2015年 · 第95卷第15期

High-quality guidelines (consensus) have strong guidance for their corresponding fields, which is conducive to the development of disciplines. But no guidelines (consensus) can be perfect or even biased. In order to improve the scientific nature of neurosurgical guidelines in the future and narrow the gap with mature guidelines in developed countries, this issue of Chinese Medical Journal published "Overall Evaluation of Chinese Neurosurgical Guidelines (Consensus)" written by Liu Weiming and other doctors^[1]One article. The author of this article used The latest international Evaluation guideline tool AGREE II (The Appraisal of Guidelines for Research & Evaluation) to evaluate The quality of Guidelines and consensus in The field of neurosurgery in China before September 1, 2014. The author evaluated the distribution and development of the guidelines for neurosurgery in China, the participants in writing the guidelines, the rigor of formulating the guidelines, whether the guidelines are convenient for specific application and popularization, and the independence in the process of writing the guidelines.

Clinical diagnosis and treatment guidelines and consensus are effective means to standardize clinical diagnosis and treatment. Good clinical guidelines can improve the overall diagnosis and treatment level and improve the curative effect, thus benefiting all parties^[1]。 As far as neurosurgery is concerned, it is a rapidly developing discipline. Originally, it was thought that all diseases that could not be treated and operated could be treated by surgery. This requires good clinical guidelines to regulate and lead the development of the discipline. With the establishment and development of evidence-based medicine, the advantages of evidence-based medicine guidelines are gradually established^[2]。

A 39-year-old male was admitted to the Department of Rheumatology and Immunology of Peking Union Medical College Hospital in June 2012 due to fatigue for 7 years, lower limb edema for 1 year, headache and diplopia for 1 month. The patient developed fatigue 7 years ago. He went to the local hospital and found that hemoglobin and platelets decreased (details unknown). He was diagnosed with Evans syndrome and was treated with prednisone and human gamma globulin. One year ago, I had low back pain, edema of both lower limbs, and increased foam in my urine. I went to Peking Union Medical College Hospital. Blood routine showed that white blood cells and hemoglobin were normal, and platelets were 56×10.⁹/L; Serum albumin 21 g/L; 24 h urine protein 9.86 g; Antinuclear antibody homogeneous 1:1 280 positive, anti-double-stranded DNA antibody, anti-human globulin (Coomb's) test positive; He was diagnosed as systemic lupus erythematosus (SLE), lupus nephritis (LN), and nephrotic syndrome (NS). He was treated with oral prednisone 60 mg/d and intravenous infusion of cyclophosphamide 1.0 g/month, but the urinary protein decreased unsatisfactorily. He was added with azathioprine 200 mg/d oral, but the urinary protein still did not disappear. 1 month ago, headache, nausea, jet vomiting, accompanied by left eye vision loss, diplopia, left eyelid swelling and hearing loss, tinnitus, loss of consciousness, epilepsy, etc. Ophthalmological examination showed abductor nerve paralysis, papilledema, retinal hemorrhage and conjunctivitis in the left eye. Admitted for further diagnosis and treatment. Past, personal, marriage and childbirth, family history are no different. Physical examination: body temperature 36.6 ℃, heart rate 90 beats/min, 20 breaths/min, blood pressure 150/104 mmHg (1 mmHg =0.133 kPa); The left eyelid was swollen, the abduction of the left eye was limited, the other directional movements and the right eye movement were normal, the vision of both eyes was slightly blurred, there was no visual field defect, the cardiopulmonary and abdominal examination was normal, and the lower limbs could be concave edema. Soft neck, negative meningeal irritation sign, pathological sign not drawn out, laboratory test: blood routine: white blood cells 6.30×10⁹/L, hemoglobin 119 g/L, platelets 12×10⁹/L. Blood biochemistry: Alanine transferase 39 U/L, aspartate transferase 18 U/L, albumin 30 g/L, creatinine 90 μ mol/L. Urine routine: protein>3.0 g/L, red blood cells 2×10^-4/L, 100% abnormal, visible tube type. 24 h urine protein 5.40 g. High sensitivity C-reactive protein 1.49 mg/L, erythrocyte sedimentation rate 27 mm/1 h. Coagulation: prothrombin time 10.8 s, activated partial thromboplastin time 21.7 s, fibrinogen 3.19 g/L, D dimer 13.13 mg/L, INR 0.93, homocysteine 10 μ mol/L. Antinuclear antibody homogeneous 1:80 positive, anti-double stranded DNA antibody 0.1 IU/L, complement C3 0.709 g/L, complement C4 0.182 g/L. Anti-phospholipid antibody negative. Bone marrow smear: active hyperplasia, no abnormalities in granules and erythroid, and many megakaryocytes. Bone marrow biopsy: hematopoietic tissue is slightly reduced, the proportion is roughly normal, and megakaryocytes are visible. Lumbar puncture: CSF pressure>330 mmH₂O (1 mmH₂O =0.0098 kPa), the routine, biochemistry and cytology of cerebrospinal fluid were normal, and the pathogenic examinations of bacteria, fungi and viruses were negative. Cranial magnetic resonance venous angiography (MRV): the left internal jugular vein, bilateral sigmoid sinus, bilateral transverse sinus, sinus sinus, straight sinus and superior sagittal sinus were not developed, and the possibility of thrombosis was high (Figure 1)。

