Zsolt Csaba Oláh, Dávid Nagy, Attila Sas, Benedek Oláh, Máté Czabajszki, Péter Tamáska, Béla Demeter
If severe cervical spinal cord injury or severe cervical vertebral fracture, subluxation or luxation is confirmed, 20-40% of the cases have vertebral artery dissection or occlusion. These can be asymptomatic, but can cause additional neurological damage in addition to cervical myelon and cervical nerve root symptoms. Vertebral artery dissection can be caused by direct injuries, stab wounds or gunshot wounds. Indirect vertebral artery dissection can occur at the same time as subluxation, luxation, or complex fractures of the cervical vertebra. CTA is the examination procedure of choice. In many cases, digital subtaction angiography examination and, if necessary, neurointerventional treatment must precede open neurosurgery. In our report, in the first patient, complete luxation of the C.VI vertebra caused unilateral vertebral artery 2-segment dissection-occlusion, while in our second patient, a stab injury caused direct vertebral artery compression and dissection. The occlusion of the vertebral artery did not cause neurological symptoms in any of the cases. In both of our cases, parent vessel occlusion was performed at the level of the vertebral artery injury before the neurosurgical operation.
{"title":"[Vertebral artery dissection during traumatic injury of the cervical spine, two case reports].","authors":"Zsolt Csaba Oláh, Dávid Nagy, Attila Sas, Benedek Oláh, Máté Czabajszki, Péter Tamáska, Béla Demeter","doi":"10.18071/isz.77.0283","DOIUrl":"https://doi.org/10.18071/isz.77.0283","url":null,"abstract":"<p><p><p>If severe cervical spinal cord injury or severe cervical vertebral fracture, subluxation or luxation is confirmed, 20-40% of the cases have vertebral artery dissection or occlusion. These can be asymptomatic, but can cause additional neurological damage in addition to cervical myelon and cervical nerve root symptoms. Vertebral artery dissection can be caused by direct injuries, stab wounds or gunshot wounds. Indirect vertebral artery dissection can occur at the same time as subluxation, luxation, or complex fractures of the cervical vertebra. CTA is the examination procedure of choice. In many cases, digital subtaction angiography examination and, if necessary, neurointerventional treatment must precede open neurosurgery. In our report, in the first patient, complete luxation of the C.VI vertebra caused unilateral vertebral artery 2-segment dissection-occlusion, while in our second patient, a stab injury caused direct vertebral artery compression and dissection. The occlusion of the vertebral artery did not cause neurological symptoms in any of the cases. In both of our cases, parent vessel occlusion was performed at the level of the vertebral artery injury before the neurosurgical operation.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 7-8","pages":"283-287"},"PeriodicalIF":0.9,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141857018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Multiple sclerosis (MS) is an autoimmune inflammatory disease of the central nervous system with demyelination and neurodegeneration. In addition to the inflammatory immune processes that characterise the onset of the disease with relapses, chronic inflammation is also present from the onset of the disease. The catabolic processes induced by chronic inflammation are responsible for the axonal degeneration that causes the progression of the disease. The activity of the disease is well defined, an important prognostic factor, and a determining factor in the indication of disease-modifying therapies. It is important to establish disease activity at the time of diagnosis and to monitor it continuously during patient care, both clinically and radiologically, as it is the basis for deciding on the current treatment. If detected on the basis of the professional guideline, it is necessary to start or switch to a highly effective therapy.
