Annals of neurology
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Annals of neurology · Sep 2014
Comparative StudyIn vivo imaging of spinal cord atrophy in neuroinflammatory diseases.
Spinal cord atrophy is prominent in chronic progressive neurologic diseases such as human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and multiple sclerosis (MS). Here we compared the spinal cord cross-sectional area (SCCSA) in HAM/TSP and MS patients to that of healthy volunteers (HVs). ⋯ These results are in accordance with the findings that whereas over half of all lesions in an MS cord are seen in the upper cervical cord, most of the pathology in HAM/TSP is seen in the thoracolumbar cord, which in turn may be responsible for the more extensive cord atrophy seen in HAM/TSP. An imaging marker such as SCCSA might serve as a surrogate endpoint in clinical trials, especially to assess the neuroprotective impact of various therapies.
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Annals of neurology · Sep 2014
New brain infarcts on magnetic resonance imaging after coronary artery bypass graft surgery: lesion patterns, mechanism, and predictors.
New brain infarcts after coronary artery bypass graft (CABG) are markedly more frequent than clinically evident stroke and have been proposed as a surrogate marker of postprocedural stroke. We sought to investigate the lesion patterns, mechanisms, and predictors of new brain infarction after CABG surgery. ⋯ Post-CABG new brain infarcts are mostly silent and cortically located. Old age, aortic arch atherosclerosis, use of cardiopulmonary bypass, and systemic inflammatory response may contribute to the pathogenesis of post-CABG new brain infarcts.
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Annals of neurology · Sep 2014
Case ReportsTargeted treatment of migrating partial seizures of infancy with quinidine.
Migrating partial seizures of infancy is an early onset epileptic encephalopathy syndrome that is typically resistant to treatment. The most common cause is a gain of function mutation in the potassium channel KCNT1. ⋯ We report the case of a child with migrating partial seizures of infancy secondary to an activating mutation in KCNT1 treated with quinidine. Treatment with quinidine was correlated with a marked reduction in seizure frequency and improved psychomotor development.
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Annals of neurology · Sep 2014
Reduced cerebral gray matter and altered white matter in boys with Duchenne muscular dystrophy.
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle weakness caused by DMD gene mutations leading to absence of the full-length dystrophin protein in muscle. Multiple dystrophin isoforms are expressed in brain, but little is known about their function. DMD is associated with specific learning and behavioral disabilities that are more prominent in patients with mutations in the distal part of the DMD gene, predicted to affect expression of shorter protein isoforms. We used quantitative magnetic resonance (MR) imaging to study brain microstructure in DMD. ⋯ Both gray and white matter is affected in boys with DMD at a whole brain level. Differences between the DMD_Dp140(-) subgroup and controls indicate an important role for the Dp140 dystrophin isoform in cerebral development.