2018年 · 第51卷第02期

In the past 10 years or so, aquaporin 4 (AQP4)^[1]and N-methyl-D-aspartate receptor (NMDAR)^[2]The discovery of isochannel or amino acid receptor autoantibodies has greatly enriched the connotation of autoimmune diseases of the nervous system. It not only makes AQP4-IgG an important marker to differentiate multiple sclerosis from neuromyelitis optica, but also further extends the clinical phenotype of neuromyelitis optica, forming a new disease spectrum of neuromyelitis optica spectrum disorders (NMOSD)^[3]。 neuronal surface-mediated antibody (NSAb)^[4]The concept of autoimmune encephalitis (AE) has become familiar to people, and it has become encephalitis and clinical new epilepsy^[5]Even rapidly progressing dementia^[6]An important differential diagnosis of.

The incidence of cardiac arrest is about (36~128) /100,000 per year, of which 86% of patients receive cardiopulmonary resuscitation, 17% ~49% of patients recover spontaneous circulation, but 80% of patients are still in coma^[1]The main cause was acute ischemic-hypoxic brain injury. A large prospective study from Europe and the United States showed that the first day survival rate of cardiopulmonary resuscitation after cardiac arrest was only 50%, and the survival rate after discharge was as low as 25%^[2]。 Severe brain injury can lead to severe neurological deficits, persistent hyporesponsiveness or vegetative states, and even death (including brain death), causing tremendous strain on society, families, and medical resources^[3]。 It is of great significance how to realize simple, quick and accurate clinical evaluation of brain function prognosis after cardiopulmonary resuscitation through technological innovation. We measured serum neuron-specific enolase (NSE) and Acute Physiology and Chronic Health Evaluation II (APACHE II) scores to provide a reference for clinical evaluation of brain function after cardiopulmonary resuscitation.

stiff person syndrome (Stiff-person syndrome, SPS)^[1,2]Is a rare disease, which was developed by Moersch and Woltman^[3]First reported in 1956, it is characterized by continuous muscle activity resulting in severe stiffness and episodic spasms of the trunk and limb muscles. One patient with SPS accompanied by ultrasound changes of substantia nigra admitted to us is reported below.

glomus jugulare tumor (GJT) is clinically rare, with an annual incidence of about 1/1.3 million, accounting for 0.60% of head and neck tumors^[1]。 The onset age of GJT is mostly 40-70 years old, and the male-female ratio is 1:6-1:3. The sporadic disease is more common, and a few are familial. The latter is related to succinate dehydrogenase (SDH) gene mutation^[2]。 GJT is mostly benign,<Multiple paragangliomas were seen in 5% of patients, with few distant metastases^[3]。 We report a patient with acute spinal cord compression due to multiple metastases of malignant GJT confirmed by pathological examination, summarized below.

Brucellosis is a zoonotic infectious disease caused by Brucella. The bacteria can invade the human body through damaged skin and mucous membranes and spread. The disease range is wide, the clinical manifestation is complicated, and it is easy to be misdiagnosed/missed. A rare case of neurobrucellosis (NB) with clinical manifestations similar to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is reported as follows.

subclavian steal syndrome (SSS) refers to a series of syndromes in which the proximal end of the subclavian artery or innominate artery is severely narrowed or completely occluded, resulting in a decrease in blood flow and pressure in the lumen of the subclavian artery, and the blood flow from the ipsilateral vertebral artery reversely flows into the subclavian artery and supplies the affected upper limb, thus inducing vertebrobasilar artery insufficiency^[1]。 SSS is an important cause of vertigo symptoms in clinic. Intermittent or persistent vertebral reverse blood flow has been reported in more than 90% of patients when the degree of stenosis of the proximal subclavian artery is>50%^[2]； Ultrasound Doppler is a simple and effective method to find reverse blood flow in vertebral arteries^[3]。 We recently treated a patient with vertigo whose ultrasound showed reverse blood flow in the left vertebral artery. He was admitted and diagnosed with SSS, but in the follow-up cerebral angiography, it was found that it was a rare proatlantal intersegmental artery (PIA), which is reported below for the reference of clinicians.

