2015年 · 第48卷第08期

Over the past 40 years, the global incidence of stroke has decreased by 42 percent in high-income countries and increased by approximately 100 percent in low-and middle-income countries, where stroke rates exceed those in high-income countries^[1]。 In China, cerebrovascular disease has become a major disease that harms the health and life of middle-aged and elderly people. According to the Third National Review Sampling Survey of Cause of Death organized by the former Ministry of Health (now the National Health and Family Planning Commission) from 2004 to 2005, cerebrovascular disease has risen to be the leading cause of death among Chinese residents^[2]。 Stroke is also the leading cause of dysfunction. For stroke patients aged 65 years or older, 26% were unable to take care of themselves in daily life and 46% had cognitive impairment at 6 months after stroke^[3]。 Stroke can not only change the patient's own life, but also change the living conditions of his family, bringing a heavy burden to society and family. Another situation that cannot be ignored is that in recent years, the prevalence of hypertension, diabetes, hyperlipidemia and other diseases among Chinese residents has increased rapidly, and these diseases are clear risk factors for stroke. Therefore, it is expected that the incidence of stroke in China will continue to rise in the near future.

Cervical artery dissection is a rare cause of cerebrovascular disease. Stroke caused by carotid artery dissection accounts for about 2% of all ischemic strokes, but the proportion is as high as 8% to 25% in young stroke population^[1,2]Carotid artery dissection is an important risk factor for stroke in young people. Previously, due to insufficient understanding of the disease and limited auxiliary examination methods, the incidence of carotid artery dissection may be underestimated, which also led to uneven treatment methods and levels. With the changes of social lifestyle and disease spectrum and the development of medical imaging technology, clinicians' understanding of carotid artery dissection is gradually deepened, and more and more patients with carotid artery dissection have been diagnosed and treated. It should be pointed out that there are not many related studies in China at present. Under this background, the Cerebrovascular Disease Group of the Neurology Branch of the Chinese Medical Association organized the compilation of the Guidelines for the Diagnosis and Treatment of Cervical Artery Dissection in China 2015^[3]This paper summarizes the research progress of carotid artery dissection in recent years, and puts forward recommendations based on the current evidence-based medical evidence for the diagnosis and treatment of carotid artery dissection, aiming to further improve the clinical understanding of carotid artery dissection, standardize the diagnosis and treatment behavior of carotid artery dissection, and improve the diagnosis and treatment level of clinicians for carotid artery dissection.

The report of the third national retrospective sampling survey of causes of death completed in 2004-2005 shows that cerebrovascular disease has jumped to the top of the causes of death in China^[1]。 Acute cerebrovascular disease (stroke) is a single disease with the highest disability rate. Its high morbidity, mortality and disability rate bring heavy burden and great pain to society, family and patients. Although the diagnosis and treatment technology of cerebrovascular disease has made great progress in recent years, and the prognosis of patients has been greatly improved, because the pathophysiological process of most stroke patients cannot be reversed, the best way to reduce the economic burden of cerebrovascular disease is prevention, especially primary prevention, that is, actively carry out early intervention and prevention according to the risk factors of cerebrovascular disease to reduce the occurrence of stroke. 2005 Guidelines for the Prevention and Treatment of Cerebrovascular Diseases in China^[2]It has been promoted nationwide, including recommendations for primary prevention of cerebrovascular diseases. In 2010, the Cerebrovascular Disease Group of the Neurology Branch of the Chinese Medical Association comprehensively revised the "Guidelines" and issued the Guidelines for Primary Prevention of Stroke in China 2010^[3]。 Based on the recent research progress at home and abroad and the update of foreign guidelines^[4]At the end of 2014, the Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association updated and revised the "Primary Prevention Guidelines" again, and the references are as of December 2014.

cervical artery dissection (CAD) refers to the intimal tear of the carotid artery, which causes blood to flow into its wall to form intramural hematoma, which then causes artery stenosis, occlusion or aneurysm-like changes, mainly internal carotid artery dissection (ICAD) and vertebral artery dissection (VAD). The incidence rate of CAD is about (2.6~3.0) /100,000 person-years, including ICAD incidence (2.5~3.0) /100,000 person-years, VAD incidence (1.0~1.5) /100,000 person-years, about 13% ~16% of patients have multiple artery dissection^[1,2]。 Despite its low incidence, CAD is an important cause of stroke in young people. Foreign data show that CAD causes about 2% of all ischemic strokes, and the proportion can be as high as 8% ~25% in young people younger than 45 years old^[3,4]。 A small sample study in China showed that acute ischemic stroke caused by CAD accounted for 7.49% of ischemic strokes in this age group in the same period^[5]。 Due to the lack of large sample studies in epidemiology, diagnosis and treatment of CAD at home and abroad, and the lack of attention by clinicians, it is easy to lead to missed diagnosis or misdiagnosis. Therefore, we have developed this guideline based on current domestic and foreign research results for clinicians' reference. CAD can be classified into traumatic (open) or spontaneous CAD depending on the cause of triggering. This guideline is mainly for spontaneous CAD (extracranial segment). The formulation principles, recommendation strength and evidence level standards of this guideline follow the relevant guidelines of Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association^[6]。

