Rev Neurol France
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Review
Surgical management of Moyamoya disease and syndrome: Current concepts and personal experience.
In this focus, we review, in the light of the recent literature, the modalities and indications of surgical cerebral revascularization for Moyamoya (MM) disease or syndrome. We also report our experience in the surgical management of adult MM. In symptomatic forms, with presence of severe disturbances of perfusion or cerebrovascular reactivity on multimodal imaging work-up, the risks of recurrent ischemic or hemorrhagic stroke is high (respectively 10-13%/yr and 2-7%/yr). ⋯ In conclusion, surgical treatment should be discussed quickly in symptomatic forms of MM (progressive or recurring) because of their poor outcome. Indirect techniques are favored in pediatric patients due to their simplicity and good clinical results. Direct, or preferentially combined techniques would be more effective in adult patients to prevent the recurrence of ischemic or hemorrhagic stroke.
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It is customary to consider that a purely sensory and painful neuropathy accompanied by normal electroneuromyographic examination may be or must be a small fiber neuropathy. This leads to perform specific tests, such as measuring the intra-epidermal nerve fiber density on skin biopsy or neurophysiological tests, such as evoked potentials to noxious stimuli (laser) or quantification of thermal sensory thresholds. However, these tests are only sensitive to the loss of small fibers (A-delta and C), which does not reflect the mechanisms responsible for peripheral neuropathic pain. ⋯ In this context, the clinical and laboratory approach must be improved to better understand the underlying mechanisms. It is imperative to interpret the data provided by laboratory tests and to correlate these data to the clinical signs and symptoms presented by the patients. Thus, one must go beyond many a priori and misinterpretations that unfortunately exist in this area at present and are not based on any solid pathophysiological basis.
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Recent advances in the understanding of Alzheimer's disease pathogenesis have led to the development of numerous compounds that might modify the disease process. Amyloid β peptide represents an important molecular target for intervention in Alzheimer's disease. The main purpose of this work is to review immunotherapy studies in relation to the Alzheimer's disease. ⋯ In both phase III solanezumab and bapineuzumab trials, PET imaging revealed that about a quarter of patients lacked fibrillar amyloid pathology at baseline, suggesting that they did not have Alzheimer's disease in the first place. So a new third phase 3 clinical trial for solanezumab, called Expedition 3, in patients with mild Alzheimer's disease and evidence of amyloid burden has been started. Thus, currently, amyloid intervention is realized at early stage of the Alzheimer's disease in clinical trials, at prodromal Alzheimer's disease, or at asymptomatic subjects or at risk to develop Alzheimer's disease and or at asymptomatic subjects with autosomal dominant mutation.