title: Size and shape of the corpus callosum in adult Niemann-Pick type C reflects state and trait illness variables.
abstract: OBJECTIVE
  Variable alterations to the structure of the corpus callosum have been described in adults with NPC, a neurometabolic disorder known to result in both white and gray matter pathology. This study sought to examine the structure of the callosum in a group of adult patients with NPC and compared callosal structure with a group of matched controls, and to relate callosal structure with state and trait illness variables.
  
  
  METHODS
  Nine adult patients with NPC were matched to control subjects (n = 26) on age and sex. The corpus callosum was segmented from the midsagittal section of T1-weighted images on all subjects, and total area, length, bending angle, and mean thickness were calculated. In addition, 39 regional thickness measures were derived by using a previously published method. All measures were compared between groups, and analyzed alongside symptom measures, biochemical parameters, and ocular-motor measures.
  
  
  RESULTS
  The callosal area and mean thickness were significantly reduced in the patient group, and regional thickness differences were greatest in the genu, posterior body, isthmus, and anterior splenium. Global callosal measures correlated significantly with duration of illness and symptom score, and at trend level with degree of filipin staining. Measures of reflexive saccadic peak velocity and gain, and self-paced saccades, correlated strongly with total callosal area.
  
  
  CONCLUSIONS
  Callosal structure and size reflect both state and trait markers in adult NPC, and they may be useful biomarkers to index both white and gray matter changes that reflect illness severity and progression.
authors: Melbourne Neuropsychiatry Centre, University of Melbourne, Melbourne, Australia. mark.walterfang@mh.org.au
authors: M
authors: M
authors: Walterfang
authors: M
authors: M
authors: Fahey
authors: L
authors: L
authors: Abel
authors: M
authors: M
authors: Fietz
authors: A
authors: A
authors: Wood
authors: E
authors: E
authors: Bowman
authors: D
authors: D
authors: Reutens
authors: D
authors: D
authors: Velakoulis
references: Br J Psychiatry. 2008 Jun;192(6):429-34
references: 18515892
references: Neurochem Res. 1999 Apr;24(4):481-9
references: 10227680
references: Mol Genet Metab. 2010 Mar;99(3):291-5
references: 19939718
references: Psychiatry Res. 2010 Jul 30;183(1):30-7
references: 20605421
references: J Comp Neurol. 2001 May 7;433(3):415-25
references: 11298365
references: Brain. 2007 Jan;130(Pt 1):120-33
references: 17003072
references: J Neurosci Res. 2008 Sep;86(12):2802-7
references: 18512758
references: J Neurol Neurosurg Psychiatry. 2005 Apr;76(4):592-5
references: 15774455
references: Exp Brain Res. 2001 Nov;141(2):218-31
references: 11713633
references: Neurology. 2009 Mar 24;72(12):1083-6
references: 19307542
references: Neurology. 1998 Nov;51(5):1381-5
references: 9818864
references: Arch Neurol. 1998 Feb;55(2):193-8
references: 9482361
references: J Inherit Metab Dis. 2009 Dec;32 Suppl 1:S221-6
references: 19609713
references: J Neurol. 2004 Apr;251(4):398-406
references: 15083283
references: Aust N Z J Psychiatry. 2009 Sep;43(9):838-45
references: 19670057
references: Clin Genet. 2003 Oct;64(4):269-81
references: 12974729
references: Mol Genet Metab. 2009 Feb;96(2):55-8
references: 19013089
references: J Neurophysiol. 1998 Jan;79(1):64-72
references: 9425177
references: J Neuropsychiatry Clin Neurosci. 2006 Spring;18(2):158-70
references: 16720792
references: Neuroscience. 2002;109(3):437-50
references: 11823057
references: Brain. 2008 Jan;131(Pt 1):196-204
references: 18056161
references: Neuroradiology. 1994;36(1):20-2
references: 8107989
references: Cereb Cortex. 2007 Oct;17(10):2276-82
references: 17164468
references: Biochim Biophys Acta. 2004 Oct 11;1685(1-3):48-62
references: 15465426
references: Pediatr Neurol. 2005 Nov;33(5):325-30
references: 16243219
references: J Cereb Blood Flow Metab. 1996 Jan;16(1):7-22
references: 8530558
references: J Neurosci Res. 1997 Aug 1;49(3):389-92
references: 9260750
references: Lancet Neurol. 2007 Sep;6(9):765-72
references: 17689147
references: J Comp Neurol. 2003 Feb 10;456(3):279-91
references: 12528192
references: J Neuropathol Exp Neurol. 2004 Jun;63(6):660-73
references: 15217094
references: Ann Neurol. 1987 Feb;21(2):138-48
references: 3827222
references: J Neurosci Res. 2005 Dec 15;82(6):811-21
references: 16273542
references: Neurology. 2010 Jul 6;75(1):49-56
references: 20484681
references: Biochim Biophys Acta. 2004 Oct 11;1685(1-3):63-76
references: 15465427
references: Schizophr Res. 2008 Aug;103(1-3):1-10
references: 18562178
references: J Neurol Neurosurg Psychiatry. 2006 Aug;77(8):997-8
references: 16844962
references: J Neurol Neurosurg Psychiatry. 1997 Oct;63(4):552
references: 9343150
references: Neuroimage. 2002 Sep;17(1):479-89
references: 12482100
references: Acta Neuropathol. 1997 Aug;94(2):164-72
references: 9255392
references: J Neurol Sci. 2006 Nov 1;249(1):1-6
references: 16814322
references: J Neurol Neurosurg Psychiatry. 2003 Apr;74(4):528-9
references: 12640083
references: J Neurol Neurosurg Psychiatry. 1998 Jul;65(1):72-9
references: 9667565
references: J Neuropathol Exp Neurol. 2001 Jan;60(1):49-64
references: 11202175
references: Psychiatry Res. 2009 Jul 15;173(1):77-82
references: 19447585