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HUNTINGTON'S DISEASE. CLINICAL CASE 亨廷顿氏病。临床病例
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.11
R. Shiraliyeva, A. K. Mammadbayli, U. A. Asadova
Huntington's disease is a neurodegenerative disease that affects basal ganglia in the white matter of the nervous system, tailed nucleus, thalamus, black substance, limbic system, cerebral cortex, cerebellum, manifested by autosome-dominant drive. In patients with early debut, the clinical course is more severe than in the classical form. In the absence of proper symptomatic treatment, neurological signs become more severe and sharply expressive.
亨廷顿氏病是一种影响神经系统白质基底节、尾状核、丘脑、黑质、边缘系统、大脑皮质、小脑的神经退行性疾病,表现为自体显性驱动。早期首发患者的临床病程比传统型更为严重。在缺乏适当对症治疗的情况下,神经系统症状会变得更加严重,表现也更加尖锐。
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引用次数: 0
THE RELATIONSHIP OF TIME PERCEPTION WITH WORKING MEMORY IN PATIENTS WITH MULTIPLE SCLEROSIS 多发性硬化症患者的时间知觉与工作记忆的关系
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.15
E.E. Deveci, O. Saraydar, S. Aksu, G. Kutlu
Objectives. Multiple Sclerosis (MS) is a chronic disease characterized by neuroinflammation of the central nervous system leading to progressive demyelination, loss of axons, and neurodegeneration. Meta-analyses have shown a cognitive impairment with a moderate effect size between healthy volunteers and MS patients, most apparently in working memory (WM), attention, and processing speed. It has recently been found that the perception of time has also been impaired in MS patients. This study aims to examine the relationship between WM and time perception in individuals with MS. Methods. Letter Number Sequencing Test (LNST), Time Interval Estimation Test, and Time Interval Production Test (TIPT) were administered to 50 MS patients. Individuals were allocated into low and high WM groups according to the median value of the LNST total score to compare time perception performance. Results. The mean age of the sample was 39.2. In the sample consisting of 14 male and 36 female participants, the mean number of educated years was 10.7 years, the age of disease onset was 31.9 years, and the duration of the disease was 7.2 years. A correlation was found between TIPT Ratio for 4 seconds and WM as well as disease severity. 29 people were in the low WM group and 21 people were in the high WM group. Age, age of disease onset, and the number of educated years were found to be higher in the high WM group. The TIPT Ratio for 4 seconds was lower in the high WM group. Conclusion. Consistent with the literature, a relationship was found between WM and time perception in individuals with MS in the 4 seconds, in which WM is considered to have a crucial role. The production of a 4- second interval was considered to be used as a marker reflecting both disease severity and WM capacity.
目的。多发性硬化症(MS)是一种慢性疾病,其特征是中枢神经系统的神经炎症导致进行性脱髓鞘、轴突丢失和神经变性。元分析表明,健康志愿者和多发性硬化症患者之间存在认知障碍,影响程度适中,最明显的是工作记忆(WM)、注意力和处理速度。最近发现,多发性硬化症患者对时间的感知也受到了损害。本研究旨在探讨多发性硬化症患者的工作记忆和时间知觉之间的关系。研究方法对 50 名多发性硬化症患者进行字母数字排序测试 (LNST)、时间间隔估计测试 (Time Interval Estimation Test) 和时间间隔制作测试 (Time Interval Production Test)。根据 LNST 总分的中位值将患者分为低 WM 组和高 WM 组,以比较时间感知能力。结果显示样本的平均年龄为 39.2 岁。样本中有 14 名男性和 36 名女性,平均受教育年限为 10.7 年,发病年龄为 31.9 年,病程为 7.2 年。研究发现,4 秒钟的 TIPT 比率与 WM 以及疾病严重程度之间存在相关性。29 人属于低 WM 组,21 人属于高 WM 组。发现高 WM 组的年龄、发病年龄和受教育年数更高。高 WM 组 4 秒钟的 TIPT 比率较低。结论与文献一致,研究发现多发性硬化症患者的 WM 与 4 秒钟的时间感知之间存在关系,而 WM 被认为在其中起着至关重要的作用。4秒间隔的产生被认为是反映疾病严重程度和 WM 能力的标志。
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引用次数: 0
METHODOLOGICAL BASIS FOR STUDYING THE MEDICOSOCIAL CONSEQUENCES OF THE FEMALE REPRODUCTIVE SYSTEM ON THE BACKGROUND OF MULTIPLE SCLEROSIS 研究多发性硬化症背景下女性生殖系统的医疗社会后果的方法论基础
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.24
N.A. Hamidova
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引用次数: 0
REGIONAL FEATURES OF THE MORBIDITY RATE OF CEREBRAL PALSY DEPENDING ON AGE AND GENDER 不同年龄和性别脑瘫发病率的地区特征
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.04
I.E. Hajiyeva, A. K. Mammadbayli
The aim of the study: to assess age-gender characteristics of morbidity rate of cerebral palsy among children in regions of the Republic of Azerbaijan. Materials and methods. Materials of Cerebral Palsy Register created by us. The prevalence (per 100 children) of pathology at different ages (up to 5 years, 5 years and older) among girls and boys in all regions and cities of Azerbaijan was determined. Statistical processing was conducted by the method of qualitative signs. Results. The prevalence of cerebral palsy was 0.27±0.005% among boys, 0.17±0.004 among girls (р<0.001). The index was higher among children aged 0-5 years (0.68±0.01%) than among children aged 5-18 years (0.06±0.001%). These indicators were different in cities and regions of the republic. Conclusions. A relatively high morbidity rate of cerebral palsy is typical for the cities Baku, Sumgayit, Mingachevir and Shirvan, also for regions Absheron, Barda, etc. Cerebral palsy is seen among boys 1.5 times more often. This feature is more pronounced especially in separate districts (1.7 times).