Langerhans histiocytosis (LCH), also known as histiocytosis X, is a non-neoplastic proliferative lesion of unknown cause, which is characterized by pathological proliferation of Langerhans cells^[1]。 LCH can involve multiple organs and systems. Hepatic Langerhans histiocytosis (HLCH) can be part of multisystem lesions of LCH, or it can be limited to the liver. The latter was also known as hepatic eosinophilic granuloma (HEG) in the past. The onset age is mainly in children, but it is rare in adults. In this study, 3 adult patients with HLCH confirmed by pathology were collected, and their clinical and imaging manifestations were analyzed to improve the understanding of this disease.

bone marrow failure syndromes (BMFS) are a group of bone marrow failure diseases arising from damage to hematopoietic stem and/or progenitor cells, involving one or more lineages of blood cells. Clinically, it can be divided into two categories: hereditary and acquired. Acquired BMFS are the most common, while hereditary BMFS are relatively rare. Acquired BMFS are divided into two categories: primary and secondary. Secondary BMFS mainly refer to bone marrow suppression caused by hematopoietic tumors, extramedullary tumors, bone marrow infiltration, radiotherapy, chemotherapy and other factors. Primary BMFS mainly include aplastic anemia (AA), immune-related cytopenia (IRH), hypoproliferative paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndrome (MDS) and unspecified cytopenia (ICUS). This paper focuses on AA and IRH, both of which belong to bone marrow hematopoietic failure caused by abnormal immune mechanism. The main immunopathogenesis of AA is T cell immune abnormality, and cytotoxic T cells (CTL) are the main effector cells. CTL apoptosis of hematopoietic stem progenitor cells through cytotoxicity, resulting in pancytopenia; The main immune pathogenesis of IRH is humoral immune abnormalities, mainly abnormal number and function of B lymphocytes, which in turn produce autoantibodies against immature hematopoietic cells of bone marrow. Bone marrow cell membrane antibodies, as the main effector molecules, destroy or inhibit bone marrow hematopoiesis, and finally cause hemopenia. However, the main pathogenesis of PNH and MDS is due to the abnormality of hematopoietic stem cell plasm, so it is not discussed in this paper.

