encephalopathy

脑病
  • 文章类型: Journal Article
    背景:已知吸收不良综合征是减肥手术的慢性并发症。因此,建议口服补充多种维生素。Wernicke脑病代表与酒精中毒或严重营养不良相关的急性神经精神综合征;文献中描述了与减肥手术相关的这种潜在并发症的散发性病例。我们介绍了一例减肥手术后严重缺乏维生素B1引起的韦尼克脑病。
    方法:一名31岁女性,从3岁开始患有聋哑症,手术前3个月,用小型胃旁路术治疗严重肥胖,进入急诊室后被转移到我们单位.在近期的病史中,视力突然迅速下降,导致视力完全丧失,明显的虚弱,和四肢的感觉异常。考虑到以前的减肥手术,非酒精性Wernicke综合征的诊断被怀疑,其中维生素B1的IV治疗开始时剂量为5瓶200毫克在100毫升的盐水溶液中(在最初的72小时内每天三次,随后1次/天)。12小时后,视力有了改善,症状在48小时内完全缓解。1个月后,所有症状完全缓解,出院。
    结论:无意识错乱或脑病的初始视力丧失是Wernicke综合征的一种不典型表现。在酒精中毒或减肥后手术的情况下,临床怀疑必须很高。早期识别非典型症状,包括视力丧失,及时给予治疗可改善这种潜在可逆但时间依赖性神经系统急症的预后.
    BACKGROUND: Malabsorption syndromes are known chronic complications of bariatric surgery. Therefore, it is recommended to take oral supplementation with multivitamins. Wernicke\'s encephalopathy represents an acute neuropsychiatric syndrome associated with alcoholism or severe malnutrition; sporadic cases of this potential complication related to bariatric surgery are described in the literature. We present a case of Wernicke\'s encephalopathy due to severe vitamin B1 deficiency after bariatric surgery.
    METHODS: A 31-year-old woman with deaf-mutism from the age of 3 years old, operated 3 months before with a mini-gastric bypass for severe obesity, was transferred to our unit after accessing the emergency room. In the immediate medical history, there was the sudden and rapid decline in vision, leading to complete loss of vision, marked asthenia, and paresthesia in the four limbs. Considering the previous bariatric surgery, the diagnosis of non-alcoholic Wernicke\'s syndrome was suspected, for which IV therapy with Vitamin B1 was started at a dosage of 5 vials of 200 mg in 100 cc of saline solution (three times a day for the first 72 hours, subsequently 1 once/day). After 12 hours, there was an improvement in visual acuity, and the symptoms completely resolved within 48 hours. She was discharged with complete resolution of all symptoms after 1 month.
    CONCLUSIONS: Initial vision loss without confusion or encephalopathy is one atypical presentation of Wernicke syndrome. Clinical suspicion must be high in case of alcoholism or post-bariatric surgery. Early recognition of atypical symptoms, including vision loss, and timely administration of therapy improves the prognosis of this potentially reversible but time-dependent neurological emergency.
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  • 文章类型: Journal Article
    脑病是Susac综合征临床三联征的一部分,但是缺乏对这种情况的神经认知和神经精神病学特征的详细了解。现有文献表明,认知缺陷的严重程度从微妙到深刻。执行功能和短期召回经常受到影响。精神病表现可能不存在或可能包括焦虑,情绪障碍或精神病。如果精神病现象在疾病过程中发展,很难弄清楚症状是否与Susac综合征的病理直接相关,还是继发于治疗相关的副作用。在这篇文章中,我们回顾了有关Susac综合征的认知和精神病发病率的已知信息,并确定了知识不足的领域。重要的是,我们还为未来的研究提供了一个框架,认为更好的表型,对病理生理学的理解,对认知和精神病学结果的治疗评估,纵向数据采集对改善患者预后至关重要。
    Encephalopathy is part of the clinical triad of Susac syndrome, but a detailed understanding of the neurocognitive and neuropsychiatric profile of this condition is lacking. Existing literature indicates that cognitive deficits range in severity from subtle to profound. Executive function and short-term recall are affected frequently. Psychiatric manifestations may be absent or may include anxiety, mood disorders or psychosis. If psychiatric phenomena develop during the disease course, it can be hard to disentangle whether symptoms directly relate to the pathology of Susac syndrome or are secondary to treatment-related side effects. In this article, we review what is known about the cognitive and psychiatric morbidity of Susac syndrome and identify areas where knowledge is deficient. Importantly, we also provide a framework for future research, arguing that better phenotyping, understanding of pathophysiology, evaluation of treatments on cognitive and psychiatric outcomes, and longitudinal data capture are vital to improving patient outcomes.