{"title":"[Theoretical approach and prognostic significance of high disease activity in multiple sclerosis].","authors":"Klotild Mátyás","doi":"10.18071/isz.77.0221","DOIUrl":"10.18071/isz.77.0221","url":null,"abstract":"<p><p><p>Multiple sclerosis (MS) is an autoimmune inflammatory disease of the central nervous system with demyelination and neurodegeneration. In addition to the inflammatory immune processes that characterise the onset of the disease with relapses, chronic inflammation is also present from the onset of the disease. The catabolic processes induced by chronic inflammation are responsible for the axonal degeneration that causes the progression of the disease. The activity of the disease is well defined, an important prognostic factor, and a determining factor in the indication of disease-modifying therapies. It is important to establish disease activity at the time of diagnosis and to monitor it continuously during patient care, both clinically and radiologically, as it is the basis for deciding on the current treatment. If detected on the basis of the professional guideline, it is necessary to start or switch to a highly effective therapy. </p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 7-8","pages":"221-225"},"PeriodicalIF":0.9,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141857017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Benedek Rónaszéki, Dániel Sandi, Ádám Ónodi, Nikolett Szabó, Lívia Dézsi, János Tajti, Klára Piukovics
Background - POEMS syndrome is a potentially well manageable disease with an ascendant therapeutic arsenal nowadays. The early recognition of the syndrome is key to prevent serious multiorgan damage, and that is still a big challenge for physicians. With the following two case reports the authors aimed to highlight the consequences of late recognition of the disease and summarize the potential therapeutic options for POEMS syndrome.
Results - We have presented two patients’ cases with a long history of examination and treatment because of uncleared polyneuropathy. Through these cases we could see how serious could be the consequences of late diagnosis and despite multiorgan impairment there are still therapeutic options which could improve the patient’s condition. Although the diagnosis of POEMS syndrome is not easy, it must raise our mind the thought and be prudent when we start a treatment in polyneuropathy.
{"title":"[A race against time: POEMS syndrome].","authors":"Benedek Rónaszéki, Dániel Sandi, Ádám Ónodi, Nikolett Szabó, Lívia Dézsi, János Tajti, Klára Piukovics","doi":"10.18071/isz.77.0212","DOIUrl":"10.18071/isz.77.0212","url":null,"abstract":"<p><p><p><strong>Background -</strong> POEMS syndrome is a potentially well manageable disease with an ascendant therapeutic arsenal nowadays. The early recognition of the syndrome is key to prevent serious multiorgan damage, and that is still a big challenge for physicians. With the following two case reports the authors aimed to highlight the consequences of late recognition of the disease and summarize the potential therapeutic options for POEMS syndrome.</p> <p><strong>Results - </strong>We have presented two patients’ cases with a long history of examination and treatment because of uncleared polyneuropathy. Through these cases we could see how serious could be the consequences of late diagnosis and despite multiorgan impairment there are still therapeutic options which could improve the patient’s condition. Although the diagnosis of POEMS syndrome is not easy, it must raise our mind the thought and be prudent when we start a treatment in polyneuropathy.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"212-216"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The aim of this study is to comprehensively determine the types of affected fibers in Parkinson’s disease (PD) patients by employing nerve conduction studies (NCS), sympathetic skin response (SSR) examinations, and current perception threshold (CPT) testing and to analyze the correlation between levodopa use and nerve involvement.
.
Methods:
This retrospective study included 36 clinically diagnosed PD patients who were recruited between January 2018 and April 2019. All patients underwent NCS, SSR testing, and CPT sensory examinations. Additionally, the PD patients were assessed for disease staging using the Hoehn and Yahr (H-Y) scale.
.
Results:
Fifteen patients were included in the tremor-dominant subtype, ten patients in the rigid-dominant subtype, and eleven patients in the mixed subtype. Eleven patients were using levodopa, while twenty-five patients had never used any anti-Parkinson’s medication. Ten patients (28%) showed abnormal sympathetic skin responses (SSR). The CPT examination revealed sensory abnormalities in twenty-four patients (67%), with eighteen patients (75%) experiencing sensory hypersensitivity and six patients (25%) experiencing sensory hypoesthesia. Twelve patients (33%) had normal CPT results. Among the patients with abnormal CPT findings, seven cases (29%) involved large myelinated fiber damage, twenty-two cases (92%) involved small myelinated fiber damage, and nineteen cases (79%) involved unmyelinated fiber damage. The rate of sensory abnormalities was 64% (7/11) in the levodopa group and 68% (17/25) in the non-levodopa group, with no statistically significant difference between the two groups.
