2018年 · 第51卷第03期

Parkinson's disease is a common neurodegenerative disease in middle-aged and elderly people, and the patient population in China and around the world is very large. The clinical symptoms of Parkinson's disease are complex, which not only impairs the exercise ability of patients, but also involves the non-motor system and causes a variety of non-motor symptoms (NMS), which affect the physical and mental health of patients in different ways. In the past, clinical studies mostly took the improvement of motor symptoms as the main observation index, and clinicians also paid more attention to the improvement of motor symptoms of patients, but neglected the overall quality of life of patients. In recent years, the concept of health-related quality of life (HRQL) has gradually received attention in the clinical research field of Parkinson's disease, and has been introduced into international large-scale multi-center clinical intervention trials many times as an important efficacy evaluation index^[1,2]。 HRQL covers multiple domains and is believed to comprehensively reflect the overall impact of disease and clinical interventions on patients^[3]It is of great significance for evaluating and optimizing clinical treatment protocols. However, HRQL is rarely used in clinical work and is only used as an observational index in clinical research. This situation may be related to the lack of understanding of HRQL by clinicians, and it also reflects the possible problems in current clinical treatment, such as the lack of effective treatment goals and efficacy evaluation tools. Therefore, combined with previous related research and literature reports, we elaborated the application value of HRQL in clinical intervention of Parkinson's disease, the current status of HRQL in patients with Parkinson's disease at home and abroad and its influencing factors, and explored the clinical intervention strategies to improve HRQL in patients with Parkinson's disease in combination with the treatment guidelines of Parkinson's disease.

myasthenia gravis (MG) is an autoimmune disease that affects the acetylcholine receptor (AChR) on the postsynaptic membrane of the neuromuscular junction (NMJ), resulting in disturbances in neuromuscular signaling^[1]The clinical characteristics are muscle pathological fatigue and light morning and heavy evening, and often accompanied by thymic involvement^[2]But its exact pathogenesis is still not fully understood. Several studies have found that miRNA is a non-coding small molecule RNA with a length of 8 to 25 nucleotides, which acts on the 3' untranslated region of target gene mRNA and transcriptionally regulates the expression of multiple genes through complete or incomplete pairing with the untranslated region^[3]。 At present, studies have shown that miRNA-125-5p, miRNA-181c and miRNA-27a-3p are involved in the pathogenesis of MG^[4,5,6]。 The association study of complex diseases using miRNA as molecular markers will provide new ideas for further exploring the pathogenesis of MG and finding new biomarkers to assist in diagnosis. We examined the expression level of miRNA-635 in peripheral blood mononuclear cells (PBMCs) of patients with MG to investigate its correlation with the onset age and clinical type of MG.

Neurological diseases have become the second leading cause of death in the world, with stroke ranking first among all neurological diseases^[1]。 In some cities with high incidence of cerebrovascular diseases in China, cerebral hemorrhage accounts for 26.7% ~51.5% of stroke patients, and it is one of the main diseases causing death and disability in China^[2]。 However, at present, the treatment of cerebral hemorrhage is limited to symptomatic treatment such as dehydration and intracranial pressure reduction, which cannot repair and fill the dead nerve cells in the necrotic area of the brain, resulting in poor prognosis of cerebral hemorrhage, often leading to incurable paralysis or even life-threatening^[3]。 Therefore, many scholars have shifted their attention to neuroprotection and repair treatment after cerebral hemorrhage, and stem cells have received widespread attention because of their self-renewal and multidirectional differentiation potential. human umbilical cord blood mesenchymal stem cells (huCB-MSCs) not only come from a wide range of sources and are convenient to collect, but also have more abundant and primitive cell content, and light transplantation-versus-host response^[4,5], is relatively sterile and is regarded as the most desirable source of cell therapy. At present, the experimental treatment of huCB-MSCs transplantation has been applied to many neurological diseases, such as Alzheimer's disease, Parkinson's disease^[6,7]Autoimmune meningitis, amyotrophic lateral sclerosis^[8,9]and spinal cord injury^[10]。 Some studies have found that huCB-MSCs have a therapeutic effect on cerebral ischemic injury in rats^[11]However, there are few reports on the study of cerebral hemorrhage. We aimed to observe the efficacy of local huCB-MSCs transplantation in the treatment of cerebral hemorrhage rats, and to provide more theoretical basis for the treatment of cerebral hemorrhage by huCB-MSCs transplantation.

nonconvulsive status epilepticus (NCSE) is a major category of traditional epilepsy classification. Because it is manifested as nonconvulsive and subclinical seizures in most cases, it is underestimated clinically and difficult to be detected^[1]Its etiology is also complicated. A case of NCSE caused by Hashimoto's encephalopathy admitted to our department is reported below.

