Wilson radiology

Wilson disease radiology

1. 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%

2. 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%

3. 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

4. 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

5. 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

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

7. 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

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

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

10. 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

11. 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

12. 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).

13. 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.

14. 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).

15. 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

16. KF RING G olden-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.

17. Kayser -Fleischer Ring E arliest 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

18. Kayser -Fleischer Ring Present in 95 % of p ts 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 .

19. 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

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

21. Arcus senilis [ gerontoxon ] W hite , 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 A rcus 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 .

22. 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.

23. 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.

24. 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.