E. O. Chekanova, Аlla А. Shabalina, T. Simaniv, R. N. Konovalov, L. A. Dobrynina, Lyudmila A. Kalashnikova, M. Gubanova, M. N. Zakharova
Introduction. Glial fibrillary acidic protein (GFAP) is the main component of intermediate astrocyte filaments. In 2016, anti-GFAP antibodies (Ab) were identified as the specific biomarker for the first established CNS inflammatory disorder subsequently called autoimmune astrocytopathy associated with anti-GFAP Ab (A-GFAP-A). Since GFAP is localized intracellularly, GFAP Ab do not appear to be directly pathogenic though serve as a biomarker of immune inflammation. Although presence of GFAP-Ab in the serum (but not in the CSF) could be observed in various CNS immune-mediated diseases, detection of GFAP-Ab in CSF is only characteristic for A-GFAP-A. A-GFAP-A usually develops after the age of 40 and mostly manifests acutely or subacutely with symptoms of meningoencephalomyelitis or its focal forms. Linear perivascular radial cerebral white matter enhancement is a specific MRI finding of A-GFAP-A. Concomitant neoplasms or autoimmune disorders, as well as co-expression of other antineuronal antibodies are not uncommon in A-GFAP-A. Usually, disease responds well to immunotherapy, and prolonged remission could be achieved, however recurrent disease course and fulminant cases are also described in the literature. In these cases, long-term immunosuppression is required. Data on epidemiology, etiological factors, and precise pathogenesis of A-GFAP-A are still limited. Due to the lack of long-term follow-up data, diagnostic criteria, generally accepted treatment strategies or prognostic risk factors for relapse and outcome of the disease have not yet been established and precised. We present the first description of a case of relapsing A-GFAP-A in Russia and an analysis of the current data on the pathogenesis, clinical features, as well as the diagnostic challenges and treatment approaches for A-GFAP-A.
{"title":"Relapsing Autoimmune GFAP Astrocytopathy: Case Report","authors":"E. O. Chekanova, Аlla А. Shabalina, T. Simaniv, R. N. Konovalov, L. A. Dobrynina, Lyudmila A. Kalashnikova, M. Gubanova, M. N. Zakharova","doi":"10.54101/acen.2023.4.11","DOIUrl":"https://doi.org/10.54101/acen.2023.4.11","url":null,"abstract":"Introduction. Glial fibrillary acidic protein (GFAP) is the main component of intermediate astrocyte filaments. In 2016, anti-GFAP antibodies (Ab) were identified as the specific biomarker for the first established CNS inflammatory disorder subsequently called autoimmune astrocytopathy associated with anti-GFAP Ab (A-GFAP-A). Since GFAP is localized intracellularly, GFAP Ab do not appear to be directly pathogenic though serve as a biomarker of immune inflammation. Although presence of GFAP-Ab in the serum (but not in the CSF) could be observed in various CNS immune-mediated diseases, detection of GFAP-Ab in CSF is only characteristic for A-GFAP-A. A-GFAP-A usually develops after the age of 40 and mostly manifests acutely or subacutely with symptoms of meningoencephalomyelitis or its focal forms. Linear perivascular radial cerebral white matter enhancement is a specific MRI finding of A-GFAP-A. Concomitant neoplasms or autoimmune disorders, as well as co-expression of other antineuronal antibodies are not uncommon in A-GFAP-A. Usually, disease responds well to immunotherapy, and prolonged remission could be achieved, however recurrent disease course and fulminant cases are also described in the literature. In these cases, long-term immunosuppression is required. Data on epidemiology, etiological factors, and precise pathogenesis of A-GFAP-A are still limited. Due to the lack of long-term follow-up data, diagnostic criteria, generally accepted treatment strategies or prognostic risk factors for relapse and outcome of the disease have not yet been established and precised. We present the first description of a case of relapsing A-GFAP-A in Russia and an analysis of the current data on the pathogenesis, clinical features, as well as the diagnostic challenges and treatment approaches for A-GFAP-A.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139383263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
R. M. Galimova, S. N. Illarioshkin, I. Buzaev, Yulia A. Sidorova, D. Krekotin, Sh.M. Safin, D. I. Nabiullina, G. Akhmadeeva, Dinara R. Teregulova
Introduction. MRI-guided focused ultrasound (MRgFUS) is approved for management of various movement disorders, primarily essential tremor and Parkinson’s disease (PD), with favorable long-term outcomes in numerous patients worldwide. However, few case studies describe the use of this modality for symptomatic treatment of dystonias that, as the third most common movement disorder, may be rather disabling. Objective: To improve outcomes in patients with cervical dystonia (CD) using MRgFUS. Materials and methods. We retrospectively analyzed 13 cases of various CD types managed with MRgFUS in single or multiple sessions. The mean age of the patients was 42 [39; 53] years. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to assess patients' statuses and severity of CD symptoms during therapy and the last available observation period. The targets included the pallidothalamic tract and the thalamic ventral oralis complex nucleus or their combination. Results. The mean follow-up period was 13.3 ± 3.4 months (July 2021 to April 2023). The mean CD severity sum score (TWSTRS score) was 22 [16; 25] before MRgFUS and 6 [4; 9] in the last observation. Therefore, we report 70.6% [55.6; 76.5] improvement (paired samples t-test p = 0.0025). Conclusion. Available data evidence that MRgFUS is efficient and sufficiently safe for symptomatic treatment in pharmacoresistant CD patients. A number of vital aspects of MRgFUS have to be specified in larger CD cohorts in the long-term follow-up.
