Advances in diagnostic and therapeutic strategies in cancer have significantly increased the survival of cancer patients, which causes cancer itself and its treatment related sequelae and side effects appearing in their later life. Patients with cancer before, during and even many years after completion of therapies can exhibit cognitive impairment, negatively affecting cancer survivors' quality of life and functional independence. Internationally, patients with cancer complaining cognitive symptoms due to the disease itself and/or its therapy are called cancer-related cognitive impairment (CRCI). However, many aspects of the association between cancer and cognitive impairment remain uncertain. The definitive connection between systemic cancer and central nervous system is yet to be established. Therefore, this review summarizes the current evidence on potential pathophysiology in these patients with CRCI, emphasizing knowledge gaps and the potential strategies to address them.

This issue is special for interpreting 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition). To standardize and unify the usage of terminology in this special issue, this article elaborately translates simplified table of 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) into Chinese. At the beginning paragraph, this article also explains the principles of Chinese translation and propose translation for some terminologies.

Migraine is a common clinical chronic neurovascular disease, which seriously affects the quality of life of patients. Studies have shown that patent foramen ovale (PFO) may be related to migraine, such as transient hypoxemia caused by patent foramen ovale related right-to-left shunt, or vasoactive substances passing through an unclosed oval foramen, avoiding lung tissue metabolism and directly entering the arterial system, and contradictory micro-embolism and other mechanisms may be related to migraines related to patent foramen ovale, especially migraine with aura. The closure of patent foramen ovale can benefit some migraine patients. This article intends to outline the relationship between patent foramen ovale and migraine, and its mechanism, prevention and treatment.

Recently, the 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) was published. It is the first time to divide diffuse gliomas into adult and pediatric types. Adulttype diffuse gliomas include three types:astrocytoma, IDH-mutant; oligodendroglioma, IDH-mutant, and 1p/19q-codeleted; glioblastoma, IDH-wildtype. Now, we review classification and diagnosis of adult-type diffuse gliomas for further accurate understanding and application of new edition tumors classification.

The 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) introduces a series of molecular biomarkers and new types/subtypes, which highlight the importance of integrated diagnoses and layered reports. The new edition tumors classification is reflections of the understanding in this field at present. Herein, we review the key molecular diagnostics and detection techniques, aiming to facilitate better understanding and more appropriate management for the tumors of the central nervous system.

Brain-computer interface (BCI) is a cutting-edge technology of interdisciplinary integration, which has been widely used in many fields. This article introduces the four key parts of BCI technology, including signal acquisition, feature extraction, feature classification and external devices manipulation. This review introduces the application status of BCI in the medical field, we focus on the application of network science and deep learning in BCI, including extracting deeper electroencephalography (EEG) signal representations and achieving high-precision classification. Lastly, we explore the application prospects and challenges of BCI in the medical field.

Treatment of neurosurgical intensive care patients infected with multidrug resistant (MDR) or extensive drug resistant (XDR) Acinetobacter baumannii presents significant challenges and is associated with high mortality rates. Sulbactam has long been an effective treatment for MDR Acinetobacter baumannii, particularly due to its ability to penetrate the inflamed blood-brain barrier, making it highly suitable for use in severe neurosurgical cases. However, in recent years, increasing resistance to sulbactam among Acinetobacter baumannii strains has become a concern. There is a lack of standardized guidelines regarding the dosage, administration methods, routes and combination therapy strategies for sulbactam in the treatment of these resistant infections. Thus, Chinese Neurosurgical Intensive Care Management Collaborative Group has developed the "Chinese expert consensus on the use of sulbactam to treat patients infected with Acinetobacter baumannii in the neurosurgical intensive care unit", through a thorough review of relevant evidence-based medical literature and extensive discussion and revision. This consensus includes 28 recommendations aimed at providing scientific and feasible clinical guidance for the application of sulbactam in the management of neurosurgical intensive care patients.

In the 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition), the term "circumscribed" was used to classify astrocytic gliomas with localized growth characteristics. So far, the descriptions of "diffuse" and "circumscribed", which define the growth mode, have been applied to the classification of gliomas. This paper interprets the content of circumscribed astrocytic gliomas in the new edition tumors classification, introduces the definitions and clinical characteristics of high-grade astrocytoma with piloid features and astroblastoma, MN1-altered, and analyzes the value and significance of molecular markers and gene changes in the diagnosis and treatment of circumscribed astrocytic gliomas.

