Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jan 2014
Impact of premorbid hypertension on haemorrhage severity and aneurysm rebleeding risk after subarachnoid haemorrhage.
Arterial hypertension (HTN) is a risk factor for subarachnoid haemorrhage (SAH). We aimed to assess the impact of premorbid HTN on the severity of initial bleeding and the risk of aneurysm rebleeding after SAH. ⋯ Premorbid HTN is associated with increased severity of the initial bleeding event and represents a significant risk factor for aneurysm rebleeding. Given that aneurysm rebleeding is a potentially fatal-but preventable-complication, these findings are of clinical relevance.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2014
Incidence and prevalence of multiple sclerosis in the UK 1990-2010: a descriptive study in the General Practice Research Database.
To estimate the incidence and prevalence of multiple sclerosis (MS) by age and describe secular trends and geographic variations within the UK over the 20-year period between 1990 and 2010 and hence to provide updated information on the impact of MS throughout the UK. ⋯ We estimate that 126 669 people were living with MS in the UK in 2010 (203.4 per 100 000 population) and that 6003 new cases were diagnosed that year (9.64 per 100 000/year). There is an increasing population living longer with MS, which has important implications for resource allocation for MS in the UK.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2014
Onset of secondary progressive phase and long-term evolution of multiple sclerosis.
To assess factors affecting the rate of conversion to secondary progressive (SP) multiple sclerosis (MS) and its subsequent evolution. ⋯ The onset of SP MS is the dominant determinant of long-term prognosis, and its prevention is the most important target measure for treatment. Baseline clinical features of early relapse frequency and age at onset can be used to select groups at higher risk of developing severe disability based on the probability of their disease becoming progressive within a defined time period.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2014
Spreading of amyotrophic lateral sclerosis lesions--multifocal hits and local propagation?
To investigate whether or not the lesions in sporadic amyotrophic lateral sclerosis (ALS) originate from a single focal onset site and spread contiguously by prion-like cell-to-cell propagation in the rostrocaudal direction along the spinal cord, as has been hypothesised (the 'single seed and simple propagation' hypothesis). ⋯ In sporadic ALS, the distribution of lower motoneuron involvement cannot be explained by the 'single seed and simple propagation' hypothesis alone. We propose a 'multifocal hits and local propagation' hypothesis instead.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2014
Clinical characterisation of Becker muscular dystrophy patients predicts favourable outcome in exon-skipping therapy.
Duchenne and Becker muscular dystrophy (DMD/BMD) are both caused by mutations in the DMD gene. Out-of-frame mutations in DMD lead to absence of the dystrophin protein, while in-frame BMD mutations cause production of internally deleted dystrophin. Clinically, patients with DMD loose ambulance around the age of 12, need ventilatory support at their late teens and die in their third or fourth decade due to pulmonary or cardiac failure. BMD has a more variable disease course. The disease course of patients with BMD with specific mutations could be very informative to predict the outcome of the exon-skipping therapy, aiming to restore the reading-frame in patients with DMD. ⋯ This study provides mutation specific data on the course of disease in patients with BMD. It shows that the disease course of patients with BMD, with a mutation equalling a 'skipped' DMD mutation is relatively mild. This finding strongly supports the potential benefit of exon skipping in patients with DMD.