2016年 · 第49卷第09期

For a long time, although it has been believed that Parkinson's disease has existed for quite some time before clinical onset, it was not until the discovery of synuclein and the pathological grade of Parkinson's disease proposed by Professor Braak of Germany that the pro-dromal stage of Parkinson's disease gradually gained recognition and attention. Throughout the development of Parkinson's disease course, the stage from the onset of non-motor symptoms to the onset of motor symptoms that meet the diagnostic criteria of Parkinson's disease before the onset is defined as the Parkinson's disease prodromal. The course of Parkinson's disease is irreversible, and at present, all drugs and surgical treatments can only improve the clinical symptoms of patients, but cannot completely cure the disease. Clinical trials of neuroprotective drugs in patients with onset have not yet obtained definite positive results. One of the possible reasons is believed to be that the intervention treatment is too late and the compensatory period of the disease has been missed. Therefore, early warning and diagnosis of patients with Parkinson's disease in the prodromal stage and intervention when the development of Parkinson's pathology has not yet lost compensation are one of the most promising keys to delay or even block the onset of patients.

Alzheimer's disease (AD) is a serious disabling and fatal neurodegenerative disease. It is also one of the most common types of dementia and one of the important causes of death in the elderly^[1]。 With the ageing of the population in China, the prevalence of AD is increasing year by year. It is estimated that there are about 300,000 new cases in China every year, and the total number of AD patients in China is expected to exceed 20 million in 2030^[2]。 In 2014, Professor Jia Jianping's research team conducted a study on the prevalence of AD among the elderly over 65 years old nationwide, and the results showed that the prevalence of AD in my country was 3.21%^[3]； Since then, the team has once again conducted a large-scale epidemiological investigation on the prevalence of mild cognitive impairment (MCI) caused by various etiologies in China, and found that the incidence of MCI in rural areas in China is significantly higher than that in urban areas, and vascular factors are the primary risk factors in MCI caused by various etiologies^[4]。 In 2016, Professor Zhang Zhenxin's team reported the epidemiological survey results of the prevalence and incidence of various types of dementia in my country. The data showed that the annual incidence of dementia among people over 65 years old in my country was as high as 12.1/1,000, of which 8.2/1,000 were confirmed AD^[5]

hereditary peripheral neuropathies (HPN) include hereditary motor and sensory neuropathies (HMSN), hereditary motor neuropathies (HMN), hereditary sensory neuropathies (HSN), and hereditary sensory and autonomic neuropathies (HSAN).^[1]。 Hereditary motor neuropathy usually has no or only mild sensory impairment, whereas hereditary sensory neuropathy is generally dominated by sensory involvement, which may be accompanied by autonomic dysfunction and skin ulcers^[2]。 Hereditary motor and sensory neuropathy, also known as Charcot-Marie-Tooth disease (CMT), is the most common type of hereditary peripheral neuropathy^[1]。 This paper briefly introduces the characteristics of CMT, especially the current diagnosis problems.

primitive trigeminal artery (PTA) originates from the cavernous sinus segment of the internal carotid artery and is an embryonic primitive vessel between the internal carotid and basilar arteries^[1,2]。 PTA may be associated with unilateral internal carotid artery occlusion^[3]At present, there are no reports of PTA combined with bilateral internal carotid artery occlusion. A patient with transient ischemic attack (LS-TIA) with PTA main artery aneurysm and chronic bilateral internal carotid artery occlusion and limb shaking caused by multiple extracranial artery stenosis is reported as follows, and the collateral compensatory role and possible pathogenesis of PTA in this case are discussed.

Brainstem encephalitis refers to an inflammatory lesion that occurs in the brainstem. We report a rare case of brainstem encephalitis with positive serum anti-GM2 IgM antibody. Its pathogenesis may be related to the occurrence of abnormal immune response against GM2 in the brainstem of the patient. This disease may belong to gangliosides (GS) antibody lineage disease. Glucocorticoids have a good therapeutic effect.

diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin's lymphoma (NHL), accounting for 30% to 40% of all adult NHL, and the incidence rate in China is higher. The 4th edition of the classification further divides them into three categories: non-specific types, special subtypes and independent diseases. Nonspecific is a group of DLBCLs that cannot be classified as a particular subtype or independent disease, and the vast majority of DLBCLs belong to this group. The well-known primary central nervous system lymphoma (PCNSL) belongs to a particular subtype of DLBCL; intravascular large B-cell lymphoma (IVLBCL) is an independent disease of DLBCL^[1]。 IVLBCL is a rare type of NHL with an average age of onset of 60 to 70 years^[2,3,4]Its clinical feature is that tumor cells only grow in small blood vessels (mainly capillaries and retrocapillary venules), and are generally difficult to find in peripheral blood and bone marrow^[5]。 In Western countries, IVLBCL occurs in the central nervous system and skin^[2,6]While in Eastern countries, it is more common in bone marrow, liver and spleen^[3,7,8]In the late stage, small blood vessels in the whole body can be involved, and multiple organ lesions can occur. The disease progresses quickly, the prognosis is poor, and the mortality rate is extremely high. The purpose of treatment is to prolong the survival time of patients. At present, there are very few cases of neurological IVLBCL reported publicly and confirmed by pathology in China, only more than 10 cases^{[9,10,11,12,13,14,15,16,17,18]}。 Therefore, a case of IVLBCL admitted to the Department of Neurology of Xiangya Hospital is reported. The diagnosis and treatment process of this patient went through several turns, and finally was diagnosed by pathology at Stanford University Medical Center in the United States.

