2017年 · 第50卷第05期

China has become the country with the largest aging population in the world. The sixth census shows that there are 178 million elderly people over 60 years old in China, accounting for 13.26% of the total population. At present, the prevalence of Alzheimer's disease (AD) among the elderly in China is 3% ~7%^[1]。 AD has a high disability rate, patients lose the ability to live independently in the late stage, and completely need the care of others, which brings a heavy economic burden to society and families. It has become one of the main diseases that seriously endanger the health of the elderly and affect the sustainable development of China. How to carry out effective prevention and treatment for it is a major challenge to cope with the aging society.

Alzheimer's disease (AD) is a neurodegenerative disease with cognitive dysfunction as the core clinical symptom. It has become an important disease that seriously affects the quality of life and physical and mental health of the elderly because of its latent onset, difficulty in early diagnosis and recognition, and limited intervention measures and treatment. According to the Global Alzheimer's Disease Report published by AD International in 2016^[1]At present, the number of AD patients in China has reached more than 8 million, and the incidence of AD among the elderly over 65 years old is 4% ~6%, which has brought a heavy social and economic burden to developing China.

vascular parkinsonism (VP) is a type of secondary parkinsonism. It was first described and named arteriosclerotic parkinsonism by Critchley in 1929, but the role of vascular factors in parkinsonism was controversial in the subsequent decades. With the progress of clinical pathology research, imaging techniques such as CT and MRI can also clearly show white matter lesions, cerebral infarction and cerebral hemorrhage, and the role of vascular factors in Parkinson's syndrome has gradually been recognized. In recent years, VP has attracted increasing attention, but at present, there is a lack of standardized diagnostic criteria and treatment options for VP in the world, and there is little evidence in clinical research and lack of evidence in domestic research. With the increase of the elderly population in China, the number of patients with diseases such as stroke and hypertension is also increasing, and the prevalence of VP will further increase. In order to better standardize the diagnosis and treatment of VP in China, we formulated an expert consensus on the diagnosis and treatment of VP in China based on relevant literature and clinical research results at home and abroad, and introduced the epidemiology, pathology, clinical manifestations and imaging characteristics of VP, so as to better guide clinical practice and standardize the diagnosis and treatment of VP.

neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system^[1,2]Aquaporin 4 antibody (AQP4-IgG) is its specific antibody^[3]。 Wingerchuk et al.^[4]A group of central nervous system demyelinating diseases whose pathogenesis and clinical features are similar to those of NMO are uniformly designated as neuromyelitis optica spectrum diseases (NMOSD). In 2015, the International NMO Diagnostic Group (IPND) eliminated the separate definition of NMO and integrated NMO into the broader category of NMOSD. We identified a case of NMOSD with fever as the first symptom, which is now reported below.

intravascular lymphoma (IVL) is a rare intravascular malignancy first developed by Pfleger and Tappeiner in 1959^[1]Description, and designated systemic vascular endotheliopathy. In 2001, WHO classified it as a subtype of diffuse large B-cell lymphoma, which belongs to the non-Hodgkin's lymphoma type^[2]。 This disease is more common in middle-aged and elderly people. Its pathological feature is that tumor cells proliferate in large numbers in the lumen of small blood vessels, usually involving the central nervous system, but also skin, lung, liver, spleen and other organs, showing malignant progression and poor prognosis. A case of intravascular large B-cell lymphoma with repeated stroke-like attacks and multiple system damage is reported.

cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive genetic disease caused by mutation of CYP27A1 gene. The incidence of CTX is low, the clinical manifestations are diverse, and it is easy to be misdiagnosed and missed. Since the first case of CTX was reported by Von Bogaert et al. in 1937, only a few hundred cases of CTX have been reported globally and only 14 cases have been reported domestically^[1]。 One patient with genetically and pathologically confirmed CTX admitted to us is reported below.

