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Radiculopathy C8-T1 atypical initial presentation: a case report 神经根病C8-T1不典型首发:1例报告
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.487
Rafael de Almeida, Sayuri Aparecida Hirayama, Francine de Paula Roberto Domingos, Lucas Silva Dias, Lorena Dias de Araújo, Paulo Henrique Martinelli Oliveira, Raderi Luiz Cardoso dos Santos, Larissa Miyashiro, Rushansky Vilela de Azevedo, Gustavo Carvalho Costa
Introduction: Radiculopathy is a common condition whose symptoms can include pain, sensory change, and motor weakness owing to mechanical and chemical irritation of the spinal nerve root. The aim is to report atypical clinical presentation of radiculopathy secondary to neoplasia. Case report: A 62-year-old male patient was admitted with pain in the right scapular region of onset 2 months ago and progressive worsening. On patient admission, he presented isolated loss of strength in the right hand with force grade V in the proximal right upper limb and right dropped hand and living reflexes in the right upper limb. Thoracic/cervical spine magnetic resonance imaging with expansive formations in the thoracic bone marrow of neoplastic aspect, compressive fracture of the vertebral body of T1 with impairment of the thoracic cord, reduction of the vertebral canal at level C3-C4. Electroneuromyography: bilateral acute C8-T1 radiculopathy, worse right and chronic radiculopathy on the left. Subsequently, he evolved with brachial paraparesis, exalted global osteotendinous reflexes, and inexhaustible clonus in the lower limbs. C4-T5 posterior cervical arthrodesis was performed. Discussion: Cervical radiculopathy may leave the clinician perplexed by lack of clinical-radiological correlation. Of special interest is the weakness of the intrinsic hand muscles without radiological evidence for C8 radiculopathy. Overlapping with the T1 root, the C8 root innervates the finger flexors and all the intrinsic hand muscles. C8 radiculopathy is characterized by radicular neck pain, hand weakness, and sensory deficit of the ulnar fingers and medial forearm. Conclusion: The lack of clinical-radiological correlation should not mislead the clinician from the correct diagnosis, and should not delay the surgical decompression of the cord and the roots.
神经根病是一种常见的疾病,其症状包括疼痛、感觉改变和运动无力,这是由于脊神经根受到机械和化学刺激所致。目的是报告继发于肿瘤的神经根病的不典型临床表现。病例报告:一名62岁男性患者因右肩胛骨区疼痛2个月前发病并逐渐加重而入院。入院时,患者表现为孤立性右手力量丧失,右上肢近端力量等级为V级,右手下垂,右上肢反射正常。胸椎/颈椎磁共振成像显示肿瘤方面胸椎骨髓膨胀,T1椎体压缩性骨折伴胸髓损伤,C3-C4节段椎管复位。神经肌电图:双侧急性C8-T1神经根病,右侧加重,左侧慢性神经根病。随后,他发展为臂膀截瘫,整体骨腱反射增强,以及下肢无穷无尽的阵挛。行C4-T5颈椎后路关节融合术。讨论:由于缺乏临床与放射学的相关性,颈椎神经根病可能使临床医生感到困惑。特别感兴趣的是手部固有肌肉无力,没有C8神经根病的放射证据。与T1根重叠,C8根支配手指屈肌和所有手部固有肌肉。C8神经根病的特征是神经根性颈痛、手部无力、尺指和前臂内侧感觉缺陷。结论:临床与放射学相关性的缺乏不应误导临床医生正确诊断,不应延误脊髓根减压手术。
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引用次数: 0
“Wake-up” onset of pharyngocervicobrachial variant of Guillain-Barré syndrome: a case report 格林-巴- <s:1>综合征咽喉颈臂变异型的“唤醒性”发病1例
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.565
Evelliny Gomes da Silva, A. Holanda, V. Arca, Daniel Alves de Oliveira, Anna Paula Paranhos Miranda Covaleski, Letícia Klabinske Marques Monteiro, Eduardo Sousa de Melo
Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in the world. There are variants. Of the cranial forms, the following stand out: the classic form, facial diplegia with distal paresthesias, pharyngo-cervico-brachial form, polyneuritis cranialis, Miller-Fisher syndrome and Bickerstaff encephalitis. This study aimed to report the case of a 73-year-old male patient, diabetic, former smoker and alcoholic, who presented at the Neurology outpatient clinic of a tertiary hospital in Pernambuco, after emergency care and 19 days of symptoms, reported as sudden cervical weakness, dysarthrophonia, dysphagia and weakness in the right hemiface, three weeks after vaccination (influenza and triple viral) and flu syndrome. He had dyspnea since the onset of the condition, with no progression or fluctuating complaints. The neurological examination showed multiple cranial nerve syndrome (right peripheral pattern facial palsy, reduced elevation of the soft palate and cervical extension paresis) associated with global hypo/arreflexia. Complementary exams showed, in addition to leukocytosis and signs of bronchopathy on chest tomography, cerebrospinal fluid with 00 cells and 48 proteins and electroneuromyography with predominantly sensitive axonal polyneuropathy, decrease in bilateral facial motor amplitude, needle with myopathic pattern. Brain magnetic resonance imaging without alterations. With the possibility of cranial polyradiculoneuritis and a history of dyspnea raised, he was admitted to the intensive care unit to monitor his breathing pattern and dysautonomia. He received antibiotic therapy for seven days due to pneumonia and pulse therapy with IVIG (2 g/kg for five days). He maintained progressive improvement of symptoms. He was discharged with a multidisciplinary outpatient follow-up scheduled. It is concluded, therefore, that the recognition of GBS and variant forms is necessary.
