Annals of neurology
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Patients with the unresponsive wakefulness syndrome (UWS; formerly vegetative state) or in a minimally conscious state (MCS) open their eyes spontaneously but show no (UWS) or only marginal (MCS) signs of awareness. Because these states can become permanent, residual information processing capacities need to be determined, and reliable outcome predictors need to be found. We assessed higher-order cortical information processing in UWS or MCS in a large group of patients using electroencephalographic event-related potentials (ERPs) and determined their long-term prognostic value for recovery. ⋯ Results specify cognitive capabilities in disorders of consciousness, and determine their prognostic value. Specifically the N400 ERP is suggested as an important tool to assess information-processing capacities that can predict the likelihood of recovery of patients in UWS or MCS.
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Annals of neurology · Apr 2013
Randomized Controlled TrialElevated temperature and 6- to 7-year outcome of neonatal encephalopathy.
A study was undertaken to determine whether higher temperature after hypoxia-ischemia is associated with death or intelligence quotient (IQ)<70 at 6 to 7 years among infants treated with intensive care without hypothermia. ⋯ Among noncooled infants of a randomized trial, elevated temperatures during the first postnatal days are associated with increased odds of a worse outcome at 6 to 7 years.
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Annals of neurology · Apr 2013
Multicenter StudyAnti-amyloid β autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies.
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is characterized by vasogenic edema and multiple cortical/subcortical microbleeds, sharing several aspects with the recently defined amyloid-related imaging abnormalities (ARIA) reported in Alzheimer's disease (AD) passive immunization therapies. Herein, we investigated the role of anti-amyloid β (Aβ) autoantibodies in the acute and remission phases of CAA-ri. ⋯ Our data support the hypothesis that the pathogenesis of CAA-ri may be mediated by a selective autoimmune reaction against cerebrovascular Aβ, directly related to autoantibody concentration and soluble Aβ. The CSF dosage of anti-Aβ autoantibodies with the technique here described can thus be proposed as a valid alternative tool for the diagnosis of CAA-ri. Moreover, given the similarities between ARIA developing spontaneously and those observed during immunization trials, anti-Aβ autoantibodies can be considered as novel potential biomarkers in future amyloid-modifying therapies for the treatment of AD and CAA.
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Annals of neurology · Apr 2013
LTBP4 genotype predicts age of ambulatory loss in Duchenne muscular dystrophy.
Duchenne muscular dystrophy (DMD) displays a clinical range that is not fully explained by the primary DMD mutations. Ltbp4, encoding latent transforming growth factor-β binding protein 4, was previously discovered in a genome-wide scan as a modifier of murine muscular dystrophy. We sought to determine whether LTBP4 genotype influenced DMD severity in a large patient cohort. ⋯ LTBP4 haplotype influences age at loss of ambulation, and should be considered in the management of DMD patients.