2015年 · 第48卷第04期

This issue of the Chinese Journal of Neurology published the Guidelines for the Diagnosis and Treatment of Acute Ischemic Stroke in China 2014 and the Guidelines for the Secondary Prevention of Ischemic Stroke and Transient Ischemic Attack in China 2014^[1,2]。 Since the search for research evidence for these two guidelines (the "new guidelines" for short) ends in 2014, they are called the "2014 edition". Since May 2014, the Neurology Branch of Chinese Medical Association and its Cerebrovascular Disease Group have organized a new round of formulation, update and revision of a series of guidelines/consensus/classifications for cerebrovascular diseases. This issue is the first batch of newly revised guidelines. The follow-up guidelines to be published include "cerebral hemorrhage", "primary prevention", "cerebral small vessel disease", "venous sinus thrombosis", "arterial dissection", "cerebrovascular disease imaging examination", "subarachnoid hemorrhage", "cerebrovascular disease classification" and "stroke risk prediction tool", etc., which are currently being actively formulated or revised and will be published in this journal one after another. By 2015, it had taken 13 years for the Cerebrovascular Disease Group of the Neurology Branch of the Chinese Medical Association to formulate a series of guidelines for the prevention and treatment of cerebrovascular diseases in China. As early as 2002, it organized the first Chinese guidelines for the prevention and treatment of cerebrovascular diseases. In early 2005, it was approved by the Ministry of Health to promote it nationwide, and later it was officially published by the People's Medical Publishing House^[3]。 The second guidelines were updated and formulated in 2008. After 2010, the Chinese Journal of Neurology successively published five guidelines, including the Guidelines for the Diagnosis and Treatment of Acute Ischemic Stroke in China 2010 and the Guidelines for the Secondary Prevention of Ischemic Stroke and Transient Ischemic Attack in China 2010^[4,5,6,7,8]It has played a positive role in standardizing the diagnosis and treatment of cerebrovascular diseases in China.

Acute ischemic stroke (acute cerebral infarction) is the most common type of stroke, accounting for about 60% to 80% of all strokes. The time division of the acute phase is not uniform, and it generally refers to within 2 weeks after the onset of the disease. Recent studies have shown that the mortality rate of hospitalized patients with acute cerebral infarction is about 3.3% ~5.2% at one month after the onset of disease in China^[1,2]The mortality rate at 3 months was 9% to 9.6%^[2,3]The death/disability rate is 34.5% ~37.1%^[2,3]The one-year mortality rate was 11.4% ~15.4%, and the death/disability rate was 33.4% ~44.6%^[2,3,4]。 The management of acute ischemic stroke should emphasize early diagnosis, early treatment, early rehabilitation and early prevention of recurrence. Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association began to organize and formulate guidelines for the prevention and treatment of cerebrovascular diseases in China at the end of 2002. In early 2005, it was approved by the Ministry of Health to promote it nationwide, and in early 2007, it was officially published by People's Medical Publishing House^[5]。 In February 2010, the Chinese Journal of Neurology published the 2010 edition of the Guidelines for the Diagnosis and Treatment of Acute Ischemic Stroke^[6]。 The above work has played a positive role in standardizing the diagnosis and treatment of cerebrovascular diseases in China. Due to the continuous publication of new research evidence in recent years, the 2010 version of the guide has also collected good suggestions for improvement during the use. Therefore, in 2014, the 2010 version of the guidelines was updated and revised by the Neurology Branch of Chinese Medical Association and the Cerebrovascular Disease Group. The writers formulated recommendations through comprehensive inquiry, analysis and evaluation of relevant research evidence, solicitation of opinions from all parties, and full discussion to reach consensus, aiming to help clinicians choose the current relatively good diagnosis and treatment methods for stroke patients. In clinical practice, physicians should refer to the principles of this guideline and carry out individualized treatment according to the specific condition of patients.