吉兰-巴罗综合征(GBS)是世界上最常见的急性弛缓性麻痹病因。有不同的变体。在颅型中,有以下几种比较突出:经典型、面瘫伴远端感觉异常、咽颈臂型、颅多神经炎、米勒-费雪综合征和比克斯塔夫脑炎。本研究旨在报告一名73岁男性患者,糖尿病患者,前吸烟者和酗酒者,他在伯南布哥一家三级医院的神经病学门诊就诊,经过紧急护理和19天的症状,报告为突然颈椎无力,关节障碍,吞咽困难和右半面无力,接种疫苗(流感和三病毒)和流感综合征三周后。他自发病以来就有呼吸困难,无进展或波动性主诉。神经学检查显示多发性颅神经综合征(右外周型面神经麻痹、软腭抬高降低和颈部伸展性轻瘫)与全身反射不足/反射不足有关。补充检查显示,除了白细胞增多和胸部断层扫描支气管病变的征象外,脑脊液有00个细胞和48个蛋白,神经肌电图以敏感轴突多神经病变为主,双侧面部运动幅度下降,针状肌病型。无改变的脑磁共振成像。随着颅多根神经炎的可能性和呼吸困难史的增加,他被送进重症监护病房监测他的呼吸模式和自主神经异常。患者因肺炎接受了7天的抗生素治疗和5天的IVIG脉冲治疗(2g /kg)。他的症状持续改善。出院时安排了多学科门诊随访。因此,有必要对GBS及其变体形式进行识别。
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引用次数: 0
Neurosyphilis causing bilateral optic perineuritis 引起双侧视神经会阴炎的神经梅毒
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.568
R. Maffei, A. Barbosa, A. Amorim, Gabriel Pinheiro Martins de Almeida e Souza, P. Fraiman, J. Ferrer, V. Faria, M. Lima, Ruan Gambardella Rosalina de Azevedo, S. Matas
Introduction: Optic perineuritis is a rare disorder with multiple possible etiologies, including systemic autoimmune or infectious syndromes and, more rarely, neurosyphilis. The aim of this report is to describe an uncommon manifestation of this reemerging infectious disease. Case report based on a retrospective analysis of the medical records of the patient. Case report: A previously healthy 35-year-old female presented to the hospital with a seven-day history of progressive right eye vision loss and pain on extraocular movement, with development of a pink maculopapular rash on the trunk. On the sixth day of her disease, similar symptoms appeared in the contralateral eye. Physical examination showed reduced fotomotor reflex and finger counting at 50 cm in both eyes. Magnetic resonance imaging revealed bilateral and circumferential thickening with enhancement of the optic nerve sheath, suggesting bilateral optic nerve perineuritis. Serum Venereal Disease Research Laboratory (VDRL) was 1/64 and cerebrospinal fluid (CSF) showed 440 cells (86% lymphocytes), proteins 97 mg/dL, glucose 47 mg/dL, lactate 21 mg/dL and VDRL 1/4. She was treated with IV crystalline penicillin for 21 days and had partial improvement of bilateral vision within two months. At follow-up, her visual acuity was 20/40 in the right eye and 20/100 in the left; her CSF normalized, with nonreactive VDRL. Conclusion: Neurosyphilis is a treatable cause of optic perineuritis, and its recognition is important to establish the right antibiotic treatment.