.
Conclusion:
The incidence of abnormal CPT findings in PD patients was higher than that of abnormal SSR responses, suggesting that nerve fiber damage primarily affects small fiber nerves (SFN).
{"title":"Study on the relationship between peripheral nerve fiber types and levodopa usage in Parkinson's disease.","authors":"Liu Nan, Zhang Jie","doi":"10.18071/isz.77.0161","DOIUrl":"https://doi.org/10.18071/isz.77.0161","url":null,"abstract":"<p><strong>Background and purpose: </strong><p>The aim of this study is to comprehensively determine the types of affected fibers in Parkinson’s disease (PD) patients by employing nerve conduction studies (NCS), sympathetic skin response (SSR) examinations, and current perception threshold (CPT) testing and to analyze the correlation between levodopa use and nerve involvement.</p>.</p><p><strong>Methods: </strong><p>This retrospective study included 36 clinically diagnosed PD patients who were recruited between January 2018 and April 2019. All patients underwent NCS, SSR testing, and CPT sensory examinations. Additionally, the PD patients were assessed for disease staging using the Hoehn and Yahr (H-Y) scale. </p>.</p><p><strong>Results: </strong><p>Fifteen patients were included in the tremor-dominant subtype, ten patients in the rigid-dominant subtype, and eleven patients in the mixed subtype. Eleven patients were using levodopa, while twenty-five patients had never used any anti-Parkinson’s medication. Ten patients (28%) showed abnormal sympathetic skin responses (SSR). The CPT examination revealed sensory abnormalities in twenty-four patients (67%), with eighteen patients (75%) experiencing sensory hypersensitivity and six patients (25%) experiencing sensory hypoesthesia. Twelve patients (33%) had normal CPT results. Among the patients with abnormal CPT findings, seven cases (29%) involved large myelinated fiber damage, twenty-two cases (92%) involved small myelinated fiber damage, and nineteen cases (79%) involved unmyelinated fiber damage. The rate of sensory abnormalities was 64% (7/11) in the levodopa group and 68% (17/25) in the non-levodopa group, with no statistically significant difference between the two groups. </p>.</p><p><strong>Conclusion: </strong><p>The incidence of abnormal CPT findings in PD patients was higher than that of abnormal SSR responses, suggesting that nerve fiber damage primarily affects small fiber nerves (SFN).</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"161-166"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sonkaya Riza, Yaşa Ertuğrul Mustafa, Korkmaz Buse, Kuz Betül, Sonkaya Zeliha Zeynep, Öztürk Bilgin, Karadaş Ömer
Background and purpose:
Parkinson’s disease (PD) is a heterogeneous neurodegenerative disorder characterized by contradictory clinical outcomes among its several subtypes. The disease can manifest with a tremor-dominant (TD) or a non-tremor-dominant (NTD) phenotype. Although the TD subtype may show a better prognosis, there is limited information on the phenotypic differences regarding the level of axial symptoms. For this reason, in this study it was aimed to make a quantitative comparison of axial posture and spinal mobility between PD with TD and NTD.
.
Methods:
This case-control study was conducted on 94 patients with diagnosed PD. A group diagnosis approach was used in the study, such that the diagnosis of each patient was confirmed, and they were assig-ned to TD and NTD groups by a neurologist expert on movement disorders. Of the patients with PD, 61 were in the TD group, and 33 were in the NTD group. Spinal mouse was used to measure spinal posture and spinal mobility in both sagittal and frontal planes.
.
Results:
Two groups of 61 patients (25 male + 36 female) with TD-PD (mean age: 64.49±10.37 years) and 33 patients (20 male +13 female) with NTD-PD (mean age: 63.45±9.11 years) were enrolled in the study. There were no significant differences between the patients with TD and NTD in terms of sagittal and frontal postures (p>0.05). In addition to this, anterior trunk tilt was found to significantly increase as the disease stage advanced in both groups. While the greatest anterior trunk tilt change in the TD-PD group was observed in the 3rd stage, NTD-PD group was in the 2.5th stage. Aside from this, the outcomes of the spinal mobility measurements in the frontal and sagittal planes were similar between the groups (p>0.05).