ischemic stroke and transient ischemic attack (TIA) are the most common types of cerebrovascular diseases. Nearly 70% of patients in China have ischemic stroke^[1]。 The latest data show that the annual recurrence rate of ischemic stroke in China is as high as 17.7%^[2]。 Effective secondary prevention is an important means of reducing recurrence and mortality. Since 2010, the Cerebrovascular Disease Group of the Neurology Branch of the Chinese Medical Association issued the Secondary Prevention Guidelines for Ischemic Stroke and Transient Ischemic Attack in China 2010^[3]Since then, abundant evidence-based medical evidence has been added in the field of secondary prevention of ischemic stroke and TIA worldwide. Countries around the world have successively issued or updated guidelines and consensus. In order to standardize the clinical practice of secondary prevention of cerebrovascular diseases in China, experts from the Cerebrovascular Disease Group of Neurology Branch of Chinese Medical Association updated and revised the 2010 secondary prevention guidelines. By reviewing relevant research evidence, combining with China's national conditions and clinical status, soliciting opinions from all parties and fully discussing to reach a consensus, the writing group collectively formulated the Guidelines for Secondary Prevention of Ischemic Stroke and Transient Ischemic Attack in China 2014, with a view to providing neurologists with reasonable and scientific secondary prevention and treatment strategies for ischemic stroke and TIA, so as to reduce the mortality, recurrence and disability rate of patients with ischemic stroke and TIA in China. The evaluation criteria of recommendation strength and evidence in this guideline are the same as those in the Guidelines for the Diagnosis and Treatment of Acute Ischemic Stroke in China 2014 published on pages 246~257 of this issue.

large artery atherosclerotic (LAA) stroke is an important type of ischemic stroke, which has the characteristics of high mortality and disability rate. Cerebral atherosclerosis is the main pathogenesis^[1]。 It is currently believed that atherosclerosis is a chronically progressive inflammatory process in which inflammatory factors interact with each other. osteoprotegerin and receptor activator of soluble nuclear factor-κ B ligand (sRANKL) play an important role in the inflammatory process of atherosclerosis^[2,3]。 The purpose of this study was to investigate the relationship between osteoprotectin, sRANKL and LAA stroke and prognosis.

Studies in recent years have confirmed that plasma miRNA molecules can be used as marker molecules for the diagnosis and efficacy evaluation of tumors and other diseases, while there are few reports on differentially expressed miRNA in plasma of patients with ischemic stroke. We used miRNA microarray to detect the expression of miRNA in plasma of patients with ischemic stroke, and further used quantitative PCR to verify some differentially expressed miRNA, and preliminarily analyzed the correlation between differentially expressed miRNA and the neurological deficit score of the National Institutes of Health Stroke Scale (NIHSS).

Parkinson's disease is a chronic neurodegenerative disease with the main clinical manifestations of motor and non-motor symptoms. Patients with early Parkinson's disease are often treated with dopamine receptor agonist monotherapy to delay levodopa-induced motor complications; Patients with advanced Parkinson's disease are mostly treated with levodopa combined with dopamine receptor agonists, which can reduce the motor complications that have occurred. Pramipexole immediate-release and sustained-release agents are non-ergot dopamine receptor agonists and have an important effect on D₂Family receptors exhibit high selectivity for D₃The receptor is more affinity, showing its advantages of good efficacy and small adverse reactions. Although international studies have confirmed the efficacy of pramipexole sustained-release and immediate-release agents, there is no comparative evaluation study of the two in Chinese patients with Parkinson's disease. We conducted a noninferiority assessment of the efficacy of pramipexole extended-release and immediate-release combined with levodopa therapy in Chinese patients with Parkinson's disease.

Neuromyelitis optica (NMO) has gradually been recognized as an inflammatory demyelinating disease that mainly invades the optic nerve and spinal cord and has obvious heterogeneity in clinical and radiographic manifestations. The advent of aquaporin 4 (AQP4) antibodies establishes NMO as a disease entity independent of multiple sclerosis (MS)^[1]。 A large number of studies have found that some NMO patients coexist with systemic autoimmune diseases (SADs) and non-organ-specific autoantibodies^[2,3,4,5]Such as systemic lupus erythematosus (SLE), Sjogren's syndrome (SS) and Hashimoto's thyroiditis (HT). Wingerchuk et al.^[6]Optic neuritis/long-segment myelitis (LM) with SADs is classified as NMO spectrum diseases (NMOSDs) together with classical NMO, recurrent optic neuritis/LM, optic neuritis/LM with characteristic brain damage, and Asian optic neuromyeloid MS. We analyzed the clinical data and imaging characteristics of patients with NMOSDs combined with SADs, further elaborated the relationship between NMO/NMOSDs and SADs, and provided the basis for early diagnosis and treatment of the disease.