In 1982, Rune Aaslid applied transcranial doppler ultrasound (TCD) to clinic. TCD gradually became an important method for diagnosing intracerebral artery stenosis. TCD microembolus monitoring began in 1990s, and was initially discovered by Spencer during intraoperative monitoring of carotid endarterectomy patients. Solid embolus signals were found^[1]Subsequently, it was found that microembolic signals (MES) could be detected in atrial fibrillation, intracranial and extracranial aortic stenosis, etc. MES is an independent risk factor predicting the occurrence of stroke events. It has been reported that MES may also occur in intracranial blood vessels of patients with connective tissue diseases, such as systemic lupus erythematosus (SLE), Behcet's disease, Takayasu arteritis, etc.^[2]This discusses the etiological mechanism related to central nervous system symptoms in connective tissue diseases, and broadens the clinical application scope of TCD microembolus monitoring. The mechanism and clinical significance of this type of MES are not yet clear. We used TCD technique to detect cerebral artery MES in SLE patients in our hospital, to explore the signal characteristics and clinical significance of cerebral artery microembolus in SLE patients.

hypertrophic cranial pachymengitis (HCP) is a rare disease characterized by focal or diffuse inflammation, fibrosis, hyperplasia and hypertrophy of the dura mater. The main clinical manifestations are chronic headache and polycerebral nerve palsy. This disease combined with orbital inflammatory pseudotumor (OIP) is rarely reported. One patient admitted to our hospital is reported as follows.

primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin's lymphoma with primary intracranial origin. Clinically, it is often manifested by progressive encephalopathy or focal central nervous system signs. Enhanced scanning on imaging can find lesion enhancement. A case of PCNSL with viral encephalitis as the main manifestation admitted to us is reported below.

The middle cerebellar foot (MCP), also known as the brachium pontificium, is mainly supplied by the anterior inferior cerebellar artery (AICA) and in part by the superior cerebellar artery (SCA)^[1]。 Brachiopontine infarction accounted for 0.9% of acute cerebral infarction and 5.2% of vertebrobasilar artery stroke, respectively^[2]While isolated bilateral infarction is clinically rare^[3,4]。 At present, the report of bilateral MCP infarction is limited to individual cases, and its mechanism is still unclear. Cerebellar ataxia and vertigo were the most common clinical manifestations. MRI examination showed bilateral symmetric circular signal changes in MCP, and most vascular examinations showed vertebrobasilar artery occlusion or severe stenosis.

At present, it is believed that anti-gamma-aminobutyric acid B (GABA-B) receptor encephalitis is a kind of borderline encephalitis, which is mainly related to cell membrane antigen antibody. GABA-B receptor is a G-protein-coupled receptor, which belongs to an inhibitory synaptic protein. It plays an important role in neurotransmitter transmission and maintaining synaptic stability, and is widely distributed in the central and peripheral nervous systems. The highest concentrations in the central nervous system were in the hippocampus, thalamus and cerebellum^[1]。 Animal studies have found that knocking or deleting the GABA-B receptor through drugs or genes can cause epilepsy and cause memory, learning and behavior disorders^[2]。 Clinically, most patients with anti-GABA-B receptor encephalitis have epilepsy as the main onset symptom, accompanied by symptoms of borderline encephalitis such as memory impairment, confusion of consciousness and behavior, disorientation, etc. A small number of patients may develop cerebellar ataxia, nystagmus, limb clonus, autonomic dysfunction, ventilation disorder, and psychological symptoms such as hallucination vision^[3,4,5,6]。 A case of anti-GABA-B receptor encephalitis admitted to our department is reported as follows.

