2015年 · 第48卷第07期

autophagy is one of the most popular research fields in life sciences after apoptosis. In recent years, the research results of autophagy have emerged one after another, which has become the focus of many scientists. At present, a large number of studies have shown that autophagy is closely related to the occurrence of neurodegenerative diseases, ischemic brain injury, epilepsy, central nervous system infection and immune abnormalities^[1]。 For example, recent studies have shown that autophagy is very critical for clearing abnormally accumulated long-lived proteins in cells. Functional abnormalities and defects of autophagy are likely to be one of the pathological mechanisms of neurodegenerative diseases. Therefore, autophagy modulators are expected to become novel drugs for the treatment of neurodegenerative diseases^[2]。 In addition, when cerebral ischemia and hypoxia occur, the mammalian rapamycin target protein (mTOR) pathway is activated, which in turn inhibits autophagy, thus increasing the neuronal sensitivity to ischemia and hypoxia^[3,4]。 Recent studies have also found that the DEPDC5 (DEPdomain containing 5) gene, which has the function of autophagy regulation, is closely related to the development of epilepsy after mutation^[5,6]。

In the 1950s, electroencephalography began to be used in neuro-intensive care units (NCUs). At the beginning of this century, NCUs in China have developed rapidly. According to incomplete statistics in 2010, 76 NCUs have reached the construction standard^[1]Therefore, it is imperative to establish EEG monitoring workstations and formulate relevant monitoring specifications. The Neurocritical Care Cooperation Group of Neurology Branch of Chinese Medical Association organized neurocritical care medicine experts and neuroelectrophysiology experts to jointly write the Recommended Opinions on EEG Monitoring Standards in Neurocritical Care Units based on evidence of evidence-based medicine and the current medical situation in China, hoping to provide help and reference for the medical practice of neurocritical care, critical care medicine and emergency medicine physicians. The writing steps of this article include: searching and reviewing relevant literature (from Medline database 1995-2013); Evidence level confirmation and recommendation confirmation were performed using the 2011 version of the Oxford Center for Evidence-Based Medicine Evidence Grading Criteria^[2]； Gradually expand the scope for full discussion, and raise the recommendation level (Level A recommendation) for opinions that are not sufficient for the time being, but have reached a high degree of consensus in expert discussion.

multiple system atrophy (MSA) is a progressive neurodegenerative disease of adult onset, which is characterized by autonomic failure, Parkinson's disease, cerebellar ataxia and other functional disorders of varying degrees. At present, there is a lack of effective treatment. MSA may have rare vocal cord dysfunction, which is characterized by slurred speech, tone change, sleep wheezing, respiratory failure and other abnormalities. Only one case has been reported in China^[1]。 Because vocal cord dysfunction can cause fatal inspiratory dyspnea, sleep apnea and sudden death, it is easy to be misdiagnosed as a respiratory disease, resulting in missed diagnosis and mistreatment, so it needs more attention. Four cases of MSA combined with vocal cord dysfunction diagnosed in our hospital from 2008 to 2013 were summarized, and their clinical manifestations, imaging changes, laryngoscopy, diagnosis and treatment process and prognosis were analyzed. The data of 4 patients are summarized inTable 1。

Behcet's disease is a systemic immune system disease with recurrent episodes^[1]When the nervous system is involved, it is called neuro-Behcet's disease (NBD), accounting for 5.3% to 14.3% of Behcet's disease patients^[2]。 Neurological lesions may be the first manifestation of Behcet's disease^[3], easily misdiagnosed. A patient with NBD who was repeatedly misdiagnosed as encephalitis is reported.

paroxysmal kinesigenic dyskinesia (PKD) is a group of involuntary dyskinesia diseases induced by sudden movement. Both genetic and sporadic cases have been reported^[1]。 At present, most studies believe that primary familial PKD is mostly genetically caused and related to PRRT2 gene mutation^[2,3,4]。 In rare cases, PKD may be secondary to other factors or coexists with other diseases, such as multiple sclerosis, head trauma, congenital myotonia, or hyperthyroidism^[5,6,7]。 Hyperthyroidism can be secondary to dyskinesia and neuromuscular disorders such as choreathetosis, tremor, hypokalemic periodic paralysis, and cases of secondary PKD are very rare^[8]。 We report a case of sporadic PKD secondary to hyperthyroidism and perform a literature review on its related clinical features.

carotid artery stenting (CAS) has become an effective method to treat carotid artery stenosis and prevent ischemic cerebrovascular disease, but plaque shedding is still one of the main complications of CAS. Plaque shedding in CAS generally occurs in the cerebral hemisphere region, but sometimes it can also cause embolism of the central retinal artery or branch retinal artery. Plaque detachment at the carotid bifurcation is the most common cause of embolism in the central or branch retinal arteries. We present a case of central retinal artery embolism caused by plaque shedding in CAS, analyze its pathogenesis and therapeutic measures.