Brain abscesses are common intracranial infectious lesions, which can appear as single or multiple, and usually have a space-occupying effect. Multiple brain abscess with hemorrhage is a rare acute and severe disease in neurology, which is often formed by hematogenous dissemination. It is difficult to distinguish from metastatic tumor stroke in the early stage of the disease, and it is easy to delay the treatment due to insufficient understanding. However, there are few relevant literature reports at present. A case of multiple hemorrhagic brain abscess caused by infectious endocarditis admitted to Xiangya Hospital of Central South University is reported as follows.

linear scleroderma is rarer in the clinic, while linear scleroderma with neurological symptoms is even rarer^[1]。 The literature shows that most of the cases are linear scleroderma involving the frontal and facial region, that is, knife-like linear scleroderma (linear scleroderma en coup de sabre, LSCS)^[2]。 A case of LSCS with neurological symptoms diagnosed in our hospital is presented.

Hashimoto's encephalopathy is a kind of encephalopathy with recurrent or progressively aggravated disorders of consciousness, seizures, cognitive impairment and mental disorders as the main clinical manifestations. The disease was first reported by Brain in 1966, and was determined to be associated with increased serum anti-thyroid antibodies, and has good responsiveness to corticosteroid therapy^[1]。 The disease is relatively rare. As of 2013, domestic scholars have reported a total of 31 cases of Hashimoto's encephalopathy, including 6 males and 25 females^[2,3,4]。 Hashimoto's encephalopathy has various clinical manifestations and is easily misdiagnosed and misdiagnosed. A patient with Hashimoto encephalopathy complicated with diffuse toxic goiter (also known as Graves disease) and Hashimoto thyroiditis (HT) admitted to our hospital was summarized and analyzed, aiming to improve the understanding of this disease.

The patient was a 40-year-old woman who was admitted to the hospital on November 7, 2013 due to "left lower limb weakness for more than 1 year, aggravated for half a year, and right lower limb weakness for 1 month". The patient progressively developed claudication of the left lower limb in the fall of 2012. Physical examination in other hospitals showed that the left anterior tibial muscle was weak; magnetic resonance imaging (MRI) of thoracic and lumbar vertebrae showed no obvious abnormalities. Left cross-domain gait developed in December 2012 and frequently tripped. Symptoms were significantly aggravated after long walking in May 2013, with the left lower limb being lifted less than 20 cm from the ground and the walking distance less than 50 m, and muscle atrophy in the left calf was found. Right lower limb weakness occurred in October 2013. Physical health in the past, running and jumping ability similar to peers. Family members do not have similar symptoms and parents are not intimate married. Physical examination after admission; No obvious abnormalities were observed in the heart, lungs and abdomen, and no rash was observed on the face, neck and dorsal side of each joint. There is no drooping of the eyelids, unlimited movement of the eyeballs in all directions, and no diplopia. Obvious bilateral atrophy of the anterior tibialis muscle (32.5 cm in circumference at 10 cm of the left subpatella and 33.5 cm on the right); Left iliopsoas muscle strength Ⅳ^＋/Grade V, left tibialis anterior muscle, extensor hallucinus longus muscle IV^–Grade, right anterior tibialis muscle and extensor hallucinus longus muscle grade IV, residual muscle strength grade V (including bilateral quadriceps and trunk muscles), and the fatigue test was negative. Decreased symmetry of tendon reflexes in the extremities. Bilateral Babinsky sign negative. The acupuncture sensation, light touch sensation, tuning fork vibration sensation, joint position sensation and figure sensation of limbs and trunk were normal. Freemasonic movement is normal. Negative Gower sign, left transdomain gait, bilateral heel and toe walking inability. After admission, serum creatine kinase was 505 U/L, lactate dehydrogenase was 186 U/L, and aspartate aminotransferase was 25 U/L.

