1 临床资料 患者男,36岁,体重100 kg,有高血压病史和家族史,平时收缩压最高达190 mmHg(1 mmHg=0.133 kPa),不规则服降压药.2014年8月25日突然起病,出现双手麻木,约1 min后跌倒在地,意识丧失,无肢体抽搐、口吐白沫等症状.外院头颅CT检查示桥脑出血,出血量约12 mL,破人第四脑室(图1).随后出现呼吸不稳,予经口腔气管插管.1周后病情逐渐加重,呼吸不规则,体温40℃,于出血后第8天转至上海长海医院.入院体格检查:体温40℃,脉搏120次/min,呼吸30次/min,血压144/90mmHg;重度昏迷,面色青紫,呼吸急促,脉搏细速;眼球有浮动,左侧瞳孔直径1.Smm,右侧瞳孔直径2.0mm,对光反射存在,双侧角膜反射消失;四肢软瘫,双侧病理征阴性;双肺听诊布满干、湿啰音.头颅CT检查示,桥脑出血,严重水肿,环池、第四脑室结构不清,水肿波及中脑,以左侧明显(图2).

>脑干听诱发电位(BAEP)由Jewett和Williston于1971年首先报道,并推测其可能来源于脑干的听觉通路。颅底骨折伴听力障碍是神经外科常见的病症,特别好发于合并脑干损伤时,BAEP可通过外周刺激记录脑皮质处的电位,反映传导通路及相应皮质部位的功能;临床上主要用于脑干听通路的评估及死亡判定。我们对颅底骨折伴听力障碍患者进行

PURPOSE:Charcot-Marie-Tooth disease (CMT) is the most common type of inherited peripheral neuropathy and has a high degree of genetic heterogeneity. CMT with concurrent diabetes mellitus (DM) is rare. The purpose of this study is to explore the genetic, clinical and pathological characteristics of the patients with CMT and concurrent DM.METHODS:We investigated gene mutations (the peripheral myelin protein 22 gene, myelin protein zero gene, lipopolysaccharide-induced tumor necrosis factor-α factor gene, early growth response gene and the neurofilament light chain gene loci) of a relatively large and typical Chinese family with CMT1 and concurrent DM2. From the literature, we also retrieved all reported families and single cases with CMT and concurrent DM. We comprehensively analyzed the characteristics of total 33 patients with CMT and concurrent DM, and further compared these characteristics with those of patients of diabetic peripheral neuropathy (DPN).RESULTS:Patients with CMT and concurrent DM had some relatively independent characteristics and pathogenic mechanisms. So we designated that kind of characteristic demyelinating CMT which accompanies DM as Yu-Xie syndrome (YXS), a new specific clinical subtype of CMT.CONCLUSION:CMT is an etiologic factor of DM, even though the intrinsic association between CMT and DM still remains further exploration.

<正>丙戊酸钠口服溶液(德巴金溶液)是目前临床上广泛用于小儿科的一线广谱抗癫痫药物,用于神经外科及神经内科并不常见。因昏迷鼻饲患者不能口服抗癫痫药,大部分癫痫发作时不易控制,使用德巴金针剂抗癫痫治疗,因长期应用费用昂贵,大部患者及家属经济上难以承受。基于上述原因,我们从2004年1月开始尝试使用德巴金溶液对昏迷癫痫患

目的 加强对Wallenberg综合征各种临床表现的识别,做到早期发现和正确治疗.方法 回顾近期2例病人的就诊经过,结合文献报道,对该综合征的各种非典型表现进行归纳总结.结果 本文报道的2例患者中,例1仅有三个主要体征,提示3个主要结构的损害,这3个结构均在延髓的背外侧靠小脑下脚处;其他结构,如疑核、脊髓丘脑束、网状结构等不受累及,这些表现是零碎的Wallenberg综合征.例2除了具有典型的6联征外,又多了个对侧面部的感觉障碍,这种表现属于扩大的Wallenberg综合征.结论 Wallenberg综合征临床表现复杂多变,了解局部的神经结构是早期识别的前提.

