2015年 · 第48卷第05期

The management of acute phase of ischemic stroke is described in detail in the 2010 edition of "Chinese Guidelines for Diagnosis and Treatment of Acute Ischemic Stroke" and the 2011 edition of "Chinese Guidelines for Diagnosis and Treatment of Ischemic Cerebrovascular Disease by Endovascular Intervention"^[1,2]。 They consistently recommended intravenous thrombolysis as the first choice in the treatment of acute ischemic stroke. However, evidence-based medicine has confirmed that the proportion of large vessel occlusive stroke can achieve recanalization after intravenous thrombolysis is relatively low^[3]。 Against this background, the successful development and clinical application of stent-like thrombectomy devices (Solitarie FR and TRVEO) made the endovascular treatment of acute ischemic stroke a brilliant achievement in 2012^[4,5]。 However, the IMS-Ⅲ trial published in 2013^[6]SYNTHESIS Expansion test^[7]MR RESCUE test^[8]The results of the study consistently show that the efficacy of endovascular intervention in the treatment of acute ischemic stroke is not superior to intravenous thrombolysis. While the MR CLEAN trial published in 2014^[9]As a result, cerebrovascular intervention practitioners found some comfort in the "academic winter": the study showed that the combination treatment group (drug combined with endovascular therapy) showed a better prognostic distribution at 90 days compared with the standard drug treatment group [modified Rankin scale (mRS) score 0-6]. Stroke has now become the first fatal disease in China^[10]In order to further standardize the early endovascular interventional diagnosis and treatment of acute ischemic stroke, the Neurovascular Intervention Collaborative Group of Neurology Branch of Chinese Medical Association led the establishment of a guideline writing group for interventional diagnosis and treatment of acute ischemic stroke, which was repeatedly discussed and revised in March and November 2014, so that the "Chinese Guidelines for Early Endovascular Intervention in Acute Ischemic Stroke" was finally finalized and published in this issue (hereinafter referred to as "the Guidelines"). This guideline stems from a systematic review of available evidence-based medicine. Compared with the previous guidelines, the biggest feature of this guideline is that it only involves the opinions and methods for the treatment of acute stroke caused by macrovascular disease, which has strong clinical relevance and operability, and updates the previous treatment views, which is worth in-depth study.

At present, the number of deaths due to stroke in China every year has surpassed that of tumors and cardiovascular diseases, becoming the first cause of death. Treatment in the acute phase is extremely important for the prognosis of stroke patients. Evidence-based medicine has confirmed that intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) within 4.5 h of onset is the preferred method for the treatment of acute ischemic stroke. However, that number of patients who can reach the hospital within the time window and have indication for thrombolysis is very limited^[1]； In addition, the recanalization rate of large vessel occlusive stroke after intravenous thrombolysis is low. For example, the recanalization rate of M1 segment of middle cerebral artery is about 30%, and the recanalization rate of terminal internal carotid artery is only 6%^[2]。 The presence of these factors largely limits the wide application of rt-PA in clinical practice.

Multiple sclerosis (MS) is an immune-mediated disease characterized by inflammatory demyelinating lesions of the white matter of the central nervous system (CNS). The etiology is still unclear, but it may be related to genetics, environment, viral infection and other factors. MRI imaging manifestations are extensive demyelination of CNS white matter accompanied by oligodendrocyte degeneration, and may also be accompanied by nerve cells and their axons degeneration. MS lesions have multiple temporal and spatial characteristics^[1,2,3]。

Unilateral transient neuralgia-like headache with conjunctival hyperemia and tearing (Short-lasting unilateral neuralgiform headache with conjuctival injection and tearing, SUNCT) It is a rare headache, which is more common in middle-aged and elderly people and rare in children. A child with SUNCT was admitted to our department and treated by botulinum toxin A injection. After 9 months follow-up, there was no recurrence. After reviewing the literature, there is no report in China.