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  • 文章类型: Journal Article
    背景:谵妄是医院老年人常见的并发症,康复和长期设施。
    目的:评估世界范围内已验证的谵妄评估工具的使用情况和谵妄管理方案的存在。
    方法:对世界谵妄意识日全球一日点患病率研究的二次分析,2023年3月15日。
    方法:包括医院在内的横断面在线调查,康复和长期设施。
    方法:参与的临床医生报告了谵妄的数据,协议的存在,谵妄评估,谵妄意识干预措施,非药物和药物干预措施,和病房/单位特定的障碍。
    结果:来自44个国家/地区的数据,分析了1664个病房/单位和36.048名患者。验证的谵妄评估用于66.7%(n=1110)的病房/单位,18.6%(n=310)使用个人判断或没有评估,10%(n=166)使用其他评估方法。66.8%(n=1094)的病房/单位报告了谵妄管理方案。各大洲的谵妄管理方案各不相同,从非洲的21.6%(21/97病房/单位)到澳大利亚的90.4%(235/260),与使用经过验证的谵妄评估相似,非洲为29.6%(29/98),北美为93.5%(116/124).有谵妄管理方案[n=1094/1664,66.8%]的病房/单位比没有方案的病房/单位更有可能使用经过验证的谵妄测试[比值比6.97(95%置信区间5.289-9.185)]。谵妄方案的存在增加了有效谵妄评估的机会,很可能,基于证据的干预措施。
    结论:报告存在谵妄管理方案的病房/单位使用经过验证的谵妄评估工具评估谵妄的可能性更高。
    BACKGROUND: Delirium is a common complication of older people in hospitals, rehabilitation and long-term facilities.
    OBJECTIVE: To assess the worldwide use of validated delirium assessment tools and the presence of delirium management protocols.
    METHODS: Secondary analysis of a worldwide one-day point prevalence study on World Delirium Awareness Day, 15 March 2023.
    METHODS: Cross-sectional online survey including hospitals, rehabilitation and long-term facilities.
    METHODS: Participating clinicians reported data on delirium, the presence of protocols, delirium assessments, delirium-awareness interventions, non-pharmacological and pharmacological interventions, and ward/unit-specific barriers.
    RESULTS: Data from 44 countries, 1664 wards/units and 36 048 patients were analysed. Validated delirium assessments were used in 66.7% (n = 1110) of wards/units, 18.6% (n = 310) used personal judgement or no assessment, and 10% (n = 166) used other assessment methods. A delirium management protocol was reported in 66.8% (n = 1094) of wards/units. The presence of protocols for delirium management varied across continents, ranging from 21.6% (on 21/97 wards/units) in Africa to 90.4% (235/260) in Australia, similar to the use of validated delirium assessments with 29.6% (29/98) in Africa to 93.5% (116/124) in North America. Wards/units with a delirium management protocol [n = 1094/1664, 66.8%] were more likely to use a validated delirium test than those without a protocol [odds ratio 6.97 (95% confidence interval 5.289-9.185)]. The presence of a delirium protocol increased the chances for valid delirium assessment and, likely, evidence-based interventions.
    CONCLUSIONS: Wards/units that reported the presence of delirium management protocols had a higher probability of using validated delirium assessments tools to assess for delirium.