导言。核磁共振成像引导聚焦超声（MRgFUS）已被批准用于治疗各种运动障碍，主要是本质性震颤和帕金森病（PD），在全球众多患者中取得了良好的长期疗效。然而，很少有病例研究描述这种方法用于肌张力障碍的对症治疗，而肌张力障碍是第三大常见运动障碍，可能会造成严重的致残。目标：改善颈椎病患者的治疗效果使用 MRgFUS 改善颈肌张力障碍 (CD) 患者的治疗效果。材料和方法。我们对 13 例不同类型的 CD 患者进行了回顾性分析，这些患者均接受过 MRgFUS 单次或多次治疗。患者的平均年龄为 42 [39; 53] 岁。我们使用多伦多西方痉挛性皮肌痉挛评定量表（TWSTRS）来评估患者在治疗期间和最后一次观察期间的状况和 CD 症状的严重程度。研究对象包括苍白球丘脑束和丘脑腹侧口腔复合核或它们的组合。研究结果平均随访时间为（13.3 ± 3.4）个月（2021年7月至2023年4月）。MRgFUS 前，CD 严重性总分（TWSTRS 评分）平均为 22 [16; 25]，最后一次观察时为 6 [4; 9]。因此，我们报告的改善率为 70.6% [55.6; 76.5]（配对样本 t 检验 p = 0.0025）。结论现有数据证明，MRgFUS 对药物耐药性 CD 患者的对症治疗是有效且足够安全的。MRgFUS 的一些重要方面还需要在更大的 CD 群体中进行长期随访。
{"title":"MRI-Guided Focused Ultrasound in Cervical Dystonia","authors":"R. M. Galimova, S. N. Illarioshkin, I. Buzaev, Yulia A. Sidorova, D. Krekotin, Sh.M. Safin, D. I. Nabiullina, G. Akhmadeeva, Dinara R. Teregulova","doi":"10.54101/acen.2023.4.3","DOIUrl":"https://doi.org/10.54101/acen.2023.4.3","url":null,"abstract":"Introduction. MRI-guided focused ultrasound (MRgFUS) is approved for management of various movement disorders, primarily essential tremor and Parkinson’s disease (PD), with favorable long-term outcomes in numerous patients worldwide. However, few case studies describe the use of this modality for symptomatic treatment of dystonias that, as the third most common movement disorder, may be rather disabling. \u0000Objective: To improve outcomes in patients with cervical dystonia (CD) using MRgFUS. \u0000Materials and methods. We retrospectively analyzed 13 cases of various CD types managed with MRgFUS in single or multiple sessions. The mean age of the patients was 42 [39; 53] years. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to assess patients' statuses and severity of CD symptoms during therapy and the last available observation period. The targets included the pallidothalamic tract and the thalamic ventral oralis complex nucleus or their combination. \u0000Results. The mean follow-up period was 13.3 ± 3.4 months (July 2021 to April 2023). The mean CD severity sum score (TWSTRS score) was 22 [16; 25] before MRgFUS and 6 [4; 9] in the last observation. Therefore, we report 70.6% [55.6; 76.5] improvement (paired samples t-test p = 0.0025). \u0000Conclusion. Available data evidence that MRgFUS is efficient and sufficiently safe for symptomatic treatment in pharmacoresistant CD patients. A number of vital aspects of MRgFUS have to be specified in larger CD cohorts in the long-term follow-up.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139384036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
R. Lyukmanov, Mikhail R. Isaev, Olesya A. Mokienko, Pavel D. Bobrov, Ekaterina S. Ikonnikova, Anastasiia N. Cherkasova, N. Suponeva
Introduction. Non-invasive brain–computer interfaces (BCIs) enable feedback motor imagery [MI] training in neurological patients to support their motor rehabilitation. Nowadays, the use of BCIs based on functional near-infrared spectroscopy (fNIRS) for motor rehabilitation is yet to be investigated. Objective: To evaluate the potential fNIRS BCI use in hand MI training for comprehensive post-stroke rehabilitation. Materials and methods. This pilot study included clinically stable patients with mild-to-moderate post-stroke hand paresis. In addition to the standard rehabilitation, the patients underwent 10 nine-minute MI fNIRS BCI training sessions. To evaluate the quality of fNIRS BCI control, we assessed the percentage of time during which the classifier