MR spectroscopy in monitoring the treatment of Wilson's disease patients.
The aim of this study was to determine the effectiveness of brain proton magnetic resonance spectroscopy ((1)H-MRS) for monitoring therapy in Wilson's disease (WD) patients. Voxels were located in the globus pallidus (right, left). We followed 17 newly diagnosed WD cases for 1-year period. During this observation period, 6 neurological and 9 hepatic patients improved, while 2 neurological patients deteriorated. The pretreatment (1)H-MRS analysis showed a statistically significant lower level of mI/Cr, NAA/Cr, and higher Lip/Cr in all WD patients with improvement compared with controls. In patients with hepatic signs, a statistically significant increase of mI/Cr and Glx/Cr was observed in the second (1 year posttreatment) (1)H-MRS. In patients with neurological improvement after treatment in the follow-up (1)H-MRS, a statistically significant increase of NAA/Cr was noted. During neurological deterioration, a decrease of Glx/Cr and NAA/Cr was seen, in contrast to another neurologically impaired patient with liver failure exacerbation, where a decrease of mI/Cr and increase of Glx/Cr was observed. The alternations of NAA/Cr ratio in neurologically impaired patients and mI/Cr and Glx/Cr in patients with liver failure could be a sensitive marker of the clinical recovery and deterioration in those WD patients. (1)H-MRS is a technique that can be used for accurate monitoring of treatment efficacy in WD patients.
II Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. tarnacka@ipin.edu.pl
Beata
B
Tarnacka
Wojciech
W
Szeszkowski
Marek
M
Golebiowski
Anna
A
Czlonkowska
D000293:Adolescent; D000328:Adult; D000931:Antidotes / Q000627:therapeutic use; D001224:Aspartic Acid / Q000032:analysis; D001921:Brain / Q000378:metabolism / Q000473:pathology; D016022:Case-Control Studies; D002794:Choline / Q000032:analysis; D003401:Creatine / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D018698:Glutamic Acid / Q000032:analysis; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D011522:Protons; D012720:Severity of Illness Index; D055815:Young Adult
D000931:Antidotes; D011522:Protons; D001224:Aspartic Acid; D018698:Glutamic Acid; D010396:Penicillamine; D003401:Creatine; D002794:Choline