myofibrillar myopathies (MFMs) are a group of inherited muscle diseases characterized by abnormal deposition of myofibrillar-associated proteins, rare progressive exacerbation, with high clinical and genetic heterogeneity. Early histopathological findings suggest the presence of desmin deposition in the muscle fibers of such patients, which is referred to as "desminopathy"^[1,2]。 Through further research, it was found that besides desmin, other myofibrillar-related proteins were also abnormally deposited. Therefore, myopathies resulting from abnormal deposition of all myofibrillar-associated proteins are defined as "MFMs". The clinical manifestations of MFMs are diverse and can occur at all ages. It is manifested as slowly progressing distal or proximal muscle weakness, which can be accompanied by multiple organ involvement such as myocardium, respiratory muscle, peripheral nerves, bones and joints, and gastrointestinal tract. At present, eight classical MFMs pathogenic genes have been found: DES, CRYAB, MYOT, LDB3, FLNC, BAG3, FHL1, TTN^[3]。 In recent years, DNAJB6, PLEC, HSPB8A and ACTA1 have also been listed as its pathogenic genes^[4]。 However, there are still some patients whose clinical and muscle pathology are consistent with the diagnosis of MFMs, and their causative genes are not found. Therefore, patients with negative genetic tests above do not rule out the diagnosis of MFMs. We mainly summarize the pathological characteristics of MFMs, the pathogenesis, clinical characteristics and auxiliary examinations (including myocardial enzymes, electromyography, electrocardiogram, cardiac color ultrasound and muscle MRI) of each subtype (Table 1^[3]), helping clinicians raise awareness of the disease.

photosensitive epilepsy (PSE), the most common type of reflex epilepsy, was first reported by Gower in 1885. PSE is a group of disorders including PSE alone and epilepsy with photosensitivity. PSE alone refers to the appearance of seizures only upon visual stimulation, whereas epilepsy with photosensitivity refers to both spontaneous seizures and photosensitivity^[1]。 photosensitivity, also known as photoparoxysmal response (PPR), refers to the abnormal EEG response to intermittent flash stimulation. The electroencephalogram of PPR showed a spinous-slow complex of 2-5 Hz, which was significant in the frontal and central regions. PSE can be induced by certain types of visual stimuli, which can occur in situations such as watching television, playing video games, or attending a dance party. In view of the absolute advantage of vision in acquiring daily information, PSE is still an important direction of medical research with the development of optical technology, video game technology and television image technology. In recent 10 years, with the development of neuroimaging and neuroelectrophysiological technology, new discoveries have been made on the etiology, pathophysiological mechanism, diagnosis and treatment of PSE. We now summarize the latest progress of PSE as follows, so as to improve clinicians' understanding of PSE.

Migraine is a common neurological condition. The results of the 2013 Global Burden of Disease Survey indicate that migraine is the third most common disease and the sixth most disabling disease in humans^[1]。 In addition to moderate to severe unilateral or bilateral recurrent pulsatile headache symptoms, patients cannot return to normal for several hours or even days before the onset of the headache, and for a long time after the headache symptoms are terminated, these symptoms are collectively referred to as non-headache symptoms of migraine. The impact of non-headache symptoms on patients' body and mind cannot be ignored, and it is paid more and more attention by clinicians and scientific researchers. We reviewed the epidemiology of migraine non-headache symptoms, the progress of research on mechanisms, the impact on headache and the treatment.

The incidence, disability and mortality of acute ischemic stroke remain high. At present, the effective treatment methods are ultra-early intravenous thrombolysis and arterial thrombectomy to achieve vascular recanalization and tissue perfusion recovery. How to screen suitable patients to maximize the benefit of treatment and reduce the risk has become a research hotspot. Modern imaging techniques provide a lot of valuable information for vascular recanalization, including large artery stenosis, collateral circulation, core infarction, penumbra^[1]Wait. Recently, it has been found that the changes of cerebral venous return have a certain correlation with the prognosis of ischemic stroke, which may become a new imaging index to guide vascular recanalization therapy. We mainly reviewed the existing imaging evaluation methods of cerebral venous return and their related research progress in the prognosis of ischemic stroke, so as to lay a foundation for further exploring the application value of cerebral venous return evaluation in endovascular treatment and related mechanism research.