.
Conclusion:
It is widely acknowledged that many clinical aspects of the TD and NTD forms of PD differ; however, in our study, it was observed that there may be no difference in the axial symptoms of the patients with PD in terms of classification according to tremor dominance.
{"title":"A comparison of quantitative parameters of axial posture and spinal mobility between motor subtypes of Parkinson's disease.","authors":"Sonkaya Riza, Yaşa Ertuğrul Mustafa, Korkmaz Buse, Kuz Betül, Sonkaya Zeliha Zeynep, Öztürk Bilgin, Karadaş Ömer","doi":"10.18071/isz.77.0187","DOIUrl":"https://doi.org/10.18071/isz.77.0187","url":null,"abstract":"<p><strong>Background and purpose: </strong><p>Parkinson’s disease (PD) is a heterogeneous neurodegenerative disorder characterized by contradictory clinical outcomes among its several subtypes. The disease can manifest with a tremor-dominant (TD) or a non-tremor-dominant (NTD) phenotype. Although the TD subtype may show a better prognosis, there is limited information on the phenotypic differences regarding the level of axial symptoms. For this reason, in this study it was aimed to make a quantitative comparison of axial posture and spinal mobility between PD with TD and NTD. </p>.</p><p><strong>Methods: </strong><p>This case-control study was conducted on 94 patients with diagnosed PD. A group diagnosis approach was used in the study, such that the diagnosis of each patient was confirmed, and they were assig-ned to TD and NTD groups by a neurologist expert on movement disorders. Of the patients with PD, 61 were in the TD group, and 33 were in the NTD group. Spinal mouse was used to measure spinal posture and spinal mobility in both sagittal and frontal planes. </p>.</p><p><strong>Results: </strong><p>Two groups of 61 patients (25 male + 36 female) with TD-PD (mean age: 64.49±10.37 years) and 33 patients (20 male +13 female) with NTD-PD (mean age: 63.45±9.11 years) were enrolled in the study. There were no significant differences bet­ween the patients with TD and NTD in terms of sagittal and frontal postures (p>0.05). In addition to this, anterior trunk tilt was found to significantly increase as the disease stage advanced in both groups. While the greatest anterior trunk tilt change in the TD-PD group was observed in the 3rd stage, NTD-PD group was in the 2.5th stage. Aside from this, the out­comes of the spinal mobility measurements in the frontal and sagittal planes were similar between the groups (p>0.05).</p>.</p><p><strong>Conclusion: </strong><p>It is widely acknowledged that many clinical aspects of the TD and NTD forms of PD differ; however, in our study, it was observed that there may be no difference in the axial symptoms of the patients with PD in terms of classification according to tremor dominance.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"187-195"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fibrinogen to albumin ratio (FAR) is thought to have a predictive effect in diseases such as cancer and myocardial infarction. We aimed to elucidate the prognostic value of FAR in ischemic stroke patients who underwent mechanical thrombectomy.
.
Methods:
A total of 103 patients hospitalized for acute stroke who underwent mechanical thrombectomy within 6 hours of symptoms’ outset have been analyzed retrospectively. Stroke severity was interpreted via the National Institutes of Health Stroke Scale (NIHSS) score during the neurological examination. Recanalization success after mechanical thrombectomy was evaluated with the TICI score (Thrombolysis in Cerebral Infarction scale), and 2b – 3 patients were recorded as those with recanalization. The patients’ modified Rankin scale (mRS) at discharge and at the end of the third month were recorded.
.
Results:
Statistically significant differences were observed in age, admission blood glucose, glomerular filtration rate and FAR according to the mRS scores of the patients in the third month (p<0.05). Significant variables in the risk factor analysis were re-evaluated in the multivariate model. The best model was determined using the backward Wald method in the multivariate model, and it was determined that differences in age, admission blood glucose, and FAR were significant.