intracranial atherosclerosis is the main pathological basis of ischemic stroke in Asian population^[1], the pathogenesis of symptomatic intracranial atherosclerotic disease is associated with hemodynamic hypoperfusion, arterial-arterial embolism, vector atherosclerotic plaques involving perforator artery openings. Among them, the pathological basis of arterial-arterial embolism mainly depends on the nature of atherosclerotic plaque, which is closely related to the instability of plaque. Histopathological studies showed that unstable plaques were characterized by lipid-rich necrotic cores, thin or ruptured fibrous caps, intraplaque hemorrhage^[2]。 Atherosclerotic plaques that are small in size and do not result in significant arterial stenosis may also be unstable plaques. However, traditional angiography techniques such as transcranial Doppler ultrasound (TCD), computed tomography angiography (CTA), magnetic resonance cerebral angiography (MRA) and digital subtraction angiography (DSA) have the characteristics of relatively low sensitivity and specificity, and cannot show the morphological and structural characteristics of arterial wall and atherosclerotic plaque, so it is difficult to evaluate the stability of atherosclerotic plaque, which is not conducive to the judgment of stroke risk. The emerging high-resolution magnetic resonance imaging (HR MRI) technique can be used to identify the presence of microarterial atherosclerotic plaques, reveal the morphological and structural characteristics of arterial wall and atherosclerotic plaques, thereby helping to identify the nature of intracranial artery occlusive lesions, assess the vulnerability of intracranial atherosclerotic plaques, clarify the etiology, mechanism and risk of recurrence of stroke, and guide antithrombotic and endovascular interventional therapy^[3]。 Based on this, we review the research progress of HR MRI and intracranial atherosclerosis in the literature as follows.

neuromyelitis optica (NMO) is an immune-mediated inflammatory demyelinating disease of the central nervous system that mainly involves the optic nerve and spinal cord. Since the discovery of the NMO serum autoantibody marker against aquaporin – 4 (AQP4) IgG antibody^[1]NMO is gradually recognized as a disease entity different from multiple sclerosis (MS). Anti-AQP4-IgG antibodies are involved in the immunopathogenic mechanism of NMO. Applying the existing detection methods, anti-AQP4-IgG antibodies can be detected in the sera of about 88% of NMO patients^[2]。 Therefore, anti-AQP4 – IgG antibodies are also known as NMO – IgG and were included as a supporting standard in 2006 by Wingerchuk et al.^[3]Revised diagnostic criteria for NMO. This diagnostic criteria requires optic neuritis and acute myelitis, and at least 2 of the 3 supporting criteria must be met simultaneously: (1) spinal cord MRI lesions ≥3 vertebral body segments; (2) Cranial MRI did not meet the diagnostic criteria of MS; (3) Serum NMO-IgG positive. However, in clinic, some patients with optic neuritis or myelitis only meet some diagnostic criteria for NMO, and serum anti-AQP4-IgG antibody is positive, which is classified as neuromyelitis optica spectrum disorders (NMOSDs), including: (1) NMO; (2) the lesions are limited to the optic nerve and spinal cord: idiopathic unitemporal or recurrent longitudinally extensive transverse myelitis (LETM; MRI spinal cord lesions ≥3 vertebral body segments), and recurrent or concurrent bilateral optic neuritis; (3) Asian type of optic neurospinal MS; (4) optic neuritis or LETM combined with autoimmune disease; (5) Intracranial lesions characterized by optic neuritis or myelitis with NMO (hypothalamus, corpus callosum, periventricular and brainstem)^[4]。

The migraine classification in the 2013 International Headache Association Headache Classification Third Edition (ICHD-3) included menstrual migraine as a subtype of migraine without aura. This is due to the difference in estrogen and progesterone levels in women of childbearing age throughout the menstrual cycle, which is a subtype of headache with a temporal relationship with menstruation. It has different prevalence and clinical characteristics. Pathogenetically, the estrogen level of patients is strongly correlated with it. Although the relationship between migraine and sex hormones has been repeatedly verified, the pathophysiological mechanisms involved in migraine have not been fully elucidated^[1]。

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective invasion of upper and lower motor neurons. Most of them die within 3 to 5 years after diagnosis. Studies confirm that nutritional status of patients with ALS is also an independent risk factor for prognosis and survival^[1,2]。 In addition to early respiratory muscle involvement, energy metabolism is already impaired in the early stage of ALS. Improving the nutritional status of patients through gastrointestinal fistula, especially solving the insufficient intake of dysphagia caused by medulla oblongata involvement, is one of the important measures to intervene in the impaired energy metabolism, maintain the nutrition of patients, and then prolong their survival time^[3,4]。 percutaneous endoscopic gastrostomy (PEG) is listed as a standard nutritional measure for ALS in the guidelines of the American Neurological Association and the European Union Neurological Society^[5,6]。 However, there is no broad consensus on the time of PEG implantation, and there are still some controversies on the advantages and disadvantages of PEG implantation and the survival time of PEG. The reasons are not only related to the insufficient clinical research of PEG in these aspects, but also to the fact that the complex relationship between energy metabolism and neurodegenerative diseases has not been revealed^[7]。 We summarize the recent research progress on the necessity of intervention for impaired energy metabolism in patients with ALS, as well as the benefits to clinical PEG and the risks associated with respiratory function, as follows.

