2015年 · 第48卷第02期

limbic encephalitis (LE) is a clinical syndrome that occurs in the limbic system of the brain. In particular, autoimmune-mediated LE often has a subacute onset in clinic, often manifested as recent forgetfulness, disorientation, mental behavior abnormalities, and often epileptic seizures and other symptoms; Sometimes it is difficult to distinguish from viral encephalitis and Hashimoto's encephalopathy. Many new viral encephalitis often have no clear etiological evidence^[1], so, more easily confused with autoimmune LE. Therefore, it is necessary to understand LE more comprehensively, master its related characteristics and pay attention to its diagnosis.

Parkinson's disease is a degenerative disease of the nervous system, mainly manifested by quiescent tremor, bradykinesia, muscle stiffness, and postural balance disorder. At present, the clinical diagnosis mainly depends on medical history and levodopa reactivity. In recent years, with the advancement of molecular imaging diagnostic technology, positron emission tomography (PET) and single photon emission computed tomography (SPECT) have also become an important basis for clinical diagnosis of Parkinson's disease. Early and correct diagnosis of Parkinson's disease can help patients to regularly treat as soon as possible and delay the progression of the disease. However, the diagnosis of this disease still needs autopsy pathological biopsy (referred to as biopsy). Therefore, it is very important to find the basis of in vivo pathological diagnosis for early and correct diagnosis of Parkinson's disease.

Wernicke encephalopathy (Wernicke encephalopathy) is a form of vitamin B₁Metabolic disorders of the central nervous system caused by deficiency. The most common cause is chronic alcoholism, but clinically many patients with Wernicke encephalopathy do not have a history of alcoholism. The disease is an acute or subacute course, with dangerous symptoms, and insufficient understanding can easily delay diagnosis and treatment, resulting in an increase in mortality rate. We collected the clinical data of 20 patients with non-alcoholic Wernicke encephalopathy in our hospital for retrospective analysis to explore the pathogenic factors, cranial imaging and clinical characteristics of Wernicke encephalopathy.

acromegaly is a rare chronic progressive endocrine disease. More than 95% of acromegaly is caused by pituitary adenomas that secrete growth hormone^[1]。 Acromegaly causes multiple peripheral neuropathies other than carpal tunnel syndrome, which are rarely reported at home and abroad. A case of peripheral neuropathy caused by pituitary adenoma combined with acromegaly was admitted to our hospital, which is reported as follows.

Kennedy's disease, also known as X-linked spinal and bulbar muscular atrophy (SBMA), was developed in 1968 by American doctors Kennedy et al.^[1]First reported, is X-linked recessive genetic disease. The patients with Kennedy disease were all male, with middle-aged onset, and their clinical manifestations were mainly atrophy and weakness of proximal limb and tongue muscles^[2]。 We report a patient with Kennedy's disease diagnosed by genetic testing. Clinically, distal upper limb involvement is predominant and there is no lingual muscle atrophy, which is relatively rare.

The megalodolichobasilar or megadolichobas-ilar artery (MDBA) is a relatively rare basilar artery disease, which is characterized by significant prolongation, thickening and twisting of the basilar artery. The clinical manifestations are mainly posterior circulatory ischemia or cranial nerve compression, which affects the circulation of cerebrospinal fluid and leads to obstructive hydrocephalus. We found one case, which is now reported below.

Rivaroxaban is a novel direct factor XA inhibitor that inhibits thrombosis by blocking thrombin production. The main adverse reaction was bleeding, and others included gastrointestinal symptoms, increased glutamyl transpeptidase and increased transaminases. The drug does not need to monitor the international standardized ratio (INR) and has been increasingly used in anticoagulant therapy.

Primary inflammatory demyelinating lesions of the central nervous system (CNS) are a group of diseases with demyelination of nerve fibers and infiltration of inflammatory cells around small blood vessels as the main pathological manifestations. For CNS inflammatory demyelinating diseases with mass-like features, the early literature lacked uniform nomenclature, and it was mostly defined as multiple sclerosis (MS) demyelinating plaques with a diameter of ≥2 cm. The disease was first reported by Van Dor Velden in 1979. In recent years, such lesions have been referred to as tumor demyelinating lesions (TDLs) or tumor-like inflammatory demyelinating lesions, demyelinating pseudotumors, pseudotumor-like demyelinating lesions. It is rarely reported and is easily misdiagnosed as brain tumor in clinic. Therefore, we review the research progress of this disease in order to improve clinicians' understanding of this disease.

Inflammatory demyelinating disease of the central nervous system (CNS) refers to a type of disease in which the axon is relatively preserved due to demyelination based on immune-mediated inflammation^[1]。 Inflammatory demyelinating diseases are divided into primary and secondary diseases according to the myelin itself or other etiologies involving myelin diseases. Primary CNS inflammatory demyelinating diseases include multiple sclerosis (MS) and its acute variant – Marburg MS, neuromyelitis optica (NMO), Balo's concentric circle sclerosis, acute disseminated encephalomyelitis (ADEM) and the hyperacute variant of ADEM – acute hemorrhagic leukoencephalitis (AHL). Common secondary CNS inflammatory demyelinating diseases include carbon monoxide poisoning, vitamin B₁₂Deficiency, mercury poisoning (Minamata's disease), alcoholism, hypoxia, radiation damage, etc. Different inflammatory demyelinating diseases have different clinical symptoms, laboratory tests and imaging, which is related to the differences in their pathological changes. Therefore, we summarized the pathological characteristics of different common primary CNS inflammatory demyelinating diseases in combination with clinical work and domestic and international progress.

