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.