Journal of Translational Neuroscience(转化神经科学电子杂志)
Cover Story   2019, Volume 4 Issue 3
   Online First

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, Volume 4 Issue 3

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Review
Pathogenic roles of alpha-synuclein in Parkinson’s disease and multiple system atrophy
Hanjiang Luo, Min Chen, Shun Yu,
Journal of Translational Neuroscience. 2019, 4 (3): 1-13.  
https://doi.org/10.3868/j. issn. 2096-0689. 2019. 03. 001

Abstract   PDF (15636KB)
Alpha-synucleinopathies (α-synucleinopathies) are a diverse group of neurodegenerative diseases comprising Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). Although in all these diseases there exist abnormal accumulation of alpha-synuclein (α-syn) aggregates in nerve tissues, the pathological lesions formed by α-syn aggregates and their cellular locations are quite different. In PD and DLB, the hallmark pathological lesions are Lewy bodies (LBs) and Lewy neurites (LNs), which are localized in the neuronal somata and processes. In MSA, the characteristic pathologic structures are glial cytoplasmic inclusions, which are deposited in the cytoplasm of oligodendrocytes. The fact that PD and MSA have distinct pathological α-syn lesions suggest that different mechanisms play a role in the pathogenesis of the two diseases. In this review article, we compare the clinical manifestations and pathological features of PD and MSA, the two common synucleinopathies, and discuss the potential mechanisms for the formation of α-syn aggregates and their pathologic roles in PD and MSA.
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Alpha-synuclein in body fluids as a diagnostic biomarker for Parkinson’s disease
Qihan Song, Wenjiao Yu, Shun Yu,
Journal of Translational Neuroscience. 2019, 4 (3): 14-23.  
https://doi.org/10.3868/j. issn. 2096-0689. 2019. 03. 002

Abstract   PDF (11330KB)
Parkinson’s disease (PD) is a common neurodegenerative disease, characterized clinically by both motor and non-motor symptoms. Pathologically, PD is hallmarked by the loss of dopaminergic neurons in the substantia nigra (SN) and the formation of α-synuclein (α-syn) containing inclusion bodies (Lewy pathology) in the surviving neurons. Diagnosis of PD is still based on clinical features. However, owing to the complexity, heterogeneity, and overlapping of its symptoms with other Parkinsonian disorders, correct diagnosis of PD remains a challenge, especially in the early stages. Therefore, there is an urgent need for biomarkers that can help correctly diagnose PD, differentiate PD from other Parkinsonian disorders, monitor the progression of the disease, and evaluate the therapeutic efficacy. Various molecules have been investigated for their utility in diagnosing PD, among which α-syn is the most extensively investigated one due to its close implication in the etiology and pathogenesis of PD and related diseases. During the past decade, various species of α-syn, including total, oligomeric, and phosphorylated α-syn in various tissues, have been investigated for their utility as a potential biomarker for PD diagnosis and differential diagnosis. Various forms of α-syn in body fluids, including cerebrospinal fluid (CSF), blood plasma, and saliva, are among the ones that are extensively investigated, since the body fluids are relatively accessible compared to the peripheral tissues. The aim of this review is to summarize the progress of studies on the utility of α-syn in body fluid as a biomarker for PD diagnosis and differential diagnosis.
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Parkinson’s disease: how should we consider the selectivity and progressivity of its neuropathology?
Wei Li, Xiaohan Yu, Shun Yu,
Journal of Translational Neuroscience. 2019, 4 (3): 24-36.  
https://doi.org/10.3868/j. issn. 2096-0689. 2019. 03. 003

Abstract   PDF (16284KB)
Parkinson’s disease (PD) is a common age-related neurodegenerative disease characterized by movement disorders. The hallmark pathological lesions of PD are the formation of Lewy pathology in selected populations of neurons throughout the nervous system. Braak and his colleagues created a staging system for PD describing the connection between Lewy pathology and disease severity. They proposed that Lewy pathology might be initially triggered by exogenous pathogens targeting the enteric or olfactory nervous system, then spread in a prion-like propagation manner from the peripheral nerves to the lower brainstem and midbrain, before finally reaching higher cortical structures, causing a sequential occurrence of the non-motor and motor symptoms, depending on the lesioned neurons. However, emerging evidence also supports a functional threshold hypothesis proposed by Engelender and Isacson in which Lewy pathology may occur parallelly in the central and peripheral nervous systems and the symptoms only begin when the functional reserve of the affected neurons (and their connecting brain regions) is unable to allow for network compensation. Consequently, early symptoms of PD reflect the loss of function in the least compensated systems, such as the enteric and olfactory nervous systems, rather than the spread of Lewy pathology from the peripheral to the central nervous systems. The current review article provides a comprehensive overview of the evidence supporting a merged mechanism that the neurodegeneration in PD happens to those neurons that are not only intrinsically vulnerable but also affected by the spread of Lewy pathology.
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Case Report
Characteristic analysis of diffuse leptomeningeal glioneuronal tumor misdiagnosed as cryptococcal meningitis in adolescent female
Yi Bao, Lei Gao, Jing Xiao, Huihui Wu, Ying Wang, Xinyu Du, Quanying Liu
Journal of Translational Neuroscience. 2019, 4 (3): 37-44.  
https://doi.org/10.3868/j. issn. 2096-0689. 2019. 03. 004

Abstract   PDF (9940KB)
Diffuse leptomeningeal glioneuronal tumor (DLGNT) in adolescent female is rare and easy to be misdiagnosed due to its nonspecificity. This article described the characteristics of an adolescent female DLGNT patient with no history of tumor whose first symptoms are headache and vision loss, and analyzed the causes why DLGNT is easy to be misdiagnosed as cryptococcal meningitis. Treatment remedies: the adolescent female presented with progressive exacerbations of headache, vomiting and vision loss after general treatment. Dynamic monitoring of routine biochemical changes in cerebrospinal fluid (CSF) found abnormal high CSF pressure and protein, repeated examination of antibodies and acid-fast bacilli were negative, and high-throughput pathogen gene examination excluded viral meningitis, tuberculous meningitis and other diseases. To save the optic nerve, the Ommaya capsule was implanted to reduce the intracranial pressure. After diagnostic antifungal treatment, the patient’s condition did not improve. To identify the etiology, extensive meningeal enhancement was eventually detected by enhanced magnetic resonance imaging (MRI), and highly atypical tumor cells were identified by repeated examination of fresh CSF cytology. Post treatment evaluating: for DLGNT, consult oncology. Meningeal biopsy and PET-CT (positron emission tomographycomputed tomography) examination were recommended, and intrathecal chemotherapy and whole-brain radiotherapy were performed according to the examination results.But the patient’s family refused to have a meningeal biopsy and asked to be released from the hospital. Conclusions: the adolescent female without a history of tumor can not rule out the disease, and cryptococcal meningitis also has meningeal enhancement. The gold standard for the diagnosis of DLGNT is to find cancer cells. There is no effective cure for DLGNT, the timely placement of Ommaya sac can significantly improve the quality of the patient’s life, and the active adoption of targeted therapy is expected to extend the patient’s survival.
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