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

Rai, Santosh and *, Suresh BV and Bhat, I. G. and *, Mithun Sekhar and Chakraborti, Shrijeet (2013) Childhood adrenoleukodystrophy – Classic and variant – Review of clinical manifestations and magnetic resonance imaging. Journal of Pediatric Neurosciences, 8. pp. 192-197. ISSN 1817-1745

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Abstract

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.

Item Type: Article
Uncontrolled Keywords: Adrenoleukodystrophy, auditory pathway, magnetic resonance imaging
Subjects: Medicine > KMC Mangalore > Pathology
Medicine > KMC Mangalore > Radio Diagnosis and Imaging
Medicine > KMC Mangalore > Medicine
Depositing User: KMCMLR User
Date Deposited: 30 Dec 2013 07:38
Last Modified: 30 Dec 2013 07:38
URI: http://eprints.manipal.edu/id/eprint/138138

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