accurately detected patient's mental state. We scored the hand function using the Action Research Arm Test (ARAT) and the Fugl-Meyer Assessment (FMA). Results. The study included 5 patients at 1 day to 12 months of stroke. All the participants completed the study. All study participants achieved BCI control rates higher than random (41–68%). While three patients demonstrated the clinically significant improvements in their ARAT scores, one of them also showed an improvement in the FMA score. All the participants reported experiencing drowsiness during training. Conclusions. Post-stroke patients can operate the fNIRS BCI system under investigation. We suggest adjusting the feedback system, extending the duration of training, and incorporating functional electromyostimulation to enhance training effectiveness.
{"title":"Brain–Computer Interface Using Functional Near-Infrared Spectroscopy for Post-Stroke Motor Rehabilitation: Case Series","authors":"R. Lyukmanov, Mikhail R. Isaev, Olesya A. Mokienko, Pavel D. Bobrov, Ekaterina S. Ikonnikova, Anastasiia N. Cherkasova, N. Suponeva","doi":"10.54101/acen.2023.4.10","DOIUrl":"https://doi.org/10.54101/acen.2023.4.10","url":null,"abstract":"Introduction. Non-invasive brain–computer interfaces (BCIs) enable feedback motor imagery [MI] training in neurological patients to support their motor rehabilitation. Nowadays, the use of BCIs based on functional near-infrared spectroscopy (fNIRS) for motor rehabilitation is yet to be investigated. \u0000Objective: To evaluate the potential fNIRS BCI use in hand MI training for comprehensive post-stroke rehabilitation. \u0000Materials and methods. This pilot study included clinically stable patients with mild-to-moderate post-stroke hand paresis. In addition to the standard rehabilitation, the patients underwent 10 nine-minute MI fNIRS BCI training sessions. To evaluate the quality of fNIRS BCI control, we assessed the percentage of time during which the classifier accurately detected patient's mental state. We scored the hand function using the Action Research Arm Test (ARAT) and the Fugl-Meyer Assessment (FMA). \u0000Results. The study included 5 patients at 1 day to 12 months of stroke. All the participants completed the study. All study participants achieved BCI control rates higher than random (41–68%). While three patients demonstrated the clinically significant improvements in their ARAT scores, one of them also showed an improvement in the FMA score. All the participants reported experiencing drowsiness during training. \u0000Conclusions. Post-stroke patients can operate the fNIRS BCI system under investigation. We suggest adjusting the feedback system, extending the duration of training, and incorporating functional electromyostimulation to enhance training effectiveness.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139383674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sergey V. Kotov, Maria M. Shcherbakova, Valentina A. Zenina, Elena V. Isakova, Alexey S. Kotov
Introduction. Various speech disorders that lead to impaired communication occur in 3050% of ischemic stroke (IS) survivors. Although most attention is traditionally paid to aphasia, speech disorders also include the following: dysarthria, dysphonia (isolated or in combination with dysarthria and/or dysphagia), fluency disorders, and non-specific speech disorders associated with the severity of condition and a cognitive disorder.