Cognitive impairment has become one of the most important diseases threatening the health of the elderly population. How to identify and evaluate it early is the key to the early diagnosis and treatment for this kind of disease. This review compares the cognitive assessment scales commonly used at home and abroad so as to provide a reference for researchers to choose a suitable scale. It also introduces the recent progress of cognitive impairment screening scales in China in order to develop a cognitive impairment screening scale for Chinese population.

The integrated and layered diagnoses of central nervous system tumors is based on the growing importance of molecular information in the classification of central nervous system tumors. The 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) updated the integrated and layered diagnoses methods based on the previous version of the WHO tumors classification (fourth revised version). Layered report feature an integrated diagnosis at the top, followed by layers that display histopathological classification, WHO classification and molecular information. This review mainly focuses on the interpretation of the integrated diagnosis and grading of central nervous system tumors in the new edition tumors classification.

In this article, we look back on history of the classification of tumors of central nervous system(CNS) in brief, and further review the evolution of WHO Classification of Tumors of CNS from the first edition to the fifth edition (WHO CNS5), including the category frame, tumor type/subtype and tumor grading. Changed from histology based classification into more biologically and molecularly defined entities, WHO CNS5 was recently released as expected, after attempt in WHO CNS4 updated version and 7 update publications upon the work of the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy-Not Official WHO (cIMPACT-NOW). One of the important changes in WHO CNS5 is the separating gliomas into pediatric-type and adult-type based on their well-established molecular genetic differences. Tracing alone the clue in WHO CNS5, we introduce evolutionary process of entities and subtypes under each categories, and try to explain why they were added, deleted or why their naming was changed from WHO CNS1 to WHO CNS5. Tumor grading in WHO CNS5 has make efforts on moving close to how grading is done for non-CNS neoplasms, and added molecular parameters as biomarkers of grading based on histological features. WHO CNS5 has set new standers for clinical management of CNS tumors, and has been expected to facilitate related clinical studies and basic research in the future.

In June 2016, in order to provide a standardized clinical reference for the diagnosis and treatment of insomnia, "Guidelines for the diagnosis and treatment of insomnia disorder in China" compiled by Chinese Sleep Research Society (CSRS) was published. The guideline was compiled according to the standards of evidence-based medicine and the latest International Classification of Sleep Disorders Third Edition (ICSD - 3). It emphasized the role of cognitive behavioral treatment (CBT) in the treatment of insomnia, and enriched the assessment and interventions for special populations. This paper intends to provide an explanation and supplement to the compiling process and key elements of the guideline in details, in order to offer some help and guidance for clinicians for better understanding and application of the guideline.

With the development of the combination of medicine and engineering and the improvement of clinical rehabilitation demand, intelligent upper limb rehabilitation robots can provide stroke patients with high intensity and repetition of active rehabilitation training to restore the upper limb motor function and achieve the remodeling of damaged brain nerves. However, many upper limb rehabilitation robot systems are still in the stage of laboratory development or clinical verification. This paper summarizes the latest research results of upper limb rehabilitation robots at home and abroad, compares the advantages and disadvantages of different types of robots, looks forward to the development trend of upper limb rehabilitation robots, and provides ideas for further promoting clinical transformation.

The 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) focused on promoting the molecular pathological diagnosis in the role of the central nervous system tumor classification. According to the tumor histopathological morphology and genetic features, the new edition tumors classification added 22 tumor types or subtypes, which reflects the latest recognition of tumor clinical pathology and molecular genetics. In this paper, we will briefly introduce the new tumors with specific histopathological features in new edition tumors classification, with the purpose of understanding the biological behavior of these tumors and improving the accuracy of diagnosis, so as to benefit patients.

Embryonal tumors of central nervous system (CNS) are a class of highly heterogeneous brain tumors and the most common malignant brain tumors in children. Researches based on histology and molecular genetics can classify them. This kind of researches only can not stratify the prognosis of embryonic tumors, but also provide treatment guidance for them. The 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) updated the classification of embryonal tumors. This article will interpret the updating from two aspects of medulloblastoma and other CNS embryonal tumors.

