title: Corpus callosum atrophy in patients with hereditary diffuse leukoencephalopathy with neuroaxonal spheroids: an MRI-based study.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan.
authors.forename: Michiaki
authors.initials: M
authors.lastname: Kinoshita
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Kondo
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yoshida
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Fukushima
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Hoshi
authors.forename: Keisuke
authors.initials: K
authors.lastname: Ishizawa
authors.forename: Nobuo
authors.initials: N
authors.lastname: Araki
authors.forename: Ikuru
authors.initials: I
authors.lastname: Yazawa
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Washimi
authors.forename: Banyu
authors.initials: B
authors.lastname: Saitoh
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Kira
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D000368:Aged; D001284:Atrophy / Q000473:pathology; D003337:Corpus Callosum / Q000473:pathology*; D005260:Female; D006801:Humans; D056784:Leukoencephalopathies / Q000175:diagnosis / Q000453:epidemiology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D012189:Retrospective Studies