Objective: to study the variety of speech disorders and their features in patients with acute IS.
Materials and methods. We examined 69 right-handed patients with mild-to-moderate acute IS and NIHSS score of 412. The patients were enrolled in the study on days 17 of the IS.
Results. We found aphasia in 27/69 patients (39.1%), dysarthria in 21/69 patients (30.4%), dysphonia (isolated or in combination with dysarthria) in 17/69 patients (24.6%), fluency disorders in 19/69 patients (27.5%; 2 patients with tachylalia and 17 patients with bradylalia). In addition, 30 patients (43.5%) had dysphagia (isolated or in combination with dysarthria). At the initial examination, patients admitted within the 17 days of the acute IS onset presented with global or severe sensory and motor aphasia. At the same time, we discovered a pronounced positive dynamics in speech recovery thanks to speech therapy sessions. A significant remission in a speech disorder component led to the development of cortical aphasia affecting either anterior or posterior language areas at the end of the most acute IS period, while aphasia severity reduced to mild or moderate.
Conclusions. A fast reduction in aphasic disorders due to the speech therapy sessions suggests that the focal and connectional diaschisis are the basis for the severe speech disorders.
{"title":"Features of speech disorders in patients with acute ischemic stroke","authors":"Sergey V. Kotov, Maria M. Shcherbakova, Valentina A. Zenina, Elena V. Isakova, Alexey S. Kotov","doi":"10.54101/acen.2023.3.2","DOIUrl":"https://doi.org/10.54101/acen.2023.3.2","url":null,"abstract":"Introduction. Various speech disorders that lead to impaired communication occur in 3050% of ischemic stroke (IS) survivors. Although most attention is traditionally paid to aphasia, speech disorders also include the following: dysarthria, dysphonia (isolated or in combination with dysarthria and/or dysphagia), fluency disorders, and non-specific speech disorders associated with the severity of condition and a cognitive disorder.
 Objective: to study the variety of speech disorders and their features in patients with acute IS.
 Materials and methods. We examined 69 right-handed patients with mild-to-moderate acute IS and NIHSS score of 412. The patients were enrolled in the study on days 17 of the IS.
 Results. We found aphasia in 27/69 patients (39.1%), dysarthria in 21/69 patients (30.4%), dysphonia (isolated or in combination with dysarthria) in 17/69 patients (24.6%), fluency disorders in 19/69 patients (27.5%; 2 patients with tachylalia and 17 patients with bradylalia). In addition, 30 patients (43.5%) had dysphagia (isolated or in combination with dysarthria). At the initial examination, patients admitted within the 17 days of the acute IS onset presented with global or severe sensory and motor aphasia. At the same time, we discovered a pronounced positive dynamics in speech recovery thanks to speech therapy sessions. A significant remission in a speech disorder component led to the development of cortical aphasia affecting either anterior or posterior language areas at the end of the most acute IS period, while aphasia severity reduced to mild or moderate.
 Conclusions. A fast reduction in aphasic disorders due to the speech therapy sessions suggests that the focal and connectional diaschisis are the basis for the severe speech disorders.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135193678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Anastasia A. Salienko, Vladimir E. Syutkin, Maria V. Lisitskaya, Denis V. Kuznetsov, Murad S. Novruzbekov
In solid organ recipients, post-transplant neurotoxicity of calcineurin inhibitors (CIs) can be manifested by brain and spinal cord demyelination with multiple sclerosis (MS)-like symptoms.
Here are presented two case reports of neurological MS-like symptoms in the long-term post-liver transplant period with different underlying causes.
CI neurotoxicity may resemble various neurological diseases, including MS. At the same time, liver transplant recipients can develop true MS regardless of the immunosuppressant use. In liver transplant recipients, adequate differential diagnosis of neurological complications avoids unnecessary medications and reverses severe neurological deficits by immunosuppressant conversion.
{"title":"Demyelinating CNS processes in late post-liver transplant period","authors":"Anastasia A. Salienko, Vladimir E. Syutkin, Maria V. Lisitskaya, Denis V. Kuznetsov, Murad S. Novruzbekov","doi":"10.54101/acen.2023.3.10","DOIUrl":"https://doi.org/10.54101/acen.2023.3.10","url":null,"abstract":"In solid organ recipients, post-transplant neurotoxicity of calcineurin inhibitors (CIs) can be manifested by brain and spinal cord demyelination with multiple sclerosis (MS)-like symptoms.