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  • 文章类型: Journal Article
    亮氨酸氨酰基tRNA合成酶1(LARS1)缺乏症(婴儿肝功能衰竭综合征1型(ILFS1))具有多系统表型,包括发热相关的急性肝功能衰竭(ALF),慢性神经系统异常,和脑病发作。为了更好地表征脑病发作和MRI改变,系统评估了13例LARS1缺乏症患者的35例头颅MRI,并分析了神经系统表型。所有个体都有发育迟缓,10/13有癫痫发作。8/13的脑病发作通常与感染有关,出现癫痫发作和意识下降,主要伴有肝功能障碍,并在17/19集恢复。肝移植后,一名患者发生无肝功能障碍的脑病。核磁共振成像,在急性脑病期间进行MRI检查的5/7个体存在深部灰质和脑干改变。幕上皮质受累(6/13)和小脑分水岭损伤(4/13)发生癫痫和/或脑病。矢状图像上脑干轮廓异常(8/13),萎缩(8/13),髓鞘形成延迟(8/13)与脑病无明显相关性。深灰质和脑干改变的模式显然是LARS1缺乏症脑病的特征,与有机酸尿症和线粒体疾病中的肝性脑病或代谢性中风的模式不同。虽然病理机制尚不清楚,感染期间的发烧和能量不足可能是致病的;因此,建议摄入足够的葡萄糖和蛋白质以及积极的发烧管理。由于在流感感染期间观察到严重发作,我们强烈建议季节性接种疫苗。
    Leucine aminoacyl tRNA-synthetase 1 (LARS1)-deficiency (infantile liver failure syndrome type 1 (ILFS1)) has a multisystemic phenotype including fever-associated acute liver failure (ALF), chronic neurologic abnormalities, and encephalopathic episodes. In order to better characterize encephalopathic episodes and MRI changes, 35 cranial MRIs from 13 individuals with LARS1 deficiency were systematically assessed and neurological phenotype was analyzed. All individuals had developmental delay and 10/13 had seizures. Encephalopathic episodes in 8/13 were typically associated with infections, presented with seizures and reduced consciousness, mostly accompanied by hepatic dysfunction, and recovery in 17/19 episodes. Encephalopathy without hepatic dysfunction occurred in one individual after liver transplantation. On MRI, 5/7 individuals with MRI during acute encephalopathy had deep gray matter and brainstem changes. Supratentorial cortex involvement (6/13) and cerebellar watershed injury (4/13) occurred with seizures and/or encephalopathy. Abnormal brainstem contour on sagittal images (8/13), atrophy (8/13), and myelination delay (8/13) were not clearly associated with encephalopathy. The pattern of deep gray matter and brainstem changes are apparently characteristic of encephalopathy in LARS1-deficiency, differing from patterns of hepatic encephalopathy or metabolic stroke in organic acidurias and mitochondrial diseases. While the pathomechanism remains unclear, fever and energy deficit during infections might be causative; thus, sufficient glucose and protein intake along with pro-active fever management is suggested. As severe episodes were observed during influenza infections, we strongly recommend seasonal vaccination.
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  • 文章类型: English Abstract
    In recent years, reports of health problems associated with nitrous oxide consumption have significantly increased. In Germany, nitrous oxide (N2O) is easily available in cartridges without legal restrictions. The main reason for its popularity in the party scene are the euphoric, psychedelic effects of the gas. In addition to severe and sometimes irreversible health problems associated with long-term use of nitrous oxide, e.g., anemia and nerve damage, life-threatening or fatal consequences of acute nitrous oxide consumption can also occur: accidents under the influence of nitrous oxide, pneumothorax, pneumopericardium and shock due to an explosive increase in airway pressure when inhaled directly from the cartridge. But the most common cause of severe complications is asphyxia as the gas is usually inhaled pure from large balloons and without oxygen. The resulting hypoxia during use may be perpetuated by the diffusion hypoxia that occurs during the reoxygenation period. Nitrous oxide as a cause in accidental or intoxication events is usually not detectable but can only be identified as a trigger based on the patient\'s history or the circumstances. Acute medical treatment is symptomatic.
