WILSONS-DISEASE WITH CEREBRAL MANIFESTATION - MONITORING THERAPY BY CSF COPPER CONCENTRATION

被引:27
作者
HARTARD, C [1 ]
WEISNER, B [1 ]
DIEU, C [1 ]
KUNZE, K [1 ]
机构
[1] DR HORST SCHMIDT KLIN,WIESBADEN,GERMANY
关键词
WILSONS DISEASE CEREBROSPINAL FLUID; COPPER DIAGNOSIS; THERAPY;
D O I
10.1007/BF00869772
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The clinical courses, cerebrospinal fluid (CSF) and serum copper concentrations and urinary copper excretions under different schemes of drug treatment in four patients with cerebral manifestations of Wilson's disease were monitored over 6-11 years. CSF copper concentration measurements were performed from the beginning of therapy onwards in three patients and from 16 months after initial treatment onwards in the fourth. CSF copper levels decreased slowly over the years in parallel with clinical improvements, and increased in one patient who interrupted therapy for 2 years. These findings confirm our hypothesis that the concentration of copper in the CSF is a valuable quantitative parameter reflecting the normalization of copper in the brain. Copper measurements during phases of initial neurological deterioration in two patients receiving D-penicillamine, and in one patient receiving D-penicillamine and zinc sulphate, revealed decreased free serum copper and CSF copper levels.
引用
收藏
页码:101 / 107
页数:7
相关论文
共 52 条
[21]   ANTIGEN-ANTIBODY CROSSED ELECTROPHORESIS [J].
LAURELL, CB .
ANALYTICAL BIOCHEMISTRY, 1965, 10 (02) :358-&
[22]  
LEE DY, 1989, J LAB CLIN MED, V114, P639
[23]   THE KAYSER-FLEISCHER RING DURING LONG-TERM TREATMENT IN WILSONS-DISEASE (HEPATOLENTICULAR DEGENERATION) - A FOLLOW-UP-STUDY [J].
LOSSNER, A ;
LOSSNER, J ;
BACHMANN, H ;
ZOTTER, J .
GRAEFES ARCHIVE FOR CLINICAL AND EXPERIMENTAL OPHTHALMOLOGY, 1986, 224 (02) :152-155
[24]  
MASON J, 1989, LANCET, V1, P1455
[25]  
MAYET IY, 1990, CLIN PHARMACY, V9, P951
[26]  
MULLERJENSEN A, 1983, ELECTROENCEPHALOGR C, V56, P139
[27]  
PALL H, 1986, LANCET, V2, P1279
[28]   DETERIORATION OF WILSONS-DISEASE FOLLOWING THE START OF PENICILLAMINE THERAPY [J].
PALL, HS ;
WILLIAMS, AC ;
BLAKE, DR .
ARCHIVES OF NEUROLOGY, 1989, 46 (04) :359-360
[29]   THE EFFECT OF ZINC SUPPLEMENTS ON PLASMA ZINC AND COPPER LEVELS AND THE REPORTED SYMPTOMS IN HEALTHY-VOLUNTEERS [J].
SAMMAN, S ;
ROBERTS, DCK .
MEDICAL JOURNAL OF AUSTRALIA, 1987, 146 (05) :246-&
[30]   VISUAL AND BRAIN-STEM AUDITORY EVOKED-RESPONSES IN WILSONS-DISEASE [J].
SATISHCHANDRA, P ;
SWAMY, HS .
ACTA NEUROLOGICA SCANDINAVICA, 1989, 79 (02) :108-113