研究目的:评估阿塞拜疆共和国各地区儿童脑瘫发病率的年龄-性别特征。材料和方法。我们创建的脑瘫登记册材料。确定了阿塞拜疆所有地区和城市不同年龄段(5 岁以下、5 岁及以上)女孩和男孩的发病率(每 100 名儿童)。统计处理采用定性符号法。结果。男童脑瘫患病率为 0.27±0.005%,女童为 0.17±0.004(р<0.001)。该指数在 0-5 岁儿童中(0.68±0.01%)高于 5-18 岁儿童(0.06±0.001%)。这些指标在共和国各城市和地区有所不同。结论巴库、苏姆加伊特、明加切维尔和希尔万等城市以及阿勃谢龙、巴尔达等地区的脑瘫发病率相对较高。男孩患脑瘫的比例是男孩的 1.5 倍。这一特点在单独的地区尤为明显(1.7 倍)。
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引用次数: 0
NEUROMYELITIS OPTICA. CASE REPORT 神经脊髓炎视网膜病变。病例报告
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.22
A. K. Mammadbayli, Sh.N. Mehtiyeva, S.C. Ismayilova
Introduction. Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. The frequency of occurrence worldwide is estimated at 1-2 people out of every 100,000 people. Case report. Our patient was a 37-year-old woman. She felt a sudden vision loss since May 2022. She related this condition to another case of unknown etiology that she encountered in January of that year - numerous vesicular-type rashes appeared on the abdomen and back and lasted for 3 days, but the traces of those rashes remained for some time in the form of hypopigmentation. In November 2022, she has a sudden vision loss again. "Vitiligo-type" white spots were visible around the eyes and on the hands. In neurological examination: eyeball movements are complete in all directions, photoreaction is preserved (D=S), nose-lip folds are symmetrical (D=S), tongue is on the midline, swallowing is not disturbed. Muscle tone and muscle strength in upper and lower limbs are normal, reflexes have increased (D=S). No changes in gait and sensory disturbances have been observed. The functions of the pelvic organs have not been disturbed. Intellect appears appropriate for age, speech has not changed. Ophthalmologist's examination, both eyes have high degree of myopia, myopic astigmatism. Contrasted Cranial MRI- Angiography revealed increased signal in the intracranial segments of the bilateral optic nerve, chiasm, and bilateral optic tract level. The MRI image was initially evaluated in favor of bilateral optic neuritis. USG of the thyroid gland showed sonographic changes in favor of Hashimoto's thyroiditis, and autoimmune thyroiditis was detected. In chest MRI, a demyelinating lesion of 3 mm at C4 level, a 2 mm broad-based protrusion at C4-5 disc that minimally compresses the spinal cord, an annular swelling at C5-6 disc that compresses the anterior subarachnoid area, Th1 – 6 mm, Th5 – 12 mm, Th9 – 10-38 mm long demyelinating lesions were seen. Discussion. According to the available literature, the detection of AQP-4 protein antibodies in serum, which has a role in the pathogenesis of the disease, can play a decisive role in differential diagnosis. In clinical practice, despite the phenotypic and serological characteristics of this disease, the diagnosis is often difficult. Because there are various autoimmune, infectious and neoplastic etiologies phenotypic, manifestations of diseases can mimic neuromyelitis optica. However, based on the clinical symptoms and the results of instrumental examinations, our patient was diagnosed with neuromyelitis optica. She was treated with hormonal therapy and plasmapheresis, and a significant positive change in neurological symptoms was observed. Conclusion. Despite the difficulties in the differential diagnosis of neuromyelitis optica with other demyelinating diseases, especially wit