    UNASSIGNED: In den letzten Jahren haben Berichte über lachgaskonsumassoziierte Gesundheitsstörungen erheblich zugenommen. Lachgas (N2O) ist in Deutschland problemlos legal und nahezu ubiquitär in Kartuschen erhältlich. Grund für die Beliebtheit in der Partyszene sind v. a. die euphorisierend-psychedelischen Effekte des Gases. Neben ernsthaften und manchmal irreversiblen gesundheitlichen Problemen bei Langzeitanwendung (Blutbild- und Nervenschädigungen) ereignen sich immer wieder auch akutmedizinisch bedeutsame, lebensbedrohliche oder tödliche Folgen des Lachgaskonsums: Unfälle unter Lachgaseinfluss, Pneumothorax, Pneumoperikard und Schock durch explosionsartige Druckerhöhung in den Atemwegen bei Inhalation direkt aus der Kartusche, aber v. a. hypoxische Komplikationen, da das Gas meist pur und ohne Sauerstoffbeimengung aus großen Ballons inhaliert wird. Die während der Anwendung auftretende Hypoxie kann zudem durch die in der Abflutungsphase auftretende Diffusionshypoxie perpetuiert werden. Lachgas als Ursache ist bei Unfällen oder Intoxikationsgeschahen meist nicht nachweisbar, sondern nur anamnestisch oder durch die Umstände als Auslöser zu identifizieren. Die akutmedizinische Therapie ist symptomatisch.
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  • 文章类型: Case Reports
    登革热(DF)和结核病(TB)带来了重大的全球健康挑战,通常具有重叠的临床特征,尤其是当并发登革热脑病等疾病时。我们提供了一个案例研究,涉及一名84岁男性,有复杂的病史,包括肺结核再激活,后来患上了登革热脑炎。这突显了在老年人群中管理此类病例的复杂性。登革热脑炎,曾经被认为是非亲神经的,越来越多的人认识到,有神经症状的患者需要考虑作为潜在的鉴别诊断,特别是在流行地区。我们的患者表现出典型的DF症状以及脑病的表现。同时,观察到继发性TB再激活,强调这些疾病之间错综复杂的相互作用。此外,下呼吸道感染(LRTI)进一步复杂化的临床表现。及时识别,综合治理至关重要,正如我们的案例所证明的,及时报告和保守措施导致了有利的结果。
    Dengue fever (DF) and tuberculosis (TB) present significant global health challenges, often with overlapping clinical features, especially when complicated by conditions like dengue encephalopathy. We present a case study involving an 84-year-old male with a complex medical history, encompassing pulmonary tuberculosis reactivation, who subsequently developed dengue encephalitis. This underscores the complexity of managing such cases in the geriatric population. Dengue encephalitis, once considered non-neurotropic, is increasingly recognized, necessitating consideration as a potential differential diagnosis in patients with neurological symptoms, particularly in endemic regions. Our patient exhibited typical DF symptoms alongside manifestations of encephalopathy. Concurrently, secondary TB reactivation was observed, emphasizing the intricate interplay between these diseases. Additionally, lower respiratory tract infection (LRTI) further complicated the clinical picture. Timely recognition and comprehensive management are crucial, as demonstrated in our case, where prompt reporting and conservative measures led to a favorable outcome.
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  • 文章类型: Case Reports
    丙戊酸脑病是一种罕见且严重但可治疗的副作用。这项研究的重点是四名女性患者,他们服用了丙戊酸药物治疗癫痫,并增加了癫痫发作的频率,加剧了意识的破坏,肠胃问题,认知功能障碍,共济失调,和心理行为异常。停用丙戊酸钠后,患者症状随时间改善。因此,使用丙戊酸钠时,人们应该意识到丙戊酸钠脑病的风险,如果任何上述病因不明的症状在临床上表现出来,就停止使用药物。我们还研究了导致丙戊酸脑病的潜在发病机制,以及一起服用抗癫痫药物会增加脑病的风险。有人强调,确定是多么重要,诊断,并尽快治疗丙戊酸钠脑病。
    Valproate encephalopathy is one of the unusual and severe but treatable side effect. This research focuses on four female patients who had valproate medication for epilepsy and developed an increased frequency of seizures, exacerbated disruption of consciousness, gastrointestinal problems, cognitive dysfunction, ataxia, and psychobehavioral abnormalities. The patient\'s symptoms improved over time once sodium valproate was stopped. As a result, when using sodium valproate, one should be aware of the risk of sodium valproate encephalopathy and cease using the medication right once if any of the above symptoms of unknown etiology manifest clinically. We also go over the potential pathogenesis that lead to valproate encephalopathy and the heightened risk of encephalopathy from taking antiepileptic medications together. It was stressed how crucial it is to identify, diagnose, and treat sodium valproate encephalopathy as soon as possible.
