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White Matter Disorders (white + matter_disorders)
Selected AbstractsWhite matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findingsACTA NEUROLOGICA SCANDINAVICA, Issue 5 2010C. Sundal Sundal C, Ekholm S, Andersen O. White matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findings. Acta Neurol Scand: 2010: 121: 328,337. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Background,,, Leukoencephalopathies are a heterogeneous group of severe encephalopathy syndromes with myelin, axonal or vascular pathology, typically with extensive white matter lesions on MRI T2-FSE and/or -FLAIR sequences. Objectives,,, This review is restricted to leukoencephalopathies with onset in adult age and a dominant inheritance. These diseases are generally severe and often lethal and present with an exacerbating or insidiously progressive course. Material and methods,,, The focus is on four syndromes with pure leukoencephalopathies, however, leukoencephalopathies with associated clinical features are included. Results,,, T2 weighted MR imaging often show features common for leukoencephalopathies, yet shows distinguishing features in transthyretin amyloidosis. Conclusion,,, The diagnosis within the group of leukoencephalopathies thus characterized by MRI relies mainly upon clinical and genetic analysis. The differential diagnosis against treatable leukoencephalopathies is increasingly relevant. [source] Magnetic resonance techniques for the in vivo assessment of multiple sclerosis pathology: Consensus report of the white matter study groupJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 6 2005Massimo Filippi MD Abstract On October 9,11, 2003, the third meeting of the White Matter Study Group of the International Society for Magnetic Resonance in Medicine was held in Venice, Italy. This article is the report of the meeting on how to use MRI in the diagnostic workup of multiple sclerosis (MS) and allied white matter disorders, and to define the nature and the extent of MS pathology in vivo. Both of these steps are central to the design of future treatment strategies aimed at limiting the functional consequences of the most disabling aspects of this disease. J. Magn. Reson. Imaging 2005;21:669,675. © 2005 Wiley-Liss, Inc. [source] A real reason for patients with pseudobulbar affect to smileANNALS OF NEUROLOGY, Issue 2 2007Howard J. Rosen MD Pseudobulbar affect (PBA) is a dramatic disorder of emotional expression and regulation characterized by uncontrollable episodes of laughing and crying that often cause embarrassment, curtailment of social activities, and reduction in quality of life. The disorder occurs in patients with brain injury caused by many types of neurological disease, including stroke, tumors, and neurodegenerative gray and white matter disorders. Although the pathophysiology is unknown, PBA may relate to release of brainstem emotional control centers from regulation by the frontal lobes. Diagnosis of PBA can be difficult and relies on careful characterization of episodes and differentiation from depression. Although there are no US Food and Drug Administration,approved treatments for PBA, several agents have been shown to be effective, including tricyclic antidepressants, selective serotonin reuptake inhibitors, and a new agent containing dextromethorphan and quinidine. The growing number of treatment options, some of great benefit to patients, highlights the importance of accurate diagnosis of this disorder. Ann Neurol 2007 [source] | ||||||||||