The document describes findings on CT and MRI in patients with Wilson's disease. Key findings include cortical atrophy, ventricular dilatation, caudate atrophy, and hypodensities in the brainstem, cerebellum, basal ganglia, and thalamus on CT. MRI shows atrophy of the cerebrum, brainstem, and cerebellum, along with signal abnormalities in the putamen, caudate, thalami, midbrain, pons, and cerebral white matter. Characteristic signs on MRI include the "face of a giant panda" and "bright claustral" signs. Differential diagnosis and clinical features like the Kayser-Fleischer ring are also discussed.

6. CT brain in Wilson Disease
• Cortical atrophy 44.8%
• Ventricular dilatation 44%
• Caudate atrophy 25%
• Brain stem atrophy 31.9%
• Cerebellar atrophy 19%
• Hemispheric hypodensities 29.3%
• Basal ganglionic hypodensities. 19.8%
• Thalamic hypodensities. 10.3%

7. MRI brain IN Wilson Disease
• Atrophy of the cerebrum, 70%
• Brainstem, 66%
• Cerebellum 52%
• Signal abnormality in putamen, 72%
• Caudate, 61%
• Thalami, 58%
• Midbrain, 49%
• Pons , 20%
• Cerebral white matter 25%
• Cortex 9%
• Medulla 12%
• Cerebellum 10%
• Face of giant panda' sign 12%
• CPM like feature 7%
• Bright claustral sign 4%

8. MRI IN Wilson disease
And B, T1-weighted axial MR images show bilateral increased signal intensity in the globus
pallidus (arrows) and midbrain (arrowhead).
Kim T et al. AJNR Am J Neuroradiol 2006;27:1373-1378
©2006 by American Society of Neuroradiology

9. Das SK and Ray K (2006) Wilson's disease: an update
Nat Clin Pract Neurol 2: 482–493 10.1038/ncpneuro0291
Hyperintensities in the bilateral basal ganglia and thalami shown by T2-weighted MRI
of the brain
MRI IN WILSON DISEASE

10. MRI WILSON DISEASE
face of a giant panda sign,
• increased signal intensity
in the midbrain
tegmentum with normally
hypointense red nucleus
(eyes, yellow arrow),
• preservation of signal
intensity of the pars
reticulate of substantia
nigra (ears, green arrow),
• Low signal intensity of
superior colliculous (chin,
white arrow).
Panda AK. BMJ 2013

11. MRI WILSON DISEASE
• There is a thin rim of T2
hyperintensity in the
claustrum known as the
bright claustrum sign
(yellow arrow).

12. Figure Brain MRI T2-weighted axial MRI demonstrates (A) symmetric hyperintense signals in
the putamen, posterior internal capsule, and thalami (arrows), (B) “face of the giant panda” in
midbrain with high signal in tegmentum and normal red nuclei (arrows), and (C) “face of the
panda cub” in pons with hypointensity of central tegmental tracts with hyperintensity of
aqueductal opening to fourth ventricle (arrows).
Shivakumar R , and Thomas S V Neurology 2009;72:e50
©2009 by Lippincott Williams & Wilkins

13. Brain Stem changes: CPM like
a. Classical: Hyperintensity of whole of the central pons sparing a peripheral
rim;
b. Bisected pontine signal change by a horizontal line and;
c. Trisected: Pontine hyperintensity trisected by a hypointense line like
‘Mercedes Benz’ sign

14. MRI other changes
a. Bilateral basal ganglionic and
thalamic hyperintensity in
addition to mild-to-moderate
degree diffuse atrophy
b. Extensive diffuse white matter
changes
c. Bilateral lentiform, thalamic,
midbrain and white matter
hyperintensity
d. Midbrain hyperintensity in the
tectal region

15. DIFERENTIAL DIAGNOSIS
• Vascular diseases:-
 Chronic hypertensive encephalopathy
 Embolic territorial infarction ( “top of the basilar artery
syndrome”)
• Primary Neoplasm:-
 Glioma
 Primary CNS Lymphoma
• Infectious Diseases:-
 Viral Diseases (Japanese encephalitis,west nile
encephalitis).
 Bacterial cause:- tuberculous meningoencephalitis

16. DIFERENTIAL DIAGNOSIS
 Fungi:- histoplasmosis and cryptococcosis
 Cerebral Malaria(Plasmodium falciparum)
• Demyelinating Disorders:-
 ADEM
 MS
• Metabolic Disorders:-
 Osmotic Demyelination Syndrome
 Wilson’s Disease
 Wernicke’s Encephalopathy
• Hypoxia
• Posterior Reversible Encephalopathy Syndrome

17. Japanese encephalitis
©2006 by American Society of Neuroradiology
.A, T2-weighted axial image: bilateral thalamic
lesions (black arrows).
Note left hippocampal tail involvement (white
arrow).
B, T2-weighted coronal image shows bilateral
thalamic (black arrows), substantia nigra (white
arrows), and left hippocampal body involvement
(large white arrow)
C, Image more posterior than B shows
hippocampal tail involvement on the left side
(arrow).
D, Axial T2-weighted image shows bilateral
substantia nigra lesions (arrows).

