Korean J Radiol.  2007 Feb;8(1):82-85. 10.3348/kjr.2007.8.1.82.

Diffusion-Weighted MR Imaging of Unusual White Matter Lesion in a Patient with Menkes Disease

• Lee ES
• Ryoo JW
• Choi DS
• Cho JM
• Kwon SH
• Shin HS

• KMID: 753871
• DOI: http://doi.org/10.3348/kjr.2007.8.1.82

Abstract

We report here on the diffusion-weighted imaging of unusual white matter lesions in a case of Menkes disease. On the initial MR imaging, the white matter lesions were localized in the deep periventricular white matter in the absence of diffuse cortical atrophy. The lesion showed diffuse high signal on the diffusion-weighted images and diffuse progression and persistent hyperintensity on the follow up imaging. Our case suggests that the white matter lesion may precede diffuse cortical atrophy in a patient with Menkes disease.

Keyword

Brain; Magnetic Resonance (MR); Menkes disease; White Matter; Diffusion study

MeSH Terms

Menkes Kinky Hair Syndrome/*diagnosis
Male
Infant
Humans
*Diffusion Magnetic Resonance Imaging
Diagnosis, Differential
Brain Diseases/*diagnosis
Atrophy

Figure

• 

  Fig. 1 Initial MR images obtained at age of 10 months. A. T2-wieghted axial image (TR/TE = 4700/104 msec, 5 mm thickness) demonstrates symmetric hyperintense lesion in both deep periventricular white matters (arrows). B. Diffusion weighted image (TR/TE = 3500/92, b maximum = 1000 s/mm2, three directions, 5 mm thickness) shows the bright signal intensity of the lesion. C. On the apparent diffusion coefficient map, the lesion shows diffuse low signal intensities, suggesting restricted diffusion. D. Three dimensional MR angiography shows markedly tortuous intracranial and extracranial vessels, which are characteristics of Menkes disease.

• 

  Fig. 2 Follow up diffusion study obtained at the age of 13 months. A, B. Consecutive diffusion-weighted images (TR/TE = 3500/95, b maximum = 1000 s/mm2) demonstrate marked extension of the white matter lesions to the adjacent white matter. The lesion also involves the corpus callosum (arrows). Note there is no definite progression of cortical atrophy. C, D. Apparent diffusion coefficient maps of the lesion show diffusely decreased apparent diffusion coefficient, similar to that of the initial lesions (Fig. 1C).

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