Paroxysmal dyskinesias are a rare heterogeneous group of conditions manifesting as abnormal involuntary movements that recur episodically and last only for a brief duration. The abnormal movements may be dystonic, choreic, ballistic, or a mixture of these [1,2]. Such conditions can be divided into three types: paroxysmal kinesigenic dyskinesia (PKD), paroxysmal nonkinesigenic dyskinesia, and paroxysmal exercise-induced dyskinesia [3]. Generally speaking, these dyskinesias are primary forms. They often occur in adolescence and respond well to antiepilepsy drugs such as phenitoin or carbamazepine. Some of these patients have a family history. Apart from these primary forms, there still are so-called secondary ("symptomatic") paroxysmal dyskinesias, which are often caused by environmental factors or other diseases [4]. Hashimoto encephalopathy (HE), presently known as Steroid Responsive Encephalopathy associated with Autoimmune Thyroiditis (SREAT), is a potentially fatal disease. Its manifestations are quite variable. Two distinct forms have been described: one was a vasculitic type characterized by multiple relapsing-remitting stroke-like episodes and mild cognitive impairment; the other was a diffuse progressive type characterized by dementia and psychiatric symptoms [5,6]. Antineuronal antibodies were thought to be associated with the pathogenesis of HE [7]. Recently, we encountered a patient whose symptoms began with PKD and finally culminated in global confusion and dementia. He was diagnosed with HE, and recovered completely after steroid treatment. A 75-year-old man was admitted to the Department of Neurology in October 2011. His wife and other family members reported that he began to develop involuntary movements on the left limbs 5 months ago. They noticed the symptoms for the first time while he was having lunch. When he wanted to take up the bowl on the table, he could not control his left arm and hand. The arm made a choreic and ballistic gesture, so he threw his bowl away. At first, this symptom occurred 2–3 times daily. Gradually, the frequency and amplitude increased day after day. They noticed that the attack was always induced by a sudden voluntary movement, for example getting up quickly to answer the telephone, or crossing the road while the traffic lights became green, but it never occurred during sleep. This condition was limited on the left side for nearly three months. He had seen doctors for several times and was prescribed carbamazepine for several days; however, this therapy could not stop the attacks. The frequency even increased to 120–150 times daily before admission. In the mean time, his family members noticed he became "stupid" and silent. He could not answer simple questions and could not even recall familiar people and places. On admission, physical examinations showed normal body temperature, breath rate, heart rate, and blood pressure. Neurological examination was remarkable for mild lethargy and unclear speech. He was unable to cooperate when tested for comprehension, orientation, memory, and calculation. Cranial nerve functions were intact. Muscle strength was normal and Babinski sign negative. There were no signs of meningeal irritation. Routine haematological results, including cancer markers, peripheral antineutrophil cytoplasmic antibodies, cytoplasmic antineutrophil cytoplasmic antibodies, erythrocyte sedimentation rate, antiphosphalipid antibody, antinuclear antibody, rheumatoid factor, systemic lupus erythematosus, serum Vitamin B12, and folic acid were normal or negative. His liver and kidney functions were unremarkable. Blood work for infectious diseases (Epstein–Barr virus, cytomegalovirus, simplex virus, Herpes zoster virus) was negative. The free thyroxine T3, T4, and TSH in the blood were normal. B-type ultrasound examination showed normal gland without nodes or calcification. His dynamic EEG for 24 h showed generalized slow waves (Figure 1), there was no focal or lateralized spike or sharp-slow waves throughout the whole recording period. The