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Annals of Indian Academy of Neurology
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IMAGES IN NEUROLOGY
Year : 2008  |  Volume : 11  |  Issue : 1  |  Page : 56-57
 

Selective tract abnormality in adrenoleukodystrophy: Uncommon MRI finding


Department of Neurology, All India Institute of Medical Sciences, New Delhi - 110 029, India

Date of Submission01-Sep-2007
Date of Decision31-Oct-2007
Date of Acceptance08-Nov-2007

Correspondence Address:
Rohit Bhatia
Department of Neurology, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.40230

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How to cite this article:
Bhatia R, Desai S, Padma M V, Tripathi M, Prasad K. Selective tract abnormality in adrenoleukodystrophy: Uncommon MRI finding. Ann Indian Acad Neurol 2008;11:56-7

How to cite this URL:
Bhatia R, Desai S, Padma M V, Tripathi M, Prasad K. Selective tract abnormality in adrenoleukodystrophy: Uncommon MRI finding. Ann Indian Acad Neurol [serial online] 2008 [cited 2020 Nov 26];11:56-7. Available from: https://www.annalsofian.org/text.asp?2008/11/1/56/40230


A 9-year-old male child presented with progressive spastic pure motor quadriparesis of one year duration without any visual, auditory or extrapyramidal dysfunction. Mild cognitive impairment was present. His hemogram, liver, renal, thyroid and parathyroid profile, serum cortisol and serum ACTH were normal. His MRI brain revealed symmetric T2W hyperintensity along the descending tracts in the genu [Figure - 1]A, the posterior limb of internal capsule and along the brainstem [Figure - 1]B-D. His MRI of spine was normal. Elevated levels of very long chain fatty acids (VLCFA) in serum confirmed the diagnosis of childhood cerebral X-linked adrenoleukodystrophy (ALD).

X-linked ALD is a peroxisomal disorder that affects the white matter of the central nervous system, adrenal cortex and testes and is caused by a deficiency of a single enzyme, acyl-CoA synthesase, that prevents the breakdown of very long chain fatty acids (C > 22:0); which (VLCFA) subsequently accumulate in the tissues and plasma, thereby leading to cerebral demyelination, peripheral nerve abnormalities and adrenocortical and testicular insufficiency. The genetic defect responsible for X-linked ALD is located in Xq28, the terminal segment of the long arm of the X chromosome. Loes et al. described five MR imaging patterns, with their relative frequencies, ages of affected patients and patterns of progression at MR imaging. [1] The MRI in adrenoleukodystrophy classically shows the primary involvement of the deep white matter in the parieto-occipital lobes and involvement of the splenium of the corpus callosum (66% of cases are mainly found in children.), which may include the lesions of the visual and auditory pathways. The involvement of the frontal lobe or genu of the corpus callosum is observed in approximately 15.5% of cases and mainly in adolescents. Primary cerebellar white matter or combined involvement of the parieto-occipital and frontal white matter has also been described. The primary involvement of the frontopontine or corticospinal projection fibers, as seen in our case, is reported in 12% of the cases, mainly in adults. However, it may also be observed with childhood cerebral ALD, as in the present case. [2],[3]

 
   References Top

1.Loes DJ, Fatemi A, Melhem ER, Gupte N, Bezman L, Moser HW, et al. Analysis of MRI patterns aids prediction of progression in X-linked adrenoleukodystrophy. Neurology 2003;61:369-74.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Kim JH, Kim HJ. Childhood X-linked adrenoleukodystrophy: Clinical-pathologic overview and MR imaging manifestations at initial evaluation and follow-up. Radiographics 2005;25:619-31.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Barkovich AJ, Ferriero DM, Bass N, Boyer R. Involvement of the pontomedullary corticospinal tracts: A useful finding in the diagnosis of X-linked adrenoleukodystrophy. AJNR Am J Neuroradiol 1997;18:95-100.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]


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