Answer for BIR CoW 15 Sep 2024
Wilson's disease
Findings
Symmetrical T2/flair hyperintensities in bilateral caudate nucleus, bilateral ventrolateral thalamus and bilateral crus cerebri. Mild T1 hyperintensities noted in bilateral basal ganglia.
Discussion
Wilson's disease, also known as hepatolenticular degeneration, is a multisystem disease due to abnormal accumulation of copper. It is characterized by early onset liver cirrhosis with CNS findings most frequently affecting the basal ganglia and midbrain. The most frequently affected sites are the basal ganglia (especially putamen), followed by midbrain, pons, and thalamus . The distribution is bilateral and symmetric Abnormal T2 hyperintensity in the putamina is the most common MRI abnormality . Additional areas of T2 signal abnormality predominantly affect the deep gray nuclei . Involvement of the midbrain tegmentum can appear as a face of the giant panda sign on axial images. Axial T2 at the pontine tegmentum may also show the face of a miniature panda sign (cub of the giant panda). This combination is referred to as the double panda sign.
Reference
1. Kim TJ, Kim IO, Kim WS, Cheon JE, Moon SG, Kwon JW, Seo JK, Yeon KM. MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. (2006) AJNR. American journal of neuroradiology. 27 (6): 1373-8. Pubmed
2. Yu XE, Gao S, Yang RM, Han YZ. MR Imaging of the Brain in Neurologic Wilson Disease. (2019) AJNR. American journal of neuroradiology. 40 (1): 178-183. doi:10.3174/ajnr.A5936 - Pubmed
Findings
Symmetrical T2/flair hyperintensities in bilateral caudate nucleus, bilateral ventrolateral thalamus and bilateral crus cerebri. Mild T1 hyperintensities noted in bilateral basal ganglia.
Discussion
Wilson's disease, also known as hepatolenticular degeneration, is a multisystem disease due to abnormal accumulation of copper. It is characterized by early onset liver cirrhosis with CNS findings most frequently affecting the basal ganglia and midbrain. The most frequently affected sites are the basal ganglia (especially putamen), followed by midbrain, pons, and thalamus . The distribution is bilateral and symmetric Abnormal T2 hyperintensity in the putamina is the most common MRI abnormality . Additional areas of T2 signal abnormality predominantly affect the deep gray nuclei . Involvement of the midbrain tegmentum can appear as a face of the giant panda sign on axial images. Axial T2 at the pontine tegmentum may also show the face of a miniature panda sign (cub of the giant panda). This combination is referred to as the double panda sign.
Reference
1. Kim TJ, Kim IO, Kim WS, Cheon JE, Moon SG, Kwon JW, Seo JK, Yeon KM. MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. (2006) AJNR. American journal of neuroradiology. 27 (6): 1373-8. Pubmed
2. Yu XE, Gao S, Yang RM, Han YZ. MR Imaging of the Brain in Neurologic Wilson Disease. (2019) AJNR. American journal of neuroradiology. 40 (1): 178-183. doi:10.3174/ajnr.A5936 - Pubmed
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!