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Unusual epileptic deterioration and extensive white matter lesion during treatment in Wilsons disease

Cited 5 time in Web of Science Cited 6 time in Scopus
Authors
Kim, Young Eun; Yun, Ji Young; Yang, Hui-Jun; Kim, Han-Joon; Jeon, Beom S
Issue Date
2013-09-25
Publisher
BioMed Central Ltd.
Citation
BMC Neurology Vol.13 No.127, pp. 1-3
Keywords
Wilson’s diseaseSeizureDystoniaCortical lesionMRI
Abstract
Background : Wilsons disease (WD) is a genetic disorder which can be controlled fairly well with decupuration therapy. However, symptoms, on rare occasions, can worsen even when WD is being treated. Herein, we report a case involving unusual neurological deterioration during decupuration therapy for WD.
Case presentation : A 28-year-old man was diagnosed with WD 13 years prior to his clinical visit; however, his drug compliance has been poor over the years. He was treated with trientine because tremors and dysarthria have presented in recent years. However, dysarthria and dystonia developed in his limbs, which were worse on the right side and had been aggravated for several weeks despite good drug compliance. His symptoms were fluctuating. It was initially misdiagnosed as dystonia; although, it turned out to be a seizure due to cortical degeneration. These symptoms were completely resolved with antiepileptic drugs. Moreover, the cortical enhancement of bifrontal degeneration has disappeared on the MRI.
Conclusion : This case showed unusual epileptic neurologic deterioration due to cortical degeneration during decupuration therapy. Seizures in WD can easily be mistaken as part of dystonia. However, the fluctuating symptoms suggest a seizure.
ISSN
1471-2377
Language
English
URI
https://hdl.handle.net/10371/83633

http://www.biomedcentral.com/1471-2377/13/127
DOI
https://doi.org/10.1186/1471-2377-13-127
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Neurology (신경과학교실)Journal Papers (저널논문_신경과학교실)
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