Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Sep 2016
Assessment of pulmonary function in amyotrophic lateral sclerosis: when can polygraphy help evaluate the need for non-invasive ventilation?
Non-invasive positive-pressure ventilation (NPPV) is an established, effective, long-term treatment for patients with amyotrophic lateral sclerosis (ALS), but the correct indicators for the establishment of NPPV have not been defined. ⋯ Our study shows that in patients not reporting dyspnoea and having an FVC of >75%, nocturnal hypoventilation was observed in nearly every second patient. Therefore, for the question of whether NPPV should be initiated, polygraphy does not provide useful additional information if the FVC is already <75% as predicted. However, in patients with more or less normal lung function parameters or where lung spirometry cannot perform adequately (eg, bulbar ALS), it can provide sufficient evidence for the need of NPPV.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2016
Randomized Controlled Trial Multicenter StudyADCOMS: a composite clinical outcome for prodromal Alzheimer's disease trials.
Development of new therapies for Alzheimer's disease (AD) is increasingly focused on more mildly affected populations, and requires new assessment and outcome strategies. Patients in early stages of AD have mild cognitive decline and no, or limited, functional impairment. To respond to these assessment challenges, we developed a measurement approach based on established scale items that exhibited change in previous amnestic Mild Cognitive Impairment (aMCI) trials. ⋯ ADCOMS is proposed as new standard outcome for pAD and mild AD dementia trials, and is progressing in a CAMD-sponsored qualification process for use in registration trials of pAD.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2016
ReviewSelective vulnerability in neurodegeneration: insights from clinical variants of Alzheimer's disease.
Selective vulnerability in the nervous system refers to the fact that subpopulations of neurons in different brain systems may be more or less prone to abnormal function or death in response to specific types of pathological states or injury. The concept has been used extensively as a potential way of explaining differences in degeneration patterns and the clinical presentation of different neurodegenerative diseases. ⋯ While cross-disease comparisons can provide insights into the differential vulnerability of networks and neuronal populations, we focus here on what is known about selective vulnerability-related factors that might explain the differential phenotypic expressions of the same disease-in this case, typical and atypical forms of Alzheimer's disease. Whereas considerable progress has been made in this area, much is yet to be elucidated; further studies comparing different phenotypic variants aimed at identifying both vulnerability and resilience factors may provide valuable insights into disease pathogenesis, and suggest novel targets for therapy.