.
Conclusion:
FAR can be used as a novel, effective, economical, and practical biomarker in patient with acute ischemic stroke who underwent mechanical thrombectomy.
.
背景和目的:纤维蛋白原与白蛋白比值(FAR)被认为对癌症和心肌梗死等疾病具有预测作用。我们旨在阐明 FAR 在接受机械血栓切除术的缺血性脑卒中患者中的预后价值:方法:我们对103名因急性脑卒中住院并在症状开始后6小时内接受机械性血栓切除术的患者进行了回顾性分析。在进行神经系统检查时,通过美国国立卫生研究院卒中量表(NIHSS)评分来解释卒中严重程度。机械血栓切除术后的再通成功率用 TICI 评分(脑梗塞溶栓量表)进行评估,2b – 3 的患者被记录为再通患者。结果:根据第三个月患者的 mRS 评分,在年龄、入院血糖、肾小球滤过率和 FAR 方面观察到显著的统计学差异(p<0.05)。在多变量模型中重新评估了风险因素分析中的显著差异。在多变量模型中使用后向 Wald 法确定了最佳模型,并确定年龄、入院血糖和 FAR 的差异显著:结论:FAR可作为一种新颖、有效、经济、实用的生物标志物,用于急性缺血性脑卒中患者的机械性血栓切除术。
{"title":"Fibrinogen to albumin ratio's prognostic value in ischemic stroke patients who underwent mechanical thrombectomy.","authors":"Ozdogru Derya, Uysal Kadir Onur, Erdem Miray, Soker Banu Elif, Ozturk Ilker, Avci Akkan, Arlier Zulfikar","doi":"10.18071/isz.77.0167","DOIUrl":"10.18071/isz.77.0167","url":null,"abstract":"<p><strong>Background and purpose: </strong><p>Fibrinogen to albumin ratio (FAR) is thought to have a predictive effect in diseases such as cancer and myocardial infarction. We aimed to elucidate the prognostic value of FAR in ischemic stroke patients who underwent mechanical thrombectomy.</p>.</p><p><strong>Methods: </strong><p>A total of 103 patients hospita­lized for acute stroke who underwent me­cha­nical thrombectomy within 6 hours of symp­toms’ outset have been analyzed retro­spectively. Stroke severity was interpreted via the National Institutes of Health Stroke Scale (NIHSS) score during the neurological examination. Recanalization success after mechanical thrombectomy was evaluated with the TICI score (Thrombolysis in Cerebral Infarction scale), and 2b – 3 patients were recorded as those with recanalization. The patients’ modified Rankin scale (mRS) at discharge and at the end of the third month were recorded. </p>.</p><p><strong>Results: </strong><p> Statistically significant differen­ces were observed in age, admission blood glucose, glomerular filtration rate and FAR according to the mRS scores of the patients in the third month (p<0.05). Significant va­riab­les in the risk factor analysis were re-evaluated in the multivariate model. The best model was determined using the backward Wald method in the multivariate model, and it was determined that differences in age, admission blood glucose, and FAR were significant.</p>.</p><p><strong>Conclusion: </strong><p>FAR can be used as a novel, effective, economical, and practical biomarker in patient with acute ischemic stroke who underwent mechanical thrombectomy.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"167-176"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gabriella Sinkó, Márton Tompa, Zsuzsanna Kiss, Bernadette Kálmán
Background - Leukodystrophies, a heterogeneous group of brain and spinal cord disorders, often pose challenges in establishing molecular etiology. Vanishing White Matter Disease (VWMD) is a rare subtype of leukodystrophies presenting with characteristic clinical and MRI features, nevertheless, achieving diagnostic certainty requires genetic studies.