视神经周围炎是一种罕见的疾病,有多种可能的病因,包括系统性自身免疫或感染性综合征,以及更罕见的神经梅毒。本报告的目的是描述这种重新出现的传染病的一种不寻常的表现。病例报告基于对患者医疗记录的回顾性分析。病例报告:一名健康的35岁女性,因右眼进行性视力丧失和眼外运动疼痛7天就诊,躯干出现粉红色斑疹。发病第6天,对侧眼出现类似症状。体格检查显示双眼50 cm处足运动反射和手指计数减少。磁共振成像显示双侧及周围增厚伴视神经鞘增强,提示双侧视神经会神经炎。血清性病研究室(VDRL) 1/64,脑脊液(CSF) 440个细胞(86%淋巴细胞),蛋白97 mg/dL,葡萄糖47 mg/dL,乳酸21 mg/dL, VDRL 1/4。静脉注射结晶青霉素21天,2个月内双侧视力部分改善。随访时,患者右眼视力20/40,左眼视力20/100;她的脑脊液正常,无反应性VDRL。结论:神经梅毒是视神经会阴炎的一种可治疗的病因,对其进行识别对确定正确的抗生素治疗具有重要意义。
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引用次数: 0
History of neurology in São Paulo at the first half of the 20th century: a systematic review 20世纪上半叶<s:1>圣保罗神经病学史:系统回顾
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.611
M. Costa, João Pedro de Sá Pereira, Laura Victória Miranda Silveira
Neurology in São Paulo was initiated at the beginning of the XX century, with the creation of the Paulista School of Medicine. This article aims to analyze the emergence of Neurology in the state of São Paulo and the influences it received. To this end, we conducted a systematic search in an electronic database, Scielo. In this context, in the 20th century, neurology studied in São Paulo was greatly influenced by the science practiced in France, since in 1882 the specialty was initiated at the University of Paris. Discussions about the diseases in this area were discussed at the Santa Casa de Misericórdia in São Paulo. Another important place for the development of neurology in São Paulo was the creation of the neuropathology and cerebrospinal fluid laboratory, in 1924, at the Hospital de Juqueri. In the 1920s, the teaching of clinical neurology was created at the Faculty of Medicine by Enjolras Vampré, known as the father of neurology in São Paulo. In the 1930s, there were major changes in the areas of brain pathology, diagnosis of diseases that affect the central nervous system, and therapy. All of this was used at Hospital de Juqueri and Faculdade Paulista de Medicina. Among the prominent methods used were pneumoencephalography, cerebral arteriography, and cerebral leucotomy. Some literary works were fundamental for the dissemination of knowledge of Neurology in the society of São Paulo, namely “Memories of the Hospital of Juqueri” from 1924 and “Archives of Assistance to Psychopaths” from 1936. Neurology underwent a major evolution at the end of the first half of the twentieth century, however, it became expensive and inaccessible to a large part of the population, especially at a time before the creation of the Unified Health System, making the poorest marginalized concerning the treatment of neurological diseases that affected the society of São Paulo at the time.
随着圣保罗医学院的建立,圣保罗的神经病学始于20世纪初。本文旨在分析神经学在圣保罗州的出现及其所受到的影响。为此,我们在Scielo电子数据库中进行了系统的检索。在这种背景下,在20世纪,在圣保罗学习的神经学深受法国科学实践的影响,因为该专业于1882年在巴黎大学创立。在圣保罗圣之家Misericórdia讨论了这一领域的疾病。神经学在圣保罗另一个重要的发展地点是1924年在Juqueri医院建立的神经病理学和脑脊液实验室。在20世纪20年代,临床神经学的教学在医学院由安灼拉·万普拉斯创立,他被称为圣保罗神经学之父。在20世纪30年代,脑病理学、影响中枢神经系统疾病的诊断和治疗领域发生了重大变化。所有这些都在Juqueri医院和Paulista de Medicina学院使用。其中使用的主要方法是气脑造影,脑动脉造影和脑白质切开术。一些文学作品是圣保罗社会传播神经学知识的基础,即1924年的《Juqueri医院的回忆》和1936年的《精神病患者援助档案》。神经病学在20世纪上半叶经历了一次重大的发展,然而,它变得昂贵,大部分人无法获得,特别是在统一卫生系统建立之前,使得最贫穷的人在神经疾病的治疗方面被边缘化,这种疾病当时影响了圣保罗社会。
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引用次数: 0
Correlation between neurological deficit at admission and functionality at discharge of patients from a clinical hospital stroke program 临床医院卒中项目患者入院时神经功能缺损与出院时功能的相关性
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.593
Tamires Cristine Bitencourt, Caio Henrique Veloso da Costa, Ana Lúcia Parizi Mello, Saulo Ribeiro, R. Domingues
Introduction: Stoke is one of the main causes of disability in Brazil, with an average hospital stay (HS) of 11 days and prolonged HS can generate functional impairment to patients. Objectives: To correlate neurological deficit at admission with functionality and days of HS. Methods: Patients were selected from the clinical program of post-stroke care, from March to December 2022 with HS time greater than or equal to 4 days. They were divided into 2 groups: Group 1, between 4 and 11 days of HS and group 2, greater than 11 days of HS. The Functional Independence Measure (FIM) and the National Institute of Health Stroke Scale (NIHSS) were applied at admission and discharge. Data analysis was performed with Microsoft Excel© 2016 with descriptive statistics, Pearson’s Correlation Test was also performed, considering P < 0.05 for the variables studied. Results: 86 subjects were included. 