As a new science and technology, artificial intelligence (AI) has been applied to the prevention, diagnosis, treatment and rehabilitation of stroke, and shows great potential. The combination of artificial intelligence and big data can accurately identify high-risk subjects, classify stroke subtype, and assist in the formulate of therapeutic strategies in acute period and prevention strategy of stroke. Sequentially, it can improve the effect of rehabilitation. This paper reviews the research progress of artificial intelligence in the prevention, diagnosis, treatment and rehabilitation of stroke.

Objective To summarize the clinical and syndrome (DWS) in children before and after peritoneal shunt. Methods and Results All 6 children with DWS admitted to Fujian Sanbo Funeng Brain Hospital from January 2016 to January 2023 were selected as the objects of observation, including one case of Dandy-Walker malformation (DWM) and 5 cases of Dandy- Walker variant (DWV). Before surgery, the main manifestations were cerebellar vermis defect, upward movement of the tentorial and sinusional area, severe dilatation of the fourth ventricle, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (one case); or cerebellar vermis dysplasia, cerebellar hemisphere compression, the fourth ventricle dilatation, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (5 cases). Right lateral ventriculoperitoneal shunt (2 cases) or posterior cranial fossa cyst-peritoneal shunt (4 cases) were performed, and the operations were successful without surgery-related complications. After 2.31- 7.00 years of follow-up, 6 cases of hydrocephalus and posterior cranial fossa cystic malformation were gradually relieved, 3 cases of good prognosis, cerebral hemisphere and cerebellum development, speech, movement and intelligence normal; there were 3 children with general prognosis, including cerebellar disequilibrium symptoms (one case), autism, claustrophobia (one case), and speech, motor and intellectual disorders (one case) complicated with neurocutaneous melanosis. Conclusions The brain structure of the children with DWS presented with hydrocephalus can be well developed after the hydrocephalus is corrected by single shunt. Among them, the cerebellar vermis of the DWV is well developed, while the development of the DWM presented with cerebellar vermis defect needs to be observed in more cases.

Post-stroke cognitive impairment (PSCI) is one of the most important factors leading to post-stroke disability. In recent years, studies have shown that patients with PSCI have gastrointestinal microbiome imbalance. Besides, gastrointestinal microbiome also plays an important role in the occurrence and development of PSCI. As a consequence, this research reviews the progress on the relationship between the PSCI and the gastrointestinal microbiome, in order to provide a new perspective for the prevention, the treatment and research direction of PSCI.

Parkinson's disease (PD) is a common neurodegenerative disease in the aged populations, which is characterized by resting tremor, rigidity, bradykinesia and abnormal gait, accompanied by a variety of non-motor symptoms (NMS). The prevalence and incidence of PD rise sharply with the increase of age. In the advent of global aging, the rapid growing of elderly populations results in a rising number of PD patients, especially in China. A correct understanding of the epidemiology of PD helps to respond to this challenge actively. This article aims to provide an overview of the prevalence, incidence and mortality of PD and their differences among regions. Special efforts are made to illustrate the current status and future trends of the epidemiology and economic burden of PD in China.

Objective To study the clinical features and treatment of acute disseminated encephalomyelitis (ADEM). Methods All patients admitted with ADEM during May 1990 to Dec 2010 were included in the study. Clinical data of 12 cases with ADEM were reviewed and analysed. The diagnosis of ADEM was made based on the clinical presentation, suggestive MRI and auxiliary examination findings. All patients were treated with intravenous steroids or immunoglobulins (IVIg). Results The sample consisted of 10 men and 2 women. The oldest patient was 69 years old and the youngest was 6 years old. Six patients had definite upper respiratory tract infection preceded the onset of neurological symptoms, 3 patients had non?specific fever, 1 patient had measles vaccination, 1 patient had measles prior to the onset 4 months ago. No preceding illness and vaccination occurred in 1 patient. The common presenting symptoms were fever, nausea, vomiting, headache. Neurological manifestations included cranial nerve involvement (the abducent nerve was the most common cranial nerve involved), paralysis (include hemiplegia, quadriplegia, paraplegia), altered sensorium, bladder involvement (both incontinence and retention), meningeal irrigation sign and conscious disturbance. Conclusion Despite the serious manifestation, ADEM in patient has good immediate outcome. Early diagnosis and treatment should be emphasized.

