White matter diseases affecting the corpus callosum; demyelinating and metabolic diseases Poster No.: C-0199 Congress: ECR 2011 Type: Educational Exhibit Authors: J. H. Yoo; Seoul/KR Keywords: Neuroradiology brain, Head and neck, CT, MR, Education DOI: 10.1594/ecr2011/C-0199 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 9
Learning objectives To illustrate the MR findings of demyelinating disease and metabolic diseases affecting corpus callosum. Background The corpus callosum is the thickest and strongest fiber bundle tract in the whole body. Moreover, corpus callosum is composed predominantly of myelinated axons, therefore, many white matter involving diseases such as demyelinating and metabolic diseases can affect the corpus callosum. Imaging findings OR Procedure details Multiple Sclerosis (MS) is one of the commonly involving site of the corpus callosum, periventricular white matter, internal capsule, and pons. Callososeptal interface, the inferior aspect of the corpus callosum and septum pellucidum, is the most characteristic sites of MS. MS plaques involving corpus callosum show similar signal findings as the usual MS plaques with nodular high signal intensities on T2-weighted and FLAIR sequences (Fig.1). Enhancement is common in the active stage, and diffusion restriction can be in acute stage. Atrophy of the corpus callosum coexist in the longstanding chronic MS as well as extensive white matter change, which can be a surrogate marker of disease worsening with significant correlation of morphological and functional impairment [1-4] (Fig. 1). Acute disseminated encephalomyelitis (ADEM) is often very similar to the MS in the MR imaging, although clinically monophasic symptom with complete recovery is typical that can be a differential point with MS. It can be helpful in differential diagnosis preferentially deep gray matter and subcortical white matter is more commonly involved in ADEM than periventricular white matter in MS. (Fig.2). Vanishing white matter disease, also called childhood ataxia with diffuse central nervous system hypomyelination, is one of the most prevalent inherited childhood white matter disorders. It is an autosomal recessive inheritance disease related to mutation in any of the five genes (EFI2B1-5) with progressive leukodystrophy with a relapsing-remitting course. The typical findings are extensive cerebral white matter progressive rarefaction Page 2 of 9
and cystic degeneration sparing gray matter. There is no contrast enhancement. [5] (Fig.3). Adrenoleukodystrophy is a rare inherited disorder that breakdown the very long chain fatty acid metabolism resulting in progressive damage the myelin and brain and failure of adrenal gland and eventually death. When the most common X-linked form shows symmetric peri-trigonal white matter, diffuse corpus callosal splenium can be involved with typical marginal contrast enhancement (Fig.4). Mucopolysacharidosis(MPS) is a part of the lysosomal storage disease family caused by the absence of lysosomal enzymes needed to break down glycosaminoglycans (formerly called mucopolysaccharides). Among the 11 enzymes, type II MPS, called Hunter, shows typical brain MR findings involving multiple peirventricular white matter cystic changes known to dilated perivascular spaces not filled with CSF but filled with MPS infiltration. It also can involve the corpus callosum with small multiple but typical round cystic lesions with the same signal intensity of periventricular white matter lesions (Fig. 5). Images for this section: Page 3 of 9
Fig. 1: Multiple sclerosis. Multifocal high signal intenities along the callososeptal interface characteristic sites of multiple sclerosis. And multiple ovoid-shaped white matter high signals in the bilateral parietal region and corpus callosum and midbrain in the different patient. Fig. 2: Acute disseminated encephalitis (ADEM. High signal intensities of corpus callosal genu and bi-frontal symmetrical subcortical white matter high signal intensities on T2- weighted axial image. T1-weighted sagittal scan shows low signal change of genu of corpus callosum with somewhat diffuse thinning corpus callosum. Page 4 of 9
Fig. 3: Vanishing white matter disease. Extensive cystic necrosis of entire corpus callosum with extensive white matter cystic encephalopathy. Page 5 of 9
Fig. 4: CNS involvement of Mucopolysaccharidosis type II (Hunter disease) with extensive white matter changes containing multiple cystic changes and atrophy along the corpus callosum and white matter. Page 6 of 9
Fig. 5: Adrenoleukodystrophy. Bilateral symmetric high signal intensities involving periventricular and subcortical white matter and bilateral posterior thalami and corpus callosal splenium in the T2-weithed axial image. Peripheral marginal enhancement of the affected region including bilateral subcortical white matter is characteristic and helpful finding in differential diagnosis. Page 7 of 9
Fig. 6: Mitochondrial disease. Highi signal intensity of corpuc callosum and bilateral periventricular white matter on the coronal FLAIR image. Also noted, bilateral symmtetric midbrain and pons and medulla. T1-weighted sagittal scan shows diffuse atrophy of corpus callosum and brainstem and medulla. Page 8 of 9
Conclusion White matter diseases such as demyelinating and metabolic diseases can affect the corpus callosum. Personal Information 1. Jeong Hyun Yoo. Department of Radiology. Ewha Womans University. School of Medicine. Seoul. Korea. 2. Jill V. Hunter. Diagnostic Imaging. Texas Childrens' Hospital. Houston. Tx. USA. References 1. Straus Farber R, Devilliers L, Miller A, et al. Differentiating multiple sclerosis from other causes of demyelination using diffusion weighted imaging of the corpus callosum. J Magn Reson Imaging 2009;30:732-736 2. Forrester MB, Coleman L, Kornberg AJ. Multiple sclerosis in childhood: clinical and radiological features. J Child Neurol 2009;24:56-62 3. Balassy C, Bernert G, Wober-Bingol C, et al. Long-term MRI observations of childhoodonset relapsing-remitting multiple sclerosis. Neuropediatrics 2001;32:28-37 4. Pelletier J, Suchet L, Witjas T, et al. A longitudinal study of callosal atrophy and interhemispheric dysfunction in relapsing-remitting multiple sclerosis. Arch Neurol 2001;58:105-111 5. Pineda M, A RP, Baquero M, et al. Vanishing white matter disease associated with progressive macrocephaly. Neuropediatrics 2008;39:29-32 Page 9 of 9