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

P. V.Santosh Rai, B. V. Suresh, I. G. Bhat, Mithun Sekhar, Shrijeet Chakraborti

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)192-197
Number of pages6
JournalJournal of Pediatric Neurosciences
Volume8
Issue number3
DOIs
Publication statusPublished - 01-09-2013

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Adrenoleukodystrophy
Magnetic Resonance Imaging
Addison Disease
Adrenal Insufficiency
Inborn Genetic Diseases
Body Fluids
Demyelinating Diseases
Consensus
Fatty Acids
Central Nervous System

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Neuroscience(all)

Cite this

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Childhood adrenoleukodystrophy - Classic and variant - Review of clinical manifestations and magnetic resonance imaging. / Rai, P. V.Santosh; Suresh, B. V.; Bhat, I. G.; Sekhar, Mithun; Chakraborti, Shrijeet.

In: Journal of Pediatric Neurosciences, Vol. 8, No. 3, 01.09.2013, p. 192-197.

Research output: Contribution to journalArticle

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