41 were excluded due to lack of informations in medical records, and 45 patients were analyzed. Group 1: Included 32 patients, 24 with ischemic stroke (IS), 2 hemorrhagic stroke (ICH) and 6 transient ischemic attack (TIA), 56.2% were female and 69 ± 15.2 years and 5.9 ± 15.9 days HS with 5.2 ± 4.8 points in the FIM/days of hospitalization and NIHSS admission of 2.9 ± 4.2. Group 2: 13 patients, 11 with IS, 2 ICH, 61.5% male, with 67±15.2 years, NIHSS admission of 7.3 ± 4.2, there was a gain of 1.14 ± 4.7 points in the FIM/days of hospitalization and 21.3 ± 15.7 days HS. There was a negative correlation between Days of Hospitalization and FIM/days of hospitalization (r = -0.3), Days of Hospitalization and FIM at Discharge (r = -0.57) and NIHSS admission and FIM of discharge (r = -0.6). Conclusion: We observed a correlation between neurological deficit at admission, functional outcome at discharge and time of HS related to systematized assistance to a clinical stroke program.
简介:斯托克是巴西致残的主要原因之一,平均住院时间(HS)为11天,HS延长会对患者产生功能损害。目的:探讨入院时神经功能缺损与HS的功能和天数之间的关系。方法:选取2022年3月~ 12月脑卒中后临床护理方案中HS时间大于等于4天的患者。将其分为2组:1组为HS发病4 ~ 11天,2组为HS发病11天以上。入院和出院时采用功能独立性量表(FIM)和美国国立卫生研究院卒中量表(NIHSS)。使用Microsoft Excel©2016进行数据分析,采用描述性统计,考虑各变量P < 0.05,进行Pearson相关检验。结果:共纳入86例受试者。41例因病历资料不足而被排除,45例患者被分析。第一组:32例患者,其中缺血性脑卒中24例,出血性脑卒中2例,短暂性脑缺血发作6例,女性56.2%,HS 69±15.2岁5.9±15.9天,住院时间FIM/d 5.2±4.8分,NIHSS入院时间2.9±4.2分。组2:13例患者,其中IS 11例,ICH 2例,男性61.5%,平均年龄67±15.2岁,NIHSS住院7.3±4.2分,住院时间FIM/d增加1.14±4.7分,HS增加21.3±15.7天。住院天数与FIM/住院天数(r = -0.3)、出院时住院天数与FIM (r = -0.57)、NIHSS入院与出院时FIM (r = -0.6)呈负相关。结论:我们观察到入院时的神经功能缺损,出院时的功能结局和HS的时间与临床卒中计划的系统化协助之间的相关性。
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引用次数: 0
Multifocal motor neuropathy atypical presentation: case report 多灶性运动神经病不典型表现:1例报告
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.594
Ana Flavia Andrade Lemos, M. C. Foloni, Rebeca Aranha Barbosa Sousa, Yasmim Nadime José Frigo, T. Alvarenga, Patrick Emanuell Mesquita Sousa Santos, Gilberto Bento Magioni Junior, Ana Beatriz Baston, Helio Aquaroni Farão Gomes, L. C. Lopes, N. Nakao
Case report: Woman, 34 years old, with paresis that started four years ago during pregnancy, predominantly distal in the lower limbs and progression to the upper limbs. No involvement of cranial nerves and sensitivity. Initially considered a diagnosis of Myasthenia Gravis and clinical gain was observed with the use of pyridostigmine. After a few months, the patient’s symptoms recurred with asymmetric flaccid tetraparesis, predominantly in the lower limbs, associated with global areflexia and oral immunosuppressive therapy was initiated, with improvement. Electroneuromyography with normal sensory nerve conduction studies, but reductions in amplitudes in proximal compound muscle action potentials, with markedly reduced persistence in F-wave studies of the four limbs. Diffuse neurogenic changes were observed on exertion and activities such as positive sharp waves, fibrillation, fasciculations and myokymia at rest. Repetitive nerve stimulation at 3Hz without changes. A hypothesis of multifocal motor neuropathy (MMN) was then made. Treatment with intravenous immunoglobulin was performed, with significant recovery of symptoms. Discussion: MMN is a rare disease (prevalence of 0.6 per 100,000 individuals), with a predominance in men and a mean age of onset around 40 years. The differential diagnosis includes motor neuron disease and other demyelinating neuropathies. It is immune-mediated by antianglioside antibodies (anti-GM1), but they are not identified in all patients and may be present in other neuropathies. It is defined by muscle weakness predominantly distal, asymmetrical, predominantly in the upper limbs, slowly progressive, associated with reduced deep reflexes in the affected regions. The main electrophysiological characteristic is the presence of motor nerve conduction blocks (CB) outside the usual sites of compression. Conclusion: The finding that CB presents in patients with MMN suggests that nerve conduction should be extensively studied in every patient with a lowermotor-neuron syndrome to identify patients who might respond favorably to immunomodulating treatment.
