Evaluation of patients with X-linked adrenoleuko-dystrophy using the next generation internet

Jimenez-Sanchez G1,3, Ingeholm ML2, Levine BA2, Eichler F3, Moser HW3

1 Howard Hughes Medical Institute and Institute of Genetic Medicine, Johns Hopkins University, Baltimore MD

2 Georgetown University Hospital, ISIS Center, Washington, DC

3 Kennedy Krieger Institute, Baltimore, MD, USA


Introduction: X-linked adrenoleukodystrophy (X-ALD) affects maily the nervous system white matter, adrenal cortex and testis. It has an estimated frequency of 1:21,000 in the male population. Magnetic resonance imaging (MRI) permits early detection of nervous system damage and is a sensitive indicator of disease progression.

Objective: To develop an MRI transmission network using the new generation internet (NGI), allowing remote “real time” assessment, in order to evaluate novel therapeutic intervention of X-ALD.

Methods: Initial results from an internet-1 based network between Baltimore and Minneapolis has shown the important benefits of electronically transmmitted MRI in the evaluation of patients with X-ALD. Recently, a NGI was developed. This new version offers significant advantages over the previous generation, including better image quality and higher transmission speed of complex MRI and MR spectroscopy images, reducing transmission time to seconds rather than hours. This latter feature makes possible a “real-time” interaction between site of origin and a central reading site, so that image acquisition can be monitored and modified as the image is being produced. 

Results: A pilot study compared 78 data transfer trials using standard Internet to 83 data transfer trials using NGI. The NGI showed significant advantages in the overall, among which, the mean transmission time of the NGI (242.5 secs) showed to be nearly an order of magnitude faster than the mean transmission time of the standard Internet (2062.5 secs).

Conclusion: Our preliminary results suggest that this will be a valuable aid for the evaluation of disease severity and effects of therapeutic interventions in X-ALD and may have application in other disease states.