2016年 · 第49卷第10期

Skeletal muscle diseases are a group of diseases in which muscle fiber destruction or dysfunction is the main pathophysiological process of skeletal muscle under the damaging action of various etiologies. Secondary skeletal muscle damage caused by peripheral nerve or vascular lesions is not included. The types of skeletal muscle diseases are divided into acquired and hereditary by etiology. Acquired skeletal muscle diseases mainly include toxic, traumatic and inflammatory, the latter including immune (dermatomyositis, immune necrotizing myopathy, polymyositis, overlapping myositis, inclusion body myositis and rare types of myositis) and infectious. Hereditary skeletal muscle disorders include muscular dystrophy including limb-band, facioscapulohumeral, oculopharyngeal, distal oculopharyngeal, distal, tonic, pseudohypertrophic and congenital, and myopathy including metabolic, congenital and myofibrillary^[1]。 The general methods involved in the diagnosis of these diseases include creatine kinase, muscle imaging, electromyography, which are mainly used for localized diagnosis, muscle biopsy (biopsy for short), gene and myositis antibody tests, which are mainly used for qualitative diagnosis. The significance and importance of the above methods have been explained in the article "Diagnosis and Treatment of Skeletal Muscle Diseases" published in this journal in 2014^[1]。 In the past few years, auxiliary examination methods have developed rapidly, and with the popularization of the concept of precision medicine, they have been integrated and learned from each other in the pathophysiological process of revealing the occurrence and development of diseases, and their value has been evaluated differently in many diagnostic guidelines for muscle diseases at home and abroad^{[2,3,4,5,6,7]}The importance of various auxiliary examinations in the process of diagnosing diseases has also changed, so it is necessary to optimize the diagnostic strategies of different skeletal muscle diseases.

intracranial atherosclerotic disease (ICAD) is the most common cause of ischemic stroke in Asia^[1]。 It is reported that about 46.6% of acute ischemic strokes in China are caused by ICAD^[2]And the annual recurrence rate of related cerebrovascular events is as high as 17.1% ~38.2%^[3]。 ICAD can involve all intracranial blood vessels, and the middle cerebral artery (MCA) is the most susceptible to involvement because of its thick main trunk and large blood supply range. Arterial remodeling is an important mechanism of prevalence of atherosclerotic diseases. Studies on coronary artery sclerosis have shown that positive remodeling is the main remodeling mode of acute coronary syndrome, and negative remodeling is more common in patients with stable angina pectoris^[4]。 In recent years, high resolution MRI (HRMRI) has been widely used in intracranial artery imaging due to its non-invasive nature and high signal-to-noise ratio, which has provided beneficial help for us to study the remodeling of intracranial arteries. We used HRMR to investigate the remodeling of atherosclerotic MCA vessel wall and its relationship with cerebral infarction.

Acute encephalitis accompanied by ovarian teratoma is not uncommon in clinical practice and has been reported as early as 1997^[1,2]。 In 2007, Dalmau et al.^[3]The relationship between acute encephalitis with ovarian teratoma and N-methyl-D-aspartate (NMDA) receptor in the brain was found, and this type of encephalitis was named anti-NMDA receptor encephalitis. Foreign data show that 38% of anti-NMDA receptor encephalitis patients can detect tumors, and ovarian teratoma is the most common^[4]Ovarian teratoma was present in 56% of female patients older than 18 years^[5]。 The proportion of anti-NMDA receptor encephalitis cases with combined tumors in China is low^[6]。 Ovarian teratoma is the only known tumor type that is clearly associated with the development of anti-NMDA receptor encephalitis. We report a case of an ovarian teratoma recently admitted to our hospital that recurred after resection and caused anti-NMDA receptor encephalitis.

Multiple sclerosis (MS) is a common demyelinating disease of the nervous system, but MS with changes of pontinal crucifixion in imaging is less common, and its specific mechanism is still unclear. A case of MS with hot cross bun sign was recently admitted to our hospital, and the report is as follows.

Anti-α-amino-3-hydroxy-5-methyl-4-isoZolpropionate 2-receptor antibody (AMPA2-R) encephalitis is a newly discovered autoimmune encephalitis against neuronal cell surface antigens. In 2009, Lai et al.^[1]Ten cases of anti-AMPAR antibody encephalitis were reported for the first time. By the end of 2015, the number of confirmed cases reported at home and abroad totalled more than 50^[2,3]。 In 2015, China Wu Hui et al^[4]One case of AMPA2-R antibody encephalitis has been reported, but anti-AMPA2-R antibody encephalitis with autoimmune thyroid disease has not been reported. The diagnosis and treatment and follow-up results of a patient admitted to our hospital are reported as follows.

primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune disease with typical symptoms of dry eyes and dry mouth. Extraglandular symptoms are associated with affected organs, which may include the musculoskeletal system, skin, kidneys, lungs, liver, and central and peripheral nervous systems^[1,2,3]。 Neurological involvement has been reported in 8.5% to 67.0% of patients^[4]Its pathogenesis may be a series of pathological changes caused by extensive acute and chronic vasculitis and non-vasculitis or inflammatory cells acting on nerve or muscle tissue due to immune dysfunction^[5]。 We report 2 admitted patients with pSS with acute cerebral infarction as follows to attract the attention of neurologists.

ovarian hyperstimulation syndrome (OHSS) is a iatrogenic complication caused by controlled ovarian stimulation (COS) in assisted reproductive technology^[1]Its pathophysiological mechanism is that the increase of systemic capillary permeability leads to a large amount of intravascular fluid extravasation, and the clinical manifestations mainly include pleural and ascites fluid, hypoalbuminemia, edema, etc., and even thrombotic events due to blood concentration. With the increasing use of assisted reproductive technology, the associated thrombosis risk should be recognized and paid attention to accordingly.

A 78-year-old male was admitted to Beijing Chaoyang Hospital on March 14, 2016 due to "dizziness, nausea for 1 d, right limb weakness with difficult speech for 8 h". One day before admission, the patient had dizziness in quiet state without obvious trigger, accompanied by double vision, rotation of vision, nausea and vomiting, and the vomit was stomach content. Eight hours before admission, he developed limb weakness in quiet state, right side, accompanied by numbness in the right limb, confusion in speech, choking and coughing when eating and drinking water, so he went to the neurology department. History of hypertension, diabetes and cerebral infarction was denied. No history of smoking or alcoholism. No family history. Physical examination at admission: blood pressure 164/97 mmHg (1 mmHg =0.133 kPa), clear consciousness, dysarthria, bilateral pupil diameter 3 mm, sensitivity to light reflex, full movement of eyeball in all directions, visible horizontal nystagmus. Bilateral nasolabial folds were symmetrical, bilateral pharyngeal reflexes were weakened, and tongue extension was left. Right side shrugging movement weakened. Left upper and lower limb muscle strength Ⅴ^－Grade, right upper and lower limb muscle strength Ⅱ^－Grade, low muscle tone. The tendon reflexes were symmetrical, and bilateral pathological signs were positive. The left finger-nose test and heel-knee-tibia test were not stable and accurate, and the right side was not cooperative. Right proprioception decreased. Soft neck, no resistance. NIHSS score 12. Essen scale score 2. Laboratory tests: total cholesterol 7.20 mmol/L, low density lipoprotein 5.5 mmol/L, glycated hemoglobin 6.6%, homocysteine 34 mmol/L, D-dimer 0.71 mmol/L; Fasting blood glucose was 6.94 mmol/L. Thyroid function, coagulation, thrombotic combination, tumor markers, etc. were roughly normal. Imaging tests: seeFigure 1，Figure 2。 Diagnosis: cerebral infarction (large artery atherosclerotic type); Bilateral occlusion of vertebral and basilar arteries; hyperlipidemia; Hyperhomocysteinemia.

Patients with malignant tumors are often complicated by various myopathies, such as myasthenia gravis, polymyositis (PM), dermatomyositis (dermatomyositis) and immune-mediated necrotizing myopathy (IMNM), etc. Especially the latter kinds of inflammatory myopathies are relatively common in clinic. The older the patient, the more opportunities to occur, and there are certain particularities in pathological morphological changes. 2015 Yang et al.^[1]A meta-analysis found that the relative risk of dermatomyositis, PM, and dermatomyositis/PM combined tumors were 5.5, 1.62, and 4.07, respectively, and their high incidence was within the first year after myositis diagnosis, and the relative risk of endomyositis and PM accompanied by malignant tumors at one year was 19.41 and 5.02, respectively. However, the specific pathogenesis of malignant tumor-related inflammatory myopathy is still unclear. Recent studies have found that there are many related antibodies in patients with malignant tumor-related inflammatory myopathy, and some antibodies are closely related to their pathogenesis. Therefore, we give a brief introduction to these antibodies.

The natural immune system is the first line of defense of the body's immune response. When the body is invaded by pathogenic microorganisms or disease-related host molecules, it can sense pathogen-associated molecular pattern (PAMP) and damage-associated molecular pattern (DAMP) through pattern recognition receptors, and play an immune regulatory role. In the central nervous system, pattern recognition receptors are expressed only in microglia, macrophages, and astrocytes. Among them, pattern recognition receptors that locate in the cytoplasm and recognize intracellular and extracellular signals are called nucleotide binding oligomerization domain (NOD) -like receptors (NLRs), mainly including NOD-like receptor protein 1 (NLRP1), NOD-like receptor protein 3 (NLRP3), NOD-like receptor protein 6 (NLRP6), NOD-like receptor protein 7 (NLRP7), NOD-like receptor protein 12 (NLRP12), NOD-like receptor C4 (NLRC4) and neuronal apoptosis inhibitory protein (NAIP)^[1]。 Its classical structure includes three parts: NLR, an apoptosis-associated speck-like protein containing CARD (ASC) adapter protein containing a caspase recruitment domain (CARD), and a caspase-1 effector protein. Among them, NLRP3 inflammasome with NLRP3 as the core is an important member of this family. A variety of stimulating factors can activate NLRP3 inflammasome, leading to the activation of caspase-1 precursor, thereby regulating the maturation and secretion of interleukin 1 β (IL-1 β) and interleukin 18 (IL-18), triggering the clearance of pathogens and the occurrence of inflammatory response. In the central nervous system, the NLRP3 inflammasome produces a wide range of immunomodulatory effects by recognizing a variety of pathogens and danger signals. Therefore, the relationship between NLRP3 inflammatory bodies and the mechanism of development of central nervous system diseases is gradually becoming a research hotspot.

