Clinical analysis and literature review of Wilson's disease with onset of mental and behavior disorder
- VernacularTitle:以精神行为异常起病的肝豆状核变性临床分析及文献复习
- Author:
Bingchuan XIE
1
;
Wenting WANG
1
;
Zhiyuan SHEN
1
Author Information
- Publication Type:Journal Article
- Keywords: Mental and behavior disorder ；Hepatolenticular degeneration ； K-F ring ； Ceruloplasmin ； Blood ATP7B gene
- From: Journal of Apoplexy and Nervous Diseases 2022;39(11):1023-1026
- CountryChina
- Language:Chinese
- Abstract: To investigate the clinical and imaging features，diagnosis，treatment and prognosis of patients with wilson disease（WD）.Methods The clinical data of wilson's disease with mental and behavior disorder were analyzed.Results The patient started the disease at the age of 16.At the beginning of the disease，she was talking to herself，laughing and in a daze，which did not attract the attention of her family.The first diagnosis and treatment was not made until the age of 22.Brain atrophy and T-2 high signal in bilateral thalamus，brainstem，cerebellum，pontine arm and putamen were shown on head MRI.K-F ring was positive under slit lamp，ceruloplasmin <3 mg/dl.Gene detection showed ATP7B gene variation.Conclusion When patients start with mental,emotional，and cognitive disorders,accompanied by intracranial symmetrical lesions and combined with systemic multiple system damage，WD should be considered.K-F loop，ceruloplasmin and gene detection are helpful for early diagnosis and treatment.
- Full text:202407232021581392Clinical analysis and literature review of Wilson's disease with onset of mental and behavior disorder.pdf