 Here are presented two case reports of neurological MS-like symptoms in the long-term post-liver transplant period with different underlying causes.
 CI neurotoxicity may resemble various neurological diseases, including MS. At the same time, liver transplant recipients can develop true MS regardless of the immunosuppressant use. In liver transplant recipients, adequate differential diagnosis of neurological complications avoids unnecessary medications and reverses severe neurological deficits by immunosuppressant conversion.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135193675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daria A. Grishina, Natalia A. Suponeva, Alina S. Arestova, Evgenia A. Melnik, Taisiya A. Tumilovich, Victoria V. Sinkova, Mikhail A. Piradov
Introduction. Despite the improving diagnostic criteria for the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), its verification is still an issue.
Objective: to study the rate and the causes of CIDP misdiagnosis.
Materials and methods. We prospectively and retrospectively analyzed the clinical and paraclinical data of 223 patients admitted to the Research Center of Neurology from 2018 to 2022 with a CIDP referral.
Results. We revised the CIDP diagnosis in 150/223 patients (67%; median age 55.5 [43; 63] years; 75 males and 75 females; 3-year follow-up history [1.75; 5.25].) Once the definitive diagnosis was clarified, we divided the patients into the following groups: polyneuropathy of other etiology (n = 94; 63%), other neuromuscular disorders (n = 39; 27%), CNS disorders (n = 10; 7%), no structural NS disease (n = 7; 5%). Patients did not meet the 2021 EAN/PNS diagnostic criteria at the history-taking stage in 65% of cases, at the neurological examination stage in 39% of cases, and at the electroneuromyography stage in 92% of cases.
Conclusions. The rate of CIDP misdiagnosis in Russia is 67%, and most often this refers to patients with polyneuropathy of other etiologies. The main cause for the CIDP misdiagnosis was inaccurate electroneuromyography. We should bear in mind that CIDP is a rare disorder with an extensive differential diagnosis, so it should be verified according to the current 2021 EAN/PNS diagnostic criteria.
{"title":"Chronic inflammatory demyelinating polyradiculoneuropathy overdiagnosis in Russia","authors":"Daria A. Grishina, Natalia A. Suponeva, Alina S. Arestova, Evgenia A. Melnik, Taisiya A. Tumilovich, Victoria V. Sinkova, Mikhail A. Piradov","doi":"10.54101/acen.2023.3.1","DOIUrl":"https://doi.org/10.54101/acen.2023.3.1","url":null,"abstract":"Introduction. Despite the improving diagnostic criteria for the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), its verification is still an issue.
 Objective: to study the rate and the causes of CIDP misdiagnosis.
 Materials and methods. We prospectively and retrospectively analyzed the clinical and paraclinical data of 223 patients admitted to the Research Center of Neurology from 2018 to 2022 with a CIDP referral.
 Results. We revised the CIDP diagnosis in 150/223 patients (67%; median age 55.5 [43; 63] years; 75 males and 75 females; 3-year follow-up history [1.75; 5.25].) Once the definitive diagnosis was clarified, we divided the patients into the following groups: polyneuropathy of other etiology (n = 94; 63%), other neuromuscular disorders (n = 39; 27%), CNS disorders (n = 10; 7%), no structural NS disease (n = 7; 5%). Patients did not meet the 2021 EAN/PNS diagnostic criteria at the history-taking stage in 65% of cases, at the neurological examination stage in 39% of cases, and at the electroneuromyography stage in 92% of cases.
 Conclusions. The rate of CIDP misdiagnosis in Russia is 67%, and most often this refers to patients with polyneuropathy of other etiologies. The main cause for the CIDP misdiagnosis was inaccurate electroneuromyography. We should bear in mind that CIDP is a rare disorder with an extensive differential diagnosis, so it should be verified according to the current 2021 EAN/PNS diagnostic criteria.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135194575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ekaterina G. Seliverstova, Mikhail V. Sinkin, Anton Y. Kordonsky, Andrey A. Grin
Introduction. Electromyography (EMG) is an important diagnostic tool for the evaluation of radiculopathy. Since 1990s a paraspinal mapping technique is used, which detects spontaneous activity in paraspinal muscles (PM) at the level of several vertebral segments. This modality seems to be highly conclusive for diagnosing radicular lesions. The main limitation of this method is spontaneous activity dependence on the disease duration.