Anton syndrome is a rare visual absence denial disorder. On the basis of bilateral cortical blindness, the patient himself cannot feel the presence of blindness and firmly believes that he can see what is actually completely fictional^[1,2]。 Although various etiologies of cortical blindness may contribute to Anton syndrome, bilateral occipital infarction is the most common cause, whereas Anton syndrome caused by bilateral posterior cortical watershed infarction is rare. We treated a patient with bilateral posterior cortical watershed infarction presenting with Anton syndrome caused by third-degree atrioventricular block combined with atrial fibrillation, which is reported below.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first developed by Vitaliani et al.^[1]Reported autoimmune encephalitis, with or without ovarian teratoma. The clinical manifestations of patients with anti-NMDAR encephalitis include prodromal hyperthermia, psychiatric symptoms, epileptic seizures, disturbance of consciousness, etc^[2]； Cerebrospinal fluid and serum anti-NMDAR antibody^[2]The test was positive, and the EEG showed specific delta brush^[3]Performance. One adult patient diagnosed in our hospital is reported as follows.

Hypertension is the most common disease with the highest incidence and the highest incidence of disability and death^[1]。 In 2013, the World Health Organization took hypertension prevention and control as the theme of World Health Day for the first time, emphasizing the need to reduce the risk of cardiovascular and cerebrovascular diseases by controlling hypertension, highlighting the importance of hypertension prevention and treatment. Hypertension not only promotes cerebral arteriosclerosis, increases the risk of stroke, but also affects cognitive function. It is estimated that there will be 266 million patients with hypertension in China in 2012, and 70% of stroke patients are related to hypertension^[2]Cerebrovascular disease has become the first cause of death among our nationals^[3]。 In recent years, it has been found that mean blood pressure has little predictive effect on coronary events and little predictive effect on stroke events, while blood pressure variability (BPV) has a positive effect on predicting cerebrovascular events. BPV research has become a hot spot in the field of blood pressure research^[4]。 The larger the BPV, the greater the impact on vital target organs. We aimed to review the relationship between BPV changes and cerebral artery, stroke, dementia and other cerebrovascular events through the research of BPV at home and abroad in the past 2 years.

International understanding of Alzheimer's disease (AD) has been developed from the 1984 National Association of Neurology and Language Disorders, Stroke and Alzheimer's Disease and Related Diseases^[1]Diagnostic criteria for AD dementia, 2007^[2]2010^[3]And 2014^[4]prodromal AD, presymptomatic AD, asymptomatic AD proposed by the international working group, and mild cognitive impairment due to AD (MCI due to AD) proposed by the National Institute on Aging and the Alzheimer's Association in 2011^[5]and preclinical AD^[6]The subjective cognitive decline (SCD) of pre-clinical AD has developed into today's^[7]Stage. The concept of SCD puts forward an important warning for us to correctly screen those with normal cognition in clinical research in the future; At the same time, it makes us realize that in the continuous course of disease from cognitive normality to dementia, in addition to the MCI stage, there can be SCD stage before it. If AD can be correctly identified in the SCD stage, it is of great significance for the early prevention and treatment of AD. So how to identify SCD in the preclinical stage of AD early?

In clinical work, we often observe that patients get better after receiving placebo treatment. At present, there is more and more evidence that the placebo effect is a neurobiological phenomenon. Placebo analgesia is the most common placebo effect in clinic, and its core is the experience of placebo relieving pain. The results of recent studies in neuropsychology, neurobiology and neuroimaging have largely confirmed that the placebo analgesic effect is real. We will explore the relevant mechanisms of placebo analgesia from the above aspects.

tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease mainly caused by hamartoma lesions and neuropsychiatric symptoms in multiple organs of the body. Facial fibroangioma, depigmentation spots, renal angiomyolipoma, cerebral cortical nodules, subependymal nodules, pulmonary lymphangioleiomyomatosis, epilepsy, autism, intellectual disability, etc. may occur according to the site of the involved lesions^[1]。 The incidence of this disease ranges from 1/10 000 to 1/6 000, and there are about 1.5 million patients worldwide. The etiology is due to the loss of function of TSC1/TSC2 protein dimer due to the mutation of TSC1 gene or TSC2 gene (encoding hamartoma protein and potato globulin, respectively), the activity of mammalian target fo rapamycin (mTOR) pathway is enhanced, and the cell growth and proliferation are out of control. Rapamycin can directly inhibit the activity of mTOR pathway. Both animal experiments and clinical studies have demonstrated that rapamycin and its derivative everolimus can improve clinical symptoms, especially neurological symptoms, in patients with tuberous sclerosis^[2,3]It has brought dawn for the research and treatment of TSC, a refractory genetic disease.