brain MR imaging, including T1, T2, and DW were all normal (not shown). Before treatment, dynamic EEG demonstrated diffuse low and medium-amplitude voltage, with the background of theta-activities. There was no sharp or spike-slow waves throughout the recording period. A lumbar puncture showed mild increase in protein 900 mg/L (normally, 150–450 mg/L), with normal cell count and glucose. The IgG, IgA, and IgM in the cerebrospinal fluid was 116 mg/L (normally, 10–50 mg/L), 18.9 mg/L (normally, 0–11.1 mg/L), and 1.4 mg/L (normally, 0–11.1 mg/L), respectively. The antithyroidglobulin antibody and the anti-TSH antibody were normal in the blood; however, the antithyroid peroxidase antibody was significantly elevated (more than 340 IU/mL), which decreased to 179.82 IU/mL after 1-month treatment. After excluding infectious diseases and Creutzfeldt–Jacob disease, HE was considered. He was administered methyprednisolone 500 mg, IV, once a day, for 15 days, 250 mg for another fortnight, and then tapered gradually till 20 mg prednisone, thrice a day. Ten days after steroid treatment, his consciousness began to improve, and the frequency of dyskinesia began to decrease; 20 days after treatment, the PKD symptom completely disappeared. Thereafter, progressive clinical improvement was observed. On the previous day of his discharge, the EEG showed normal (Figure 2). After treatment for 1 month, routine EEG demonstrated medium-amplitude voltage, with the background of alpha-activities. This is the first report of HE, which begins with PKD to our knowledge. PKD is often primary (genetic or idiopathic), which occurs at the age between 5 and 20 years and responds well to antiepilepsy drugs. However, Blakeley et al. found that a specific cause had been identified in some cases. These causes include peripheral trauma, central trauma, vascular lesions, multiple sclerosis, menigovascular syphilis, and kernicterus [7]. Such secondary dyskinesias were easily controlled with anticonvulsant therapy, requiring only one agent. What is different in our patient is that his PKD could not be stopped, because it is unresponsive to carbamazepine or other antiepilepsy drugs, such as valproate, or clonazepam. Further, it is just the initial symptom of the whole course. As the disease progressed, other mental disorders such as global confusion and lethargy predominated over the clinical aspects. The clinical features, laboratory findings, and his responsiveness to steroid treatment are in complete accordance with the revised criteria for SREAT made by Castillo et al. [8]. The pathogenesis and etiology of HE has not yet been identified. Currently, the most broadly accepted hypothesis is that an autoimmune etiology results in either cerebral vasculitis or direct injury from autoantibodies [8]. Although we could not see any abnormalities in the MR imaging, there must be some subcellular changes in the brain, at least some functional disturbance, as seen in the EEG before treatment. Zhao et al. performed brain biopsy in a patient and found reduced density of cortical tissue with neuron loss, swelling of surviving neurons, reactive gliosis, and angiogenesis. Transmission electron microscopy showed vacuolar degeneration of some neurons with swollen mitochondria and microglia activation. Formation of small cysts, demyelination, and sponge-like changes within white matter were also found [9]. However, Castillo et al. performed brain biopsy in two patients, and found the histological findings were entirely normal in one patient, with only meningeal thicking and dural enhancement [8]. In conclusion, this report is interesting and has important clinical significance. Facing a patient with PKD, which is not responsive to antiepilepsy drugs, and especially if the patient undergoes progressive global confusion, clinicians should consider the possibility of HE. The elevated antithyroid antibodies in the serum, especially anti-TPO antibody, are important markers in evaluating this condition. The authors have no conflict of interest.