Sleep-related rhythmic movement disorder (SRMD) is a sleep-related syndrome manifested by repeated rhythmic stereotypical actions in multiple parts of the body. This phenomenon was first described by Wepfer in 1727, and further elaborated by Zappert and Cruchet in 1905^[1]。 In the latest International Classification of Sleep Disorders (ICSD-3) released in 2014, SRMD is classified as sleep dyskinesia^[2]。 SRMD has the characteristics of episodic, repetitive, stereotypical, and inability to recall the episodic process after the attack. It is often misdiagnosed as other episodic diseases such as epilepsy. In fact, SRMD is not uncommon, but due to the lack of relevant knowledge and the rarity of domestic research and reports, its clinical recognition rate is low, and it is easy to be misdiagnosed and missed. Clinical studies on SRMD are reviewed below.

medication overuse headache (MOH) is a form of chronic headache. A large number of studies have shown that the incidence of MOH is 0.9% ~1.8% worldwide^[1]。 In headache clinics or tertiary headache centers, MOH patients have become the largest headache patient group along with migraine and tension headache patients. Although MOH is not a life-threatening disease, it can become a chronic disabling disease that has a much greater impact on the daily function of patients than migraine^[2]。 At present, the pathogenesis of MOH has not been elucidated. In recent years, there have been many research reports on MOH abroad. We summarize the research progress of MOH pathogenesis as follows.

The World Health Organization (WHO) defines quality of life as the experience of individuals in different cultures and value systems with regard to their goals, expectations, standards, concerns, and survival situations. Among them, health related quality of life (HRQoL) is an important index to evaluate disease outcomes^[1]The clinical application is increasingly widespread. Parkinson's disease is a neurodegenerative disease with the death of dopamine neurons in the substantia nigra as the main pathological change. For this chronic disease, which is difficult to cure, the aim of treatment is mainly to improve the quality of life of patients^[2], rather than just improving the patient's motor symptoms. Parkinson's disease HRQoL scale can be used to evaluate the quality of life and disease severity of patients with Parkinson's disease^[3,4,5]We will review the HRQoL rating scales for various Parkinson's diseases.

Parkinson's disease is a common neurodegenerative disease, with an incidence of about 300/100 000 in the general population, mostly after the age of 50, and the incidence increases gradually with age^[1]。 The etiology of Parkinson's disease is still unknown. Studies have shown that its main pathological features are the progressive loss of dopaminergic neurons in the substantia nigra and the formation of Lewy bodies in residual neurons. At present, it is believed that aging, environmental toxins, genetic factors and endogenous factors are the main mechanisms leading to Parkinson's disease. However, it is still unclear how environmental toxins and genetic factors lead to the damage of dopaminergic neurons in substantia nigra. In recent years, in the study of the pathogenesis of Parkinson's disease, it has been found that the selective deletion of dopaminergic neurons is closely related to the action of dopamine endogenous toxin in neurons in substantia nigra. Among them, vesicular monoamine transporter-2 (VMAT-2), as a transport carrier of dopamine, is responsible for transporting dopamine into vesicles for storage and avoiding the damage of dopamine to cytoplasmic organelles. It is particularly important in the study of the pathogenesis of Parkinson's disease.

neurodegeneration with brain iron accumulation (NBIA) mainly refers to a group of hereditary diseases of the nervous system caused by specific gene mutations. The clinical manifestations are mainly dyskinesia, abnormal gait, spasm, cognitive impairment, etc. Its common imaging feature is abnormal iron ion deposition signals in specific parts of the brain. At present, 9 types have been found (Table 1)^[1]。 NBIA is an imaging classification. There are several types of NBIA whose clinical manifestations belong to the category of hereditary Parkinson's disease (FPD). Therefore, its clinical classification overlaps with FPD. For example, ATP13A2 gene mutation and PLA2G6 gene mutation can lead to FPD. Therefore, ATP13A2 is also called PARK9 and PLA2G6 is also called PARK14. Among the genes that cause NBIA, only ceruloplasmin gene and ferritin L chain gene can directly cause iron ion metabolism disorder, and the rest genes are mainly related to fat metabolism and lysosomal activity disorder. We mainly summarized the clinical manifestations and imaging features of each type of NBIA for clinical reference.

The 18th International Conference on Parkinson's Disease and Movement Disorders was held in Stockholm, Sweden from June 8 to 12, 2014. More than 2,000 experts, scholars, physicians and researchers from all over the world attended the conference. Nearly 30 neurologists, surgeons and researchers in China attended the conference. The participants of this conference exchanged and introduced the progress made in this field in the past year. Now a brief introduction.

The F-wave is the late incubation period potential generated by reverse activation of the return discharge of anterior horn cells. This delayed response can be used not only to detect the full length of motor nerve including the proximal end, but also to assess the excitability of spinal motor neurons, and it is also a complement to conventional nerve conduction detection methods. The incubation period of F wave is closely related to the nerve length detected, and it cannot be generalized in clinical application. The purpose of our study is to establish the normal reference value of F-wave incubation period and height in Chinese people, so as to provide an accurate judgment of F-wave incubation period in clinical practice.