病例报告:女性,34岁,四年前怀孕期间开始出现瘫,主要是下肢远端,并进展到上肢。没有脑神经和敏感性受累。最初认为诊断重症肌无力和临床增益观察使用吡哆斯的明。几个月后,患者症状复发,表现为不对称弛缓性四肢全瘫,以下肢为主,伴有全身反射,开始口服免疫抑制治疗,病情有所改善。正常感觉神经传导的神经肌电图,但近端复合肌动作电位的振幅降低,四肢f波研究的持久性明显降低。在运动和活动中观察到弥漫性神经源性改变,如正锐波、纤颤、束状搏动和静息时肌无力。重复3Hz神经刺激无变化。提出多灶性运动神经病(MMN)假说。静脉注射免疫球蛋白治疗后症状明显恢复。MMN是一种罕见疾病(患病率为每10万人中0.6人),以男性为主,平均发病年龄约为40岁。鉴别诊断包括运动神经元病和其他脱髓鞘神经病变。它是由抗angli苷类抗体(anti-GM1)免疫介导的,但并非在所有患者中都发现,可能存在于其他神经病变中。主要表现为远端肌肉无力,不对称,主要发生在上肢,缓慢进展,伴有受影响区域深反射减少。主要的电生理特征是在通常的压迫部位外存在运动神经传导阻滞(CB)。结论:脊髓灰质炎在MMN患者中出现的发现表明,应该对每一位低运动神经元综合征患者的神经传导进行广泛的研究,以确定可能对免疫调节治疗有良好反应的患者。
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引用次数: 0
Renal predictors of mortality in stroke patients: partial results of a transversal study 中风患者死亡率的肾脏预测因素:一项横向研究的部分结果
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.621
A. Novais, Beatriz Santos Rogério, R. Branco
Introduction: Renal factors are known as important, and a controllable factor, to define prognosis,mortality and hospitalization time in most of diseases. It still lacks studies that clarify in a specific way the role of this factors. Objectives: This study aims to elucidate the impact of renal factor in outcomes of stroke patients (hospitalization time, CTI time, delirium, infection and death during internment). Methods: We performed a transversal study, part of a cohort. Data of previous renal insufficiency,admission,highest and last creatinine and urea and the highest creatinine clearance during hospitalization. We performed univariate analysis, with T test, Mann-Whitney or chi-square tests;after, multivariate analysis with logistic regression (considering P < 0.05). Results: We obtained 190 patients. Admission creatinine was significant to death (P = 0.05), delirium (P = 0.07) and infection (P = 0.06); highest creatinine to hospitalization time, delirium, infection (P < 0.001) and death (P = 0.008);and last creatinine to death (P < 0.001), delirium (P = 0.003), infection (P = 0.01) and CTI time (P = 0.1). Previous renal insufficiency showed significance to death (P = 0.03), delirium and infection (P < 0.001). ). Highest urea showed significance to all outcomes (P < 0.001), including CTI time (P = 0.004); admission urea to death (P = 0.01); and last urea to death (P < 0.001), hospitalization time (P = 0.1) and delirium (P = 0.06). Clearance only did not show significance to CTI time.Multivariate analysis to each outcome obtained: last creatinine to death (P = 0.03); highest creatinine to hospitalization time (P = 0.02); admission (P = 0.01), highest (P = 0.01) creatinine, admission urea (P = 0.03) and clearance (P = 0.01) to delirium; renal insufficiency (P = 0.009), admission (P = 0.007), highest (P = 0.002) and last (P = 0.02) creatinine, highest urea (P = 0.005) and clearance (P = 0.003). None was significant to CTI time. Conclusion: All parameters of renal function showed to be important measures of prognosis in stroke patients; thus, it is a factor we need to be aware of during hospitalization time.