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  • 文章类型: Journal Article
    背景:伴有可逆性脾病变(MERS)的轻度脑炎/脑病是一种临床放射学综合征,其特征是在磁共振(MR)上出现短暂的中枢神经系统症状和call体(SCC)的可逆性病变。我们报告了一例罕见的成人发作的MERS,并回顾了现有文献。
    方法:我们描述了一例具有罕见症状和体征的成人发作MERS,并对包括四名以上成人发作(>14岁)MERS患者的病例系列进行了系统评价。从2000年1月到2022年12月。我们总结了临床,实验室,成像和治疗数据。
    结果:我们纳入了7项符合条件的研究,共51例成人发作的MERS患者。大多数患者(88%)的神经系统表现先于前驱症状,主要是发烧(78%)。头痛是最常见的症状(50%),其次是癫痫(22%)和意识障碍(22%)。一半的患者出现脑脊液炎症改变,所以定义脑炎病例。诊所康复,除两名在急性期需要呼吸机支持的严重意识障碍患者外,所有患者均可获得。MR显示92%的患者在SCC中出现孤立性病变,而8%的患者也表现出关节外病变,随访影像时,所有病变均得到缓解或改善.
    结论:MERS是脑炎/脑病的一种形式,具有广泛的中枢神经系统表现,通常有轻微的症状,比如头痛,这可能会导致一些案件被忽视,大多数患者预后良好。
    BACKGROUND: Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) is a clinical-radiological syndrome characterized by transient central nervous system symptoms and a reversible lesion in the splenium of the corpus callosum (SCC) on magnetic resonance (MR). We reported a case of adult-onset MERS with uncommon presentation and reviewed the existing literature.
    METHODS: We described a case of adult-onset MERS with uncommon symptoms and signs and performed a systematic review of case series including more than four patients of adult-onset (> 14 years old) MERS, from January 2000 to December 2022. We summarized the clinical, laboratory, imaging and therapy data.
    RESULTS: We included seven eligible studies for a total of 51 adult-onset MERS patients. Neurological manifestations were preceded by prodromal symptoms in most of the patients (88%), mainly with fever (78%). Headache was the most common symptom (50%), followed by seizures (22%) and disturbance of consciousness (22%). Inflammatory changes on cerebrospinal fluid were present in a half of patient, so defining encephalitis cases. Clinal recovery, was achieved in all patients but two with severe disturbance of consciousness who required ventilator support in the acute phase. MR showed isolated lesion in the SCC in 92% of patients, while 8% of patients showed also extracallosal lesions, all the lesions resolved or improved on follow-up imaging.
    CONCLUSIONS: MERS is a form of encephalitis/encephalopathy with a broad range of central nervous system manifestation, often with mild symptoms, such as headache alone, that can lead to overlooked some cases, with an excellent prognosis in most patient.