Case presentation - Our patient is a nine year old girl, who developed progressive gait difficulties at around 3-4 years of age. Her brain MRI showed confluent lesions with increased signal intensity in the cerebral and cerebellar white matter on T2/FLAIR sequences, within which hypointense regions appeared with signal intensity resembling that of the cerebrospinal fluid on T1 sequences. Whole exome sequencing identified a homozygous likely pathogenic variant within the EIF2B5 gene in the proband, which was present in a heterozygous state in both asymptomatic parents. Having the clinical and molecular genetic diagnosis established, we explored therapeutic possibilities for the patient.
Conclusion - VWMD is a severe form of leukodystrophies with little or no disease modifying therapy available until recently. A better understanding of its molecular pathogenesis offers some hope for new inventive therapies.
{"title":"[Vanishing white matter disease, a rare leukodystrophy with mutation in the EIF2B5 gene].","authors":"Gabriella Sinkó, Márton Tompa, Zsuzsanna Kiss, Bernadette Kálmán","doi":"10.18071/isz.77.0207","DOIUrl":"10.18071/isz.77.0207","url":null,"abstract":"<p><p><p style=\"text-align: justify;\"><strong>Background -</strong> Leukodystrophies, a hete­ro­­ge­neous group of brain and spinal cord dis­orders, often pose challenges in es­tab­li­shing molecular etiology. Vanishing White Matter Disease (VWMD) is a rare sub­type of leu­ko­dys­trophies presenting with characteristic clinical and MRI features, ne­ver­theless, achieving diag­nostic certainty requires genetic studies.</p> <p style=\"text-align: justify;\"><strong>Case presentation -</strong> Our patient is a nine year old girl, who developed progressive gait difficulties at around 3-4 years of age. Her brain MRI showed confluent lesions with in­­creased signal intensity in the cerebral and cerebellar white matter on T2/FLAIR se­quen­ces, within which hypointense regions ap­peared with signal intensity resembling that of the cerebrospinal fluid on T1 sequences. Whole exome sequencing identified a homozygous likely pathogenic variant within the EIF2B5 gene in the proband, which was present in a heterozygous state in both asymptomatic parents. Having the clinical and molecular genetic diagnosis established, we explored therapeutic possibilities for the patient.</p> <p style=\"text-align: justify;\"><strong>Conclusion - </strong>VWMD is a severe form of leukodystrophies with little or no disease modifying therapy available until recently. A better understanding of its molecular pathogenesis offers some hope for new inventive therapies. </p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"207-211"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Josef Michael Kupka, Patrick Thurner, Tilman Schubert, Nicolin Hainc, Menno Robbert Germans, Zsolt Kulcsár
Background and purpose:
Chronic subdural hematoma (cSDH) is a challenging pathology with high recurrence rate after surgical treatment and may seriously affect the patient’s quality of life. Membrane formation with angiogenesis plays an important role in the evolution of the disease, providing a promising target for endovascular therapy. Our goal is to categorize angiographic patterns of chronic subdural hematoma for standardized reporting purposes.
.
Methods:
In our retrospective analysis of prospective data collection, we analyzed angiographic properties of all high recurrence risk patients with cSDH, who were treated by embolization in our hospital between February 2019 and June 2020. Altogether 17 patients were included in the analysis.
.
Results:
Based on superselective angiography of the middle meningeal artery (MMA) in the two standard, AP and lateral views, three distinct categories of dural supply were defined: normal vascular pattern (Grade I), cottonwool appearance without enlargement of the MMA branches (Grad II) and strong cottonwool like staining with dilatative remodelling of the MMA branches (Grade III).
.
Conclusion:
The proposed grading system of the angiographic appearance of cSDH, representing the pathophysiological evolution of the disease should be correlated to therapeutic success rates and could be applied in future clinical studies.
