Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Neuroradiology
Published in: Neuroradiology 9/2006

01-09-2006 | Diagnostic Neuroradiology

Wilson’s disease: cranial MRI observations and clinical correlation

Authors: S. Sinha, A. B. Taly, S. Ravishankar, L. K. Prashanth, K. S. Venugopal, G. R. Arunodaya, M. K. Vasudev, H. S. Swamy

Published in: Neuroradiology | Issue 9/2006

Login to get access

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<0.001) but did not correlate with the duration of illness.

Conclusion

MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.
Literature
1.
King AD, Walshe JM, Kendall BE et al (1996) Cranial MR changes in Wilson’s disease. AJR Am J Roentgenol 167(6):1579–1584PubMed
2.
Saatci I, Topcu M, Baltaoglu FF et al (1997) MR findings in Wilson’s disease. Acta Radiol 38(2):250–258PubMedCrossRef
3.
Starosta-Rubinstein S, Young AB, Kluin K et al (1987) Clinical assessment of 31 patients with Wilson’s disease. Correlations with structural changes on MRI. Arch Neurol 44:365–370PubMed
4.
Magalhaes AC, Caramelli P, Menezes JR et al (1994) Wilson’s disease: MRI with clinical correlation. Neuroradiology 36(2):97–100PubMedCrossRef
5.
Schwab RS, England AC (1969) Projection technique for evaluating surgery in Parkinson’s disease. In: Gillingham FJ, Donaldson MC (eds) Third symposium on Parkinson’s disease. Livingstone, Edinburgh, pp 152–157
6.
Meenakashi-Sundaram S, Taly AB, Kamat V, Arunodaya GR, Rao S, Swamy HS (2002) Autonomic dysfunction in Wilson’s disease – a clinical and electrophysiological study. Clin Auton Res 12:185–189CrossRef
7.
8.
Selwa LM, Vanderzant CW, Brunberg JA, Brewer GJ, Drury I, Beydoun A (1993) Correlation of evoked potential and MRI findings in Wilson’s disease. Neurology 43(10):2059–2064PubMed
9.
Brewer GJ (2000) Recognition, diagnosis, and management of Wilson’s disease. Proc Soc Exp Biol Med 223:39–46PubMedCrossRef
10.
Walshe JM (1989) Wilson’s disease. In: Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 49. Elsevier, Amsterdam, pp 223–238
11.
Roh JK, Lee TG, Wie BA, Lee SB, Park SH, Chang KH (1994) Initial and follow-up brain MRI findings and correlation with the clinical course in Wilson’s disease. Neurology 44(6):1064–1068PubMed
12.
van Wassenaer-van Hall HN, van den Heuvel AG, Jansen GH, Hoogenraad TU, Mali WP (1995) Cranial MR in Wilson disease: abnormal white matter in extrapyramidal and pyramidal tracts. AJNR Am J Neuroradiol 16(10):2021–2027PubMed
13.
Giagheddu M, Tamburini G, Piga M et al (2001) Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson’s disease. Acta Neurol Scand 103(2):71–81PubMedCrossRef
14.
Oder W, Prayer L, Grimm G et al (1993) Wilson’s disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology 43(1):120–124PubMed
15.
Page RA, Davie CA, MacManus D et al (2004) Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease. Neurology 63(4):638–643PubMed
16.
Grimm G, Oder W, Prayer L, Ferenci P, Madl C (1991) Comparison of functional and structural brain disturbances in Wilson’s disease. Neurology 41:272–276PubMed
17.
Prayer L, Wimberger P, Kramer J, Grimm G, Oder W, Imhof H (1990) Cranial MRI in Wilson’s disease. Neuroradiology 32(3):211–214PubMedCrossRef
18.
Barbosa ER, Caramelli P, Bacheschi LA et al (1993) Wilson’s disease: magnetic resonance imaging (MRI) with clinical correlations in 16 cases. Rev Paul Med 111(3):407–411PubMed
19.
Denny-Brown D (1964) Hepatolenticular degeneration (Wilson’s disease) – two different components. New Engl J Med 270:1149–1156PubMedCrossRef
20.
Sener RN (1993) Wilson’s disease: MRI demonstration of cavitations in the basal ganglia and thalami. Pediatr Radiol 23(2):157PubMedCrossRef