Cerebrovascular disease is the second cause of death in the world^[1]In recent years, it has jumped to the top cause of death in China^[2]Moreover, it is the main cause of long-term disability in adults, and its high morbidity, disability and mortality have become global public health problems^[3]。 Epidemiological research can provide information on which groups of people and geographical regions have high risk of disease and poor prognosis outcome, which is conducive to the formulation of prevention and treatment strategies^[3]。

The incidence of pain in patients with Parkinson's disease is about 67.6%. Like depression, it is considered to be one of the most distressing non-motor symptoms for patients. More than one third of patients have more than one chronic pain of different nature, which seriously affects the quality of life of patients. Clinically, the pain symptoms of Parkinson's disease are often masked or replaced by classical motor symptoms, and most of them are not taken seriously by clinicians as independent problems. There is greater blindness about the nature, classification and treatment of Parkinson's disease pain, resulting in inaccurate treatment effect and nearly half of patients do not receive analgesic treatment^[1,2,3]。 Therefore, strengthening the understanding of Parkinson's disease pain, further optimizing treatment measures, alleviating pain symptoms, and improving the quality of life of patients with Parkinson's disease have become hot spots in the research field of non-motor symptoms of Parkinson's disease. We reviewed the recent research progress on the clinical characteristics, therapeutic measures and pathogenesis of Parkinson's disease with pain in recent years.

Kennedy's disease, also known as spinal and bulbar muscular atrophy (SBMA), is a rare adult-onset sex-linked recessive inherited motor neuron degeneration disease. It is characterized by slowly progressing muscular weakness and atrophy of the medulla oblongata, face and extremities. 1968 by Kennedy et al.^[1]Firstly, it was reported that the pathogenic gene was localized at the proximal end of the long arm of the sex chromosome (Xqll ~12), mainly due to the abnormal amplification of CAG encoding polyglutamine in exon 1 of the androgen receptor (AR) gene^[2]。 When the number of CAG repeats of the trinucleotide sequence in the coding region is over-amplified beyond a certain limit, the disease occurs, but the specific pathogenic mechanism has not been uniformly determined. We review and prospect the clinical characteristics, pathogenesis, diagnosis and treatment of Kennedy's disease.

Susac syndrome (Susac syndrome) is a relatively rare vascular endotheliopathy with a typical clinical triad of "acute encephalopathy, branch retinal artery occlusion (BRAO) and impaired hearing". So far, more than 300 cases have been reported worldwide, mainly distributed in North America and Europe. The incidence ratio of male to female is about 1:3.5, and the onset age is 7-72 years old, but the most common occurrence is 20-40 years old^[1,2]。 Individual cases have been reported in China. In order to improve clinicians' understanding of this disease, so as to carry out early diagnosis and treatment, we now make a review and analysis of this disease.

The 13th National Conference on Electromyography and Clinical Neuroelectrophysiology and Standardization Symposium was held in Guiyang, Guizhou Province on May 28 – 30, 2014. The conference was co-sponsored by the Electromyography and Clinical Electrophysiology Group of Neurology Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Neurology, and co-organized by the Neuroelectrophysiology Center of Guizhou Provincial People's Hospital. A total of 148 papers were accepted at the conference, of which 48 were exchanged at the conference. Cui Liying, Fan Dongsheng, Wang Yuping, Huang Xusheng, Shen Dingguo, Zhou Hui, Lu Zuneng and other well-known domestic experts attended the conference and gave special reports. Tang Xiaofu, Shen Dingguo and other veterans of clinical neuroelectrophysiology in China were invited to attend the conference. This conference also specially invited Mark Hallett, a famous neurology professor from NIH in the United States, president of the International Society of Clinical Neuroelectrophysiology, and Sanjeev Dattatraya Nandedkar, an American electrophysiology engineer, to give keynote speeches. The opening ceremony was chaired by Professor Cui Liying, head of the study group, and Wang Mouyue, director of the editorial department of Chinese Journal of Neurology, delivered a welcoming speech. The contents of some of the topics of the meeting are briefly described below.