Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study

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概要

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

• 社団法人 日本内科学会の論文

著者

• Kira Jun-ichi

  Department Of Neurology Graduate School Of Medical Sciences Kyushu University

• Ikeda Shu-ichi

  Department Of Internal Medicine (neurology And Rheumatology) Shinshu University School Of Medicine

• Araki Nobuo

  Department Of Neurology Keio University School Of Medicine

• Washimi Yukihiko

  Department Of Neurology National Center For Geriatrics And Gerontology

• Ishizawa Keisuke

  Department Of Medical Pharmacology Institute Of Health Biosciences The University Of Tokushima Gradu

• Fukushima Kazuhiro

  Department Of Medicine (neurology And Rheumatology) Shinshu University School Of Medicine

• Yoshida Kunihiro

  Department of Brain Disease Research, Shinshu University School of Medicine, Japan

• Kinoshita Michiaki

  Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan

• Kondo Yasufumi

  Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan

• Hoshi Ken-ichi

  Department of Neurology, Nagano Red-Cross Hospital, Japan

• Yazawa Ikuru

  Laboratory of Research Resources, Research Institute for Longevity Sciences, National Center for Geriatrics and Gerontology, Japan

• Saitoh Banyu

  Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan

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