{"title":"Angiographic classification of chronic subdural hematoma.","authors":"Josef Michael Kupka, Patrick Thurner, Tilman Schubert, Nicolin Hainc, Menno Robbert Germans, Zsolt Kulcsár","doi":"10.18071/isz.77.0196","DOIUrl":"https://doi.org/10.18071/isz.77.0196","url":null,"abstract":"<p><strong>Background and purpose: </strong><p>Chronic subdural hematoma (cSDH) is a challenging pathology with high recurrence rate after surgical treatment and may seriously affect the patient’s quality of life. Membrane formation with angiogenesis plays an important role in the evolution of the disease, providing a promising target for endovascular therapy. Our goal is to categorize angiographic patterns of chronic subdural hematoma for standardized reporting purposes.</p>.</p><p><strong>Methods: </strong><p>In our retrospective analysis of prospective data collection, we analyzed angiographic properties of all high recurrence risk patients with cSDH, who were treated by embolization in our hospital between February 2019 and June 2020. Altogether 17 patients were included in the analysis. </p>.</p><p><strong>Results: </strong><p>Based on superselective angiography of the middle meningeal artery (MMA) in the two standard, AP and lateral views, three distinct categories of dural supply were defined: normal vascular pattern (Grade I), cottonwool appearance without enlargement of the MMA branches (Grad II) and strong cottonwool like staining with dilatative remodelling of the MMA branches (Grade III).</p>.</p><p><strong>Conclusion: </strong><p>The proposed grading system of the angiographic appearance of cSDH, representing the pathophysiological evolution of the disease should be correlated to therapeutic success rates and could be applied in future clinical studies.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"196-200"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zsolt Csaba Oláh, Gyula János Papp, Attila Sas, Benedek Oláh, Máté Czabajszki, Béla Demeter
Chronic subdural hematoma is one of the most common diseases requiring a neurosurgical operation that affect elderly and fragile patients. In addition to standard neurosurgical operations (trepanation and craniotomy), embolization of the meningeal artery media is an alternative solution. Several review aerticles have confirmed the very high rate of success and safety of the endovascular treatment. We present the technical details and results of our 10 consecutive selective media meningeal artery embolization procedures for residual chronic subdural hematomas. Our interventions were performed without complications and all resulted in complete recovery.
{"title":"[Az arteria meningea media embolisatio szerepe a krónikus subduralis haematoma kezelési algoritmusában, legújabb evidenciák és saját tapasztalataink].","authors":"Zsolt Csaba Oláh, Gyula János Papp, Attila Sas, Benedek Oláh, Máté Czabajszki, Béla Demeter","doi":"10.18071/isz.77.0201","DOIUrl":"10.18071/isz.77.0201","url":null,"abstract":"<p><p><p>Chronic subdural hematoma is one of the most common diseases requiring a neurosurgical operation that affect elderly and fragile patients. In addition to standard neurosurgical operations (trepanation and craniotomy), embolization of the meningeal artery media is an alternative solution. Several review aerticles have confirmed the very high rate of success and safety of the endovascular treatment. We present the technical details and results of our 10 consecutive selective media meningeal artery embolization procedures for residual chronic subdural hematomas. Our interventions were performed without complications and all resulted in complete recovery. </p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"201-206"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Long Covid is a complex condition characterised by symptoms that persist for weeks and months after the Covid infection, accompanied by cognitive impairment that negatively affects daily life. Understanding this complex condition is important for the development of diagnostic and therapeutic strategies.
This article aims to provide a comprehensive overview of cognitive impairment in long-COVID, including its definition, symptoms, pathophysiology, risk factors, assessment tools, imaging abnormalities, potential biomarkers, management strategies, long-term outcomes, and future directions for research.
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Methods:
The search methodology used in this review aimed to include a wide range of research on cognitive impairment related to both COVID-19 and long-COVID. Systematic searches of PubMed and Google Scholar databases were conducted using a mixture of MeSH terms and keywords including ‘cognition’, ‘cognitive impairment’, ‘brain fog’, ‘COVID-19’ and ‘long-COVID’. The search was restricted to studies published in English between 1 January 2019 and 11 February 2024, which presented findings on neurological manifestations in human participants.
