Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · May 2014
Review Meta AnalysisCan fMRI safely replace the Wada test for preoperative assessment of language lateralisation? A meta-analysis and systematic review.
Recent studies have shown that fMRI (functional magnetic resonance imaging) may be of value for pre-surgical assessment of language lateralisation. The aim of this study was to systematically review and analyse the available literature. A systematic electronic search for studies comparing fMRI with Wada testing was conducted in the PubMed database between March 2009 and November 2011. ⋯ Language production or language comprehension tasks and different regions of interest did not yield statistically significant different results. It can be concluded that fMRI is reliable when there is strong left-lateralised language. The Wada test is warranted when fMRI fails to show clear left-lateralisation.
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J. Neurol. Neurosurg. Psychiatr. · May 2014
ReviewGenetic counselling in ALS: facts, uncertainties and clinical suggestions.
The clinical approach to patients with amyotrophic lateral sclerosis (ALS) has been largely modified by the identification of novel genes, the detection of gene mutations in apparently sporadic patients, and the discovery of the strict genetic and clinical relation between ALS and frontotemporal dementia (FTD). As a consequence, clinicians are increasingly facing the dilemma on how to handle genetic counselling and testing both for ALS patients and their relatives. On the basis of existing literature on genetics of ALS and of other late-onset life-threatening disorders, we propose clinical suggestions to enable neurologists to provide optimal clinical and genetic counselling to patients and families. ⋯ Presently, genetic testing should not be proposed to asymptomatic at-risk subjects, unless they request it or are enrolled in research programmes. Genetic counselling in ALS should take into account the uncertainties about the pathogenicity and penetrance of some genetic mutations; the possible presence of mutations of different genes in the same individual; the poor genotypic/phenotypic correlation in most ALS genes; and the phenotypic pleiotropy of some genes. Though psychological, social and ethical implications of genetic testing are still relatively unexplored in ALS, we recommend multidisciplinary counselling that addresses all relevant issues, including disclosure of tests results to family members and the risk for genetic discrimination.
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J. Neurol. Neurosurg. Psychiatr. · May 2014
Myasthenia in pregnancy: best practice guidelines from a U.K. multispecialty working group.
A national U. K. workshop to discuss practical clinical management issues related to pregnancy in women with myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provided that their myasthenia is under good control before pregnancy, the majority of women can be reassured that it will remain stable throughout pregnancy and the postpartum months; (4) spontaneous vaginal delivery should be the aim and actively encouraged; (5) those with severe myasthenic weakness need careful, multidisciplinary management with prompt access to specialist advice and facilities; (6) newborn babies born to myasthenic mothers are at risk of transient myasthenic weakness, even if the mother's myasthenia is well-controlled, and should have rapid access to neonatal high-dependency support.