the KD-associated central-peripheral distal axonopathy...

..It can also prolong tracheostomy-free survival...

..These findings contradict assumptions of a generally negative impact of the disease on the emotional disposition and may indicate compensatory cognitive or neuroplastic changes...

..Overall, high-resolution (1)H-MRS allows a sensitive spatial and temporal metabolite profiling in the presymptomatic phase of ALS even before significant neuronal cell loss occurs...

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..Here, we demonstrate that in G93A-SOD1 mice the MRI signal intensities of nucleus V, VII, XII, and nucleus ambiguus show a time-dependent increase starting around day 90, parallel to first behavioral signs of a motoneuron disorder...

..It can be concluded that the SOD1 G93A mutation causes early alterations of tau expression in cns, which are not exclusively restricted to the upper and lower motor neuron...

..While both T2 and ADC might prove useful as progression markers and enable the longitudinal non-invasive evaluation of ALS in G93A-SOD1 mice, the potential is limited by age-dependent effects in case of ADC mapping...

..The decrease in 2N expression resulted in lower levels of total tau mRNA and tau protein. No changes in tau expression were observed in spinal cord of studied animals...

..This makes TARDBP, the gene encoding for TDP-43, a candidate for genetic screening in ALS...

..has also been linked with ALS foci in Japan (oral use) and Guam (topical use), with the additional consumption on Guam of food items prepared from Cycas sp. seed or animals that consume cycad seed components...

..INTERPRETATION: Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy...

..We excluded the involvement of the ALS and FTD-linked genes for copper/zinc superoxide dismutase (SOD1) and tau. The R1101K sequence alteration of the DCTN1 gene may predispose subjects to ALS and FTD...

..In conclusion we find no evidence from analyses in erythrocytes that the putative protective factor in recessive families acts by simply downregulating the synthesis or altering the molecular structure or turnover of the mutant enzyme...

..Our results support the view that there are only subtle cognitive deficits in ALS patients and we assume a possible effect of practice on cognitive tasks following reduced daily motor activity...

..Disease progression is more rapid than in the ALS2 phenotype cases described to date. The patient's consanguineous parents carry the mutation in the heterozygous state as do his two unaffected brothers...

..We found that the sequential analysis offered a gain in time of 38%. We conclude that the sequential trial design may in certain situations be superior to the classical design...

..These features resemble the course of sporadic amyotrophic lateral sclerosis (ALS) in humans and suggest that modified function of glutamate AMPA channels may be causally linked to pathogenesis of ALS...

..Additionally, the authors attempted to confirm previous studies that had shown quality of life to be underestimated by partners or caregivers...