High quality guidelines/consensuses have always been a key focus of professional journals. Since 2000, especially in the past decade, the Chinese Journal of Neurology has published a series of disease related diagnostic standards and treatment guidelines/consensuses, providing clinical doctors with standardized, efficient, and scientifically economical diagnosis and treatment plans. The article discusses the conditions that high-quality guidelines/consensuses should meet and what situations should not occur, and encourages more and better high-quality guidelines/consensuses articles to be published in the Chinese Journal of Neurology in the new year.

Since its inception over 60 years ago, the Chinese Journal of Neurology has published many readable and usable high-level clinical papers, including many of the first reported cases of neurological diseases in China, the results of large sample clinical studies, practical and authoritative guidelines for the diagnosis and treatment of diseases or techniques, consensus, standards and norms (hereinafter referred to as guidelines/consensus). Through reading these articles, clinicians have increased their new understanding of nervous system diseases, standardized the diagnosis and treatment of nervous system diseases, and improved the level of diagnosis and treatment. However, there is still a gap between the articles received and some clinical papers published in the journal in recent years and the high standard and standard clinical requirements. Therefore, we propose that complete and standard case data be the cornerstone; authenticity of case data be the bottom line; high-level authoritative guidelines/consensus be published; precision education, active promotion of guidelines/consensus be couducted. Hope we can continue to work hard in the New Year to do a better job with the magazine.

For the Chinese Journal of Neurology, it is faced with an opportunity for the state encouraging Chinese authors to publish their papers in domestic journals, but it is also faced with the challenges of Science Citation Index journals competing for manuscript sources and changing of readers′ reading habits as well as the competition of domestic similar medical journals. With the establishment of the Seventh Editorial Board of Chinese Journal of Neurology, the Journal will mainly take the following measures to recreate brilliance in the next four years: attracting more high-quality original articles by strengthening solicitation of manuscripts and shortening publishing delay, improving the quality of editorials and review articles, enriching the content and columns of the Journal, publishing more high-level guidelines and consensuses, being more open and inclusive, improving digital construction, enhancing academic service capacity, taking into account both domestic and international orientation.

Axial myopathy is a general term for a group of myopathy involving the axial muscles. It refers to a group of skeletal myopathy in which paraspinal muscles are individually or significantly affected, with or without the involvement of whole body skeletal muscles. Axial muscle weakness is often ignored in clinical practice. The evaluation of axial muscle is mainly the evaluation of the paraspinal muscles (erector spinae) in current literature. The clinical manifestations of paraspinal muscle weakness are dropped head syndrome and camptocormia. Physical examination and skeletal muscle magnetic resonance imaging, especially the whole body muscle magnetic resonance scan, are vital for the evaluation of axial muscle. It is of great clinical significance to increase attention to the diagnosis and differentiation of axial myopathy, which is helpful to avoid missing treatable diseases and improve the understanding and early recognition of associated myopath.

Objective:To summarize the clinical, thigh magnetic resonance (tMRI) and electromyographic (EMG) characteristics in patients with immune-mediated necrotizing myopathy (IMNM).Methods:A total of 32 IMNM patients who were admitted to the Department of Neurology from April 2019 to April 2021 were enrolled at the First Medical Centre of Chinese PLA General Hospital. According to the type of antibody, the patients were divided into anti-SRP antibody positive (SRP +) group, anti-HMGCR antibody positive (HMGCR +) group and seronegative (SN) group. The gender, age, course of disease, myositis antibodies, extramuscular manifestations, EMG were collected and analyzed among three groups. The characteristics of skeletal muscle were assessed by tMRI inflammatory edema and fat infiltration scores. Analysis of variance, Kruskal-Wallis test and Chi-square test were used to compare the differences in different clinical characteristics and tMRI scores among the three groups. When there was a statistical difference among the three groups, the comparison between the two groups was corrected by the Bonferroni method. Result:(1) Of the 32 patients, 20 were females (62.5%).The median age of onset was 47±14 years, 25 (78.1%) patients had an acute or subacute course.There were 17 (53.1%) with SRP +, 8 (25.0%) with HMGCR +, and 7 (21.9%) with MSAs (myositis specific antibodies) negative. Anti-Ro52 antibody was the most common combined antibody (12/32, 37.5%), among which 10 were in SRP +group.(2) The CK of all patients were elevated, median was 5 948 (4 229, 7 664) U/L. There was no statistical difference of MMT scores among three groups. The proximal limb score was lower than distal limb ( P<0.01). The axial muscle score was lower than the distal limb score ( P<0.05).(3) Extramuscular manifestations of HMGCR + group were lower than those of the other two groups (12.5% vs. 71.4% and 76.5%, P<0.017). Rash (60.0% vs.14.3%, P<0.05) and interstitial pulmonary diseases (70.0% vs. 14.3%, P<0.05) were more common in patients with anti-SRP coexistence with anti-Ro52 than those with isolated anti-SRP. Connective tissue disease was more common in SN group (57.1% vs. 11.8% and 0, P<0.017).(4) tMRI showed fascial edema of SN group was more obvious than that of the other two groups ( P<0.017). There was no statistical difference in the degree of fat infiltration and inflammatory edema among three groups, but SRP + group had more cases of early fat infiltration.(5) Myotonic potentials (25.0% vs. 0 and 0, P<0.017) and compound repetitive discharges (CRDs) (50.0% vs. 5.9% and 0, P<0.017) were common in HMGCR + group. Proteomic analysis found significantly different expressed proteins in skeletal muscle of patients with myotonic potentials or CRDs were associated with cytoskeleton, cell junction and extracellular matrix. Conclusion:IMNM with pure anti-SRP antibody positive and anti-HMGCR positive were mainly affected by skeletal muscles. Those who were co-positive for anti-SRP antibody and anti-Ro52 antibody had more extramuscular manifestations, which might be a special subtype of SRP + group. This study proposed for the first time that myofascial inflammatory edema is an early sign of SN-IMNM injury. EMG of HMGCR +group were more prone to myotonia potential and CRDs.