Interventional embolization of intracranial dural arteriovenous fistula (DAVFs) has become a mature technique. Clinical treatment needs to select surgical approach according to the characteristics of its blood supply artery and drainage vein, so as to cure arteriovenous fistula. DAVFs generally have multiple supply arteries, and arteriovenous fistulas with single supply arteries are relatively rare. Where the existing catheter technique fails to completely embolize all branch supply arteries, the remaining supply arteries, despite their small size, are often a factor in the recurrence of arteriovenous fistula^[1]。 Although some arteriovenous fistulas have multiple supply arteries, their drainage veins merge into one anterior sinus vein, so embolization of drainage veins through venous approach becomes a good way to cure this kind of arteriovenous fistula. We retrospectively analyzed a patient with intracranial arteriovenous fistula with interventional embolization through intravenous approach. After six months follow-up, there was no recurrence.

paroxysmal sympathetic hyperactivity syndrome (PSH) is a paroxysmal transient sympathetic excitation and motor symptom after severe brain injury^[1]。 The causes were brain trauma, cerebral hypoxia, stroke and others. One case misdiagnosed as epilepsy that we treated is summarized as follows.

Parkinson's disease is a common neurodegenerative disease with an incidence of approximately 1.7% among people over 65 years old in China^[1]。 Parkinson's disease is characterized by progressive and extensive loss of dopaminergic neurons in the dense part of the substantia nigra of the midbrain and the appearance of Lewy bodies containing α-synuclein inclusion bodies in the cells. The pathogenesis of Parkinson's disease is the result of genetic, environmental, oxidative stress, abnormal protein expression, ubiquitin-proteasome system (UPS) and autophagy. Among them, protein overexpression and abnormal aggregation are considered to be one of the important pathogenesis of Parkinson's disease.

SWEDDs is an abbreviation for Scans Without Evidence of Dopaminergic Deficits. SWEDDs patients refer to those patients who have been diagnosed with Parkinson's disease based on clinical features and whose dopamine transporter (DAT) scan results are normal, that is, early, uncertain Parkinson's-like patients. More often than not, they are not really Parkinson's patients^[1]。 Therefore, it is of great significance to improve the recognition rate of SWEDDs and diagnose it as soon as possible.

Alzheimer's disease (AD) is the most common neurodegenerative dementia at present, and is clinically characterized by impairment of multiple cognitive domains such as episodic memory, language, visual space and executive function. Although the diagnostic criteria and pathological characteristics of AD have long reached the consensus of experts, its clinical symptoms may be heterogeneous. More than 15% of patients do not present with memory impairment early^[1]While onset with atypical clinical features is called atypical AD or variant AD syndrome^[2]These atypical clinical manifestations pose challenges to the diagnosis and condition monitoring of diseases. At present, atypical AD syndrome mainly includes four categories: posterior cortical atrophy (PCA), logopenic variant primary progressive aphasia (lvPPA), frontal variant AD (FV-AD) and Down syndrome, among which the first three categories are the main^[2]。 With the gradual understanding of typical AD by neurologists and the continuous revision of diagnostic criteria, the most common diagnostic criteria for atypical AD are promoted. Of course, the specificity and sensitivity of these diagnostic criteria need to be further verified by clinical practice. The author will make a detailed description and comparative analysis of the clinical features and diagnostic criteria of the main atypical AD syndrome.

juvenile myoclonic epilepsy (JME) is a common idiopathic generalized epilepsy (IGE) that occurs in people aged 12 to 18 years old. The incidence rate is 5% to 11% in epileptic people. The typical EEG manifestations during the seizure are bilateral symmetrical irregular 4-6 Hz diffuse multi-spinous slow wave or spike slow-wave discharges (SWDs)^[1,2]。 JME clinical episodes are characterized by bilateral, single or repeated multiple, irhythmic myoclonic tics, evident in the upper limbs, and usually combined with tonic-clonic and atypical absence episodes in addition to myoclonic episodes^[3]。 Among them, 90% of JME patients are effective with antiepileptic drugs, but they usually relapse after stopping the drug, and most patients need lifelong treatment^[4]。