简介神经脊髓炎视网膜谱系障碍(NMOSD)是一种自身免疫性疾病,会导致严重脱髓鞘,尤其是视神经和脊髓,典型临床表现为急性视神经炎和横贯性脊髓炎。全世界的发病率估计为每 10 万人中有 1-2 人。病例报告。患者是一名 37 岁的女性。自 2022 年 5 月起,她突然感到视力下降。她的这种情况与当年 1 月遇到的另一个病因不明的病例有关--腹部和背部出现大量水泡型皮疹,持续了 3 天,但这些皮疹的痕迹以色素沉着的形式保留了一段时间。2022 年 11 月,她的视力再次突然下降。眼周和手部出现 "白癜风型 "白斑。神经系统检查:眼球在各个方向的运动都很完整,光反应得以保留(D=S),鼻唇沟对称(D=S),舌头位于中线上,吞咽不受干扰。上下肢肌张力和肌力正常,反射增强(D=S)。步态和感觉障碍没有变化。盆腔器官的功能没有受到干扰。智力与年龄相符,语言能力也没有改变。经眼科医生检查,双眼高度近视,近视散光。对比颅脑磁共振成像(Cranial MRI)- 血管造影显示,双侧视神经颅内段、视交叉和双侧视束水平信号增强。核磁共振成像初步评估为双侧视神经炎。甲状腺 USG 声像图显示桥本氏甲状腺炎和自身免疫性甲状腺炎。胸部核磁共振成像显示,C4水平有一个3毫米的脱髓鞘病变,C4-5椎间盘有一个2毫米的宽基突起,压迫脊髓的程度很小,C5-6椎间盘有一个环状肿物,压迫蛛网膜下腔前区,Th1-6毫米、Th5-12毫米、Th9-10-38毫米长的脱髓鞘病变。讨论根据现有文献,血清中 AQP-4 蛋白抗体的检测在该病的发病机制中起着决定性作用,可用于鉴别诊断。在临床实践中,尽管该病具有表型和血清学特征,但诊断往往比较困难。因为有各种自身免疫性、感染性和肿瘤性病因的表型、疾病表现可与神经脊髓炎视网膜炎相似。然而,根据临床症状和仪器检查的结果,我们的患者被诊断为神经脊髓炎视网膜病变。她接受了激素治疗和血浆置换术,神经症状出现了明显的积极变化。结论尽管神经脊髓炎视网膜病变与其他脱髓鞘疾病,尤其是多发性硬化症的鉴别诊断存在困难,但通过详细了解临床、放射学和预后方面的差异,还是可以正确评估诊断标准的。
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引用次数: 0
LATERAL AMYOTROPHIC SCLEROSIS IN THE PRACTICE OF A CARDIOLOGIST 一位心脏病专家在临床上的侧肌萎缩性硬化症病例
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.10
E. V. Khorolets, S.V. Shlyk
Comorbid pathology affects the course and outcome of the disease. The observation of a patient after acute myocardial infarction with the development of amyotrophic lateral sclerosis is a special case. The progression of neurological symptoms in amyotrophic lateral sclerosis leads to decompensation of respiratory and heart failure and an unfavorable outcome of the disease.
并发症会影响疾病的进程和结果。观察急性心肌梗死后并发肌萎缩侧索硬化症的患者就是一个特殊病例。肌萎缩性脊髓侧索硬化症的神经症状不断加重,导致呼吸衰竭和心力衰竭失代偿,对疾病的预后不利。
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引用次数: 0
SPINAL SURGERY: HISTORICAL ASPECTS AND CONTEMPORARY APPROACHES IN THE TREATMENT OF DEGENERATIVE DISORDERS 脊柱外科:治疗退行性疾病的历史和现代方法
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.03
A.M. Furman, O.M. Komarov, M.V. Khyzhnyak, I.J. Garayev
The issue of treating discogenic neurocompression syndromes remains highly relevant in modern medicine due to the variability in clinical manifestations and the lack of a unified approach to its treatment. Lumbar microdiscectomy remains the standard treatment for intervertebral disc herniations in the lumbar spine. The primary goal of surgical treatment for degenerative disorders is to achieve complete decompression of neural structures with minimal surgical trauma. The widespread adoption of advanced minimally invasive endoscopic techniques for intervertebral disc herniations has significantly reduced the number of disabled individuals worldwide, thus addressing the societal challenges associated with this condition. This literature review is dedicated to the development and evolution of endoscopic technologies, transitioning from open surgical interventions to percutaneous endoscopic procedures.
