Giant Axonal Neuropathy: Diffusion-weighted Imaging Features Of The Brain

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J Child Neurol. 2006 Oct;21(10):912-5.

Giant Axonal Neuropathy: Diffusion-weighted Imaging Features Of The

Brain.

Alkan A, Sigirci A, Kutlu R, Doganay S, Erdem G, Yakinci C.

Giant axonal neuropathy is a rare autosomal recessive childhood

disorder characterized by a peripheral neuropathy and features of

central nervous system involvement. Magnetic resonance imaging (MRI)

of an 11-year-old boy with giant axonal neuropathy revealed high

signal intensity in the white matter of the cerebrum and cerebellum

on T(2)-weighted imaging.

An apparent diffusion coefficient map revealed increased apparent

diffusion coefficient values in the periventricular, deep, and

cerebellar white matter, basal ganglia, and thalamus. Increased

apparent diffusion coefficient values in distinct locations suggest

increased mobility of water molecules in the brain of a patient with

giant axonal neuropathy.

This finding could indicate a myelin disorder such as demyelination.

Diffusion-weighted imaging should be performed to reveal apparent

diffusion coefficient changes and determine brain involvement in

patients with giant axonal neuropathy.