Animal models are an important way to study Alzheimer's disease (AD). Since the 20th century, with the emergence of various animal models, people's understanding of the disease has reached an unprecedented height^[1,2]。 Animal models have made important contributions to the exploration of disease etiology, pathogenesis and clinical drug screening. However, due to the current technical level, it is still difficult to realize animal models that can completely simulate the pathological process and clinical characteristics of human Alzheimer's disease. Each animal model can only show some aspects of pathological characteristics or clinical characteristics of human diseases. In addition, as an indirect study, the research results obtained by model experiments are not necessarily fully applicable to explaining the disease process of humans themselves. This theory obtained from animal experiments and its transformation and application effects finally need to be verified on humans. We aimed to summarize the progress of AD animal models, and to explore their translational utility and value in AD research, so as to provide reference for the development of AD animal models in the future.

Parkinson's disease (Parkinson's disease) is a group of neurodegenerative diseases with substantia nigra neuronal degeneration as the main pathological feature and bradykinesia as the prominent clinical manifestation. It mostly occurs after the age of 50. However, it has been found that many patients with Parkinson's disease have an early onset age and have a relatively unique disease phenotype. Therefore, according to the age of onset, Parkinson's disease can be divided into early-onset Parkinson's disease (EOPD) and late-onset Parkinson's disease (LOPD). The incidence of EOPD is relatively low, accounting for about 10% of Parkinson's disease. Compared with LOPD, EOPD had differences in onset symptoms, response to dopamine treatment, course of disease, and incidence of cognitive impairment. The relationship between genes and EOPD is very close. Up to now, there are 11 genes with exact relationship with EOPD. The molecular biological characteristics of these genes, epidemiological distribution and the relationship between genes and disease phenotype are the hot topics of research at present. Besides genetic factors, the role of other factors (such as environmental factors) on EOPD is not clear.

From March 27 to 29, 2014, China Cerebrovascular Disease Conference (CCCD) 2014 was held in Changsha, Hunan Province. The conference was sponsored by Chinese Medical Association and Neurology Branch of Chinese Medical Association, undertaken by Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association, Hunan Medical Association and Neurology Professional Committee of Hunan Medical Association, and co-organized by the National Key Discipline of Neurology of Central South University. The opening ceremony was presided over by Professor Xiao Bo, Standing Member of Neurology Committee of Chinese Medical Association and Chairman of Hunan Neurology Committee. Professor Liu Ming, Leader of Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association, delivered a welcome speech, Professor Pu Chuanqiang, Chairman of Neurology Branch of Chinese Medical Association, delivered an opening speech, Professor Liu Jiawang, President of Hunan Medical Association, and Professor Rao Keqin, Secretary of the Party Committee of Chinese Medical Association, also delivered congratulatory speeches on behalf of Hunan Medical Association and Chinese Medical Association respectively. Professor Tian Yongquan, Vice President of Central South University, Long Kaichao, Deputy Director of Hunan Provincial Health Department, and Xiao Ping, Secretary of Xiangya Hospital of Central South University, visited the venue for guidance. More than 2,500 well-known experts and scholars engaged in cerebrovascular diseases and related fields at home and abroad, including Professor Pu Chuanqiang, Chairman of Neurology Branch of Chinese Medical Association, Professor Jia Jianping, Former Chairman, Professor Cui Liying, Chairman-designate, and Professor Stephen Davis, Chairman of World Stroke Organization, gathered in Changsha, the star city. A total of 14 conference reports, 59 special speeches, 8 case discussions, 57 oral papers, 236 posters and 317 written papers were arranged at the conference. The latest experimental results, research progress, clinical guidelines and some hot issues in clinical work of cerebrovascular diseases were comprehensively discussed. The main contents of the meeting are divided into the following aspects.

The 17th National Conference on Neurology of Chinese Medical Association was held in Xiamen, Fujian Province from September 18 to 20, 2014. This annual conference is sponsored by the Chinese Medical Association and the Neurology Branch of the Chinese Medical Association, and undertaken by Xiamen Medical Association and Fujian Neurology Branch. The conference attracted more than 3,760 doctors in neurology and related disciplines in China to sign up enthusiastically, and received a total of 3,567 papers; The number of participants and manuscripts are the highest in all previous annual meetings. The annual meeting fully demonstrated new views, new technologies and new achievements in the field of neurology at home and abroad; At the same time, this year marks the 20th anniversary of the establishment of the Neurology Branch of the Chinese Medical Association. The conference also specially invited the chairmen and chairmen of the World Federation of Neurology Societies and the Neurosurgery Branch, Psychiatry Branch and Medical Genetic Diseases Branch of the Chinese Medical Association to attend the conference and make keynote speeches. Relevant leaders of the Chinese Medical Association also came to celebrate.