18. Infectious Diseases:-•
• West Nile virus
encephalitis:-basal ganglia
and thalamus, more
seldom in the brain stem.
48-year-old woman with West
Nile encephalitis.
Axial T2W shows hyperintensity
& expansion of both thalami.

19. • Cerebral Malaria (Plasmodium
falciparum):-
• bilateral edema or infarctions in
thalamus, In addition only the
cerebellum can be affected.
• However, most frequent finding
in cerebral malaria is diffuse
brain edema.
CECT scan obtained in a 20-
year-old male patient
shows (A) bilateral
symmetric cerebellar
hypoattenuation (arrows)
& (B) associated bithalamic
hypoattenuation (arrows).

20. Kayser-Fleischer Ring
• Named after Dr.
Bernhard Kayser (1902)
and Dr. Bruno
Fleischer(1903)
• Initially thought to be
due to the
accumulation of silver
• they were first
demonstrated to
contain copper in 1934

21. KF RING
• Golden-brown, ruby-red, or green band of 1.0 to
3.0 mm
• starts at the limbus in Descemet's membrane of
cornea.
• The color of the ring is presumably caused by
scattering and reflection of incident light and by
photointerference effects created by the layers
of copper granules.
• Such variables as size, shape, and unit density of
the granules may account for the different
appearances of the Kayser-Fleischer ring.

22. Kayser-Fleischer Ring
• Earliest pigment deposition
occur as an arc in the
superior periphery of the
cornea from the 10- to 2-
o'clock meridian.
• The arc spreads slowly
toward the horizontal
plane and gradually
broadens.
Later a band appears inferiorly as a crescent stretching from
the 5- to 7-o'clock positions. finaly the two arcs meet
In early stages may be detected by slit lamp examination

23. Kayser-Fleischer Ring
• Present in 95 % of pts with Wilson disease
with CNS involvement and upto 50% of
patient with hepatic involvment
• KF rings tend to decrease after 3–6 months
and disappear by 2 years.
• a pitted or beaten silver pattern may become
apparent at the previous site of the ring.
• This is an indication that treatment has
produced a negative copper balance.

24. CAUSES OF KF RING
• WILSON DISEASE
• primary biliary cirrhosis
• progressive intrahepatic cholestasis of
childhood
• chronic active hepatitis
• poorly differentiated adenocarcinoma of the lung,
associated with IgG monoclonal gammopathy
these diseases cause an elevated level of copper in
the blood, urine, and liver, only in Wilson's disease
are subnormal levels of ceruloplasmin present

25. D/D Of KF Ring
• Arcus senilis (or arcus senilis corneae)
• Fleischer ring
• Hudson-Stahli line
• Limbal ring

26. Arcus senilis [gerontoxon]
• White, grey, or blue opaque
ring in the cornea [STROMA]
• it is quite commonly present
in the elderly
• It can also appear earlier in life
as a result of
hypercholesterolemia
• Arcus deposits tend to start at
6 and 12 o'clock and fill in
until becoming completely
circumferential.
• There is a thin, clear section
separating the arcus from the
limbus, known as the lucid
interval of Vogt.d/d KF Ring
Younger people with the same abnormality at the edge of the
cornea would be termed arcus juvenilis.

27. Fleischer rings
• Pigmented rings in the
peripheral cornea,
resulting from iron
deposition in basal
epithelial cells, in the
form of hemosiderin
• They are usually yellowish
to dark-brown, and may
be complete or broken.
• Fleischer rings are
indicative of keratoconus
• Best seen using cobalt
blue light.

28. Hudson-Stahli line
• Iron deposition line in
the corneal epithelium,
which commonly seen
in the junction
between middle and
lower third cornea
• Often seen in elderly.
• Causes no symptoms or
clinical significance.

29. Limbal ring
• A limbal ring is a dark ring
around the iris of the eye.
• It is a dark-colored
manifestation of the corneal
limbus resulting from optical
properties of the region.
• Limbal rings become less
pronounced with age, thus
darker rings imply
youthfulness and are
considered attractive.[
• Some contact lenses are
colored to simulate limbal
rings.