Psychogenic pseudoepileptic seizures (PNES) refer to a group of episodic events caused by psychological factors, including motor, sensory, autonomic, behavioral, cognitive and other changes, which are similar to epileptic seizures but do not have corresponding EEG abnormalities. An et al.^[1]A study of PNES patients in southwestern China found that 31.3% of patients were misdiagnosed with epilepsy. Due to insufficient knowledge of the disease, the average diagnosis time took about 7 years^[2]As a result, most patients with PNES inappropriately take antiepileptic drugs, which not only fail to control the disease, but may induce or aggravate seizures and be misdiagnosed as "refractory epilepsy". Video electroencephalography (vEEG) is the "gold standard" for diagnosis of PNES^[3]However, it is not a routine examination and is not widespread nationwide. Therefore, the relevant clinical data provided by patients or eyewitnesses is particularly important for the early diagnosis of PNES. We reviewed the clinical characteristics and research progress of PNES, aiming to improve the understanding of clinicians and facilitate the early diagnosis and treatment of PNES.

Amyotrophic lateral sclerosis (ALS) is a fatal chronic progressive neurodegenerative disease. The etiology and pathogenesis are not yet clear. Studies have shown that its mechanism may involve the disruption of DNA/RNA and protein homeostasis, hyperphosphorylation of tau protein, inflammatory response, iron metabolism and calcium homeostasis disorders, etc. Although 90% ~95% of ALS have no family genetic history, different genotypes of ALS can affect the treatment, progression and prognosis of the disease, so genetics is still a hot research topic. So far, the incidence of ALS related genes from high to low are C9orf72, SOD1, TARDBP, FUS, ANG, ATXN2, UBQLN2 and VCP, and the main proteins of pathological inclusion bodies are TDP-43, SOD1, FUS and tau^[1]。 The study found that ALS partially overlapped with neurodegenerative diseases such as frontotemporal dementia (FTD), Alzheimer's disease (AD), Parkinson's disease and inclusion body myopathy (IBM) in genes, pathology, pathogenesis and clinical manifestations. We review the research progress of ALS overlapping with other neurodegenerative diseases through the above aspects.

From August 29th to September 1st, 2014, the 2014 3rd International Symposium on Parkinson's Disease and Movement Disorder, sponsored by the First People's Hospital affiliated to Shanghai Jiaotong University and co-organized by the World (Parkinson's Disease) Movement Disorder Association and Shanghai Branch of Chinese Medical Doctors Association, was held in Shanghai. The conference was supported by the Education Project for Developing Countries of the World (Parkinson's Disease) Movement Disorder Association (2014), the 985 (Shanghai Jiaotong University) sub-project of the Ministry of Education of China and Wang Kuancheng Medical Education Talent Fund (2014). Professor Wang Xiaoping (Shanghai) and Professor Chen Haibo (Beijing) served as the chairmen of this conference, and Mark Hallett, director of the Institute of Neurology and Stroke, National Institutes of Health, Louis CS Tan, president of the Asia-Pacific Branch of the World (Parkinson's Disease) Movement Disorder Association, and Professor Ivan Bodis-Wollner, director of the Department of Movement Disorders, New York University School of Medicine, were invited as honorary chairmen. More than 10 internationally renowned experts from the United States, Canada, Singapore, Australia, New Zealand and other places and more than 30 domestic experts were invited to attend this conference. There were also more than 200 representatives from experts and clinicians in related fields from all over the country. The opening ceremony of the conference was presided over by Professor Wang Xiaoping of the First Affiliated People's Hospital of Shanghai Jiaotong University. Zhang Kan, Director of Science and Education Department of Shanghai Municipal Health Bureau, Yu Zhuowei, Director of Shanghai Institute of Geriatrics and President of East China Hospital, and Li Zheng, Director of Songjiang District Health Bureau, delivered welcome speeches respectively. The conference was covered by the Chinese International Channel of CCTV. The special lectures of this conference covered the basic and clinical research on the etiology, diagnosis and treatment of Parkinson's disease, amyotrophic lateral sclerosis (ALS), hepatolenticular degeneration and other movement disorders and rare diseases, which promoted the exchange and integration of basic research and clinical practice, and the participants gained more gains and experiences. The following is a brief introduction to the main contents of the meeting.