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Results:
Long-COVID is characterized by persistent symptoms following COVID-19 infection, with cognitive impairment being a prominent feature. Symptoms include brain fog, difficulties with concentration, memory issues, and executive function deficits. Pathophysiological mechanisms involve viral persistence, immune responses, and vascular damage. Risk factors include age, pre-existing conditions, and disease severity. Cognitive assessment tools such as the Montreal Cognitive Assessment (MoCA) are essential for diagnosis. Imaging studies, including MRI, PET, and SPECT, reveal structural and functional brain alterations. Potential biomarkers include C-reactive protein, interleukin-6, and neuron-specific enolase. Management strategies encompass cognitive rehabilitation, occupational therapy, medications, and lifestyle modifications.
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Conclusion:
Long-COVID poses a multifaceted challenge, and cognitive impairment significantly impacts patients’ lives. A multidisciplinary approach, including cognitive rehabilitation and medication when appropriate, is essential for effective management. Future research should focus on validating biomarkers and understanding long-term cognitive outcomes.
Conclusion – Long-COVID is a global health concern, and cognitive impairment is a distressing symptom. While pharmacological interventions have potential, they require careful consideration. Continued research is crucial for improving the understanding and treatment of cognitive impairment in long-COVID.
{"title":"Cognitive impairment in long-COVID.","authors":"Tozkir Julide, Turkmen Cigdem, Topcular Baris","doi":"10.18071/isz.77.0151","DOIUrl":"https://doi.org/10.18071/isz.77.0151","url":null,"abstract":"<p><strong>Background and purpose: </strong><p>Long Covid is a complex con­dition characterised by symptoms that per­sist for weeks and months after the Co­vid infection, accompanied by cognitive im­pairment that negatively affects daily life. Understanding this complex condition is im­portant for the development of diagnostic and therapeutic strategies.</p> <p>This article aims to provide a comprehensive overview of cognitive impairment in long-COVID, including its definition, symptoms, pathophysiology, risk factors, assessment tools, imaging abnormalities, potential biomarkers, management strategies, long-term outcomes, and future directions for research.<br><br><br><br><br></p>.</p><p><strong>Methods: </strong><p>The search methodology used in this review aimed to include a wide range of research on cognitive impairment related to both COVID-19 and long-COVID. Systematic searches of PubMed and Google Scholar databases were conducted using a mixture of MeSH terms and keywords including ‘cognition’, ‘cognitive impairment’, ‘brain fog’, ‘COVID-19’ and ‘long-COVID’. The search was restricted to studies published in English between 1 January 2019 and 11 February 2024, which presented findings on neurological manifestations in human participants.</p>.</p><p><strong>Results: </strong><p>Long-COVID is characterized by persistent symptoms following COVID-19 infection, with cognitive impairment being a prominent feature. Symptoms include brain fog, difficulties with concentration, memory issues, and executive function deficits. Pa­tho­physiological mechanisms involve vi­ral persistence, immune responses, and vas­cular damage. Risk factors include age, pre-existing conditions, and disease seve­rity. Cognitive assessment tools such as the Montreal Cognitive Assessment (MoCA) are essential for diagnosis. Imaging studies, including MRI, PET, and SPECT, reveal structural and functional brain alterations. Potential biomarkers include C-reactive protein, interleukin-6, and neuron-specific enolase. Management strategies encompass cognitive rehabilitation, occupational therapy, medications, and lifestyle modifications.</p>.</p><p><strong>Conclusion: </strong><p>Long-COVID poses a multifaceted challenge, and cognitive impairment significantly impacts patients’ lives. A multi­disciplinary approach, including cognitive rehabilitation and medication when appropriate, is essential for effective management. Future research should focus on validating biomarkers and understanding long-term cognitive outcomes.</p> <p>Conclusion – Long-COVID is a global health concern, and cognitive impairment is a distressing symptom. While pharmacological interventions have potential, they require careful consideration. Continued research is crucial for improving the understanding and treatment of cognitive impairment in long-COVID.</p>.</p>","PeriodicalId":50394,"journal":{"name":"Ideggyogyaszati Szemle-Clinical Neuroscience","volume":"77 5-6","pages":"151-159"},"PeriodicalIF":0.8,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}