Abstract
Introduction: Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. Methods: MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. Results: The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), "Face of giant panda" sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P
| Original language | English |
|---|---|
| Pages (from-to) | 613-621 |
| Number of pages | 9 |
| Journal | Neuroradiology |
| Volume | 48 |
| Issue number | 9 |
| DOIs | |
| Publication status | Published - Sep 2006 |
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Keywords
- MRI
- Wilson's disease
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging
- Clinical Neurology
- Radiological and Ultrasound Technology
Cite this
Wilson's disease : Cranial MRI observations and clinical correlation. / Sinha, Sanjib; Taly, A. B.; Ravishankar, S.; Prashanth, L. K.; Venugopal, K. S.; Arunodaya, G. R.; Vasudev, M. K.; Swamy, H. S.
In: Neuroradiology, Vol. 48, No. 9, 09.2006, p. 613-621.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Wilson's disease
T2 - Cranial MRI observations and clinical correlation
AU - Sinha, Sanjib
AU - Taly, A. B.
AU - Ravishankar, S.
AU - Prashanth, L. K.
AU - Venugopal, K. S.
AU - Arunodaya, G. R.
AU - Vasudev, M. K.
AU - Swamy, H. S.
PY - 2006/9
Y1 - 2006/9
N2 - Introduction: Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. Methods: MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. Results: The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), "Face of giant panda" sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P
AB - Introduction: Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. Methods: MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. Results: The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), "Face of giant panda" sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P
KW - MRI
KW - Wilson's disease
UR - http://www.scopus.com/inward/record.url?scp=33748479472&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33748479472&partnerID=8YFLogxK
U2 - 10.1007/s00234-006-0101-4
DO - 10.1007/s00234-006-0101-4
M3 - Article
C2 - 16752136
AN - SCOPUS:33748479472
VL - 48
SP - 613
EP - 621
JO - Neuroradiology
JF - Neuroradiology
SN - 0028-3940
IS - 9
ER -