Childhood adrenoleukodystrophy - Classic and variant - Review of clinical manifestations and magnetic resonance imaging.
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.
Department of Radiodiagnosis, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India.
P V
PV
Santosh Rai
Department of Neurology, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India.
B V
BV
Suresh
Department of Neurology, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India.
I G
IG
Bhat
Department of Radiodiagnosis, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India.
Mithun
M
Sekhar
Department of Pathology, Kasturba Medical College Mangalore, Unit of Manipal University, Manipal, Karnataka, India.
Shrijeet
S
Chakraborti
Adrenoleukodystrophy; auditory pathway; magnetic resonance imaging