在大多数疾病中,肾脏因素被认为是决定预后、死亡率和住院时间的一个重要的、可控的因素。目前还缺乏明确这些因素具体作用的研究。目的:本研究旨在探讨肾因素对脑卒中患者预后(住院时间、CTI时间、谵妄、住院期间感染和死亡)的影响。方法:我们进行了一项横向研究,是队列研究的一部分。既往肾功能不全、入院情况、住院期间最高、末次肌酐、尿素及最高肌酐清除率资料。单因素分析采用T检验、Mann-Whitney检验或卡方检验,多因素分析采用logistic回归(考虑P < 0.05)。结果:获得190例患者。入院肌酐与死亡(P = 0.05)、谵妄(P = 0.07)、感染(P = 0.06)有显著性差异;最高肌酐值与住院时间、谵妄、感染(P < 0.001)、死亡(P = 0.008)有关;最后肌酐值与死亡(P < 0.001)、谵妄(P = 0.003)、感染(P = 0.01)、CTI时间(P = 0.1)有关。既往肾功能不全与死亡(P = 0.03)、谵妄和感染(P < 0.001)有显著关系。. 最高尿素对所有结局均有显著性意义(P < 0.001),包括CTI时间(P = 0.004);入院尿素致死(P = 0.01);最后尿素死亡(P < 0.001)、住院时间(P = 0.1)和谵妄(P = 0.06)。仅间隙对CTI时间无显著影响。各结果的多因素分析:最后肌酸酐至死亡(P = 0.03);最高肌酐与住院时间的关系(P = 0.02);入院时肌酐(P = 0.01)、尿素(P = 0.03)、清除率(P = 0.01)最高;肾功能不全(P = 0.009)、入院(P = 0.007)、最高(P = 0.002)和最后(P = 0.02)肌酐、最高尿素(P = 0.005)和清除率(P = 0.003)。对CTI时间无显著影响。结论:肾功能各项指标是判断脑卒中患者预后的重要指标;因此,这是我们在住院期间需要注意的一个因素。
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引用次数: 0
Pure motor-variant CIDP associated with immune checkpoint inhibitor therapy 纯运动变异性CIDP与免疫检查点抑制剂治疗相关
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.640
Laís Ciribelli Yamaguchi, Jéssica Cristina Silveira Damasceno, Bruna Queiróz Vieira, Laura Altomare Fonseca Campos, Marcelo Sobrinho Mendonça, T. Vale
Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease with many possible etiologies. There are only a few reports of CIDP secondary to the use of immune checkpoint inhibitor therapy. The aim is to describe a case of CIDP secondary to the treatment of metastatic melanoma with immune checkpoint inhibitors (ipilimumab and nivolumab). Case report: A 52-year-old male patient, with arterial hypertension and hypothyroidism, presented with paresthesia and pain in the hands and forearms in November/22, that progressively spread and affected the feet in a one-month period. He then presented proximal and distal tetraparesis in January/23, leading him to depend on a wheelchair. At the time, he was being treated with immunotherapy for metastases in the pectoral muscles due to a melanoma. Symptoms’ onset and progression coincided with the therapy infusions. On examination, there were a grade 4 strength in flexion, extension, abduction and adduction of the thighs and grade 5 in other movements, with global areflexia and tactile hypoesthesia in the feet. Cerebrospinal fluid examination (CSF) showed 11 cells, predominantly with lymphocytes, and 283 mg/dL of proteins. Electromyographic studies revealed focal demyelinating neuropathy of the medians at the wrist level, with moderate to severe intensity on the right and moderate on the left, suggestive of pure motor-variant CIDP. Methylprednisolone 1g/day was given for five days with significant improvement of the condition. Results: The 2021 EFNS/PSN criteria provide diagnostic guidelines for CIDP based on clinical, electromyographic and CSF studies. Conclusion: CIDP secondary to the use of immune checkpoint inhibitors has distinct characteristics such as lymphocytic pleocytosis with slightly increased CSF cellularity and severe neuropathic pain as an initial symptom.
