Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Review Meta AnalysisDeep brain stimulation for dystonia.
The few controlled studies that have been carried out have shown that bilateral internal globus pallidum stimulation is a safe and long-term effective treatment for hyperkinetic disorders. However, most recent published data on deep brain stimulation (DBS) for dystonia, applied to different targets and patients, are still mainly from uncontrolled case reports (especially for secondary dystonia). This precludes clear determination of the efficacy of this procedure and the choice of the 'good' target for the 'good' patient. ⋯ In the former group, we review the main results for primary dystonia (generalised/focal) and highlight recent data on myoclonus-dystonia and tardive dystonia (as they share, with primary dystonia, a marked beneficial effect from pallidal stimulation with good risk/benefit ratio). In the latter group, poor or variable results have been obtained for secondary dystonia (with a focus on heredodegenerative and metabolic disorders). From this overview, the main results and limits for each subgroup of patients that may help in the selection of dystonic patients who will benefit from DBS are discussed.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Multicenter StudyCognitive domain deficits in patients with aneurysmal subarachnoid haemorrhage at 1 year.
Cognitive domain deficits can occur after aneurysmal subarachnoid haemorrhage (aSAH) though few studies systemically evaluate its impact on 1-year outcomes. ⋯ In patients with aSAH, cognitive domain deficits worsened functional outcomes at 1 year. Delayed cerebral infarction was an independent risk factor for two or more domain deficits at 1 year.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
ReviewTumefactive demyelination: an approach to diagnosis and management.
Tumefactive lesions are an uncommon manifestation of demyelinating disease and can pose a diagnostic challenge in patients without a pre-existing diagnosis of multiple sclerosis. Choosing when to biopsy a tumefactive lesion to exclude alternative pathology can be difficult. ⋯ This article aims to review the available literature for tumefactive demyelination and to propose an approach to diagnosis and management. We argue that disease modifying therapy should be considered for acute tumefactive demyelinating lesions only once criteria of dissemination in time and space are fulfilled and the diagnosis of multiple sclerosis is confirmed.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
White matter imaging helps dissociate tau from TDP-43 in frontotemporal lobar degeneration.
Frontotemporal lobar degeneration (FTLD) is most commonly associated with TAR-DNA binding protein (TDP-43) or tau pathology at autopsy, but there are no in vivo biomarkers reliably discriminating between sporadic cases. As disease-modifying treatments emerge, it is critical to accurately identify underlying pathology in living patients so that they can be entered into appropriate etiology-directed clinical trials. Patients with tau inclusions (FTLD-TAU) appear to have relatively greater white matter (WM) disease at autopsy than those patients with TDP-43 (FTLD-TDP). In this paper, we investigate the ability of white matter (WM) imaging to help discriminate between FTLD-TAU and FTLD-TDP during life using diffusion tensor imaging (DTI). ⋯ These neuroimaging and neuropathological investigations provide converging evidence for greater WM burden associated with FTLD-TAU, and emphasise the role of WM neuroimaging for in vivo discrimination between FTLD-TAU and FTLD-TDP.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2013
Rapidly progressive scoliosis and respiratory deterioration in Ullrich congenital muscular dystrophy.
To characterise the natural history of Ullrich congenital muscular dystrophy (UCMD). ⋯ The natural history of scoliosis, respiratory function and walking ability in UCMD patients were characterised. Although the age of onset varied, scoliosis, as well as restrictive respiratory dysfunction, progressed rapidly within years, once they appeared.