Corpus callosum atrophy in patients with hereditary diffuse leukoencephalopathy with neuroaxonal spheroids: an MRI-based study.
作者： Michiaki Kinoshita , Yasufumi Kondo , Kunihiro Yoshida , Kazuhiro Fukushima , Ken-ichi Hoshi
DOI: 10.2169/INTERNALMEDICINE.53.0863
关键词: Central nervous system 、 Splenium 、 Vascular dementia 、 Autopsy 、 Medicine 、 Corpus callosum 、 Hyperintensity 、 Pathology 、 Atrophy 、 White matter
摘要: Objective Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is an adult-onset white matter disease that presents clinically cognitive, mental and motor dysfunction. Several autopsy reports have indicated the corpus callosum (CC), largest bundle of matter, severely affected in patients HDLS. The aim this study was to evaluate atrophy (CCA) quantitatively HDLS patients. Methods We assessed CCA six genetically-proven (HDLS group), comparison observed 20 vascular dementia (VaD group) 24 age-matched without organic central nervous system (CNS) (non-CNS group). Using midsagittal MR images, five measurements CC were obtained: width rostrum (aa'), body (bb') splenium (cc'), anterior posterior length (ab) maximum height (cd). Next, index (CCI) calculated as (aa' + bb' cc')/ab. Results All had lesions frontoparietal lobes on initial MRI scans. Compared VaD non-CNS groups, CCI significantly decreased group (with group, p<0.01; p<0.01). Conclusion This showed significant all scans obtained 6-36 months after onset. propose early appearance CCA, frequently accompanied by high-intensity genu and/or splenium, T2 images important diagnostic clue
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