In 2021, the WHO released the Classification Tumors of the Central Nervous System (fifth edition). Compared with the revised version of the fourth edition of the WHO Classification of Tumor of the Central Nervous System in 2016, classification of ependymal tumors has many adjustments. Ependymal tumors are classified according to anatomical sites. Several new molecular subtypes are added. Myxopapillary ependymoma is adjusted to WHO grade 2 instead of grade 1. Definition of the ependymoma is deleted. Combined with the relevant literature in recent years, we interpret the updates in this new edition tumors classification about ependymal tumors in order to deepen the understanding of ependymal tumors and standardize the clinical practice.

WHO Classification of Tumors of the Central Nervous System (CNS) has been developed in the past more than 40 years after 6 versions published, which powerfully advances the clinical management of CNS tumors. We summarize the publishing process, classification characteristics and major modifications in each versions before the current edition. The classification tables of each edition complied in this summary are refreshed and dedicated to comply with the original English version, and referred to translated version as well as interpretation article in Chinese. The diverse name for the same entity in different versions are unified according to current nomenclature.

Guillain-Barré syndrome (GBS) is an acute inflammatory disorder of the peripheral nervous system and consists of at least four subtypes of acute peripheral neuropathy namely, acute inflammatroy demyelinating polyneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and Miller-Fisher syndrome. Major advances have been made in the understanding of the mechanism of some subtypes. GBS is thought to be due to autoimmunity for which immunotherapy is usually prescribed. About 20% patients died or had persistent disability despite normative immunotherapy. More research is needed to identify better treatment regimens and new therapeutic strategies. In this review, we summarized the epidemiology, diagnosis, pathogenesis, and treatment of the principal subtypes.

The 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition) was released. One of the biggest differences between new edition tumors classification and the previous editions is that the pediatric-type diffuse low-grade gliomas and the pediatric-type diffuse high-grade gliomas are classified into independent tumor families. This change is based on the fact that although pediatric-type diffuse gliomas morphologically resemble their adult counterparts, while their molecular genetics features are completely different, and the prognosis of these tumors with different molecular genetics features is different accordingly, so different treatment strategies are needed. Physicians and surgeons need to know of the features of these pediatric-type diffuse gliomas. This paper reviews the existing literature, and the pathological reports and imaging data of our hospital, summarizes the imaging and pathological diagnostic features, as well as the clinical manifestations, prognostic features and treatment suggestions of these pediatric-type diffuse gliomas. This review will provide an annotation from the view of diagnosis and treatment on pediatric-type diffuse gliomas for the physicians and surgeons.

Platelet glycoprotein (GP) Ⅱ b/Ⅲ a receptor is the final common pathway of platelet aggregation and thrombosis, which can specifically and rapidly inhibit platelet aggregation. In recent years, more and more evidences support that tirofiban, a GP Ⅱ b/Ⅲ a receptor antagonist, can be used as a treatment option for thromboembolic diseases, but its application experience in clinical practice is still relatively insufficient. In order to apply tirofiban more reasonably and normatively in clinical practice, the Chinese Stroke Society organized a writing committee of well-known experts in the field of neurological intervention in China, and wrote the consensus of experts on the clinical application of tirofiban in atherosclerotic cerebrovascular diseases. It provides clinical experience and guidance for the application of tirofiban in the field of cerebrovascular disease, and guarantees the safe and effective use of drugs in medical behaviors.

Hereditary ataxia (HA) is a clinically and genetically heterogeneous group of neurodegenerative disorders with high mortality and morbidity. It is characterized by progressive cerebellar ataxia of gait and limbs variably associated with ophthalmoplegia, pigmentary retinopathy, pyramidal and extrapyramidal signs, dementia and peripheral neuropathy. The molecular diagnosis process is proposed based on molecular classification. So far, symptomatic treatment is the mainly approach, with the lack of effective therapeutic method.

Multiple sclerosis (MS) is an inflammatory demyelinating disease in central nervous system. The etiology is still unknown. The pathogenesis may be related to autoimmune response. Clinical features are dissemination in time (multiple attacks) and dissemination in space (multifocal episode). The common used examinations including brain or spinal MRI, CSF analysis and evoked potentials. The latest diagnostic criteria is McDonald criteria published in 2010. It is now accepted that neuromyelitis optica (NMO) is different from MS in clinical, pathology, imaging and so on. Due to the diversity in clinical manifestation and the lack of specific biological parameters, the diagnosis of multiple sclerosis is still challenging.

