Revista de neurologia
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To present a review of the neurological manifestations, diagnosis and treatment of Fabry disease. ⋯ Fabry disease must be included in the differential diagnosis of stroke in young people, particularly in those with criptogenic etiology, vertebrobasilar location and renal dysfunction. The diagnosis has important therapeutic implications since it is available a replacement therapy with agalsidase, a recombinant enzyme which has been effective in lipid tissue clearance and clinical improvement in Fabry patients.
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Revista de neurologia · Nov 2006
Review[What can we expect of Collaborative Review Groups of Cochrane Collaboration in neuropaediatrics?].
Cochrane Collaboration (CC) contains detailed, critical and up-to-date systematic reviews (SR) of the best scientific evidence available. ⋯ Paediatric neurology SR are infrequent (3.6% of the 2.231 SR published in CC), and helps an evidence-based decision-making in a few areas: pharmacologic treatment of epilepsy, management of intraventricular haemorrhage of preterm infants and bacterial meningitis. Many therapies in paediatric neurology persist with no supporting evidence, and we detected no SR about important neurological issues in childhood as attention-deficit hyperactivity disorder, mental retardation and hypotonia.
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Revista de neurologia · Oct 2006
Review Case Reports[Hypertrophic cranial and spinal pachymeningitis. A description of four new cases and a review of the literature].
Hypertrophic pachymeningitis is an infrequent disease that is characterised by inflammatory hypertrophy of the dura mater. There are cranial, spinal and craniospinal forms of the disease. They may be due to underlying infectious, autoimmune or neoplastic processes, although most of the cases reported in recent years have no base pathology and are known as idiopathic hypertrophic pachymeningitis. The ideal treatment is unknown, but most cases usually respond well to therapy with corticoids. ⋯ On observing pachymeningitis in neuroimaging tests it becomes necessary to conduct a comprehensive aetiological study in search of infectious, autoimmune and neoplastic diseases. In the cranial forms there may be parenchymatous oedema, which would explain the epileptic seizures and the cognitive deterioration of our patients. Response to corticoids is spectacular, although it is often necessary to continue to administer them for prolonged periods of time.
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Revista de neurologia · Jun 2006
Review[Acquired neuromuscular syndrome in critically ill patients].
To analyse the causes and signs of the muscle weakness that critically ill patients develop during their stay in the ICU and to review the literature. ⋯ On many occasions 'polyneuropathy of critically ill patients' and 'acute myopathy in intensive care' can be differentiated and to do so neurophysiological and, if necessary, muscle biopsy studies are a valuable aid. Although some authors prefer to group these conditions under the name 'polyneuromyopathy', we propose the general term 'acquired neuromuscular syndrome in critically ill patients' -a more descriptive expression that does not presuppose a particular mechanism or a single aetiology.