2016年 · 第49卷第06期

neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSD) are a class of autoimmune inflammatory demyelinating diseases with typical clinical manifestations of optic neuritis and/or acute transverse myelitis. aquaporin-4 (AQP4) antibody (AQP₄The discovery of IgG) distinguishes it from multiple sclerosis (MS) as an independent disease entity. At present, AQP4-IgG is considered to be the most specific biomarker of NMO/NMOSD, but some patients with NMO/NMOSD still have serum AQP4-IgG negative. In addition, other autoimmune antibodies have been found in NMO/NMOSD patients, suggesting that other immune factors other than AQP4-IgG are involved in the pathogenesis of NMO/NMOSD.

myasthenia gravis (MG) is a typical neuromuscular junction autoimmune disease mediated by acetylcholine receptor (AChR) antibodies, T cell-dependent, and complement-involved. experimental autoimmune myasthenia gravis (EAMG) is an ideal animal model to study the pathogenesis and clinical manifestations of MG^[1]。 The production of autoreactive B cells and pathological antibodies play a key role in the pathogenesis of MG; However, the production of pathological antibodies is inseparable from CD4^＋helper T (Th) cells. According to its expressed transcription factors and secreted cytokines, CD4^＋Th cells can differentiate into different cell subpopulations, including Th1 cells (CD4^＋IFN-γ^＋), Th2 cells (CD4^＋IL-4^＋), regulatory T (Treg) cells (CD4^＋CD25^＋Foxp3^＋) and newly discovered Th17 cells (CD4^＋ROR γ t^＋CCR6^＋) and follicular assist T (T follicular helper, Tfh) (CD4^＋CXCR5^＋ICOS^＋) cells, etc. Previous studies have suggested that Th2 cells play a leading role in the production of pathological autoantibodies and the pathogenesis of MG; However, with the discovery of new helper T cell subsets, we and other scholars have shown that Tfh and Th17 cells are crucial in B cell assistance, MG ectopic germinal center formation, and anti-AChR antibody production^[2,3,4]。 Let's just call CD4^＋The role of helper T cell subsets and their cytokines in the pathogenesis of MG is discussed in this paper, in order to find the key pathogenic cell subsets, block the activation of these cell subsets and target the cytokines produced by them, so as to provide a theoretical basis for biological targeting therapy of MG.

Brait-Fahn-Schwartz disease is a rare neurodegenerative disease involving both motor system and extrapyramidal system. Its clinical manifestation is typical of Parkinson's disease with amyotrophic lateral sclerosis (ALS). A case of this disease admitted to our hospital is reported.

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant disease, a subtype of spinocerebellar ataxias (SCA). It can involve multiple parts of the cranium, with "ataxia, epilepsy and dementia" as common clinical symptoms. It is extremely rare in China. We identified a family through genetic diagnosis, which is reported below.

The medial temporal lobe structure refers to the arcuate area at the base of the temporal lobe and within the lateral accessory sulcus. Many neurological disorders are closely related to the medial temporal lobe structure and are found in magnetic resonance imaging (MRI) T₂The weighted images showed hyperintensity in the medial temporal lobe of one or both sides. During the acute phase of the disease, T₂Weighted image hyperintensity is mainly seen in viral encephalitis, paraneoplastic limbic lobe encephalitis, vasculitis and status epilepticus; The hyperintensity of chronic phase with atrophy is mainly seen in medial temporal lobe sclerosis and neurodegenerative diseases^[1]。 Neurosyphilis manifesting as unilateral medial temporal lobe lesions is rare. A case of neurosyphilis with unilateral hippocampus and amygdala involvement admitted to our department is reported below.

chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a class of immune-mediated acquired motor sensory nerve demyelinating polyneuropathy. The course of the disease is chronic progression or relief and recurrence, which may be accompanied by cerebrospinal fluid protein-cell separation. The neuroelectrophysiological manifestations are slowed down the conduction velocity of peripheral nerves, conduction block and abnormal dispersion of waveforms. The pathology shows the characteristics of multifocal demyelination of myelinated fibers, intimal edema, inflammatory cell infiltration, coexistence of demyelination and myelination regeneration with onion ball-like changes, etc.