Background Mesenchymal malignancies prefer hematogenous spread, while epithelial malignancies tend to prefer lymphatic spread. However, mesenchymal malignancies occasionally generate lymphatic metastases. Objective To investigate the clinicopathological features of the epithelioid malignant peripheral nerve sheath tumor (EMPNST). Methods The clinical data, histopathological, histochemical and immunohistochemical features in one case of EMPNST with lymph node metastases were retrospectively analyzed, and the related literatures were reviewed. Results A 42-year-old woman presented right upper arm and shoulder numbness with a right axillary nodule measuring 4 cm × 4 cm × 3 cm for 2 months. Type-B ultrasonic examination showed substantive lesions in right axillary which appeared to be a neurofibroma. CT scan showed soft tissue masses in right axillary with multiple enlarged lymph nodes were considered to be malignant lesions. MR image showed nodules in right armpit muscles were considered to be malignant tumor. The multiple nodules were identified in right axillary and the largest one was about 5 cm × 3 cm × 3 cm intraoperatively. The section of specimen was hard, gray-red and sallow in color with focal necrosis. The nodules were partly circumvoluted with blood vessel and without distinct border. The surrounding nerves were not obviously compressed. Microscopically, the structure of lymph node was damaged. The tumor invaded the surrounding fibrous connective tissue and adipose tissue with focal hemorrhage and necrosis. Tumor cells arranged in sheets, nests, and were separated by thin fibrous and blood vessels tissue. Most tumor cells were epithelioid. They were large in size with abundant cytoplasm. Cell border was indistinct, and the cytoplasm consisted of fine granules. Nuclei were invariably intensely hyperchromatic with coarse chromatin, and were vacuolated with prominent nucleoli in the center of the nuclei. Tumor cells showed frequent mitotic activity. Multinucleated giant cells and interstitial mucous degeneration were presented. Immunohistochemical staining showed that tumor cells were positive for S-100 protein, strong nuclear reactivity for Sox-10, and were positive for vimentin and Col-Ⅳ, and were negative for CK, CEA, EMA, Melan-A, CD56, CD3 and CD20. P53 positive revealed by 40% nuclear of tumor cells. Ki-67 labeling index was above 90%. Histochemical staining showed that the tumor cells were surrounded by reticular fibers. The patient had a medical history which recorded a tumor at the right wrist was removed in other hospital 8 years ago, and the wrist tumor recurred 5 years ago. The pathological diagnosis of the recurred wrist tumor was EMPNST with focal hemorrhage and necrosis. According to the history and the final pathological diagnosis, axillary tumor was diagnosed as lymph node metastasis of EMPNST in right axillary. Conclusion The metastasis of MPNST is mostly through the blood channel. This is the first case of the lymph node metastasis of EMPNST. In clinical work, it should be differentiated from other tumors with similar histopathology.

Lipid metabolism may play a role in the onset and progression of Parkinson's disease (PD), involving various pathological mechanisms such as abnormal folding and accumulation of α-synuclein (α-Syn), ferroptosis, GBA1 gene mutation, and mitochondrial dysfunction, etc.. This article reviews the lipid metabolism abnormalities associated with Parkinson's disease and explores potential related pathophysiological mechanism, aiming to provide new insights into the early diagnosis and treatment of Parkinson's disease.

The word "dementia" originates with Dr. Philippe Pinel of France. Back in 1801, Pinel reported a young female patient, aged 34, who was exhibiting some strange symptoms for someone in her age group. Pinel described this woman's condition as "demence", or an incoherence of mental faculties. It was believed that the term "dementia" came from this association. More than a century later (1906), Dr. Alois Alzheimer published a paper on one of his patients who had very similar symptoms and behaviors as Pinel's patient. This description, of a woman known as Auguste D, was the first scientific report of the disease now known as Alzheimer's disease. It is now clear that Alzheimer's disease is a major cause of dementia in elderly people as well as in relatively young adults. Research in the last two centuries has led to a greatly improved understanding of what Alzheimer's disease is, who gets it, and how it develops and affects the brain. Those works are beginning to pay off with better diagnostic techniques, improved treatments, and even potential ways of preventing these diseases.

