Wilson's disease: (31)P and (1)H MR spectroscopy and clinical correlation.
BACKGROUND
Proton ((1)H) magnetic resonance spectroscopy (MRS) changes are noted in Wilson's disease (WD). However, there are no studies regarding membrane phospholipid abnormality using (31)P MRS in these patients. We aimed to analyze the striatal spectroscopic abnormalities using (31)P and (1)H MRS in WD.


METHODS
Forty patients of WD (treated, 29; untreated,11) and 30 controls underwent routine MR image sequences and in vivo 2-D (31)P and (1)H MRS of basal ganglia using an image-selected technique on a 1.5-T MRI scanner. Statistical analysis was done using Student's t test.


RESULTS
The mean durations of illness and treatment were 6.2 ± 7.4 and 4.8 ± 5.9 years, respectively. MRI images were abnormal in all the patients. (1)H MRS revealed statistically significant reduction of N-acetyl aspartate (NAA)/choline (Cho) and NAA/creatine ratios in striatum ((1)H MRS) of treated patients compared to controls. The mean values of phosphomonoesters (PME) (p < 0.0001), phosphodiesters (PDE) (p < 0.0001), and total phosphorus (TPh) (p < 0.0001) were elevated in patients compared to controls. Statistically significant elevated levels of ratio of PME/PDE (p = 0.05) observed in the striatum were noted in treated patients as compared to controls in the (31)P MRS study. The duration of illness correlated well with increased PME/PDE [p < 0.001], PME/TPh [p < 0.05], and PDE/TPh [p < 0.05] and decreased NAA/Cho [p < 0.05] ratios. There was correlation of MRI score and reduced NAA/Cho ratio with disease severity. The PME/PDE ratio (right) was elevated in the treated group [p < 0.001] compared to untreated group.


CONCLUSIONS
There is reduced breakdown and/or increased synthesis of membrane phospholipids and increased neuronal damage in basal ganglia in patients with WD.
Sinha
Sanjib
S
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bangalore, 560029, India. sanjib_sinha2004@yahoo.co.in
Taly
A B
AB
Ravishankar
S
S
Prashanth
L K
LK
Vasudev
M K
MK
D015415:Biomarkers / Q000032:analysis; D001923:Brain Chemistry; D002462:Cell Membrane / Q000737:chemistry*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D008297:Male; D010743:Phospholipids / Q000032:analysis*; D010759:Phosphorus Isotopes; D011522:Protons; D013223:Statistics as Topic
D015415:Biomarkers; D010743:Phospholipids; D010759:Phosphorus Isotopes; D011522:Protons