A Case of ADEM Mimicking Cerebral Adrenoleukodystrophy Based on Supratentorial MRI Findings

Abstract

A 9-year-old male admitted for syncope also had the complains of pain and numbness in his legs and frequent falling down. There was a history of upper respiratory tract infection 10 days before. On neurologic examination, paraparesia and fall a sleep were identified. On magnetic resonance imaging, the symetric signal increases were seen in biparieto-occipital white matter intented to corpus callosum at T2-weighted sequences and cytotoxic edema was seen at diffusion-weighted images. Heterogeneous contrast enhancement was seen on these areas. In addition, at the C7-Th5 vertebrae levels, spinal cord had diffuse increased signal intensity and contrast enhancement. Acute disseminated encephalomyelitis was thought based on clinical and radiological findings. Steroid therapy was started. Significant improvement was shown after treatment. On 2-year follow-up, there was no recurrence. In conclusion, it must be kept in mind that acute disseminated encephalomyelitis can rarely present with biparieto-occipital involvement which extends to corpus callosum and can mimic adrenoleukodystrophy. For the differential diagnosis butterfly glioma, tumefactive demyelinating lesions or multiple sclerosis should be considered.

Keywords: Acute disseminated encephalomyelitis; Adrenoleukodystrophy; Leukodystrophy; MRI; Tumefactive demyelinating lesion.

Figure 1

Bilateral and symmetrical signal increases in the parieto-occipital white matter on the axial FLAIR (A) and axial T2-weighted (B) images. The lesion also includes the isthmus and splenium of the corpus callosum on sagittal T2-weighted images. The lesion area is mildly hypointense on axial T1-weighted image (D). Diffuse, mildly heterogeneous contrast enhancement is observed following intravenous injection of contrast material on axial (E) and sagittal (F) T1-weighted images.

Figure 2

Increased signal intensity on b: 1000 images secondary to diffusion restriction on DWI (A), decreased signal on ADC images (B)

Figure 3

Diffuse signal increase and edema continuing from the level of the 7^th cervical vertebrae to the level of the 5^th thoracal vertebrae in the spinal cord on sagittal T2-weighed image (A). Patchy contrast enhancement is observed in these areas following intravenous injection of contrast material on the sagittal image (b).

Figure 4

Marked improvement is observed in the lesion areas on axial FLAIR (A) and sagittal T2-wieghted (B) images.