Ocular lateropusion, referring to ocular motor bias toward (ipsipulsion) or away from (contrapulsion) the lesion side without limitation of eye movement, is uncommon [1]. Truncal lateropulsion, a compelling sensation of being pulled toward the side of lesion, is a well-known transient feature of dorsal lateral medullary infarction (LMI) [2]; and isolated truncal lateropulsion is attributed to lesions in the flocculonodular lobe [3], reticular formation [4], red nucleus of midbrain [5], cerebellar peduncles, or olivocerebellar fibers at the level of inferior cerebellar peduncle [6]. However, persistent and simultaneous ocular and truncal lateropulsion associated with caudal LMI has not been reported previously, to our knowledge. A 54-year-old man developed acute onset of dizziness while he was watching TV. He had no nausea, diplopia, tinnitus, and deafness. One hour later, he felt the dizziness becoming more severe and the left-side limbs and trunk becoming numb. Although feeling giddy, he was still able to walk by a cane. Three hours after the onset, he began vomiting. On the following day, he experienced mild dysphagia and severe hiccup. He had no hypertension, cardiac vascular disease, and diabetes mellitus previously. Neurological examination showed mild horizontal, sometimes rotatory, nystagmus. His eyes were gazing (biased) to the right at most times, but they were able to move to the left or right, up or down freely, with the target movement. Both of the ocular rimae were the same width. The pupils were equal in bright environment, but the left pupil was apparently larger than the right one in dark environment (Figure 1, top). Both pupils were sensitive to light. The right palate is mildly paraparetic and the right throat reflex is mildly decreased. The tongue moved well and there was no facial weakness. Pain and heat sensation was deceased drastically in the left trunk and limbs, but mildly in the right face. Bilateral corneal reflexes were almost equally sensitive. Bilateral vibration and joint position sensation was preserved. Finger–nose test and the heel–knee-shin test on both sides were surprisingly coordinated when examined on the bed. When standing uprightly without closing the eyes, he suffered severe lateropulsion to the right. He would certainly fall down if not supported by another person (Figure 1, left). There was no weakness of the limbs and Babinski sign on both sides was negative. Right pupil is smaller than the left one in dark environment, but both ocular rimae are equal (upper). Severe right lateropulsion on admission (lower left); By slightly pulling his garment, one could support the patient to walk almost normally after one-month treatment (lower middle); MRA shows right vertebral dysplasia and stenosis (lower right). Cerebral MRI detected a lacunar infarction in the right midlateral periphery of the caudal medulla, with preservation of the upper medulla, inferior cerebellar peduncle and dorsal funiculus (Figure 2). Angiographic sequences disclosed whole length dysplasia and stenosis of the right vertebral artery. (Figure 1, right). MR T2W Images of sequential medullary sections showing normal signals at the upper medulla (top A and B) and hypersignals (arrows) in the periphery of caudal lateral funiculus of medulla (top C and D). MR DW Images of sequential medullary sections showing normal signals at the upper medulla (bottom A and B) and hypersignals (arrows) in the periphery of caudal lateral funiculus of medulla (bottom C and D). One week after treatment, the symptoms of dysphagia, hiccup, and hypesthesia in the left limbs improved quickly, but the nystagmus worsened and oscilopsia appeared. The latter symptoms lasted for one week. The ocular and truncal lateropulsion still remained. After one-month treatment, the patient could walk almost normally, if his garment was slightly pulled from the left side by another person (Figure 1, middle). Caudal LMI is less common than rostral LMI. The symptoms of upper LMI are rather different from those of lower LMI. For upper LMI, patients often manifest severe vertigo, nystagmus, limb ataxia, crossed superficial sensory defect accompanied by decreased corneal reflex, Horner sign, even dysphagia and/or dysarthria. However, at the level of lower medulla, the inferior cerebellar peduncle has not formed and the vestibular nucleus has not appeared. The most prominent feature in the present patient is the long-lasting ocular and truncal lateropulsion. Ocular ipsipulsion has been reported in Wallenberg syndrome by Kim et al. [1], and is ascribed to damage to fibers that project from the contralesional inferior olivary nucleus via the inferior cerebellar peduncle to the Purkinje cells in the cerebellar cortex. Disinhibition of the Purkinje cells leads to decreased activity of the ipsilateral fastigial nucleus in the vermis. The fastigial nucleus projects, via the uncinate fasciculus in the superior cerebellar peduncle, upon the medial and superior vestibular nuclei and the paramedian pontine reticular formation of the contralateral side and accelerates contralateral saccades. Its inhibition results in a motor bias of horizontal eye movements toward the side of the lesion [7, 8]. In our patient, the ocular ipsilateral pulsion did occur, and we thought it was gazing palsy at first; however, the eyes could move freely in all directions. The upper medulla, including inferior olivary nucleus and inferior cerebellar peduncle, was unaffected. Therefore, there must be another mechanism lying behind this phenomenon, which has not been elucidated as yet. As for the long lasting lateropulsion, we speculate it was caused by the lesion of the right spinocerebellar tracts. Using three-dimensional brainstem mapping, Thomke et al. recently found that involvement of the ascending posterior spinocerebellar tract was associated with lateropulsion with limb ataxia; but the involvement of the descending lateral vestibulospinal tract was associated with lateropulsion without limb ataxia [9]. We cannot analogize that the lateropulsion in our patient is associated with lateral vestibulospinal tract involvement, although the patient manifested lateropulsion without limb ataxia. Because the lesion in the present patient was situated in the periphery of the midlateral medulla, it may damage the spinothalamic tract (the reason for the patient's transient sensory defect on the opposite limbs), rather than the lateral vestibulospinal tract, which descends more ventromedially. There may be another mechanism concerning the lateropulsion in our case. The fact, that the patient's left pupil was smaller than the right one in dark environment, actually indicated that the right medullary reticular formation had been involved, at least in part. Therefore, the medullary (lateral) reticulospinal tract, which derives from the medial two-third of the reticular formation and descends in the anterior part of the lateral funiculus in lower medulla, may be damaged. The tract can influence muscle tone by acting upon γ motor neurons. The extensor muscle tone will be inhibited if this tract is damaged [10]. This mechanism may, in part, explain the lateropulsion in our case. In conclusion, persistent ocular and truncal lateropulsion may be associated with the caudal LMI. The authors declare no conflict of interest.

