Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson's disease.
BACKGROUND
Magnetic resonance imaging (MRI) helps in the diagnosis of neurologic Wilson's disease (WD). The literature regarding MR spectroscopy (MRS) and diffusion-weighted imaging (DWI) in WD is limited.


OBJECTIVE
To evaluate the clinical features and neuroimaging findings in drug-naïve neurologic WD and to find correlation between clinical stage and disease duration with different imaging findings.


METHODS
The study subjects included consecutive and follow-up neurologic WD patients attending movement disorder clinic. The initial clinical and MRI features before commencement of chelation therapy were noted. Of 78 patients, 34 underwent DWI study and MRS was done in 38 patients and in 32 control subjects.


RESULTS
Dystonia, dysarthria, tremor, and behavioral abnormality were common presenting features. All patients had MRI abnormality with major affection of basal ganglia. The clinical severity and anatomical extent of MRI abnormalities were positively correlated (P < 0.001; r s = 0.709). Presence of diffusion restriction was inversely related to duration of disease (P < 0.001; r s = 0.760). WD patients had reduced N-acetylaspartate/creatine (Cr) and choline (Cho)/Cr ratio (P < 0.001) as compared with control subjects in MRS study.


CONCLUSIONS
Dystonia, dysarthria and tremor are common neurological features of WD. In this study, MRI abnormalities were positively correlated with disease severity; diffusion restriction was inversely correlated with the duration of the disease process. MRS was also a sensitive tool for diagnosing patient of neurologic WD.
Satyabrata
S
Pulai
Department of Neurology, Bangur Institute of Neurosciences, Kolkata, India.
Atanu
A
Biswas
Arijit
A
Roy
Deb Sankar
DS
Guin
Alak
A
Pandit
Goutam
G
Gangopadhyay
Prabhat Kumar
PK
Ghorai
Sujit
S
Sarkhel
Asit Kumar
AK
Senapati
D000293:Adolescent; D002648:Child; D004421:Dystonia / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D014202:Tremor / Q000175:diagnosis / Q000503:physiopathology; D055815:Young Adult