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  • 文章类型: Case Reports
    癫痫发作可因感染而恶化;然而,它们有时在急性病毒感染后消失或减少,虽然这是罕见的。我们报道了一名患有DNM1脑病的男孩在诺如病毒引起的病毒性胃肠炎后癫痫发作的自发缓解。从两个月大开始,他每天都有阵挛性癫痫发作,并在14个月大时出现癫痫性痉挛;此时他被送进医院。体格检查显示肌张力减退,斜视,舌头突出下垂,和宽间隔的牙齿。尽管脑部磁共振成像并不明显,脑电图显示频繁的枕骨尖峰。入院三天后,病人出现频繁腹泻而不发烧。粪便样本中诺如病毒的快速免疫层析检测呈阳性。腹泻出现后立即出现,癫痫发作消失了。目前,五岁的时候,患者有严重的精神运动发育迟缓;他没有言语表达,无法行走。自10个月大以来,他经历了肌阵挛症的非自愿运动。患者DNA的全外显子组测序显示存在DNM1的杂合从头变体:c.709C>T(p。Arg237Trp)。虽然从我们的病人的研究结果表明,潜在的神经网络异常被改善的免疫机制作为病毒感染的结果,需要进一步的研究来阐明癫痫自发缓解背后的机制.
    Epileptic seizures can be worsened by infections; however, they sometimes disappear or decrease after an acute viral infection, although this is rare. We report the spontaneous remission of epileptic seizures following norovirus-induced viral gastroenteritis in a boy with DNM1 encephalopathy. He had clonic seizures daily from the age of two months and developed epileptic spasms at 14 months of age; he was admitted to the hospital at this time. A physical examination revealed hypotonia, strabismus, tongue protrusion with drooping, and widely spaced teeth. Although brain magnetic resonance imaging was unremarkable, electroencephalography revealed frequent occipital spikes. Three days after admission, the patient developed frequent diarrhea without a fever. A rapid immunochromatographic test of norovirus in a stool sample was positive. Immediately after the appearance of diarrhea, the epileptic seizures disappeared. Currently, at the age of five years, the patient has a profound psychomotor developmental delay; he has no verbal expression and is unable to walk. He has experienced involuntary movements of the myoclonus since 10 months of age. Whole-exome sequencing of the patient\'s DNA revealed the presence of a heterozygous de novo variant of DNM1: c.709C>T (p.Arg237Trp). Although the findings from our patient suggest that underlying neural network abnormalities were ameliorated by immunological mechanisms as a result of the viral infection, further research is needed to clarify the mechanisms behind this spontaneous remission of seizures.
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  • 文章类型: Journal Article
    造影剂诱发的脑病是与血管内手术或计算机断层扫描(CT)中使用的造影剂相关的神经系统并发症。主要危险因素是动脉高血压,糖尿病,慢性肾脏病(CKD),高渗性对比,注入造影剂的量及其在大脑后部的直接注入,或血脑屏障损伤的病理。症状学是非特异性的,可能表现为意识水平的改变,神经病灶或癫痫发作。在排除缺血性或出血性中风后,通过排除进行诊断;CT或MRI可用于鉴别。一般来说,它在暴露后不久出现,症状持续48-72小时,完全恢复,尽管已经描述了症状持续或持续时间较长的病例。治疗包括监测,慢性血液透析(HD)患者的支持措施和肾脏替代治疗(KRT)。考虑到患者对HD的易感性以及其在这些患者中的潜在治疗作用,肾脏病学家必须意识到该实体。
    Contrast-induced encephalopathy is a neurological complication related to contrast used in endovascular procedures or computed tomography (CT). The main risk factors are arterial hypertension, diabetes mellitus, chronic kidney disease (CKD), hyperosmolar contrasts, the amount of infused contrast and its direct infusion in the posterior cerebral territory, or pathologies with blood-brain barrier damage. Symptomatology is non-specific and may present as altered level of consciousness, neurological focality or seizures. Diagnosis is done by exclusion after ischemic or hemorrhagic stroke has been ruled out; CT or MRI are useful for differentiation. Generally, it appears shortly after exposure and the symptoms lasts 48-72h with complete recovery, although cases with persistence of symptoms or longer duration have been described. Treatment consists of monitoring, supportive measures and kidney replacement therapy (KRT) with hemodialysis (HD) in patients in chronic KRT program. It is important for the nephrologist to be aware of this entity given the susceptibility of the patient on HD as well as its potential therapeutic role in these patients.
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