2015年 · 第54卷第09期

China is the worst country with cerebrovascular disease in the world, and its mortality rate is five times that of North America. The onset age of cerebrovascular disease in China is 10 years earlier than that of the Caucasian population, and delaying the onset time is the most important task faced by Chinese neurologists. Since 1900, the mortality rate of cerebrovascular disease in the United States has declined year by year^[1]While the mortality rate of cerebrovascular disease in China is increasing day by day. With the improvement of economic level and medical level, the proportion of mild cerebrovascular disease is increasing day by day, while the proportion of severe cerebrovascular disease is relatively decreasing. This trend is basically consistent all over the world. The rate of cerebral hemorrhage in hospitalized patients with cerebrovascular disease in urban hospitals in China is three times that in western countries. Atherosclerosis is still the main foundation of ischemic cerebrovascular disease, but unlike Western populations, intracranial atherosclerosis is the main foundation of Chinese population^[2]While carotid atherosclerosis contributed more in the Caucasian population. In the past few years, due to the introduction of a series of prevention and control policies by the state, the medical quality of cerebrovascular diseases in China has been greatly improved, but the pressure of prevention and control is still huge.

motor neuron disease (MND) is a malignant neurodegenerative disease that selectively damages the anterior horn cells of the spinal cord, the motor nucleus of the lower brain stem and the pyramidal cells of the cerebral cortex. Clinically, it includes amyotrophic lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, primary lateral sclerosis and flail arm syndrome. Riluzole is currently the only proven effective treatment, but it can only prolong patients' survival for several months. More and more studies have shown that non-invasive positive-pressure ventilation (NIPPV) treatment for MND patients can significantly prolong the survival time and greatly improve the quality of life of patients. Therefore, it should be highly valued and strengthened in clinical practice.

fibromuscular dysplasia (FMD) is a rare, non-inflammatory, non-arteriosclerotic vascular disease of unknown etiology, most commonly found in the extracranial segments of renal artery and internal carotid artery^[1]。 This disease is relatively benign, and most patients are found only accidentally in angiography. In patients with carotid artery fibromuscular dysplasia (CFMD), ischemic stroke occurs only when the collateral branches are decompensated^[2]Therefore, it causes less stroke. In recent years, 9 patients with cerebral infarction caused by CFMD have been admitted to our hospital. These patients are not complicated with renal artery disease, but all of them are unilateral lesions, and the terminal intracranial segment of the internal carotid artery on the diseased side is occluded. The clinical manifestations, imaging characteristics and follow-up of this group of patients are reported below.

Insulin resistance (IR) and decreased beta cell function are the main pathogenesis of type 2 diabetes mellitus (T2DM). It is believed that IR exists prior to the development of abnormal blood glucose in T2DM^[1,2]Therefore, some scholars have proposed to divide the process of T2DM into three stages: normoglycemic hyperinsulinemia (NG-HINS) stage, impaired glucose tolerance (IGT) and diabetic stage^[1]。 That is to say, NG-HINS and IGT are the same "reserve army" of T2DM. In this study, the "gold standard" high insulin positive glucose clamp technique (HECT) was used to evaluate IR levels in different groups of normal glucose tolerance (NGT), NG-HINS and IGT. The purpose of this study was to deeply elucidate the pathogenesis of T2DM and lay a theoretical foundation for early prevention of T2DM.

June 9, 2015Neurobiol AgingThe clinical-basic comprehensive research (He J, Tang L, Benyamin B, et al.) led by Professor Fan Dongsheng, Department of Neurology, Peking University Third Hospital and jointly completed by three international scientific research institutions was published online. C9orf72 hexanucleotide repeat expansions in Chinese sporadic amyotrophic lateral sclerosis. Neurobiol Aging, 2015.), defining the genetic, epigenetic, and clinical characteristics of hexanucleotide (GGGGCC) repeat expansion in the C9orf72 gene in Chinese patients with sporadic amyotrophic lateral sclerosis (ALS). This is currently the largest and most in-depth single-center ALS genetic study in non-European populations, and the first innovative comprehensive verification of the epigenetic characteristics of Asian sporadic ALS population located on the C9orf72 gene fragment.

SAP is one of the most common inpatient medical complications after stroke^[1]The birth of its concept has gone through a long process. Initially, "post-stroke pneumonia" was commonly used to describe the pathophysiological process of pneumonia occurring early after stroke. Until 2003, Professor Hilker of Germany used "stroke-associated pneumonia" for the first time to describe the clinical phenomenon of infectious lung parenchyma inflammation within 72 hours of admission in stroke patients without previous lung infection. According to its time course, SAP can be divided into acute SAP (SAP occurring within 1 month after stroke) and chronic SAP (SAP occurring 1 month after stroke).

