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Images in Neurology |

Cortical Cystic Necrosis in Wilson Disease

Aakash S. Shetty, MBBS, MD, DM1; Ritu Kashikar, MBBS, DNB2; Aabha Nagral, MBBS, MD, DNB3; Pettarusp M. Wadia, MD, DM, DNB1
[+] Author Affiliations
1Department of Neurology, Jaslok Hospital and Research Centre, Mumbai, India
2Department of Radiology, Jaslok Hospital and Research Centre, Mumbai, India
3Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India
JAMA Neurol. 2016;73(3):350-351. doi:10.1001/jamaneurol.2015.3595.
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This case report discusses a patient with Wilson disease with unusual extensive cortical cystic lesions and partial poor response to decupuration and seizures.

Wilson disease is an autosomal recessive neurodegenerative disease secondary to abnormal copper metabolism. It is caused by mutations in ATB7B and up to 500 mutations are known to date. Magnetic resonance imaging changes are very common in Wilson disease predominantly affecting the subcortical structures and basal ganglia. Cortical involvement is known to occur pathologically; however, it is rarely demonstrated on magnetic resonance imaging. This case shows unusual extensive cortical cystic lesions with partial poor response to decupuration and seizures.

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Figure.
T2-Weighted Axial Magnetic Resonance Image (MRI) and Fluid-Attenuated Inversion Recovery (FLAIR) Coronal Image

A, T2-weighted axial magnetic resonance image (MRI) demonstrating extensive bifrontal cortical and subcortical cystic changes and diffuse confluent hyperintensities in bilateral frontal periventricular deep and subcortical white matter with leukomalacia and resultant dilatation of lateral ventricles. Cortical atrophic changes were also identified. B, Fluid-attenuated inversion recovery (FLAIR) coronal image demonstrating additional changes in the basal ganglia along with cortical changes. Similar findings of extensive bifrontal gliosis were noted. Additional subtle hyperintensities in the putamen (arrowheads) were also seen.

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Cortical Cystic Necrosis in Wilson Disease. JAMA Neurol 2016;73(3):350-1.
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