Headache is the most frequent (80–90%) and often the presenting symptom of cerebral venous sinus thrombosis (CVST) [1]. It has no specific features in the mode of onset, quality of pain, extent and duration, and can be the sole clinical manifestation at the onset and during the course [2]. Headache attributed to CVST is sometimes misleading, mimicking migraine, cluster-like headache, even thunderclap headache [3–5]. Here, we report two patients with chronic, recurrent, atypical headache, who were finally diagnosed as CVST and underwent a benign course after anticoagulation treatment. Case 1. A 48-year-old Chinese woman complained of headache in the last 2 years before admission. The headache was diffuse and dull, lasting several days to a week, not accompanied by fever, nausea, and vomiting, then it could resolve spontaneously or after taking analgesic drug. However, the headache had never been really cured. After a few days of headache free, it may recur. And with the time passing by, the headache became increasingly severe and longer in duration. CT scan was performed for several times, but no obvious abnormality was found. During the past 6 months, she even experienced several attacks of thunderclap headache with nausea and vomiting, which was aggravated by standing upright. This drove her to our emergency department. She did not experience visual problem during the course; and she did not have headache attack 2 years before, with no history of oral contraceptive pills. Neurological examination revealed no apparent abnormality except for the papilledema. Subsequent lumbar puncture showed opening pressure 30 cm, but the cerebral spinal fluid was normal in components. Routine haematological results, including cancer markers, PANCA, CANCA, ESR, antiphosphalipid antibody, were normal or negative. Her blood D-dimer was 0.71 μg/mL (normal range <0.5 μg/mL), and the blood homocysteine level was 85.10 nmol/mL (normal range 5–15 nmol/mL). MRI showed normal brain parenchyma, except for the delta sign in the superior sagittal sinus on T2 image, and MR angiography showed extensive CVST with filling defects in the superior sagittal sinus, with extensive cortical venous collateral formation (Figure 1). After administration of low molecular weight heparin (4000 u, hypodermical injection, bid) for 3 weeks, the headache dissipated gradually and did not recur in the next 6 months follow-up. MR angiography revealed (A) superior sagittal sinus thrombosis with cortical venous collateral formation (indicated by arrowhead) and (B) delta sign in T2 MR imaging (indicated by arrow). Case 2. This 78-year-old Chinese woman was admitted for 4 months of episodic headache. She had no prior history of hypertension. The headache was deep and localized around the left temporal bone, not accompanied by fever, nausea, and vomiting. It is episodic and pin-prick in quality at first, lasting from half an hour to 4 h each time. The symptom was mild initially, but aggravated occasionally. Two months later, the headache gradually became persistent, pulsatile, sometimes drilling, and severer at night. She even could not go into sleep in the nights before seeing the doctor. She did not experience visual problem during the course. Neurological examination was normal. Bilateral fundascopy did not reveal papilledema. A noncontrast brain CT showed no abnormal signs. Routine haematological results were normal or negative. Her blood D-dimer was 0.91 μg/mL (normal range <0.5 μg/mL), and blood fibrin degradation products (FDP) was 3.96 μg/mL (normal range <6 μg/mL). The patient refused lumbar puncture throughout the course. MRI and MR angiography showed CVST in the left transverse sinus with filling defects on GD-DTPA enhanced imaging (Figure 2). After administration of low molecular weight heparin (4000 u, hypodermical injection, bid) for 3 weeks, the headache gradually disappeared and did not recur in the next 6 months follow-up. MR angiography revealed (A) left lateral sinus thrombosis (indicated by arrow) and (B) filling defect in left lateral sinus and superior sagittal sinus on GDDTPA enhanced MR imaging (indicated by arrowhead). Headache occurs in about 80–90% of patients with CVST. Agostoni E. reviewed 49 patients and found that the onset was acute (<48 h) in 45% of cases, subacute (48 h–1 month) in 40% and chronic (>1 month) in 11%[1]. The International Headache Society (IHS) Classification defined criteria of the diagnosis of headache attributed to CVST: (A) Any new headache (with or without other neurological signs) fulfilling criteria C and D. (B) Neuroimaging evidence of CVST. (C) Headache and other neurological signs (if present) develop in close temporal relation to CVST. (D) Headache resolves within 1 month after appropriate treatment [6]. How to explain the “new onset” in our patients? The mechanism of headache in CVST is thought to be caused by local involvement of the pain-sensitive fibers of the dura mater by distension of the sinus wall, cerebral venous dilation or leakage of blood on the surface of the brain, as well as intracranial hypertension. The mechanism of this chronic, recurrent headache in our patients may reflect the course of thrombosis and spontaneous thrombolysis at the early stage of the disease. As this mild type undergoes a chronic course, there is enough time for the collateral veins in the brain to compensate. Thus, these patients only exhibit isolated intracranial hypertension, even isolated headache [2,7,8]. As the disease progresses, the compensatory mechanism becomes no longer sufficient, and the headache becomes increasingly frequent and severe. Are there any other causes that can explain this chronic, recurrent headache in our patients? This was impossible. In case 1, the headache is not in accordance with migraine and tension type headache, or with cluster headache. In case 2, the headache is initially episodic and pin-prick in nature and gradually became persistent, pulsatile, sometimes drilling, and severer at night. This profile in the older often raises the possibility of giant cell arteritis, but the patient had no temporal tenderness, and no elevated ESR. These features can exclude the diagnosis of giant cell arteritis. Moreover, the headache in our patients resolved in 1 month after appropriate treatment, and it did not recur ever since. Our observations have important practical consequences. CVST should be considered when chronic, recurrent headache becomes increasingly frequent and severe. As the typical signs of CVST (delta sign and cord sign) are seldom found in plain CT scan, the best investigation is MRI and MR angiography [9,10].