Gliosarcoma is one of the malignant tumors in the central nervous system. It is a primary tumor containing two mixed components: glioblastoma and sarcoma. Headache, hemiplegia, epilepsy, nausea, and personality changes are common. It is mostly in the temporal lobe, but it is rare to occur in bilateral lateral ventricles, and there are no relevant reports at home and abroad. One case we treated is now reported as follows.

Parkinson's disease-cognitive impairment (PD-CI) refers to cognitive impairment that occurs in patients with Parkinson's disease. PD-CI not only reduces the quality of life of patients with Parkinson's disease^[1]It also improves the mortality rate of patients with Parkinson's disease^[2]。 Therefore, it is particularly important to identify the risk factors of PD-CI and intervene in time. rapid eye movement sleep behavior disorder (RBD) is one of the important non-motor symptoms of Parkinson's disease, and its relationship with PD-CI has attracted gradual attention in recent years, so we review it.

Alzheimer's disease (AD) is a common degenerative disease of the central nervous system in the elderly. Its characteristic pathological changes include amyloid plaques formed by abnormal aggregation of amyloid-β (A β) between nerve cells and neurofibrillary tangles (NFTs) formed by abnormally phosphorylated microtubule-associated protein (tau) aggregation in cells. mammalian target of rapamycin (mTOR) is a highly conserved serine/threonine protein kinase, which plays an important role in cell growth, proliferation and metabolism. Abnormalities in the mTOR signaling pathway are closely associated with many diseases, such as cancer, metabolic diseases, and neurological diseases. A large number of in vitro and in vivo studies have confirmed that the abnormal increase of mTOR activity is an important reason for inducing a large number of A β and tau protein aggregation, while mTOR inhibitor rapamycin can alleviate the pathological changes of AD and improve its cognition level. We combined recent findings to explore the mechanism of action of the mTOR pathway in AD and the potential therapeutic effects of mTOR inhibitors on AD.

leukoencephalopathy with vanishing white matter (VWM), also known as childhood ataxia with central nervous system hypomyelination (CACH), is an autosomal recessive leukodystrophy that has been gradually recognized in recent years, with more onset than childhood and great clinical heterogeneity. Some female patients, who onset in adulthood, are often accompanied by premature ovarian failure in addition to VWM manifestations. First written in 1962 by Eicke;^[1]Report, 1997 Schiffmann et al.^[2]Similar cases were summarized and this disease was designated as ovarian leukodystrophy (OLD), which is now considered a variant of VWM. At present, about 30 cases of OLD have been described in detail in foreign literature, and more than 20 cases have been genetically confirmed, while only one case has been reported in China^[3]。 The clinical characteristics, imaging manifestations, pathological and genetic characteristics of OLD are reviewed systematically in order to improve the awareness and attention of relevant doctors and avoid misdiagnosis or misdiagnosis.

Encephalitis is one of the common diseases of the central nervous system. According to the cause of the disease, it can be roughly divided into two categories: infectious and non-infectious encephalitis. autoimmune encephalitis (AE) is an important component of non-infectious encephalitis, which generally refers to a large class of diseases caused by the immune system responding to central nervous system antigens^[1]。 According to the specificity of antibodies, they are divided into specific antigen-antibody-related AE and non-specific antigen-antibody-related AE. The former is divided into cell surface antibody and neuronal antibody (cytoplasmic antibody) according to the different types of antibody mediation. According to the involvement site of the lesion, it can be divided into marginal lobe type, outside marginal lobe type, mixed type and no significant change type^[2]。 GABABR antibodies against γ-aminobutyric acid B type receptor (GABABR) antibody-associated encephalitis belong to cell surface antibodies, and their associated encephalitis often manifests as limbic lobe encephalitis, and more than half of them are accompanied by tumors (mostly small cell lung cancer). 2007 Dalmau et al.^[3]After the concept of autoimmune "anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis" was first proposed, a series of new receptors such as leucine-rich glioma inactivating 1 protein (IGI1) receptor, GABABR, IGLON family protein 5 antibody (IGLON5) receptor have been discovered and reported successively. A case report of anti-GABABR antibody-associated encephalitis was reported in 2010 by Lancaster et al.^[4]First published inLancet NeurologyOn. With the concept of AE and the continuous discovery of new antibodies, people's understanding of this type of immune-mediated encephalitis is deepening, and more and more cases have been discovered and reported. In this paper, we will summarize the latest literature at home and abroad, summarize and analyze its pathogenesis, clinical manifestations, auxiliary examination, diagnosis, differential diagnosis, treatment and prognosis, so as to provide reference for the clinical diagnosis and treatment of this disease.