Voltage-gated potassium channel (VGKC) antibodies mainly include contactin-associated protein 2 (Caspr2) and leucine-rich, glioma inactivated protein 1 (LGI1) antibodies, which are expressed in central and peripheral nervous systems^[1]。 In recent years, this kind of antibody-positive related diseases are more and more recognized by clinicians, but the diagnosis is still difficult, and it is easy to be misdiagnosed in clinical practice. We report a case of spinal radiculopathy associated with Caspr2 antibody positivity for clinical reference.

Steroid-responsive chronic lymphocytic inflammation with pontic perivascular enhancement (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, CLIPPERS) It is a chronic inflammatory disease of the central nervous system characterized by perivascular lymphocyte infiltration, and its combination with extracentral lesions is relatively rare. A case of CLIPPERS with interstitial lung lesions is reported as follows.

Neurosyphilis is an infectious disease of the central nervous system caused by Treponema pallidum with diverse and non-specific manifestations, but rare persistent myoclonic manifestations. This paper reports the clinical data of a patient with neurosyphilis with persistent myoclonus as the main manifestation admitted to our department in June 2015 and analyzes its characteristics, so as to improve the understanding of the special manifestation of neurosyphilis.

Alzheimer's disease (AD) is a central nervous degenerative disease that often has episodic memory impairment as the first symptom and gradually progresses to comprehensive cognitive decline, accompanied by mental behavior abnormalities, and ultimately leads to loss of daily life ability. It is the most common cause of dementia, accounting for 60% ~70% of dementia people^[1]。 There has been evidence that the pathological process of AD begins 20 to 30 years before the onset of cognitive dysfunction^[2]。 NIA-AA AD diagnostic criteria revised by the National Institute of Aging (NIA) and Alzheimer's Association (AA) in 2011^[3,4,5]and IWG-2 AD diagnostic criteria revised by the International Working Group (IWG) in 2014^[6]Abnormal biomarkers of AD have been included in the diagnostic criteria of AD, and "preclinical AD" has been included in the disease spectrum of AD. Among them, abnormal deposition of beta-amyloid protein (A β) in the brain is the most important biomarker for the diagnosis of AD.¹¹C-Pittsburgh complex (PIB) -early positron emission computed tomography (PET) molecular imaging has confirmed that A β is most likely to deposit in posterior cingulate gyrus, precuneus lobe, inferior parietal lobule, medial prefrontal cortex (MPFC) and lateral temporal lobe, and these brain regions are highly overlapping with default mode network-related brain regions, and the strength of default mode network functional connection gradually weakens with the increase of A β deposition^[7]。 Therefore, default network function abnormalities are expected to be a biomarker for diagnosing "preclinical AD" and "clinical AD". We review the discovery history of default networks and their progress in early diagnosis of AD.

rapid eye movement sleep behavior disorder (RBD) is a kind of abnormal sleep that repeatedly exhibits complex behavior and/or vocalization accompanied by dream deduction in the rapid eye movement (REM) phase. Its characteristic clinical manifestations and the appearance of rapid eye movement sleep without atonia (RWA) in polysomnography (PSG) are necessary conditions for the diagnosis of RBD^[1]。 The disease is not uncommon in middle-aged and elderly people. In the Chinese population, a large-scale community epidemiological survey based on the RBD scale found that the prevalence of suspected RBD is 4% to 5%^[2]。 The prevalence of RBD increases with age, and is more common in men, about 4 times or more than in women^[3]。 In recent years, some idiopathic RBD (iRBD) may occur years to decades earlier than synucleinopathies, including Parkinson's disease, multiple system atrophy (MSA), and dementia with Lewy bodies (DLB), suggesting that RBD may be one of the more specific and sensitive clinical markers in the early stage of synucleinopathy^[4]。 We will review the link between RBD and synucleinopathy.

Central nervous system (CNS) infection is a common neurological disease. Due to the limitation of clinical laboratory testing, more than half of CNS infection cases cannot be diagnosed definitively. At present, the most widely used methods for detecting pathogens of CNS infection in clinic mainly include morphological identification of cerebrospinal fluid pathogens, peripheral blood or cerebrospinal fluid culture, detection of pathogen antibodies in cerebrospinal fluid and serum, and genetic detection of cerebrospinal fluid. Various methods have their limitations, among which culture and pathogen antibody detection have limited scope of application, especially for viruses. Studies have shown that only 1.9% of CSF virus cultures are positive, and less than 0.1% excluding enterovirus and herpesvirus infections^[1,2]。 Genetic testing detects the presence of pathogen nucleic acid sequences in cerebrospinal fluid samples by molecular biology methods to confirm the diagnosis of CNS infection. As a molecular diagnostic method, genetic testing provides accurate results for determining the pathogens of CNS infection, which is of great significance.

The 18th National Neurology Conference of Chinese Medical Association was held at Century City International Conference Center in Chengdu, Sichuan Province from September 17th to 20th, 2015. This conference was sponsored by Chinese Medical Association and Neurology Branch of Chinese Medical Association, hosted by Sichuan Medical Association and Neurology Professional Committee of Sichuan Medical Association, and co-organized by West China Hospital of Sichuan University. 2015 coincides with the 100th anniversary of the founding of the Chinese Medical Association. The majority of doctors from the Neurology Branch actively participated in this conference. More than 5,200 doctors from neurology and related disciplines signed up for the conference and submitted 3,567 papers, celebrating the 100th anniversary of the Chinese Medical Association with practical actions. The conference activities are rich and colorful. Due to the limitation of space, this article is only a selection of some wonderful contents.