fungal rhinosinusitis (FRS) is a disease of the nose and sinuses caused by fungal infection, also known as rhinosinusomycosis^[1]。 According to the duration of the disease course, it is classified as acute (symptom duration<1 month) or chronic (symptom duration>3 months); Invasive or non-invasive depending on the presence or absence of involvement of surrounding structures and tissues^[2]。 acute invasive fungal rhinosinusitis (AIFRS) is pathologically characterized by fungal invasion of sinus mucosa, submucosa, blood vessels or bone, often with intracranial and extracranial complications^[3]。 Clinically, cases of nervous system damage caused by AIFRS are prone to misdiagnosis and delay treatment. Two cases of cavernous sinus-orbital apex syndrome caused by AIFRS admitted to us are reported to suggest that the majority of clinicians should pay attention to the diagnosis and treatment of this disease.

In recent years, with the continuous development of neuroimmunology, more and more autoimmune encephalitis-related antibodies have been discovered. It is now known that anti-contactin-associated protein 2 (CASPR2) antibodies are distributed in the central and peripheral nervous systems and appear in patients with limbic lobe encephalitis and/or Morvan's syndrome. There are many clinical symptoms associated with this antibody, and most of the symptoms are superimposed. leucine-rich glioma inactivated 1 (LGI1) antibody mainly exists in patients with limbic lobe encephalitis and a few Morvan's syndrome, with epilepsy and cognitive dysfunction as common symptoms. We report a case of CASPR2 antibody-associated encephalitis with LGI1 antibody positivity with rare presentation of Morvan's syndrome for clinical reference.

Parkinson's disease (Parkinson's disease) is a common neurodegenerative disease, and depression is one of the most important non-motor symptoms, with an incidence rate of up to 40%^[1]。 Its onset is insidious, even earlier than motor symptoms, which seriously affects the quality of life, daily function and prognosis of patients with Parkinson's disease. At present, drugs are mainly used to treat depression in Parkinson's disease in clinic, but there are problems such as drug interaction, adverse drug reactions and poor patient compliance, which bring severe challenges to the treatment.

Parkinson's disease is a common neurodegenerative disease in the elderly. As the course of the disease progresses, the exercise capacity of patients with Parkinson's disease decreases significantly, along with multiple non-motor symptoms^[1]。 The progressive degeneration and loss of dopaminergic neurons in the dense part of the substantia nigra of the midbrain and the progressive attenuation of dopamine neurotransmitters in the striatum are the main pathological basis of Parkinson's disease. Although levodopa and other drug treatments can effectively improve the motor symptoms of patients, with the progression of the disease, patients with Parkinson's disease gradually develop axial motor symptoms such as slurred speech, dysphagia, gait balance disorder and non-motor symptoms such as progressive cognitive impairment^[2]。 These clinical symptoms have poor effect on levodopa drug therapy (L-dopa nonresponsive symptoms), which are important factors affecting the quality of life and survival longevity of patients with advanced Parkinson's disease. The results suggest that the appearance of these symptoms may be related to other etiological and pathological changes besides the degeneration of dopaminergic neurons in the nigrostriatum^[3]。 cerebral small vessel disease (CSVD) is a syndrome with clinical, neuroimaging and pathological changes caused by intracranial arterioles, arterioles, capillaries, venules and arteriovenous anastomotic branches. With the development of neuroimaging technology, people's awareness of CSVD is gradually increasing. CSVD is a common type of cerebrovascular disease, which occurs in the elderly. In recent years, related studies have continuously confirmed that Parkinson's disease and CSVD are comorbid. The study found that CSVD and community elderly people have mild parkinsonism sign (MPS)^[4]And the progression of motor symptoms and some non-motor symptoms in patients with Parkinson's disease are closely related. We will review the related clinical studies in recent years from the occurrence of Parkinson's disease in patients with CSVD, the occurrence of CSVD in patients with Parkinson's disease, the factors affecting the occurrence of CSVD in patients with Parkinson's disease, and the clinical characteristics of CSVD in patients with Parkinson's disease, so as to further clarify the relationship between Parkinson's disease and CSVD, and provide a new direction for clinical intervention and research of Parkinson's disease in the future.