21.
Juan CJ, Chen CY, Liu YJ et al (2005) Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings. Neuroradiology 47(6):401–405PubMedCrossRef
22.
Mochizuki H, Kamakura K, Masaki T et al (1997) Atypical MRI features of Wilson’s disease: high signal in globus pallidus on T1-weighted images. Neuroradiology 39(3):171–174PubMedCrossRef
23.
Engelbrecht V, Schlaug G, Hefter H, Kahn T, Modder U (1995) MRI of the brain in Wilson disease: T2 signal loss under therapy. J Comput Assist Tomogr 19(4):635–638PubMedCrossRef
24.
Morgan MY (1998) Cerebral magnetic resonance imaging in patients with chronic liver disease. Metab Brain Dis 13(4):273–290PubMedCrossRef
25.
Hitoshi S, Iwata M, Yoshikawa K (1991) Mid-brain pathology of Wilson’s disease: MRI analysis of three cases. J Neurol Neurosurg Psychiatry 54(7):624–626PubMedCrossRef
26.
Drayer B, Burger P, Darwin R, Riederer S, Herfkens R, Johnson GA (1986) MRI of brain iron. AJR Am J Roentgenol 147(1):103–110PubMed
27.
Meenakshi-Sundaram S, Mahadevan A, Taly AB, Arunodaya GR, Swamy HS, Shankar SK (2003) Pathology of Wilson’s disease: revisited (abstract). Ann Indian Acad Neurol 6:67
28.
Alanen A, Komu M, Penttinen M, Leino R (1999) Magnetic resonance imaging and proton MR spectroscopy in Wilson’s disease. Br J Radiol 72(860):749–756PubMed
29.
Sinha S, Jha DK, Sinha KK (2001) Wilson’s disease in Eastern India, a study of 49 cases. J Assoc Physicians India 49:881–884PubMed
30.
Polson RJ, Rolles K, Calne RY, Williams R, Marsden D (1987) Reversal of severe neurological manifestations of Wilson’s disease following orthotopic liver transplantation. Q J Med 64:685–691PubMed
31.
van Wassenaer-van Hall HN, van den Heuvel AG, Algra A, Hoogenraad TU, Mali WP (1996) Wilson disease: findings at MR imaging and CT of the brain with clinical correlation. Radiology 198(2):531–536PubMed
32.
Thuomas KA, Aquilonius SM, Bergstrom K, Westermark K (1993) Magnetic resonance imaging of the brain in Wilson’s disease. Neuroradiology 35(2):134–141PubMedCrossRef
33.
Prashanth LK, Taly AB, Sinha S et al (2005) Prognostic factors in patients presenting with severe neurological forms of Wilson’s disease. Q J Med 98:557–563
34.
Kendall, BE, Pollock, SS, Bass NM, Valentine AR (1981) Wilson’s disease. Clinical correction with cranial computed tomography. Neuroradiology 22(1):1–5PubMedCrossRef
Metadata
Title
Wilson’s disease: cranial MRI observations and clinical correlation
Authors
S. Sinha
A. B. Taly
S. Ravishankar
L. K. Prashanth
K. S. Venugopal
G. R. Arunodaya
M. K. Vasudev
H. S. Swamy
Publication date
01-09-2006
Publisher
Springer-Verlag
Published in
Neuroradiology / Issue 9/2006
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI
https://doi.org/10.1007/s00234-006-0101-4

Other articles of this Issue 9/2006

Neuroradiology 9/2006 Go to the issue

Diagnostic Neuroradiology

Impact of diffusion-weighted MRI-measured initial cerebral infarction volume on clinical outcome in acute stroke patients with middle cerebral artery occlusion treated by thrombolysis

Functional Neuroradiology

Ictal SPECT in neocortical epilepsies: clinical usefulness and factors affecting the pattern of hyperperfusion

Diagnostic Neuroradiology

Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging

Diagnostic Neuroradiology

eLearning in education and advanced training in neuroradiology: introduction of a web-based teaching and learning application

Diagnostic Neuroradiology

The emerging role of multidetector row CT angiography in the diagnosis of cervical arterial dissection: preliminary study

Interventional Neuroradiology

Does endoluminal coil embolization cause distension of intracranial aneurysms?