慢性炎症性脱髓鞘性多根神经病变(CIDP)是一种自身免疫性疾病,有多种可能的病因。使用免疫检查点抑制剂治疗继发的CIDP只有少数报道。目的是描述一个用免疫检查点抑制剂(ipilimumab和nivolumab)治疗转移性黑色素瘤继发的CIDP病例。病例报告:一名52岁男性患者,患有动脉高血压和甲状腺功能减退症,于11月22日表现为手部和前臂感觉异常和疼痛,并在一个月内逐渐扩散并影响足部。23年1月,他出现了近端和远端四肢全瘫,导致他依赖轮椅。当时,他正在接受由黑色素瘤引起的胸肌转移瘤的免疫治疗。症状的发生和进展与治疗输注一致。检查时,大腿屈曲、伸展、外展和内收的力量为4级,其他运动为5级,伴有全身反射和足部触觉感觉减退。脑脊液检查显示11个细胞,以淋巴细胞为主,蛋白283 mg/dL。肌电图显示局灶性脱髓鞘神经病变位于手腕水平,右侧中度至重度,左侧中度,提示纯运动变异性CIDP。甲强的松龙1g/天,连续5天,病情明显改善。结果:2021年EFNS/PSN标准提供了基于临床、肌电图和脑脊液研究的CIDP诊断指南。结论:使用免疫检查点抑制剂继发的CIDP具有明显的特征,如淋巴细胞增多,脑脊液细胞量轻微增加,首发症状为严重的神经性疼痛。
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引用次数: 0
A467T variant of the polg gene: description of two clinical cases polg基因的A467T变异:两例临床病例的描述
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.669
Ana Marina Dutra Ferreira da Silva, Igor Dias Brockhausen, Alessandra Lima Nogueira Tolentino, Ana Laura Moura, A. A. Carvalho
Introduction: Variations in the POLG gene are the most common causes of mitochondrial disease of autosomal inheritance, and may be present in about 2% of the population. Case report: Case 1. CMAM, male, 48-year-old, complaining of bilateral eyelid ptosis with onset in adolescence. Since the age of six, he has been diagnosed with epilepsy. After five years of follow-up, he developed sensory ataxia. After 10 years he began to present dysarthria, dysphagia, tremor and pyramidal syndrome. Case 2. ASB, female, 42 years old, at 20 years old presented generalized clonic tonic crisis during the second and third trimesters of pregnancy; at 35 years of age she complained of tingling in plants and legs; at 37 years she noticed bilateral eyelid ptosis and at 39 years she noticed the presence of slurred speech and fatigue on small efforts. He has 3 siblings with similar symptoms and great difficulty walking. No history of consanguinity. Propedeutics: Normal serum lactate and CPK dosage; muscle biopsy showed variation in the caliber of muscle fibers, with the presence of “ragged red fibers” in Gomori’s Trichrome stain. Cranial magnetic resonance imaging: mild cerebellar atrophy in patient 1 and normal in patient 2. Electroneuromyography reveled absence of sensory action potentials in all nerves studied in both cases. New generation sequencing myopathy panel revealed pathogenic variant in homozygosis in the POLG c.1399G>A gene (p.Ala467Thr). Results: The patients received the diagnosis of mitochondrial disease, presenting complex clinical phenotype. Conclusion: DNA polymerase gamma is the enzyme responsible for replicating and maintaining mitochondrial DNA, encoded by nuclear DNA. The c.1399G>A variant in exon7 causes a replacement of an alanine with threonine (A467T), and is one of the causes of ataxia, such as spinocerebellar ataxia with epilepsy; autosomal recessive mitochondrial ataxia, sensory neuropathy, dysarthria and ophthalmoparesis and myoclonic epilepsy, myopathy and sensory ataxia. However, most of the time, they present a continuum between the phenotypes described.
