Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease
Abstract Objective To study the aggravation of clinical symptoms after discontinuation of metal chelating agent therapy in Wilson's disease (WD) patients, analyze the causes of aggravation, and observe the prognosis. Methods 40 WD patients (cerebral type 30 cases and hepatic type 10 cases) who...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-09-01
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| Series: | Brain and Behavior |
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| Online Access: | https://doi.org/10.1002/brb3.3170 |
| _version_ | 1797687642465239040 |
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| author | Xiangxue Zhou Jian Liao Yinjie Liu Haolin Qin Xia Xiao |
| author_facet | Xiangxue Zhou Jian Liao Yinjie Liu Haolin Qin Xia Xiao |
| author_sort | Xiangxue Zhou |
| collection | DOAJ |
| description | Abstract Objective To study the aggravation of clinical symptoms after discontinuation of metal chelating agent therapy in Wilson's disease (WD) patients, analyze the causes of aggravation, and observe the prognosis. Methods 40 WD patients (cerebral type 30 cases and hepatic type 10 cases) who stopped using metal chelating agent were selected, 40 WD patients with normal therapy, and 10 normal control cases were selected. All patients underwent neurological symptom evaluation using modified Young scale, Child‐Pugh liver function grading, metal metabolism, and disease typing. Magnetic sensitivity imaging (SWI), diffusion tensor imaging (DTI), and magnetic resonance spectroscopy imaging (MRS) were performed. According to the imaging results, WD patients were divided into metal deposition stage, fiber damage stage, and neuron necrosis stage. All patients were treated with metal chelating agent for 6 months. Results The score of modified Young scale in drug withdrawal group was lower than that in normal treatment group before drug withdrawal (p = .032). The score of modified Young scale was higher after drug withdrawal than before (p = .011). The number of Child‐Pugh B‐grade patients after drug withdrawal was more than that before drug withdrawal and in normal treatment group. The proportion of patients in the stage of neuronal necrosis after drug withdrawal (25%) was higher than that before drug withdrawal (10%) (p = .025). After drug withdrawal, urine copper was significantly higher than that before drug withdrawal and in the normal treatment group (p = .032, .039). After the withdrawal group resumed metal chelating agent treatment, 34.2% of neurological symptoms worsened. Conclusions WD patients showed neurological symptoms aggravation and increased liver injury after metal chelating agent withdrawal. Increased metal deposition and new nerve injury occurred in the brain. After re‐treatment, the aggravated neurological symptoms of WD patients are difficult to reverse. |
| first_indexed | 2024-03-12T01:20:42Z |
| format | Article |
| id | doaj.art-a0457d9fc6c24847b4549a5fcc3271a8 |
| institution | Directory Open Access Journal |
| issn | 2162-3279 |
| language | English |
| last_indexed | 2024-03-12T01:20:42Z |
| publishDate | 2023-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | Brain and Behavior |
| spelling | doaj.art-a0457d9fc6c24847b4549a5fcc3271a82023-09-13T06:30:24ZengWileyBrain and Behavior2162-32792023-09-01139n/an/a10.1002/brb3.3170Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's diseaseXiangxue Zhou0Jian Liao1Yinjie Liu2Haolin Qin3Xia Xiao4Department of Neurology The First Affiliated Hospital, Sun Yat‐sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology GuangzhouChinaDepartment of Neurology The First Affiliated Hospital, Sun Yat‐sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology GuangzhouChinaDepartment of Neurology The First Affiliated Hospital, Sun Yat‐sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology GuangzhouChinaDepartment of Imaging The First Affiliated Hospital of Sun Yat‐sen University GuangzhouChinaDepartment of Neurology The First Affiliated Hospital, Sun Yat‐sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology GuangzhouChinaAbstract Objective To study the aggravation of clinical symptoms after discontinuation of metal chelating agent therapy in Wilson's disease (WD) patients, analyze the causes of aggravation, and observe the prognosis. Methods 40 WD patients (cerebral type 30 cases and hepatic type 10 cases) who stopped using metal chelating agent were selected, 40 WD patients with normal therapy, and 10 normal control cases were selected. All patients underwent neurological symptom evaluation using modified Young scale, Child‐Pugh liver function grading, metal metabolism, and disease typing. Magnetic sensitivity imaging (SWI), diffusion tensor imaging (DTI), and magnetic resonance spectroscopy imaging (MRS) were performed. According to the imaging results, WD patients were divided into metal deposition stage, fiber damage stage, and neuron necrosis stage. All patients were treated with metal chelating agent for 6 months. Results The score of modified Young scale in drug withdrawal group was lower than that in normal treatment group before drug withdrawal (p = .032). The score of modified Young scale was higher after drug withdrawal than before (p = .011). The number of Child‐Pugh B‐grade patients after drug withdrawal was more than that before drug withdrawal and in normal treatment group. The proportion of patients in the stage of neuronal necrosis after drug withdrawal (25%) was higher than that before drug withdrawal (10%) (p = .025). After drug withdrawal, urine copper was significantly higher than that before drug withdrawal and in the normal treatment group (p = .032, .039). After the withdrawal group resumed metal chelating agent treatment, 34.2% of neurological symptoms worsened. Conclusions WD patients showed neurological symptoms aggravation and increased liver injury after metal chelating agent withdrawal. Increased metal deposition and new nerve injury occurred in the brain. After re‐treatment, the aggravated neurological symptoms of WD patients are difficult to reverse.https://doi.org/10.1002/brb3.3170disease stagedrug discontinuancemetal chelating agentsymptoms aggravatedWilson disease |
| spellingShingle | Xiangxue Zhou Jian Liao Yinjie Liu Haolin Qin Xia Xiao Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease Brain and Behavior disease stage drug discontinuance metal chelating agent symptoms aggravated Wilson disease |
| title | Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease |
| title_full | Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease |
| title_fullStr | Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease |
| title_full_unstemmed | Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease |
| title_short | Symptom aggravation after withdrawal of metal chelating agent therapy in patients with Wilson's disease |
| title_sort | symptom aggravation after withdrawal of metal chelating agent therapy in patients with wilson s disease |
| topic | disease stage drug discontinuance metal chelating agent symptoms aggravated Wilson disease |
| url | https://doi.org/10.1002/brb3.3170 |
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