由于临床表现的多变性和缺乏统一的治疗方法,椎间盘源性神经压缩综合征的治疗问题在现代医学中仍具有重要意义。腰椎显微椎间盘切除术仍是治疗腰椎间盘突出症的标准方法。手术治疗退行性病变的主要目标是以最小的手术创伤实现神经结构的完全减压。先进的微创内窥镜技术在治疗椎间盘突出症方面的广泛应用,大大减少了全球残疾人数,从而解决了与该疾病相关的社会难题。这篇文献综述专门介绍内窥镜技术的发展和演变,从开放式手术干预过渡到经皮内窥镜手术。
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引用次数: 0
MYELOPROLIFERATIVE DISEASES AS A CAUSE OF ACUTE CEREBROVASCULAR ACCIDENT 骨髓增生性疾病是急性脑血管意外的病因之一
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.13
Y. Shatokhin, I. Snezhko, E. V. Ryabikina, E. V. Degtereva
Among the hemorheological forms of thrombophilia, chronic myeloproliferative diseases (CMPD) play an important role: polycythemia vera, essential thrombocythemia, primary myelofibrosis, the complications of which are arterial and venous thrombosis, including acute cerebrovascular accident (ACV). Identification of changes in the hemogram in such patients requires a thorough examination to exclude CMPD: monitoring of hemogram parameters in dynamics, hemostasiological examination, and determination of the level of erythropoietin, spleen size, and molecular-genetic study. Patients with chronic MPD and stroke need to start cytoreductive therapy (α-interferons, hydroxyurea) in a timely manner, conduct active long-term antithrombotic treatment (anticoagulants, antiplatelet agents) in order to prevent thromboembolism and improve the prognosis of the disease.
在血液流变学形式的血栓性疾病中,慢性骨髓增生性疾病(CMPD)起着重要作用:真性多血细胞增多症、原发性血小板增多症、原发性骨髓纤维化,其并发症是动脉和静脉血栓,包括急性脑血管意外(ACV)。要确定此类患者的血象变化,需要进行全面检查以排除骨髓增生性疾病:动态监测血象参数、止血检查、确定促红细胞生成素水平、脾脏大小和分子遗传学研究。慢性骨髓增生性疾病合并中风的患者需要及时开始细胞再生治疗(α-干扰素、羟基脲),进行积极的长期抗血栓治疗(抗凝药物、抗血小板药物),以预防血栓栓塞,改善疾病预后。
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引用次数: 0
NEUROFIBROMATOSIS TYPE 1. CLINICAL CASE 神经纤维瘤病 1 型。临床病例
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.08
R. Shiraliyeva, A. K. Mammadbayli, U. A. Asadova
The relevance of the topic covered in the article is related to the fact that doctors have little knowledge about this nosology. For this reason, a timely and incorrect diagnosis can result in wrong treatment, reduce the patient's quality of life and even lead to death. The diagnosis of the patient we investigated was determined according to the clinical criteria developed by the National Institute of Health of the United States. If genetic examination is not performed, the established diagnosis can be considered justified. It is imperative that several medical professionals (neurologist, traumatologist, ophthalmologist, surgeon, pediatrician, gynecologist, etc.) are aware of a disease like neurofibromatosis (NF). Basing on the clinical criteria developed by the US National Institutes of Health provides favorable conditions for diagnosing patients.
这篇文章所涉及的话题之所以具有现实意义,是因为医生们对这一病名知之甚少。因此,及时而错误的诊断可能导致错误的治疗,降低患者的生活质量,甚至导致死亡。我们调查的患者的诊断是根据美国国家卫生研究院制定的临床标准确定的。如果没有进行基因检查,可以认为既定诊断是合理的。当务之急是让多位医疗专业人士(神经科医生、创伤科医生、眼科医生、外科医生、儿科医生、妇科医生等)了解神经纤维瘤病(NF)这种疾病。美国国立卫生研究院制定的临床标准为诊断患者提供了有利条件。
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引用次数: 0
DYNAMIC MONITORING OF MULTIMODAL EVOKED POTENTIALS IN PATIENTS WITH MULTIPLE SCLEROSIS AND OPTIC NEURITIS AT ONSET 动态监测多发性硬化症和视神经炎患者发病时的多模态诱发电位
Pub Date : 2023-07-30 DOI: 10.61788/njn.v1i23.26
R. Shiraliyeva, N.A. Nasrullayeva
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引用次数: 0
期刊
NATIONAL JOURNAL OF NEUROLOGY
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