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Amyotrophic lateral sclerosis.
Tremendous progress has been made in the field of ALS based on recent neuropathological and genetic discoveries and the role of metabolism and nutrition in the pathogenesis of the disease is debated and may potentially serve as a future therapeutic target.
Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis
To see whether the distribution patterns of phosphorylated 43kDa TAR DNA‐binding protein (pTDP‐43) intraneuronal inclusions in amyotrophic lateral sclerosis (ALS) permit recognition of…
Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients
This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients.
Contribution of cerebrovascular disease in autopsy confirmed neurodegenerative disease cases in the National Alzheimer's Coordinating Centre.
- J. Toledo, S. Arnold, J. Trojanowski
- Medicine, PsychologyBrain : a journal of neurology
- 1 September 2013
Concurrent cerebrovascular disease is a common neuropathological finding in aged subjects with dementia, is more common in Alzheimer's disease than in other neurodegenerative disorders, especially in younger subjects, and lowers the threshold for dementia due to Alzheimer’s disease and α-synucleinopathies, which suggests that these disorders should be targeted by treatments for cerebroVascular disease.
Amyotrophic lateral sclerosis—a model of corticofugal axonal spread
- H. Braak, J. Brettschneider, A. Ludolph, V. Lee, J. Trojanowski, K. Tredici
- BiologyNature Reviews Neurology
- 1 December 2013
It is posits that the trajectory of the spreading pattern is consistent with the induction and dissemination of pTDP-43 pathology chiefly from cortical neuronal projections, via axonal transport, through synaptic contacts to the spinal cord and other regions of the brain.
Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion
The study indicates a pathophysiological link between C9ORF72 expansions and UBQLN proteins in ALS and FTLD-TDP that is associated with alterations in clinical phenotype, and suggests that the presence of C9ORN72 repeat expansions may indicate a worse prognosis in ALS.
Spreading of pathology in neurodegenerative diseases: a focus on human studies
- J. Brettschneider, K. Tredici, V. Lee, J. Trojanowski
- BiologyNature Reviews Neuroscience
- 1 February 2015
Recent evidence that supports the notion of neuron–neuron protein propagation is reviewed, with a focus on neuropathological and positron emission tomography imaging studies in humans.
Axonal damage markers in cerebrospinal fluid are increased in ALS
- J. Brettschneider, A. Petzold, S. Süssmuth, A. Ludolph, H. Tumani
- Biology, MedicineNeurology
- 28 March 2006
It is proposed that axonal damage markers in CSF may discriminate between subtypes of ALS and that they could be used as markers for therapeutic trials.
The chemokine CXCL13 in acute neuroborreliosis
- M. Senel, T. Rupprecht, H. Tumani, H. Pfister, A. Ludolph, J. Brettschneider
- MedicineJournal of Neurology, Neurosurgery & Psychiatry
- 3 December 2009
The study confirms the relevance of CXCL13 as a diagnostic biomarker of NB and suggests that CSF CX CL13 in NB is linked to duration of disease and could be a marker of disease activity and response to antibiotic therapy.
Cognitive decline and reduced survival in C9orf72 expansion frontotemporal degeneration and amyotrophic lateral sclerosis
- D. Irwin, C. McMillan, M. Grossman
- Medicine, PsychologyJournal of Neurology, Neurosurgery & Psychiatry
- 31 October 2012
C9P cases may have a shorter survival in ALS and more rapid rate of cognitive decline related to frontal and parietal disease in FTLD, and C9orf72 genotyping may provide useful prognostic and diagnostic clinical information for patients with ALS and FTLD.