Magnetization transfer MRI demonstrates spinal cord abnormalities in adrenomyeloneuropathy.
BACKGROUND
In adrenomyeloneuropathy (AMN) conventional MRI detects only spinal cord atrophy in the late stages.


OBJECTIVE
To apply a magnetization transfer-weighted (MTw) imaging to patients with AMN and AMN-like syndrome in order to visualize and quantitatively assess the pathology of white matter tracts in the cervical spinal cord.


METHODS
MTw studies were conducted in nine men with AMN, eight symptomatic heterozygous women, and 10 age- and sex-matched controls and compared to the Expanded Disability Status Scale (EDSS) and quantitative tests of vibratory sense and postural sway. MTw data sets were obtained at the level of C1 to C3 using a three-dimensional gradient echo acquisition technique, these images were then standardized between subjects by using the in-slice CSF signal as a normalization reference, allowing a quantitative assessment of the MTw signal.


RESULTS
In contrast to conventional MRI, MTw images showed signal hyperintensities in the lateral and dorsal columns of all patients. The MT signal quantified in the dorsal column showed significant differences between patients with AMN, X-linked adrenoleukodystrophy heterozygotes, and controls. MT hyperintensity in the dorsal column correlated with EDSS, vibratory sense, and postural sway.


CONCLUSIONS
Magnetization transfer-weighted imaging is a sensitive modality for the visual and quantitative assessment of spinal cord pathology in adrenomyeloneuropathy, and is a potential tool for evaluation of new therapies.
Fatemi
A
A
Neurogenetics Research Center, Kennedy Krieger Institute, Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
Smith
S A
SA
Dubey
P
P
Zackowski
K M
KM
Bastian
A J
AJ
van Zijl
P C
PC
Moser
H W
HW
Raymond
G V
GV
Golay
X
X
D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology; D000328:Adult; D001284:Atrophy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D002574:Cervical Vertebrae; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology; D011237:Predictive Value of Tests; D020886:Somatosensory Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology* / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology