Current opinion in neurology
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Curr. Opin. Neurol. · Dec 2009
ReviewPseudoprogression and pseudoresponse in the treatment of gliomas.
Treatment response of brain tumours is typically evaluated with gadolinium-enhanced MRI using the Macdonald criteria. These criteria depend on changes in the area of enhancement. However, gadolinium enhancement of brain tumours primarily reflects impairment of the blood-brain barrier. ⋯ Both pseudo-phenomenona confuse the assessment of outcome of brain tumours in clinical practice and in clinical trials. To overcome these issues, alternative endpoints and response criteria are being developed by an international working party [response assessment in neuro-oncology (RANO)]. It is as yet unclear to what extent alternative imaging tools (positron emission tomography and MRI techniques) provide more reliable indicators of outcome.
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This review briefly discusses the definition and clinical presentation of neuropathic pain and highlights recent advances in the treatment of neuropathic pain. ⋯ Treating neuropathic pain remains a great challenge, and the treatment has to be individualized to the single patient, taking into account side effects, pain type, comorbidities, and drug interactions.
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Curr. Opin. Neurol. · Oct 2009
ReviewEmerging genetic therapies to treat Duchenne muscular dystrophy.
Duchenne muscular dystrophy is a progressive muscle degenerative disease caused by dystrophin mutations. The purpose of this review is to highlight two emerging therapies designed to repair the primary genetic defect, called 'exon skipping' and 'nonsense codon suppression'. ⋯ These novel therapies that act to correct the primary genetic defect of dystrophin deficiency are among the first generation of therapies tailored to correct specific mutations in humans. Thus, they represent paradigm forming approaches to personalized medicine with the potential to lead to life changing treatment for those affected by Duchenne muscular dystrophy.
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The term 'cerebellar ataxias' encompasses the various cerebellar disorders encountered during daily practice. Patients exhibit a cerebellar syndrome and can also present with pigmentary retinopathy, extrapyramidal movement disorders, pyramidal signs, cortical symptoms (seizures, cognitive impairment/behavioural symptoms), and peripheral neuropathy. The clinical diagnosis of subtypes of ataxias is complicated by the salient overlap of the phenotypes between genetic subtypes. The identification of the causative mutations of many hereditary ataxias and the development of relevant animal models bring hope for effective therapies in neurodegenerative ataxias. ⋯ Recent molecular advances have direct implications for research and daily practice. We provide a framework for the diagnosis of ataxias. For the first time, the therapeutic agents under investigation are targeted to deleterious pathways.
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Curr. Opin. Neurol. · Jun 2009
ReviewNeuroimaging in trigeminal autonomic cephalgias: when, how, and of what?
Trigeminal autonomic cephalgias (TACs) are characterized by frequent, short-lasting headache attacks with ipsilateral facial autonomic features. They include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. The pathogenesis of TACs is largely unknown, but many case reports in the literature suggest that TACs are secondary to structural lesions. Thus, the question arises whether TAC patients should undergo neuroimaging. Here, we review the recent literature on secondary TACs and attempt to formulate guidelines for neuroimaging. ⋯ Even clinically typical TACs can be caused by structural lesions. There are no 'typical' warning signs or symptoms. Neuroimaging should be considered in all patients with TAC or TAC-like syndromes, notably in those with atypical presentation. Depending on the degree of suspicion, additional imaging should be considered assessing intracranial and cervical vasculature, and the sellar and paranasal region.