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Identification of the occipito-pontine tract using diffusion-tensor fiber tracking in adult-onset adrenoleukodystrophy with topographic disorientation.

Uchida Y, Kimura E, Hirano T, Nishi S, Maeda Y, Yamashita S, Ueno-Shuto K, Tokutomi N, Kitajima M, Hirai T, Uchino M - Case Rep Neurol (2011)

Bottom Line: Although there are different magnetic resonance (MR) findings which reflect various phenotypes in adrenoleukodystrophy, some cases present with specific symmetrical occipital white-matter lesions.The occipito-pontine tract and lateral lemnisci were clearly detected using diffusion-tensor fiber tracking, suggesting that the topographic disorientation of this patient might be related to the occipito-pontine tract.MR tractography can effectively identify the occipito-pontine tract and may help to localize the fibers associated with clinical symptoms.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Graduate School of Medical Sciences, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

ABSTRACT
X-linked adrenoleukodystrophy is a severe and progressive neurodegenerative disease caused by the peroxisomal transporter ATP-binding cassette, subfamily D, member 1 gene mutations. The defect of this gene product results in accumulation of very-long-chain fatty acids in organs and serum, central demyelination, and peripheral axonopathy. Although there are different magnetic resonance (MR) findings which reflect various phenotypes in adrenoleukodystrophy, some cases present with specific symmetrical occipital white-matter lesions. We describe a patient with adult-onset X-linked adrenoleukodystrophy with topographic disorientation, whose brain MR images revealed T2-signal hyperintensity along the occipito-pontine tract and lateral lemnisci, but not in the cortico-spinal tract in the brainstem. The occipito-pontine tract and lateral lemnisci were clearly detected using diffusion-tensor fiber tracking, suggesting that the topographic disorientation of this patient might be related to the occipito-pontine tract. MR tractography can effectively identify the occipito-pontine tract and may help to localize the fibers associated with clinical symptoms.

No MeSH data available.


Related in: MedlinePlus

a T2-weighted image showing large, hyperintense lesions in the bilateral cerebral white matter (arrow), predominantly in the parietal lobe (asterisk). b FLAIR image showing bilateral, small-spot, hyperintense lesions in the lateral portion of the cerebral peduncle (arrows). c–e DTI images. The hyperintense lesion in b corresponds with the yellow spot (arrowhead), not the red one (arrow), in c. DT fiber-tracking images using the right cerebral peduncle as the starting point showing the occipito-pontine tract and lateral lemnisci as yellow and the cortico-spinal tract as red in the midbrain (d) and internal-capsule (e) levels.
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Figure 1: a T2-weighted image showing large, hyperintense lesions in the bilateral cerebral white matter (arrow), predominantly in the parietal lobe (asterisk). b FLAIR image showing bilateral, small-spot, hyperintense lesions in the lateral portion of the cerebral peduncle (arrows). c–e DTI images. The hyperintense lesion in b corresponds with the yellow spot (arrowhead), not the red one (arrow), in c. DT fiber-tracking images using the right cerebral peduncle as the starting point showing the occipito-pontine tract and lateral lemnisci as yellow and the cortico-spinal tract as red in the midbrain (d) and internal-capsule (e) levels.

Mentions: We report the case of a 46-year-old Japanese man whose wife first realized that he occasionally took the wrong way, got lost near his house, and made mistakes in reading and writing about 18 months prior to our seeing him. Thereafter, he often lost his way home and to the office and, about a year later, got lost within his office building and also made mistakes in reading, writing, and arithmetic. About 6 months later, he visited our outpatient clinic and was admitted to our hospital for investigation. Physical examination revealed no abnormal signs; eye fields and eye pursuit movements were normal in all directions, and a Mini-Mental State Examination score of 24 indicated that his intellectual function was within normal limits. However, he could not draw the room arrangement of his house or the way from home to the office. Laboratory tests were normal, except for elevated serum VLCFA ratios (C26/C22 = 1.318, C25/C22 = 0.034, and C24/C22 = 0.017), biochemically confirming a diagnosis of ALD. Visual evoked potentials were normal. T2-weighted and FLAIR brain MR images showed diffuse hyperintense lesions of the white matter, predominantly in the posterior regions of the parietal and occipital lobes (fig. 1a), and hyperintensities in the bilateral occipito-pontine tracts and lateral lemnisci (fig. 1b), which were seen more clearly using DT fiber tracking (fig. 1c–e).