neuromyelitis optica spectrum diseases, including classical neuromyelitis optica (NMO) and its spectrum disorder (NMOSD), are immune-related demyelinating diseases of the central nervous system that mainly involve the optic nerve, spinal cord and special parts of the brain. The clinical characteristics are optic neuritis and long-segment myelitis, with high recurrence rate and disability rate.

homocysteine (Hcy), also known as homocysteine, is a sulfhydryl-containing amino acid that is normally excreted by the kidneys after catabolism. At present, it is generally believed that when the metabolic pathway is blocked and the plasma Hcy concentration is greater than 10~15 μ mol/L, a series of vascular pathological damage will be induced, and the plasma concentration greater than this range is called hyperhomocysteinemia (HHcy)^[1,2]。 At present, a large number of studies have confirmed that HHcy is an independent risk factor for stroke, and can synergistically increase the risk of ischemic cerebral infarction with hypertension^[3]。 The higher the concentration of Hcy in plasma, the more obvious the effect on intracranial blood vessels. In the past three years, some scholars have further found that HHcy has less effect on stroke caused by atherosclerotic plaque formation in large arteries^[4]And plays an important role in the occurrence and evolution of cerebral small vessel disease (CSVD)^[5,6]。 We review the recent research progress of HHcy and CSVD at home and abroad, and summarize the relationship between them as follows.

neuromyelitis optica (NMO) is an acute or subacute inflammatory demyelinating disease of the central nervous system. Previous diagnostic criteria for NMO required optic nerve and spinal cord involvement, but more localized or extensive CNS involvement may also occur clinically. Therefore, in June 2015, the International panel for NMO diagnosis (IPND) revised the nomenclature and diagnostic criteria of NMO, determined to apply the unified terminology of NMO spectrum disorders (NMOSD), stratified into aquaporin-4 (AQP4) -IgG positive and negative groups according to serological results, and formulated new diagnostic criteria. In this paper, the concept evolution of NMO and NMOSD, the characteristics and clinical application of the new diagnostic criteria in 2015 are reviewed.

On July 10-12, 2015, the China Medical Summit and the 8th National Conference on Young and Middle-aged Neurology were grandly held in Chongqing Yuelai International Conference Center. This conference is a celebration of the 100th anniversary of the founding of the Chinese Medical Association. It is sponsored by the Chinese Medical Association and the Neurology Branch of the Chinese Medical Association, undertaken by the Youth Committee of the Neurology Branch of the Chinese Medical Association and Chongqing Medical Association, and co-organized by Daping Hospital of the Third Military Medical University. Professor Pu Chuanqiang, chairman of the Neurology Branch of Chinese Medical Association, served as the chairman of the conference, and Professor Wang Yanjiang, Zhu Yicheng, Jiang Wen and Wang Xiaoshan, deputy chairmen of the Youth Committee of the Neurology Branch of Chinese Medical Association, served as the executive chairmen. Leaders of the Chinese Medical Association, the Neurology Branch of the Chinese Medical Association, the Chongqing Municipal People's Congress, the Health Planning Commission and the Medical Association, and famous experts and scholars at home and abroad attended the meeting, including Academician Chen Zhu, Vice Chairman of the National People's Congress and President of the Chinese Medical Association, Rao Keqin, Secretary of the Chinese Medical Association, a group of international famous experts from Europe and America andScience、Nature CommunicationsEditor-in-chief and editor of the magazine, Professor Cui Liying, chairman-designate of Neurology Branch of Chinese Medical Association, Professor Jia Jianping, former chairman, all standing members and youth members. Before the meeting, President Chen Zhu met with experts from the Neurology Branch of Chinese Medical Association and had a conversation for more than an hour.