From July 2003 to July 2009, 15 cases of subdural hematoma with swirl signs were treated in our hospital and their clinical data were retrospectively analysed. The mortality was compared between these patients and those with typical acute subdural hematoma who were treated at the same time in our hospital. Among the 15 cases, full recovery was achieved in 4 cases, slight disability in 2, grave disability in 2 and death in 7 (46.7%). The mortality of these patients was conspicuously higher than that of typical subdural hematoma (14/83, 16.9%, P < 0.01). Subdural hematoma with swirl signs is often suggestive of hazardous pathogenetic condition and early diagnosis and prompt surgical intervention is essential to reduce mortality.

BACKGROUND:Spinocerebellar ataxia type 7 (SCA7) is known as an autosomal dominant cerebellar ataxia; patients with genetically confirmed diagnoses of SCA7 have increased rapidly in recent years. However, SCA7 is a rare subtype of SCA, and most data available about SCA7 are those of white people. The aim of the present study was to systematically review the prevalence and clinical and genetic aspects of SCA7 patients in East Asian population.METHODS:A search for publications on SCA7 was performed by using the "PubMed" database with the published language limited in English. Publications mainly focusing on the prevalence of SCA7 in patients with SCA and the clinical and genetic features of SCA7 patients were fully reviewed and analyzed.RESULTS:The prevalence of SCA7 in SCA patients ranged from 0 to 7.7%, which was similar to those reported previously. The clinical manifestations were typically present at the 30's of its victims (median, 29 years; interquartile range (IQR), 19.5-36.5 years), and the symptoms appeared 15 years ((15.17+/-4.22) years) earlier on average in the offspring than in the parents. Gait ataxia and visual impairment were both found in all patients of whom the clinical features were described. Mutant SCA7 alleles contained 40-100 CAG repeats, with a median of 47 repeats (IQR, 44.5-50.0); and the offspring had 13 more repeats on average compared with their parents (12.62+/-19.03). A strong negative correlation was found between CAG repeat size and the onset age of patients (r=-0.739, P=0.000). In addition, no significant difference was found in CAG repeat sizes between patients with visual impairment as the initial symptom and those with gait disturbance as their initial symptom (P=0.476).CONCLUSIONS:The prevalence of SCA7 in SCA patients, the age at onset and CAG repeats of SCA7 patients in East Asia are consistent with those of white people. However, larger population study is needed to assess the correlation between the CAG repeat size and initial symptoms of SCA7 patients in East Asia.