Identification of the occipito-pontine tract using diffusion-tensor fiber tracking in adult-onset adrenoleukodystrophy with topographic disorientation.

Uchida Y, Kimura E, Hirano T, Nishi S, Maeda Y, Yamashita S, Ueno-Shuto K, Tokutomi N, Kitajima M, Hirai T, Uchino M - Case Rep Neurol (2011)

a T2-weighted image showing large, hyperintense lesions in the bilateral cerebral white matter (arrow), predominantly in the parietal lobe (asterisk). b FLAIR image showing bilateral, small-spot, hyperintense lesions in the lateral portion of the cerebral peduncle (arrows). c–e DTI images. The hyperintense lesion in b corresponds with the yellow spot (arrowhead), not the red one (arrow), in c. DT fiber-tracking images using the right cerebral peduncle as the starting point showing the occipito-pontine tract and lateral lemnisci as yellow and the cortico-spinal tract as red in the midbrain (d) and internal-capsule (e) levels.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3104859&req=5

Figure 1: a T2-weighted image showing large, hyperintense lesions in the bilateral cerebral white matter (arrow), predominantly in the parietal lobe (asterisk). b FLAIR image showing bilateral, small-spot, hyperintense lesions in the lateral portion of the cerebral peduncle (arrows). c–e DTI images. The hyperintense lesion in b corresponds with the yellow spot (arrowhead), not the red one (arrow), in c. DT fiber-tracking images using the right cerebral peduncle as the starting point showing the occipito-pontine tract and lateral lemnisci as yellow and the cortico-spinal tract as red in the midbrain (d) and internal-capsule (e) levels.
Mentions: We report the case of a 46-year-old Japanese man whose wife first realized that he occasionally took the wrong way, got lost near his house, and made mistakes in reading and writing about 18 months prior to our seeing him. Thereafter, he often lost his way home and to the office and, about a year later, got lost within his office building and also made mistakes in reading, writing, and arithmetic. About 6 months later, he visited our outpatient clinic and was admitted to our hospital for investigation. Physical examination revealed no abnormal signs; eye fields and eye pursuit movements were normal in all directions, and a Mini-Mental State Examination score of 24 indicated that his intellectual function was within normal limits. However, he could not draw the room arrangement of his house or the way from home to the office. Laboratory tests were normal, except for elevated serum VLCFA ratios (C26/C22 = 1.318, C25/C22 = 0.034, and C24/C22 = 0.017), biochemically confirming a diagnosis of ALD. Visual evoked potentials were normal. T2-weighted and FLAIR brain MR images showed diffuse hyperintense lesions of the white matter, predominantly in the posterior regions of the parietal and occipital lobes (fig. 1a), and hyperintensities in the bilateral occipito-pontine tracts and lateral lemnisci (fig. 1b), which were seen more clearly using DT fiber tracking (fig. 1c–e).

Bottom Line: Although there are different magnetic resonance (MR) findings which reflect various phenotypes in adrenoleukodystrophy, some cases present with specific symmetrical occipital white-matter lesions.The occipito-pontine tract and lateral lemnisci were clearly detected using diffusion-tensor fiber tracking, suggesting that the topographic disorientation of this patient might be related to the occipito-pontine tract.MR tractography can effectively identify the occipito-pontine tract and may help to localize the fibers associated with clinical symptoms.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Graduate School of Medical Sciences, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

ABSTRACT
X-linked adrenoleukodystrophy is a severe and progressive neurodegenerative disease caused by the peroxisomal transporter ATP-binding cassette, subfamily D, member 1 gene mutations. The defect of this gene product results in accumulation of very-long-chain fatty acids in organs and serum, central demyelination, and peripheral axonopathy. Although there are different magnetic resonance (MR) findings which reflect various phenotypes in adrenoleukodystrophy, some cases present with specific symmetrical occipital white-matter lesions. We describe a patient with adult-onset X-linked adrenoleukodystrophy with topographic disorientation, whose brain MR images revealed T2-signal hyperintensity along the occipito-pontine tract and lateral lemnisci, but not in the cortico-spinal tract in the brainstem. The occipito-pontine tract and lateral lemnisci were clearly detected using diffusion-tensor fiber tracking, suggesting that the topographic disorientation of this patient might be related to the occipito-pontine tract. MR tractography can effectively identify the occipito-pontine tract and may help to localize the fibers associated with clinical symptoms.

No MeSH data available.


Related in: MedlinePlus