Journal of neurology
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Journal of neurology · Feb 2014
Regional alterations in cortical thickness and white matter integrity in amyotrophic lateral sclerosis.
Previous neuroimaging studies have revealed that both gray matter (GM) and white matter (WM) are altered in several morphological aspects in amyotrophic lateral sclerosis (ALS). However, the relations between GM and WM measures and their contributions to clinical features remain in doubt. In this study, we acquired high-resolution diffusion tensor imaging along with structural magnetic resonance imaging data on 20 patients with clinical evidence of ALS and 21 matched healthy controls. ⋯ Finally, patients with faster clinical progression showed more severe cortical thinning of the left precentral gyrus and FA reduction of the left CST. Together, these findings suggest that ALS is multisystem degeneration involving both the widespread cortices and the underlying WM fibers. GM and WM changes might play distinct roles in the disease progression.
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Journal of neurology · Jan 2014
ReviewUpdate on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS).
Neuromyelitis optica (NMO, Devic's syndrome), long considered a clinical variant of multiple sclerosis, is now regarded as a distinct disease entity. Major progress has been made in the diagnosis and treatment of NMO since aquaporin-4 antibodies (AQP4-Ab; also termed NMO-IgG) were first described in 2004. In this review, the Neuromyelitis Optica Study Group (NEMOS) summarizes recently obtained knowledge on NMO and highlights new developments in its diagnosis and treatment, based on current guidelines, the published literature and expert discussion at regular NEMOS meetings. ⋯ Azathioprine and rituximab are suggested as first-line treatments, the latter being increasingly regarded as an established therapy with long-term efficacy and an acceptable safety profile in NMO patients. Other immunosuppressive drugs, such as methotrexate, mycophenolate mofetil and mitoxantrone, are recommended as second-line treatments. Promising new therapies are emerging in the form of anti-IL6 receptor, anti-complement or anti-AQP4-Ab biologicals.
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Journal of neurology · Jan 2014
Randomized Controlled TrialThe cost-effectiveness of deep brain stimulation in combination with best medical therapy, versus best medical therapy alone, in advanced Parkinson's disease.
Parkinson's disease (PD) is a complex progressive movement disorder leading to motor and non-motor symptoms that become increasingly debilitating as the disease advances, considerably reducing quality of life. Advanced treatment options include deep brain stimulation (DBS). While clinical effectiveness of DBS has been demonstrated in a number of randomised controlled trials (RCT), evidence on cost-effectiveness is limited. ⋯ Total discounted costs in the DBS and BMT groups over 5 years were £68,970 and £48,243, respectively, with QALYs of 2.21 and 1.21, giving an incremental cost-effectiveness ratio of £20,678 per QALY gained. Utility weights in each health state and costs of on-going medication appear to be the key drivers of uncertainty in the model. The results suggest that DBS is a cost-effective intervention in patients with advanced PD who are eligible for surgery, providing good value for money to health care payers.
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Journal of neurology · Jan 2014
Distributed corpus callosum involvement in amyotrophic lateral sclerosis: a deterministic tractography study using q-ball imaging.
Diffusion tensor imaging (DTI) has become a useful tool for investigating early white matter (WM) abnormalities in motor neuron disease. Furthermore, fiber tracking packages that apply multi-tensorial algorithms, such as q-ball imaging (QBI), have been proposed as alternative approaches to overcome DTI limitations in depicting fiber tracts with different orientations within the same voxel. We explored motor and extra-motor WM tract abnormalities in phenotypically heterogeneous amyotrophic lateral sclerosis (ALS) cases aiming to establish a consistent QBI-based WM signature of disease. ⋯ ALS patients showed significantly decreased fiber density and volume, and increased tract length in all regions of CC and left CST (p < 0.05, corrected). In CC body, pyramidal impairment was inversely correlated to fiber density (p = 0.01), while in CC splenium, clinical disability (p = 0.01) and progression (p = 0.02) were inversely correlated to tract length. Our findings further suggest that QBI tractography might represent a promising approach for investigating structural alterations in neurodegenerative diseases and confirm that callosal involvement is a consistent feature of most ALS variants, significantly related to both pyramidal dysfunction and disease disability.
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Journal of neurology · Dec 2013
Clinical TrialVestibular rehabilitation outcomes in patients with and without vestibular migraine.
Vestibular rehabilitation programs do appear to play a beneficial role in the treatment of dizziness in patients with vestibular migraine. Anecdotally, however, patients with vestibular migraine may report persistent significant symptoms at the end of a standard treatment period where other non-migrainous patients are accomplishing their treatment goals. Therefore, the objective of this study was to assess the efficacy of vestibular rehabilitation in patients with vestibular migraine compared to patients with vestibular symptoms without migraine. ⋯ The same degree of improvement was observed in the migraine group regardless of medication regime. This study has validated vestibular rehabilitation as an effective treatment in dizzy patients both with and without vestibular migraine where the use of medication did not preclude benefit from therapy. However, further research is required to clarify the role of specific vestibular suppressant medications and the scheduling of their use in relation to physical therapy.