<正>脑干损伤是基层医院常见病的类型之一,其病死率高达44.4%～71.1%[1],重型脑干损伤的病死率更高。笔者探讨了双侧颞底去大骨瓣减压抢救重型脑干损

目的探讨颅内动脉瘤血管内栓塞治疗的效果,栓塞时机、技巧及并发症的防治。方法采用血管内栓塞治疗31例(32个颅内动脉瘤),其中电解可脱性弹簧圈栓塞颅内动脉瘤27例(28个),球囊辅助瘤颈重塑型技术和微导丝辅助瘤颈成形技术栓塞治疗2个宽颈动脉瘤,电解可脱性弹簧圈闭塞载瘤动脉1个,可脱性球囊闭塞载瘤动脉1个。结果致密栓塞29例,疏松栓塞2例;术中动脉瘤破裂出血1例;1例术后死亡。结论电解可脱性弹簧圈栓塞颅内动脉瘤具有微创、安全、效果可靠等优点;采用微导丝辅助瘤颈成形技术等方法,可以明显提高宽颈动脉瘤的致密栓塞率。

<正>黄韧带骨化症(ossified yellow ligament,OYL)是脊柱外科的常见病,但不少患者因肢体麻木、走路不稳等神经症状首先就诊于神经内科。一些神经内科医师由于专业的局限性,对此病缺乏应有的了解.导致误诊、误治。现报道我科最近诊断的2例OYL患者,结合国外相关文献,就本病的病因、临床表现、治疗和预后进行讨论。

目的探讨经蝶窦显微手术对垂体泌乳素腺瘤的治疗意义。方法对我院经蝶窦显微手术治疗垂体泌乳素腺瘤12例病人进行总结。结果肿瘤全切除11例,次全切1例。无死亡及病残发生,泌乳素全部恢复正常。结论对于垂体泌乳素腺瘤,由于神经外科显微技术的进步,经蝶垂体瘤手术目前成为神经外科疗效确切、安全的治疗方法之一。而微腺瘤,也采取积极的手术治疗,极大部分病人激素能恢复正常。

原发性颅内低压(Spontaneous intracranial hypotension,SIH)病因尚未查明,随近年来放射影像学检杏技术以及放射核素造影的应用,对SIH影像学的改变逐渐有所认识.