Though affected by the COVID-19 epidemic in 2020, the Chinese Journal ofNeurology successfully overcame the difficulties and successfully completed the annual editing and publishing work. In 2020, editorial board meetings were held monthly to decide whether or not to publish a manuscript, 12 theme issues were published, nine guidelines and consensuses by the Chinese Society of Neurology were published, 10 continuing medical education articles by famous experts were published, and several COVID-19 related articles were quickly published. In 2021, efforts will be made to make the journal to a higher level.

Tumor markers (TMs) in blood are important tools for the diagnosis, prognosis prediction and monitoring therapeutic efficacy of malignant tumor. Measurement of TMs in pleural and peritoneal fluid has been widely used. However, the significance of common TMs associated with systemic solid tumors in cerebrospinal fluid (CSF) has been overlooked to some extent. TMs in CSF are within extremely low limits in patients without intracranial malignant diseases. Slightly elevated TMs in CSF usually indicate abnormal intrathecal synthesis. Measurement of TMs in CSF is a useful and feasible ancillary diagnostic tool for intracranial metastasis, especially for leptomeningeal metastasis.

Objective:To analyze the clinical manifestations, skeletal muscle pathology, electromyography, skeletal muscle magnetic resonance imaging and gene mutations of a family with distant-onset DnaJ (heat-shock protein 40) homolog subfamily B member 6 (DNAJB6) myopathy.Methods:A total of three generations with three cases of the disease in a family, inherited by autosomal dominant inheritance, were collected. The examination of muscle enzymes, left biceps biopsy, skeletal muscle magnetic resonance imaging (MRI) and electromyography, etc,were performed for the family 's proband. Whole-exon sequencing was performed to screen the proband for pathogenic genes, and Sanger sequencing technology was performed to verify mutation sites of the proband′s family members. Results:The proband is a 30-year-old male who began to show weakness in the distal muscles as a teenager, and then gradually developed to the proximal muscles, accompanied by muscle atrophy of the limbs, mainly affecting small muscles in the hands and distal muscles of the lower limbs. Muscle enzymes of the proband were slightly elevated. Skeletal muscle MRI indicated muscle atrophy and fatty degeneration in the proximal and distal extremities, which in the distal extremities were more severe, mainly affecting the muscle groups of the posterior group. Electromyography indicated chronic myopathic damage. Muscle pathology suggested chronic muscle fiber damage and rimmed vacuoles could be found. The proband was found a heterozygous mutation [c.298T>G(p.F100V)] in DNAJB6 gene by all-exon sequencing. Sanger sequencing confirmed that his brother (similar medical history) and the second daughter also had the same mutation, and the eldest daughter was not detected the mutation at the above site. The second daughter is not ill and is a carrier of the mutation. The father of the proband died of pancreatic cancer and had similar symptoms during his lifetime.Conclusions:The above mutation of DNAJB6 gene is the pathogenic gene of this family. The clinical features are adolescence-onset muscle weakness and atrophy in distal extremities. This is the first family report of distal-onset DNAJB6 myopathy caused by mutations at this site in China.

In the coming new year, more innovative researches are encouraged to be published in the journal. We hope researchers pay more attention to ethic issues and conflicts of interest statement, details on case ascertainment and control selection method (such as diagnosis, inclusion and exclusion criteria). We also hope to publish more systematic reviews in the journal.

Based on the Chinese guidelines for diagnosis and treatment of cerebral venous sinus thrombosis 2015, combined with the relevant progress in China and abroad in recent years, the revised Chinese guidelines for diagnosis and treatment of cerebral venous thrombosis 2019 mainly updated the background information to December 2019, refined and supplemented the recommendations, including the etiology and risk factors, clinical manifestations and imaging diagnosis, treatment and prevention. It is a new basis for the diagnosis and treatment of cerebral venous thrombosis in China.