Atypical neuroimaging in Wilson’s disease

Patell, Rushad; Dosi, Rupal; Joshi, Harshal; Storz, Dennis

doi:10.1136/bcr-2013-200100

Cited by 8 publications

(4 citation statements)

References 9 publications

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“…The most frequent findings are increased density on computerized tomography or hyperintensity on T2 MRI in the basal ganglia [3]. In recent years, some unusual MRI findings have been reported, such as face of the giant panda [16–18], eye of tiger [19], central pontine myelinolysis (CPM)-like signal changes [20], and so on. Therefore, MRI is an important and useful tool in WD.…”

Section: Discussionmentioning

confidence: 99%

Characteristics of neurological Wilson’s disease with corpus callosum abnormalities

Zhou

Cao

et al. 2019

BMC Neurol

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Background Wilson’s disease (WD) is an autosomal recessive disease of impaired copper metabolism. Previous study demonstrated that WD with corpus callosum abnormalities (WD-CCA) was limited to the posterior part (splenium). This study aimed to compare clinical features between WD-CCA and WD without corpus callosum abnormalities (WD-no-CCA). Methods Forty-one WD patients who had markedly neurological dysfunctions were included in this study. We retrospectively reviewed clinical, biochemical characteristics and MRI findings in the 41 WD patients. All patients were assessed using the Unified Wilson’s Disease Rating Scale. Results Nine patients had corpus callosum abnormalities, 4 of 9 patients had abnormal signal in the genu and splenium, 5 of 9 patients had abnormal signal only in the splenium. WD-CCA had longer course (9.9 ± 4.0 years vs. 3.4 ± 3.6 years, p <0.01), more severe neurological dysfunctions (37.6 vs. 65.9, p <0.01) and higher psychiatric symptoms scores (11.2 vs. 22.5, p <0.01) than WD-no-CCA. The MRI findings indicated that WD-CCA had higher ratio than WD-no-CCA in globus pallidus (88.9% vs. 43.8%, p = 0.024) and thalamus (100% vs. 59.4%, p = 0.038). The index of liver function and copper metabolism had no significant in WD-CCA and WD-no-CCA patients. Conclusion Our findings indicate Wilson’s disease can involve the posterior as well as the anterior part of CC and patients with CC involvement had more extensive brain lesions, more severe neurological dysfunctions and psychiatric symptoms.

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Section: Discussionmentioning

confidence: 99%

Characteristics of neurological Wilson’s disease with corpus callosum abnormalities

Zhou

Cao

et al. 2019

BMC Neurol

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“…in a 14-year-old girl who had chronic liver disease, unlike our patient who had no evidence of hepatitis. [ 9 ]…”

Section: Discussionmentioning

confidence: 99%

White matter changes in Wilson’s disease: A radiological enigma

Mukherjee

Solanki

Guha

et al. 2016

Journal of Neurosciences in Rural Practice

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Wilson's disease is a metabolic disorder which presents with hepatitis or hepatic decompensation commonly. Neurologic manifestations are late and include movement disorders, personality changes, and seizures. Magnetic resonance imaging (MRI) brain shows high signal changes in putamen, lentiform nucleus, thalamus, and brainstem. White matter lesions are rare. We report a child of Wilson's disease who presented to us with dystonia, rigidity, myoclonus and had symmetrical white matter changes in the fronto-parietooccipital region. Diffusion restriction in bilateral frontoparietal areas was also seen which is rare in chronic cases like ours. Atypical MRI characteristics should be considered in patients with clinical signs of neurological involvement in Wilson's disease as it is a devastating but treatable disease.

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“…74 WD can rarely cause confluent white matter abnormalities. 76,77 Brain atrophy and susceptibility-weighted imaging abnormalities are also common at diagnosis and correlate with neurological severity at diagnosis. 78 T1-weighted hyperintensities in the basal ganglia, which likely represent manganese deposition and can occur with cirrhosis of any cause, may also be seen.…”

Section: Neuroimagingmentioning

confidence: 99%