The aim of the study is to assess if PM EMG with motor unit potential (MUP) analysis is conclusive for diagnosing lumbar radiculopathy.
Materials and methods. The study examined 58 patients (26 men and 32 women) aged 2673 years with MRI-confirmed symptomatic L5 mono-radiculopathy due to L4L5 herniated discs. The study assessed the neurological status and needle EMG of m. tensor fasciae latae (TFL) and PM at L4L5 and L3L4 levels on both symptomatic and healthy sides immediately before radicular microscopic decompression surgery. Surgery outcomes were evaluated by early and late postoperative questioning.
Results. In PMs of the affected level and side, the average MUP duration was significantly different from opposite MUPs at the higher segment (р 0.001). At 3-month disease duration, a neurogenic pattern was significantly more frequent in affected PMs (p = 0.031) with neurogenic PM MUP rearrangement in 73.3% of patients. In the TFL (L5), neurogenic changes were reported only in 47.4% of patients. When compared to normal values, significant differences were found in the average duration of TFL MUPs (р = 0.001) and PM MUPs of the affected level and side (р 0.001) both in patients with motor disorders and those with isolated pain syndrome or sensory disorders.
Conclusions. For diagnosing radiculopathy, the sensitivity of needle PM EMG is 82.6% (48/58; 95% CI 70.691.4%). Compared to limb myotome assessment, the highest informative value of PM EMG was reported in patients with the disease duration for up to 3 months. PM EMG was conclusive for diagnosing radicular lesions in patients with isolated pain syndrome or sensory disorders.
{"title":"Value of paraspinal muscle myography in diagnosing L5 radiculopathy","authors":"Ekaterina G. Seliverstova, Mikhail V. Sinkin, Anton Y. Kordonsky, Andrey A. Grin","doi":"10.54101/acen.2023.3.8","DOIUrl":"https://doi.org/10.54101/acen.2023.3.8","url":null,"abstract":"Introduction. Electromyography (EMG) is an important diagnostic tool for the evaluation of radiculopathy. Since 1990s a paraspinal mapping technique is used, which detects spontaneous activity in paraspinal muscles (PM) at the level of several vertebral segments. This modality seems to be highly conclusive for diagnosing radicular lesions. The main limitation of this method is spontaneous activity dependence on the disease duration.
 The aim of the study is to assess if PM EMG with motor unit potential (MUP) analysis is conclusive for diagnosing lumbar radiculopathy.
 Materials and methods. The study examined 58 patients (26 men and 32 women) aged 2673 years with MRI-confirmed symptomatic L5 mono-radiculopathy due to L4L5 herniated discs. The study assessed the neurological status and needle EMG of m. tensor fasciae latae (TFL) and PM at L4L5 and L3L4 levels on both symptomatic and healthy sides immediately before radicular microscopic decompression surgery. Surgery outcomes were evaluated by early and late postoperative questioning.
 Results. In PMs of the affected level and side, the average MUP duration was significantly different from opposite MUPs at the higher segment (р 0.001). At 3-month disease duration, a neurogenic pattern was significantly more frequent in affected PMs (p = 0.031) with neurogenic PM MUP rearrangement in 73.3% of patients. In the TFL (L5), neurogenic changes were reported only in 47.4% of patients. When compared to normal values, significant differences were found in the average duration of TFL MUPs (р = 0.001) and PM MUPs of the affected level and side (р 0.001) both in patients with motor disorders and those with isolated pain syndrome or sensory disorders.
 Conclusions. For diagnosing radiculopathy, the sensitivity of needle PM EMG is 82.6% (48/58; 95% CI 70.691.4%). Compared to limb myotome assessment, the highest informative value of PM EMG was reported in patients with the disease duration for up to 3 months. PM EMG was conclusive for diagnosing radicular lesions in patients with isolated pain syndrome or sensory disorders.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135193129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
5q spinal muscular atrophy (SMA) is a rare autosomal recessive neuromuscular disease characterized by gradual loss of motor neurons with progressive muscle weakness and atrophy. A specific therapy has changed the prognosis for such patients, prevented worsening disability, and improved the quality of life. Here are presented follow-up data for 13 patients with SMA aged 1942 years receiving oral therapy for 20212023. Changes in motor functions were assessed using a Revised Upper Limb Module (RULM) every 6 months. According to the follow-up data for risdiplam use in adult patients with SMA in the Moscow region, condition can be stabilized and motor functions can be improved even in patients with a severe neurological deficit at advanced disease stages.