简介:POLG基因变异是常染色体遗传的线粒体疾病最常见的原因,可能存在于约2%的人群中。病例报告:病例1。CMAM,男,48岁,主诉双侧眼睑下垂,青春期发病。从六岁起,他就被诊断患有癫痫。5年后,他出现了感觉性共济失调。10年后,他开始出现构音障碍、吞咽困难、震颤和锥体综合征。例2。ASB,女,42岁,20岁时在妊娠中晚期出现全身性阵挛性强直危象;35岁时,她抱怨植物和腿部有刺痛感;37岁时,她注意到双侧眼睑下垂,39岁时,她注意到言语不清和轻微的疲劳。他有3个兄弟姐妹也有类似的症状,而且行走非常困难。没有血缘史。预后:血清乳酸、CPK剂量正常;肌肉活检显示肌纤维直径的变化,在Gomori三色染色中存在“粗糙的红色纤维”。颅脑磁共振成像:患者1轻度小脑萎缩,患者2正常。两例患者的神经肌电图均显示感觉动作电位缺失。新一代测序肌病面板显示POLG c.1399G>A基因纯合子的致病变异(p.a ala467thr)。结果:本组患者均诊断为线粒体疾病,临床表现复杂。结论:DNA聚合酶γ是由核DNA编码的负责线粒体DNA复制和维持的酶。外显子7中的c.1399G>A变异导致丙氨酸被苏氨酸取代(A467T),是共济失调的原因之一,如脊髓小脑性共济失调伴癫痫;常染色体隐性线粒体共济失调,感觉神经病变,构音障碍和眼麻痹,肌阵挛性癫痫,肌病和感觉共济失调。然而,大多数时候,它们在所描述的表型之间呈现连续统一体。
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引用次数: 0
Evaluation of the NINDS-CSN 5-minutes protocol as a cognitive screening test to detect Parkinson’s disease dementia: a study of a Brazilian sample 评价NINDS-CSN 5分钟方案作为检测帕金森病痴呆的认知筛查试验:一项巴西样本研究
IF 1.4 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.5327/1516-3180.141s1.679
I. Teixeira, Vanessa Pereira de Alencar Souza, V. Borges, H. Ferraz
Introduction: Many cognitive screening tests have been investigated for the diagnosis of Parkinson’s disease dementia (PDD), due to its prevalence and its interference in the evolution of the disease, quality of life and in the treatment response of the patients with Parkinson’s disease (PD). Therefore, more effective and faster cognitive screening tests are needed. Objectives: To evaluate the usefulness of the NINDS-CSN 5-minutes protocol assessment (NC5MPA) in PD patients as screening test for the detection of PDD, as well as to test if the association with the cube drawing test (CDT) can increase the test accuracy. Methods: A total of 98 patients with PD were evaluated using the NC5MPA, combined with the CDT, Mini Mental State Examination and the Montreal Cognitive Assessment (MoCA). These patients were also evaluated for mild cognitive impairment (MCI) and dementia by the Clinical Dementia Rating Scale (CDR). Results: There was a good correlation (with P value < 0.00) between the test scores and PDD, but the results of the 3 tests for MCI was > 0.05. The NC5MPA test has had sensitivity of 78.5%, specificity of 85.7%, accuracy of 82.6%, positive predictive value of 80.4% and negative predictive value of 84.2%, in addition to demonstrate an average performance time of 3.2 minutes (3.08–3.31). The association with the CDT has led to a little significant increase in sensitivity and has showed a decrease in specificity and accuracy, besides increase the test performance time. In assessing the interference of education level, the results were influenced by the small sample size of the 5 to 8 years of education group. Conclusion: The NC5MPA test has proven up to be a good screening test for PDD, being even faster and easier to perform, but more tests with larger populations are necessary to assess the accuracy of this test for MCI and to assess if there is interference of education level in the test accuracy.
由于帕金森病(PD)的患病率及其对疾病发展、生活质量和治疗反应的干扰,许多认知筛查测试已被研究用于诊断帕金森病痴呆(PDD)。因此,需要更有效、更快速的认知筛选测试。目的:评价NINDS-CSN 5分钟方案评估(NC5MPA)在PD患者中作为PDD筛查试验的有用性,并检验与立方体绘制试验(CDT)联合是否能提高检测的准确性。方法:采用NC5MPA,结合CDT、Mini Mental State Examination、Montreal Cognitive Assessment (MoCA)对98例PD患者进行评估。这些患者还通过临床痴呆评定量表(CDR)评估轻度认知障碍(MCI)和痴呆。结果:测试分数与PDD之间有良好的相关性(P值< 0.00),但MCI的3项测试结果为bb0 0.05。NC5MPA检测的敏感性为78.5%,特异性为85.7%,准确率为82.6%,阳性预测值为80.4%,阴性预测值为84.2%,平均表现时间为3.2 min(3.8 ~ 3.31)。与CDT的关联导致敏感性略有显著增加,特异性和准确性下降,同时增加了测试执行时间。在评估受教育程度的干扰时,受5 ~ 8年受教育群体的样本量较小,结果受到影响。结论:NC5MPA检测已被证明是一种较好的PDD筛查方法,而且更快、更容易进行,但需要更多的人群进行更多的检测,以评估该检测对MCI的准确性,并评估是否存在教育水平对测试准确性的干扰。
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Sao Paulo Medical Journal
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