According to the research progress in recent years, "Chinese guidelines for diagnosis and treatment of acute ischemic stroke 2018" has greatly revised and supplemented the criteria for the diagnosis and treatment of acute ischemic stroke. Based on the new clinical research evidence published in the past four years, this paper interprets the revisions and supplements of the guidelines 2018.

Chinese researchers have published some studies in English journals since 2013 on the secondary prevention of stroke. These studies focused on implementation of a structured guideline-based program for the secondary prevention of ischemic stroke in China, clopidogrel with aspirin in acute minor stroke or transient ischemic attack (TIA), higher risk of recurrent ischemic events in patients with intracranial in-stent restenosis, association of hypertension with stroke recurrence depends on ischemic stroke subtype, impact of quality of anticoagulation control, and so on. In this review, we outline the data of these high-qualitied studies on secondary prevention of stroke.

Stroke is the second major diseases leading to death, among which ischemic stroke is the most common. The key to the treatment of ischemic stroke replies on early diagnosis and treatment. With the development of technology, artificial intelligence (AI) has shown great application value in the early screening, lesion detection, ischemic penumbra evaluation, vascular occlusion judgment, treatment assistance and prognosis prediction of ischemic stroke, which is expected to further improve the diagnostic efficiency and therapeutic effect of ischemic stroke.

Neuromodulation technique not only provides a new idea for the treatment of many diseases, but also has a profound influence on the innovation and development of rehabilitation medicine. Nowadays, the neuromodulation technique in China is in the stage of rapid development, facing great opportunities and challenges, and has broad application prospects. This review intends to describe the mechanism of different types of neuromodulation technologies, and its application status and future development in the field of rehabilitation medicine, in order to promote the application and development of neuromodulation technique in the field of neurorehabilitation in China.

Objective To analyze the relevant risk factors for intracranial infection after craniotomy for resection of skull base neoplasms. Methods The clinical data of 159 patients with skull base neoplasms who underwent craniotomy from May 2010 to January 2020 in The Second Affiliated Hospital of Anhui Medical University were analyzed retrospectively. The relevant risk factors for intracranial infection were analyzed by univariate and multivariate forward Logistic regression. Results Postoperative intracranial infection occurred in 81 out of 159 patients, the infection rate was 50.94%. Multivariate forward Logistic regression analysis showed the operation time was ≥ 5 h (OR=3.438, 95%CI: 1.371-8.625; P=0.008), blood loss ≥ 400 ml (OR=2.308, 95%CI: 1.115-4.777; P=0.024), frontal sinus or mastoid air chamber breach (OR=16.817, 95%CI: 3.689-76.658; P=0.000) were the risk factors for intracranial infection after craniotomy for resection of skull base neoplasms. Conclusions There was a higher risk of intracranial infection after craniotomy for resection of skull base neoplasms in patients who had long operation time, more intraoperative blood loss as well as frontal sinus or mastoid air chamber breach. It is necessary to adopt effective prevention and control measures to reduce the risk of intracranial infection.

Objective To evaluate the therapeutic efficacy and side effects of various treatments for amyotrophic lateral sclerosis (ALS) in order to formulate the best therapeutic regimen. Methods ALS, Riluzole, Gabapentin, Lamotrigine, neurotrophic factor, antioxidant and free radical scavenger gene therapy, neural stem cell treatment, treatment were appointed as retrieval words. MEDLINE, Cochrane Library, Wanfang Database for Scientific Journals in China and Chinese National Knowledge Infrastructure (CNKI) for Scientific Journals Database were used for retrieval. Related clinical guidelines, systematic reviews, randomised controlled trials, controlled clinical trials and case-observation studies were collected following the corresponding inclusion criteria and exclusion criteria and evaluated by Jadad Scale to judge the authenticity and reliability of the conclusion. Manual searching was also used. Results After screening, 39 related articals were selected as follow: 4 systematic reviews, 18 randomised controlled trials, 11 controlled clinical trials, and 6 case-observation studies. Twenty-seven articals were of high quality (according to Jadad Scale, 11 with score 4, 13 with score 5, and 3 with score 7), while 12 were of low quality with score 3. According to the evaluation of therapeutic efficacy and side effects of various therapies, it is suggested that: 1) The Riluzole is the only drug approved by American FDA, lacking of more effective treatments. 2) When the patients' condition is not relieved, medicine-combined therapies and symptomatic treatments should be used. Conclusion Evidence-based medicine can provide best clinical evidence on ALS treatment.