Journal of the neurological sciences
-
To assess microstructural alterations in white matter (WM) in amyotrophic lateral sclerosis (ALS) using diffusion tensor imaging (DTI). ⋯ WM microstructural alteration is a common pathology in ALS, which can be detected by both intra- and inter-voxel diffusion metrics. The extent of abnormalities in several WM tracts such as ATR and LIFOF may be better assessed through the inter-voxel diffusion measurement.
-
Stiff-person syndrome (SPS), first described in 1956 by Moersch and Woltman, is a progressive autoimmune disorder with core features of chronic fluctuating progressive truncal and limb rigidity and painful muscle spasms leading to gait difficulties, falls and an appearance that resembles tin soldiers. The syndrome is a rare, highly disabling disorder of the central nervous and frequently results in significant disability. Understanding of the etiology, clinical spectrum, diagnostic workup and therapeutic modalities for this painful and disabling disorder has vastly evolved over the past few years with more confidence in classifying and treating the patients. The purpose of this review is to increase the awareness, early detection, and treatment of this disabling disease. ⋯ In this article we will discuss the epidemiology, presentation and classification. Explain the pathophysiology of SPS and the autoimmune mechanisms involved. Discuss the diagnostic approach and treatments available, as well as, the prognosis and outcome.
-
Despite the strong evidence for the efficacy of surgery to treat temporal lobe epilepsy, the number of such surgeries has plateaued in western countries. This study examined trends in the number of epilepsy cases diagnosed, number of surgeries performed, and certain characteristics of surgery patients in western China between January 2009 and December 2017. ⋯ Our analysis suggests that in western China, as in western countries, the number of temporal epilepsy surgeries has plateaued. In contrast, disease-modifying factors have not changed over time in western China. Further studies are needed to explore what may contribute to these findings.
-
Multicenter Study Clinical Trial Observational Study
Late-onset hereditary ATTR V30M amyloidosis with polyneuropathy: Characterization of Brazilian subjects from the THAOS registry.
Despite growing numbers of patients diagnosed with late-onset hereditary ATTR V30M amyloidosis with polyneuropathy (ATTRv-PN), this condition remains poorly characterized in Brazil. ⋯ LO-V30M ATTRv-PN is not unusual in Brazil, tending to be more difficult to diagnose and present with a more severe phenotype, with more large nerve fibers and cardiac involvement than EO-V30M.
-
National institutes of Health Stroke Scale (NIHSS) score and the presence of successful recanalization are crucial determinants of clinical outcome in patients with major artery occlusion. However, it is unknown whether successful recanalization rate after endovascular therapy (EVT) depends on NIHSS score. ⋯ NIHSS score may serve as a predictor of successful recanalization. Recanalization is relatively easier in mild stroke than in those with severe stroke.