1989, Caplan^[1]A new pathological concept of branch atheromatous disease (BAD) was first proposed based on the pathological findings of 3 patients. BAD is a single cerebral infarction in the deep part of the brain caused by stenosis or occlusion of the perforator artery ostium caused by atherosclerosis. It is mainly used to distinguish lacunar infarction (LI) or small vessel disease caused by fibrohyalinization of small arteries. Although this concept has been put forward for more than 20 years, it is difficult to obtain histopathological specimens of this kind of patients in clinic, which leads to no obvious progress in the pathological research of this disease. The progress of imaging, especially MRI technology, has opened up a new way for the research of BAD imaging. We can indirectly determine BAD and the type of BAD by observing the carrier artery by magnetic resonance diffusion weighted imaging (DWI) and by observing the carrier artery by MRA. At present, the research on BAD imaging is gradually increasing, but the definition of BAD imaging is not uniform, and the results obtained are still different. Due to the limitation of existing imaging techniques, we cannot directly observe the structure of perforator artery wall and lumen at present. Therefore, in order to facilitate clinical practice, the Chinese ischemic stroke subclassification (CISS) classification^[2]Perforator artery disease is listed separately as a type one in, and BAD and small vessel disease are not specifically subdivided. However, the development of imaging technology (high resolution MRI and high field strength MRI) still brings hope for our future research. Perforator artery disease includes BAD and small vessel disease, and the definition of BAD is true to the definition of its original pathology. We review the definition of BAD and advances in clinical and imaging aspects.

acute spinal cord injury (ASCI) is a common type of severe trauma in neurosurgery and emergency departments. Because of its irreversibility^[1]Therefore, the discussion of ASCI has always been a major research hotspot in the field of neuroscience. At present, there are endless researches in this field, including the continuous improvement of surgical techniques and programs^[2]Clinical study of drugs to protect and promote nerve growth^[2]Clinical application of hormones^[3]And the exploration of emerging transplant therapies^[4,5]Etc. However, there is currently no well-established and effective treatment that can improve the functional outcome of patients^[2,6]。 However, the potential benefits of hypothermia therapy in preventing and limiting central nervous system injury have gradually gained widespread attention. In this paper, we systematically combed the research history, treatment scheme, mechanism of action and problems existing in the application of therapeutic hypothermia in ASCI, and put forward constructive coping strategies, and then boldly prospect its application prospect in ASCI, hoping to provide a systematic theoretical basis for the scientific positioning of therapeutic hypothermia in traditional treatment scheme.

The use of animal models to simulate human body has a long history of participating in experimental research. Experimenters try to simulate clinical diseases through various animal models, and then study the pathological mechanism of the disease or improve the treatment plan. Brain injury models mainly include traumatic brain injury, cerebral infarction, cerebral hemorrhage, white matter injury, etc. Animals commonly used in clinical experimental studies include rats, mice, rabbits, dogs and sheep. We reviewed various brain injury models of the most widely used rats as subjects, and summarized the specific manufacturing methods, advantages and disadvantages of the models, and the corresponding test indexes of each model.

On April 7-9, 2016, the 16th China Cerebrovascular Disease Conference (CCCD) 2016 was held in Hangzhou, Zhejiang 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, Zhejiang Medical Association and Neurology Branch of Zhejiang Medical Association, and co-organized by the First Affiliated Hospital of Zhejiang University School of Medicine. The opening ceremony was presided over by Professor Luo Benyan, member of Neurology Branch of Chinese Medical Association and chairman of Neurology Branch of Zhejiang Medical Association. Professor Liu Ming, head of cerebrovascular diseases group of Neurology Branch of Chinese Medical Association, Professor Pu Chuanqiang, chairman of Neurology Branch of Chinese Medical Association, Professor Wang Weilin, president of the First Affiliated Hospital of Zhejiang University School of Medicine, Academician Duan Shumin, director of Zhejiang University School of Medicine, Professor Luo Huawei, secretary-general of Zhejiang Medical Association, Professor Huang Jiaxing, secretary-general of World Stroke Organization, and Du Zhiqin, deputy secretary-general of Chinese Medical Association, respectively delivered speeches to the conference.