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Treatment Monitoring of Brain Creatine Deficiency Syndromes: A 1H- and 31P-MR Spectroscopy Study

M.C. Bianchia,b, M. Tosettib, R. Battinic, V. Leuzzie, M.G. Alessandri’d, C. Carduccif, I. Antonozzif and G. Cionic,d,e,f,g

a Department of Neuroradiology, Santa Chiara Hospital, Pisa, Italy
b Laboratory of Magnetic Resonance, IRCCS Stella Maris, Pisa, Italy
c Department of Developmental Neuroscience, IRCCS Stella Maris, Pisa, Italy
d Laboratory of Neurochemistry, IRCCS Stella Maris, Pisa, Italy
e Department of Child Neurology and Psychiatry, University "La Sapienza," Roma, Italy
f Department of Experimental Medicine and Pathology, University "La Sapienza," Roma, Italy
g Department of Child Neurology and Psychiatry, University of Pisa, Pisa Italy


Figure 1
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  		Fig 1. Metabolic pathway of creatine/phosphocreatine.


Figure 2
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  		Fig 2. Single-voxel proton spectroscopy (1H-MRS) and whole-brain phosphorous spectroscopy (31P-MRS) of patient 2, before (A and B) and after (C and D) 6 months of therapy with Cr at 400 mg/kg/bw/day and guanidinoacetate (GAA)-lowering diet restrictions.

The baseline studies demonstrate, besides a strong reduction of tCr and PCr peaks, an abnormal peak of guanidinoacetate (GAA) at 3.8 ppm on proton spectrum (A), of GAA-phosphate (GAA-P) at –0.5 ppm on phosphorous spectrum (B), and a high level of brain ATP. In contrast, in the on-therapy spectra, the GAA and GAA-P peak are not resolved, ATP turns to normal but there is an incomplete recovery of the tCr and PCr signal intensity.


Figure 3
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  		Fig 3. Total Cr (right axis, empty dot) and PCr (left axis, solid square) modifications recorded in patients 1 (A), 3 (B), and 5 (C) with different Cr daily amounts. Normative data in the bars are presented as mean ± SD.


Figure 4
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  		Fig 4. AGAT-d versus GAMT-d CK reaction rate evaluation. The angular coefficient (b value) of the fitting lines is an index of the CK system efficacy (b [pt 1] = 0.16 and b [pt 5] = 1.04). The CK system is down-regulated in GAMT-d (pt 1) with respect to AGAT-d (pt 5) because PCr remains low even when tCr availability is almost 90% of normal. These measures were recorded when patient 1 was treated only with Cr.





Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X