Expert review of neurotherapeutics
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X-linked adrenoleukodystrophy (X-ALD; OMIM #300100) is caused by defects of the ABCD1 gene on chromosome Xq28, resulting in an impairment of peroxisomal beta-oxidation and the accumulation of saturated very long chain fatty acids (VLCFAs). Primary manifestations occur in the CNS, the adrenal cortex and the testes' Leydig cells. The clinical presentation shows a marked variability which is not explained by the different X-ALD genotypes. ⋯ Hematopoietic stem cell transplantation has been reported to be effective in presymptomatic or early symptomatic CCALD, and may well also be a final therapeutic option in early ACALD patients. Early detection of mutation carriers and timely initiation of therapy is important for the effectiveness of all therapeutic efforts. Gene therapy of endogenous hematopoietic stem cells, pharmacological upregulation of other genes encoding proteins involved in peroxisomal beta-oxidation, reduction of oxidative stress, and possibly lovastatin are candidates for future X-ALD therapies.
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Expert Rev Neurother · Sep 2008
ReviewProinflammatory mediators and migraine pathogenesis: moving towards CGRP as a target for a novel therapeutic class.
Migraine is a complex neurological disorder in which genetic and environmental factors interact. At present, frontline therapies in migraine's acute treatment include the use of NSAIDS and triptans. Restrictions in the use of frontline drugs for migraine treatment and evidence concerning CGRP's key role led research towards new pathways involved in migraine pathophysiology. ⋯ This development can be considered the most important pharmacological breakthrough for migraine treatment since the introduction of sumatriptan in the early 1990s. These results are important since they confirm the current pathophysiological concept of migraine. The future introduction of CGRP antagonists in clinical practice could represent a progress for migraine therapy.
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Expert Rev Neurother · Sep 2008
ReviewLaser-evoked potentials in primary headaches and cranial neuralgias.
Using neurophysiological methods to explore nociceptive pathways may improve knowledge of the functional changes subtending pain processing in the different forms of headache and facial pain. Laser-evoked potentials (LEPs) are a reliable neurophysiological assay for the clinical assessment of pain syndromes. ⋯ In migraine, a normal amplitude of basal LEPs with reduced habituation and altered attentive modulation seems to express a general dysfunction of cortical pain processing, which may also contribute, other than to predispose, to the persistence of migraine. LEPs may be employed in the clinical evaluation of the neurophysiological and psychophysiological aspects of pain in the different forms of headaches and facial pain to improve the therapeutic approach and provide an objective measure of treatment efficacy.