Stroke is the leading cause of death or disability in Chinese residents, among which ischemic stroke accounts for about 80%. The treatment of acute phase is extremely important for the prognosis of stroke patients. Intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) is recommended as the first choice for the treatment of acute ischemic stroke (AIS) within 4.5 hours of onset^[1]。 However, evidence-based medicine has proved that the number of patients who can arrive at the hospital within the time window and have indications for thrombolysis is very limited, and the proportion of large vessel occlusive stroke that can achieve recanalization after intravenous thrombolysis is low^[2]。 Against this background, multimodal revascularization methods have emerged in recent years. We review the research progress of AIS revascularization therapy, aiming to improve clinicians' understanding of this treatment method, so as to facilitate the individualized treatment of AIS patients.

muscle ultrasound is the use of ultrasound technology to perform muscle imaging, which is non-invasive, painless, safe and convenient. Muscle ultrasound is widely used in clinic, including evaluating muscle trauma, hematoma, myopathy, differentiating abnormal muscle movements (such as fascicular fibrillation) or assisting in the localization of electromyography of special sites (such as phrenic nerve conduction or diaphragmatic needle electromyography). Muscle ultrasound can also be used to detect denervation of muscles, assessing the progression of denervated diseases, such as spinal muscular atrophy^[1]And amyotrophic lateral sclerosis (amyotrophic lateral sclerosis, ALS)^[2,3,4]。

January 4, 2017 in Authoritative Journal of Cardiovascular FieldCirculationThe Epidemiological Survey of Stroke in China conducted by the Ness-China (National Epidemiological Survey of Stroke in China) research collaboration group was published online^[1]。 The research was funded by the "12th Five-Year Plan" Science and Technology Support Program of the Ministry of Science and Technology of China, led by Professor Wang Wenzhi of the National Cerebrovascular Disease Prevention and Treatment Research Office, and jointly completed by the Chronic Disease Center of the National Center for Disease Control and Prevention (CDC), organizing the personnel of CDC in various provinces and neurology departments in more than 30 hospitals. In order to ensure the national representation, the researchers adopted a multi-stage stratified sampling method, taking into account both the geographical distribution and the level of economic development. The survey points were selected from six layers in the east, middle, west, urban and rural areas. Finally, the results of 155 survey points in 31 provinces in China were included in the analysis, and the total sample population of the survey was nearly 600,000.

Nervous system antigen-specific autoimmune disease is a subacute onset that can involve the entire nervous system and is sensitive to immunotherapy. The presentation of subacute or occult onset makes it susceptible to misdiagnosis as infectious, degenerative, demyelinating, neoplastic or vascular disease. Detection of specific IgG in neurons, glial cells, skeletal muscle in serum or cerebrospinal fluid can help in diagnosis and guide the selection of appropriate treatment options. The occurrence of paraneoplastic syndrome is related to the immune response stimulated by neural antigens produced by occult tumors, and the abundant autoantibody spectrum predicts the possibility of high cancer incidence. Therefore, the detection of nervous system-specific autoimmune antibodies can not only help in diagnosis, but also provide clues for clinicians to detect occult tumors early. Recently, the research team of Dr. Fang Boyan of the Neuroimmune Laboratory of Mayo Medical Center, with the support of Professor Lennon, discovered an astrocyte autoantibody-glial fibrillary acidic protein antibody (GFAP-IgG), which can be used as a new specific biomarker of recurrent autoimmune meningoencephalomyelitis, which has been published inJAMA NeurolMagazine^[1]The main contents of the full text are now introduced as follows.