Since British scientist Fleming developed penicillin in 1928, the battle between bacteria and antibiotics has begun. In the following nearly 80 years, the history of human anti-infection continued to unfold the story of "antibiotic-drug-resistant bacteria-new antibiotic-new drug-resistant bacteria" until 2010, when the first report of New Delhi-metallo-1 (NDM-1), a metalloenzyme resistant to carbapenem, in Klebsiella pneumoniae strain caused global panic, making this story seem difficult to continue. The emergence of extensively drug-resistant (XDR) bacteria, especially resistant to carbapenes (CR) strains, has put clinicians in a dilemma. On the one hand, there are multi-drug-resistant bacteria, and on the other hand, there are no new sensitive antibiotics. How to choose "old" antibiotics? To the pan-drug-resistant Gram negative bacilli (XDR-GNB) strain, how to choose the treatment plan and reasonable and effective antibiotic application to improve the cure rate of patients is the goal of clinicians. The following is a summary of the treatment protocols for XDR-GNB reported in relevant clinical literature at home and abroad, hoping to provide assistance to clinicians in their treatment choices.

Patients treated with antimicrobial drugs develop diarrhea whose primary disease is not easily explained, which is called antibiotic-associated diarrhea (AAD). Most patients have loose or watery stools lasting more than 3 times a day for 2 to 3 days within 2 to 8 weeks after using antibacterial drugs for 2 to 7 days, and a few patients can have loose or watery stools lasting more than 3 times a day for 2 to 3 days. If other causes of diarrhea can be ruled out, AAD can be diagnosed.

The large number of intestinal flora in the digestive tract plays a crucial role in host adaptation to environmental changes and maintenance of homeostasis. irritable bowel syndrome (IBS) is a functional gastrointestinal disease whose pathogenesis is not completely understood. Intestinal flora is involved in its pathogenesis, which has become a research hotspot in recent years. The problems and challenges faced in the study of intestinal flora and IBS are briefly described.

Intestinal flora and human body are interdependent and symbiotic, constituting intestinal micro-ecosystem. Intestinal micro-ecology is involved in metabolism, energy balance and immune regulation. It is believed that the changes of intestinal micro-ecology are related to the occurrence of many diseases^[1]。 The micro-ecology in different parts of the human gastrointestinal tract has different composition and function, which is influenced by many factors such as age, sex, race and diet structure. The constitution of bacterial profiles in different parts of the gastrointestinal tract and the corresponding metabolic characteristics are involved in maintaining human health^[2]。 It is intended to expound the important characteristics of microecology related to human health, which is of great significance for studying human health and preventing diseases.

Fabry disease is a rare hereditary lysosomal storage disease. Due to the mutation of GLA in the gene encoding alpha-galactosidase A (alpha-galactosidase A), partial or complete deficiency of enzyme activity leads to excessive intracellular glycosphingolipid accumulation, thus causing various system and organ lesions^[1]。 Since 2001, enzyme replacement therapy (ERT) has been approved for official marketing in Europe and the United States. As a milestone in the treatment of Fabry's disease, ERT can improve heart function and stabilize renal function, reduce pain and improve patients' quality of life in the early stage of Fabry's disease^[2]。 In 2013, the China Fabry Disease Expert Collaboration Group also recommended the clinical use indications of ERT in the expert consensus on the diagnosis and treatment of this disease^[3]。 However, ERT-infused enzymes could not be distributed to all cells of the target organ^[4]。 Therefore, incomplete efficacy coupled with high price limits the clinical application of ERT. In recent years, new treatments are being continuously developed, and the current treatment progress of Fabry's disease is summarized below.

Hypophosphorus rickets/osteomalacia (previously known as hypophosphorus vitamin D-resistant chondrosis/rickets) is a group of diseases characterized by poor bone mineralization, osteomalacia or rickets due to low blood phosphorus levels and insufficient production of active vitamin D. At present, it has been found that FGF-23 is associated with a variety of low-phosphate rickets/osteomalacia, so low-phosphate rickets/osteomalacia can be classified into FGF-23 related and non-related rickets^[14], FGF-23-related/unrelated hypophosphorus rickets typing and related pathogenic genes are presented inTable 1。

peripheral neuropathy (PN) has a high incidence in patients with multiple myeloma (MM). With the improvement of the diagnosis and treatment level of MM, the incidence of primary disease of MM and treatment-related PN gradually increases. However, there is no unified international diagnosis and treatment guideline for multiple myeloma peripheral neuropathy (MMPN) for reference on how to better diagnose and treat patients with PN, improve the curative effect and improve the prognosis. Therefore, we have reached the following consensus on the basis of widely soliciting the opinions of relevant domestic experts and taking into account the latest international progress.

[Website] http: / /www.sfn.org/

[URL] http: / /journals.cambridge.org/action/displayJournal? jid = BBS

A randomized controlled trial studied the long-term effects of fingolimod for multiple sclerosis, comparing the efficacy of continued fingolimod and placebo switching to fingolimod. The results showed that the annual recurrence rate, brain attrition volume, and disability disease progression were all low with continuous use of fingolimod, and that long-term use of fingolimod had good efficacy and tolerability (Neurology, 2015, 84:1582 – 1591).