The mechanism for neurological symptom deterioration in Wilson' s disease during the treatment of D-penicillamine
- VernacularTitle:青霉胺治疗引起肝豆状核变性神经症状加重
- Author:
Xiangxue ZHOU
;
Xunhua LI
;
Xiuling LIANG
;
Xiaoyong PU
;
Songlin CHEN
;
Bing LIU
;
Yinying LIANG
;
Li LI
;
Chunge XIE
- Publication Type:Journal Article
- Keywords:
Hepatolenticular;
Penicillamine;
Copper;
Phosphopyruvate hydratase
- From:
Chinese Journal of Neurology
2008;41(10):674-677
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the mechanism of the secondary deterioration of neurological symptoms in Wilson' s disease (WD) at early stage of treatment using D-penicillamine. Methods Forty non-treated WD patients, 32 of encephalic and 8 hepatic type respectively, were enrolled in the study. Their neural symptoms were scored using modified Young grade. Cerebrospinal fluid (CSF) copper, serum copper, urinary copper, neuron specific enolase (NSE) in CSF and the albumin ratio CSF/serum (AR) were measured at the same time. After 3 months of treatment with D-penicillamine, neural symptoms of patients were scored again. All dates were analyzed. Results After 3 months of treatment with D-penicillamine, 15 patients (46. 9%) developed a secondary deterioration in neurological symptoms. The concentration of copper and the NSE in CSF of patients whose neural symptom was increasingly deteriorated. The serum copper declined after treatment((0. 37± 0. 09) vs (0. 25 ± 0. 08) mg/L, t = 3. 17, P < 0. 05). The 24 hours urinary copper of patients whose symptoms had deteriorated was much lower than that of patients who had not. No significant change was found in AR ratio before and after the treatment (9. 53 ± 3.18vs12.24±3.17) in the worsened group (t=1.45, P>0. 05). Conclusions The degree of the injury in the neural system and the dose of penicillamine may affect the deterioration of the neural symptom.