Pheochromocytoma (PHEO) /paraganglioma (PGL) originates from sites with chromogenic tissue distribution of the sympathetic and parasympathetic chains of the neural crest, PHEO is in the adrenal medulla; The PGL is located in the accessory ganglion of the adrenal diplomatic or parasympathetic nerves from the base of the skull to the pelvis, most commonly in the head and neck, near the abdominal renal vessels or in the Zuckerkandl body, which is located at the onset of the inferior mesenteric artery and is the largest chromiophilic tissue outside the adrenal gland^[1,2]。 pheochromocytoma/paraganglioma (PPGL) accounts for about 0.2% to 0.6% of hypertensive people and about 7% of sympathetic nervous system tumors, with an annual incidence of 0.3/1 000 000. It occurs more often in the age of 20 to 50, with more women than men. Up to 75% were never suspected before their lives, and about 15% were familial^[1]。 The genetic background, clinical presentation and degree of malignancy of PPGL were all different. About 10% to 20% of cases are diagnosed in childhood, slightly more in males. Among children with hypertension, up to 1.7% had catecholamine-secreting tumors^[3]Most of them are pheochromocytomas. PGL from sympathetic nerves is functional, while PGI from parasympathetic nerves is mostly non-functional, depending on the location and type of mutation^[1]。 About 56% of PHEO patients under 18 years old are caused by genetic mutations;<At 10, it is as high as 70%. Hereditary PHEO is often bilateral, while PPGL is often multifocal. In general, multicentric tumors are often highly suggestive of familial disease and are more common in PPGL patients^[4]。 A total of 14 PPGL susceptibility genes have been reported so far: NF1, RET, VHL, SDHD, SDHC, SDHB, EGLN1/PHD2, KIF1, SDH5/SDHAF2, IDH1, TMEM127, SDHA, MAX and HIF2^{[5,6,7,8,9,10,11,12]}。 Among them, the roles of EGLN1/PHD2, KIF1 and IDH1 have not been confirmed by other studies at present, indicating that these gene mutations are not a common cause of hereditary PPGL. EPAS1/HIF2A mutations have also only been reported in a small number of patients^[12], to be confirmed by numerous reports.

The management of primary non-induced epilepsy is a matter of great concern for epilepsy patients and their families, and whether to give intervention is also a question of confusion for clinicians. The study found that 1/3 of the patients with the first non-induced epileptic seizure had a recurrent seizure, and 3/4 of the patients with the second to third seizure had a recurrent seizure^[1]； With the increase of the number of seizures and the prolongation of the natural course of epilepsy before treatment, the effect of drug treatment became worse and worse, and the time between the two seizures was gradually shortened, so the possibility of evolving into active epilepsy increased^[2,3]Therefore, early treatment is recommended for epilepsy patients with more early seizures. However, it is still inconclusive how to deal with epilepsy patients with initial non-induced epileptic seizures or rare early seizures. The new clinical practical definition of epilepsy recently published by the International Anti-Epilepsy Alliance (ILAE) has attracted widespread attention in the epileptic community at home and abroad. The definition is that epilepsy is a brain disease, which can be identified as epilepsy if any of the following conditions meets: (1) at least two non-induced (or reflex) seizures with an interval of>24 h. (2) one non-induced (or reflex) episode and the risk of recurrence is comparable (at least 60%) to the risk of recurrence after two non-induced episodes within the next 10 years. (3) Diagnosis of some epileptic syndrome^[4]。 However, in many cases, it is impossible to accurately judge whether the risk of recurrence is>50%, 60% or 80%. For specific patients, the influence of other clinical factors should be considered, so as to have a more accurate grasp of the patient's treatment opportunity and prognosis. The progress of the factors affecting the prognosis of patients with epilepsy is reviewed.

The Cervical Spine Research Society (CSRS) was founded in 1973. It is an interdisciplinary communication platform involving multidisciplinary organizations to promote the development of basic and clinical Research on Cervical spondylosis. At present, CSRS Europe and CSRS Asia Pacific have developed. The 2014 CSRS Annual Meeting was held in Orlando, USA from December 4 to 6. The Chair of this annual meeting was Professor Darden from Carolina Orthopedic Spine Center in Charlotte, USA. The co-executive chairs are Professor Lehman and Professor Hsu, a Chinese American. The conference selected 58 papers as speeches, 15 posters and 36 electronic posters. This annual meeting continued the tradition of CSRS, and started the 19th cervical spine surgery teaching course on the first day of the annual meeting. Systematic explanation and video demonstration were given from four aspects: cervical spine surgery technique, prognosis evaluation, treatment and complications of cervical spine trauma and cervical spondylotic myelopathy. During the formal meeting, the participants discussed the research progress in the diagnosis, treatment strategy selection, efficacy and prognosis, surgical complications and basic research of cervical spine diseases. This conference attracted more than 300 cervical spine surgeons from all over the world to participate, and the number of Chinese scholars participating is also increasing year by year. This year, more than 20 Chinese scholars attended the conference, including Professor Tian Wei of Jishuitan Hospital and Professor Liu Xiaoguang, Sun Yu and Liu Zhongjun of the Third Hospital of Beijing University. The following is an overview of the hot spots and key issues of this annual meeting.