Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Sep 2012
Comparative StudyNeuropsychological outcome after selective amygdalohippocampectomy: subtemporal versus transsylvian approach.
In the context of discussions on the optimal cognitive outcome of temporal lobe epilepsy surgery, and stimulated by recent reports on the beneficial effects of a selective subtemporal approach to memory function, this study evaluated the cognitive consequences of subtemporal versus transsylvian selective amygdalohippocampectomy (SAH) in patients with mesial temporal lobe epilepsy, taking verbal/figural memory and language functions into account. ⋯ Different from previous optimistic reports, this study demonstrates that subtemporal surgery, such as transsylvian surgery, poses similar risks for verbal memory. Differences between the approaches appear to reflect the effect of different collateral temporal lobe lesions due to the approach. Different cognitive outcomes across studies on the subtemporal approach are discussed as being in part due to study design and the chosen dependent functional measures.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2012
Case definition for progressive multifocal leukoencephalopathy following treatment with monoclonal antibodies.
Novel immunosuppressive/modulating therapies with monoclonal antibodies (MABs) have been associated with progressive multifocal leukoencephalopathy (PML), a potentially fatal disease of the brain caused by the JC virus. Taking the complex diagnostic testing and heterogeneous clinical presentation of PML into account, an agreed case definition for PML is a prerequisite for a thorough assessment of PML. ⋯ The proposed case definition permits data comparability among different medicinal products and among active as well as passive surveillance settings. It may form a basis for meaningful risk analysis and communication for regulators and healthcare professionals.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2012
Adult-onset spinocerebellar ataxia syndromes due to MTATP6 mutations.
Spinocerebellar ataxia syndromes presenting in adulthood have a broad range of causes, and despite extensive investigation remain undiagnosed in up to ∼50% cases. Mutations in the mitochondrially encoded MTATP6 gene typically cause infantile-onset Leigh syndrome and, occasionally, have onset later in childhood. The authors report two families with onset of ataxia in adulthood (with pyramidal dysfunction and/or peripheral neuropathy variably present), who are clinically indistinguishable from other spinocerebellar ataxia patients. ⋯ MTATP6 sequencing should be considered in the workup of undiagnosed ataxia, even if other investigations do not suggest a mitochondrial DNA disorder.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2012
Visuospatial deficits in posterior cortical atrophy: structural and functional correlates.
To determine the neural substrates of biased spatial orienting in posterior cortical atrophy (PCA) by using a combined structural and functional neuroimaging approach. ⋯ The results indicate that rightward bias (sign of left-sided neglect) in PCA depends on dysfunction of a large fronto-parietal network in the right hemisphere, related to both cortical atrophy and decreased cerebral perfusion.
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J. Neurol. Neurosurg. Psychiatr. · Sep 2012
Midcingulate involvement in progressive supranuclear palsy and tau positive frontotemporal dementia.
Progressive supranuclear palsy (PSP) patients often exhibit cognitive decline and behavioural changes during the disease course. In a subset, these symptoms may be the presenting manifestation and can be similar to those in frontotemporal dementia (FTD). However, correlation studies between quantitative imaging measures and detailed neuropsychological assessment are scarce. The aim of this study was to investigate the functional role of affected brain regions in cognition in PSP compared with controls and subsequently examine these regions in FTD patients with known tau pathology (FTD tau). ⋯ The posterior part of the MCC is prominently involved in the neurodegenerative process of PSP, and the severity of its hypoperfusion correlated with the extent of executive dysfunction. In FTD tau, this cognitive domain was associated with anterior MCC involvement. The degree of hypoperfusion in these regions did not differ between PSP and FTD tau. These observations provide insight into the role of the cingulate cortex in cognitive dysfunction in these neurodegenerative disorders and warrant further investigations.