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 NEUROIMAGE
Year : 2013  |  Volume : 8  |  Issue : 3  |  Page : 192-197

Childhood adrenoleukodystrophy - Classic and variant - Review of clinical manifestations and magnetic resonance imaging


1 Department of Radiodiagnosis, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India
2 Department of Neurology, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India
3 Department of Pathology, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India

Correspondence Address:
P V Santosh Rai
Department of Radiodiagnosis, Kasturba Medical College Hospitals, Ambedkar Circle, Kasturba Medical College, Unit of Manipal University, Mangalore - 575 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.123661

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Adrenoleukodystrophy (ALD) is a genetic disease associated with demyelination of the central nervous system, adrenal insufficiency, and accumulation of very long-chain fatty acids in tissue and body fluids. The attempt of this paper is to review the classical and not-so-classical MR imaging manifestations of adrenoleukodystrophy. A review of literature is done to describe the pathophysiology of the disease and the imaging differences between childhood and adult-onset of the disease. The literature is reviewed to explain the link with Addison's disease. In consensus the magnetic resonance imaging (MRI) findings of symmetrical occipital white matter lesions which progress in a rostro-caudal direction is the classical appearance of ALD. Familiarity with the clinico-pathologic manifestations and progressive MR imaging features of childhood cerebral X-linked ALD will be helpful in evaluating the affected patients.






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