Parkinson's disease (Parkinson's disease) is a common neurodegenerative disease with progressive development. The pathological changes of the disease are degeneration of substantia nigra and striatum pathways, and the clinical manifestations are motor symptoms mainly such as retardation, myotonia, tremor and postural balance disorder, and non-motor symptoms mainly such as cognitive decline and depression^[1,2,3]。 At present, the preferred treatment for Parkinson's disease is drug therapy, but it can only improve the symptoms of patients, but cannot prevent the progression of the disease^[4,5]。

IgG4-related disease (IgG4-RD) is a chronic autoimmune-mediated inflammatory and fibrotic disease of unknown cause. It was first proposed by scholars in 2001 in the relationship between autoimmune pancreatitis (AIP) and IgG4-positive plasma cells. 2003 Kamisawa et al.^[1]It was found that in patients with AIP, IgG4^＋Plasma cells not only exist in the pancreas, but also appear in bile ducts, gastrointestinal mucosa, salivary glands and lymph nodes, thus recognizing that IgG4-RD is a systemic disease and introducing the concept of IgG4 systemic disease for the first time. Year 2010Autoimmun RevMagazine officially named it IgG4-RD^[2]。 IgG4-RD to extensive IgG4 in tissue and organs^＋It is characterized by massive proliferation and infiltration of lymphocytes, sclerosis and fibrosis, with or without elevated serum IgG4 levels, which can involve multiple organs or systems, causing organ enlargement and tissue destruction, thus manifesting as AIP, sclerosing sialadenitis, inflammatory pseudotumor of liver and breast, retroperitoneal fibrosis, Mikulic disease, etc.^[3]。

Tremor is one of the common manifestations of neurological diseases, and extrapyramidal lesions are most likely to be thought of in clinic. In fact, tremor is not uncommon in peripheral neuropathy. Literature reports that tremor can be seen in 10.9% ~40% of hereditary sensorimotor neuropathies^[1]。 In recent years, tremor in patients with acquired inflammatory neuropathy, such as IgM paraproteinemic neuropathy (IgMPN), has received increasing attention^[2,3]Multifocal motor neuropathy (MMN), and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)^[4,5]Etc. CIDP is the most common chronic immune-mediated peripheral neuropathy, with a prevalence of (1~9) /100,000^[1]。 Typical CIDP manifests as recurrent or chronic progressive motor sensory neuropathy lasting more than 8 weeks^[6,7]。 Recent cohort studies have shown that 55% to 80% of CIDP patients have postural tremor^[5,8,9]。

Transthyretin-related familial amyloid polyneuropathies (TTR-FAP) are an autosomal dominant genetic disease caused by abnormal deposition of transthyretin-type amyloid in tissues due to TTR gene mutations. It is mainly characterized by neurological dysfunction that progresses symmetrically from the distal to the proximal end of the limb, which can involve the motor, sensory and autonomic nerves, and may be accompanied by other organ dysfunctions, such as amyloid myocardium and/or kidney disease. The disease can occur in all age groups. It is reported abroad that the onset of disease is common in patients around the age of 20, and there are also a few elderly patients, and usually die within 7 to 12 years after the onset of disease^[1,2]。 The disease is globally distributed with an unknown prevalence, and the prevalence varies greatly between different regions. Portugal, Sweden and Japan are areas with high incidence of this disease, with the prevalence rate ranging from 1/10 000 to 1/1 000. The prevalence rate in non-high incidence areas such as the United States and France is less than 1/10 000. The prevalence rate in China has not been reported yet^[3]。 We analyzed the characteristics of TTR-FAP patients in China, introduced the commonly used treatment options and clinical trial results in the world, and summarized the treatment selection process and follow-up process, in order to provide reference for the treatment and follow-up management of TTR-FAP patients in China.

The 21st International Conference on Parkinson's Disease and Movement Disorders was held in Vancouver, Canada from June 2 to 9, 2017. More than 3,000 experts, scholars, doctors and researchers from all over the world attended the conference. More than 40 neurosurgeons and scientific researchers in China attended the meeting. During this meeting, we exchanged and introduced the progress made in this field in the past year, so that participants had more gains and experiences. What is more worth mentioning is that the International Society of Movement Disorders (MDS) was changed in 2017, and Chinese scholar Professor Chen Shengdi was elected as the executive committee of MDS for the first time, and Professor Zhang Baorong was elected as the director of MDS Asia-Pacific for the first time.

The 20th National Neurology Conference of Chinese Medical Association was held in Suzhou, Jiangsu Province from September 7 to 10, 2017. This conference was sponsored by Chinese Medical Association and Neurology Branch of Chinese Medical Association, and hosted by Jiangsu Medical Association and Neurology Branch of Jiangsu Medical Association.

At the end of the year, the inaugural meeting of the sixth editorial committee of Chinese Journal of Neurology was successfully held in Nanning, Guangxi Province, a beautiful border city, on December 16th, 2017. Jiang Yongmao, president of Chinese Medical Association Magazine, and nearly 80 experts, including consultant, honorary editor-in-chief, editor-in-chief, deputy editor-in-chief and editorial board of the sixth editorial board, and all members of the editorial department of Chinese Journal of Neurology attended the meeting.