1. Peripheral neuropathy in copper deficient Wilson′s disease due to excessive copper removal: a case report and literature review
Jie ZHANG ; Xuen YU ; Wenlong AI ; Yongguang SHI ; Liangliang ZHANG ; Bin SONG ; Congbo SHA ; Lin CHEN ; Jiyuan HU ; Renmin YANG
Chinese Journal of Neurology 2019;52(11):919-924
Objective:
To report a case of peripheral neuropathy secondary to copper deficiency (CD) by long-term decoppering chelation in Wilson′s disease (WD) to enhance understanding of the disease, and to pay more attention to individualized treatment of WD.
Methods:
A case of WD diagnosed 12 years ago confirmed by gene detection and since then treated with anti-copper agent was diagnosed as CD based peripheral neuropathy and significant neutropenia and followed up for six months, and the clinical manifestations, laboratory examination, electrophysiology, imaging features were summarized. The related literatures were reviewed.
Results:
A total of 16 cases of WD complicated with CD were reviewed and analyzed, including seven males and nine females aged 13-56 years. All of them were treated with zinc for 1-38 years, and nine cases with peripheral neuropathy. Hematological indicators can be significantly improved and neurological symptoms can be partially alleviated after stopping copper removal treatment.
Conclusions
Peripheral neuropathy in a WD with treatment-related CD may occur in blind treatment, irregular treatment monitoring and without individualized treatment adjustment. It is necessary to monitor blood routine, copper and zinc metabolism regularly and advocate individualized treatment of WD.
2.Mechanism of Traditional Chinese Medicine in Prevention and Treatment of Parkinson's Disease:A Review
Xiaolong SHANG ; Yu WANG ; Chenchen XU ; Jianjian DONG ; Wenlong AI ; Liangjie ZHANG ; Chunling CI ; Xun WANG
Chinese Journal of Experimental Traditional Medical Formulae 2023;29(17):248-259
Parkinson's disease (PD) is a progressive chronic neurodegenerative disorder with a complex pathogenesis involving oxidative stress, neuroinflammation, mitochondrial dysfunction, and other factors. Currently, the clinical treatment of PD mainly includes levodopa, dopamine receptor agonists, monoamine oxidase B inhibitors, catechol-O-methyltransferase inhibitors, and anticholinergic drugs, but there is a lack of disease-modif g therapies that can definitively improve disease progression. According to the understanding of traditional Chinese medicine (TCM), PD is characterized by asthenia in origin and sthenia in superficiality. It is primarily caused by liver-kidney Yin deficiency, Qi-blood insufficiency, and closely related to wind, fire, phlegm, and blood stasis. Numerous clinical practices have shown that TCM has significant clinical value in the prevention and treatment of PD, the management of motor and non-motor symptoms, and the neuroprotection of dopaminergic neurons. The underlying mechanisms of TCM include antioxidative stress, anti-neuroinflammation, and regulation of mitochondrial dysfunction. This article categorized and summarized the pathogenesis of PD, systematically elucidated the pharmacological actions and molecular mechanisms of TCM monomer extracts and compounds in the prevention and treatment of PD, and provided the latest clinical research progress, aiming to provide references for the development and clinical use of TCM for PD.
3.O'Sullivan-McLeod Syndrome: A Case Report
Yu WANG ; Suyue WANG ; Ping JIN ; Yulong ZHU ; Kun XIA ; Dandan SUN ; Wenlong AI ; Xiaoming FU ; Qunrong YE ; Kai LI ; Xun WANG
JOURNAL OF RARE DISEASES 2022;1(2):189-195
O'Sullivan-Mcleod syndrome is a very rare variant of MND with a good prognosis. Its clinical feature is distal lower motor neuron syndrome of both upper limbs, and there is no effective treatment at present. We reported a case of O'Sullivan-Mcleod syndrome in this paper.The patient exhibited with middle-aged progressive distal muscle weakness and atrophy of both upper limbs, without sensory, cognitive or behavioral impairment and without pyramidal tract sign. Laboratory examination, imaging and genetic tests showed no obvious abnormalities. EMG revealed neurogenic damage to the small muscles of both hands. Now we retrospectively analyzed the clinical features of a patient with O'Sullivan-McLeod syndrome, and data from 18 cases for comparative analysis, in order to improve its understanding by clinicians.