目的探讨血管内介入治疗颅内动脉瘤的疗效。方法回顾性分析采用血管内介入植入电解可脱弹簧圈术治疗的25例颅内动脉瘤患者的临床资料。结果25例患者瘤腔致密栓塞16例,栓塞90%以上7例,栓塞85%～90%2例。术中出血1例,术后半年内再次出血2例,在外院行GDC致密栓塞,留有严重的神经功能障碍;余患者无再出血事件发生。结论血管内介入治疗颅内动脉瘤具有微创、安全、近期疗效佳的优点,其中球囊辅助成形技术的应用,可有效提高宽颈动脉瘤的致密栓塞和减少并发症的发生。

A 52-year-old man was diagnosed with general paresis, whose HIV antibodies were negative. After initiation of treatment with penicillin on the first day, no obvious clinical Jarisch-Herxheimer reaction was found. However, 6 days after treatment, the patient was found more irritable and was unable to fall asleep at night. On the seventh day, worsened magnetic resonance imaging (MRI) abnormalities in the bilateral medial and anterior temporal lobes were unexpectedly discovered. These worsened MRI abnormalities improved quickly after the addition of dexamethasone treatment. We consider that these transient and slight mental symptoms may be associated with the transiently worsening phenomenon in cerebral MRI findings during the early period of treatment with penicillin. This indicates that some nonspecific inflammatory process has happened in the early stage of treatment, which necessitates the use of corticosteroids after the occurrence of systemic or mental symptoms.

1998～2005年,我们采用单孔钻颅双管引流的方法治疗老年慢性硬膜下血肿(CSDH),取得满意疗效.现报告如下.

<正>正常压力性脑积水(normal pressure hydrocephalus,NPH)是一种脑室虽扩大而脑脊液压力正常的交通性脑积水综合征[1]。目前本病常采用分流手术治疗,但手术效果受诸多因素影响,仍存在争议。我们对此类患者根据

On admission she was normotensive, and her general examination was normal. Neurological examination showed left Horner’s syndrome, with the left eye mildly ptotic ( fig. 1 a). There was no nystagmus in both eyes. The left pupil was about 3 mm and the right one 4 mm in diameter; both pupils were alert to light. The left palate was paraparetic, and the left throat reflex was decreased. The tongue moved well and there was no facial weakness. Sensation to pain and heat was decreased substantially in the right trunk and limbs, but decreased mildly in the right face, and normal in the left face and the left body. Bilateral vibratory and postural sensation was preserved. The left finger-nose test and the heel-knee-shin test were minimally incoordinated, compared with those of the right. When standing upright and closing the eyes, she felt mild lateropulsion to the left. There was no weakness of the limbs. Cerebral MRI detected a small bandlike ischemic lesion in the left retro-olivary midlateral tegmentum, with preservation of the far dorsal vestibular nucleus and inferior cerebellar peduncle ( fig. 1 c). Angiographic sequences disclosed congenital dysplasia of the left vertebral artery and segmental stenosis at the origin of the posterior inferior cerebellar artery ( fig. 1 b). Dear Sir, Lateral medullary infarction is one of the most frequently encountered cerebral infarctions involving the brainstem and often manifests as Wallenberg syndrome. Typically, it presents with vertigo, hoarseness and dysphagia, Horner’s syndrome and cerebellar ataxia on the same side of the lesion, and hypalgesia [1] , which usually involves the ipsilateral face and the opposite trunk and limbs (crossed sensory symptoms). Hypalgesia that involves the contralateral face, trunk, arm and leg without affecting the ipsilateral side of the face has seldom been reported [2] . Recently, we have encountered a female patient, who suddenly experienced hypesthesia in the entire contralateral body half with the ipsilateral side of the face unaffected.