{"title":"Oral risdiplam for specific therapy in adult patients with 5q spinal muscular atrophy in the Moscow region","authors":"Ekaterina S. Novikova","doi":"10.54101/acen.2023.3.11","DOIUrl":"https://doi.org/10.54101/acen.2023.3.11","url":null,"abstract":"5q spinal muscular atrophy (SMA) is a rare autosomal recessive neuromuscular disease characterized by gradual loss of motor neurons with progressive muscle weakness and atrophy. A specific therapy has changed the prognosis for such patients, prevented worsening disability, and improved the quality of life. Here are presented follow-up data for 13 patients with SMA aged 1942 years receiving oral therapy for 20212023. Changes in motor functions were assessed using a Revised Upper Limb Module (RULM) every 6 months. According to the follow-up data for risdiplam use in adult patients with SMA in the Moscow region, condition can be stabilized and motor functions can be improved even in patients with a severe neurological deficit at advanced disease stages.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135194564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ekaterina V. Orlova, Alexandr B. Berdalin, Vladimir G. Lelyuk
Introduction. Microembolus detection by transcranial Doppler (TCD) is the only non-invasive modality for visualization of cerebral embolism. Currently, there is no unified classification of recorded microembolic signals (MES) that could be used in clinical practice.
The aim of the study is to investigate biophysical MES parameters in patients with ischemic stroke, as well as to assess approaches to microemboli differentiation by structure and origin to improve the diagnostic accuracy of the method and to reduce the risk of recurrent ischemic events.
Materials and methods. The inclusion criterion was TCD-detected signs of MES. We analyzed the data of 28 patients with ischemic stroke (9 women and 19 men; mean age was 58 years 13). We recorded power, duration, and frequency for each MES, and calculated an energy index.
Results. A total of 938 MES were reported. In patients with cardioembolic stroke and all other pathogenetic stroke subtypes, biophysical parameter limits were as follows: 14.65 dB for the average power, 9.45 ms for the average duration, and 0.16 J for the average energy index. For patients with atrial fibrillation, characteristic MES power was found to be 13 dB. The MES frequency limit was determined to be 650 Hz for microemboli differentiation by acoustic density.
Conclusion. The data obtained can be used to further search for optimal limit ranges for biophysical parameters of various MES in order to establish a single MES classification, which will increase the diagnostic value of microembolus detection by TCD in stroke treatment practice.
{"title":"Approaches to classification of microembolic signals in patients recovering from ischemic stroke","authors":"Ekaterina V. Orlova, Alexandr B. Berdalin, Vladimir G. Lelyuk","doi":"10.54101/acen.2023.3.9","DOIUrl":"https://doi.org/10.54101/acen.2023.3.9","url":null,"abstract":"Introduction. Microembolus detection by transcranial Doppler (TCD) is the only non-invasive modality for visualization of cerebral embolism. Currently, there is no unified classification of recorded microembolic signals (MES) that could be used in clinical practice.
 The aim of the study is to investigate biophysical MES parameters in patients with ischemic stroke, as well as to assess approaches to microemboli differentiation by structure and origin to improve the diagnostic accuracy of the method and to reduce the risk of recurrent ischemic events.
 Materials and methods. The inclusion criterion was TCD-detected signs of MES. We analyzed the data of 28 patients with ischemic stroke (9 women and 19 men; mean age was 58 years 13). We recorded power, duration, and frequency for each MES, and calculated an energy index.
 Results. A total of 938 MES were reported. In patients with cardioembolic stroke and all other pathogenetic stroke subtypes, biophysical parameter limits were as follows: 14.65 dB for the average power, 9.45 ms for the average duration, and 0.16 J for the average energy index. For patients with atrial fibrillation, characteristic MES power was found to be 13 dB. The MES frequency limit was determined to be 650 Hz for microemboli differentiation by acoustic density.
 Conclusion. The data obtained can be used to further search for optimal limit ranges for biophysical parameters of various MES in order to establish a single MES classification, which will increase the diagnostic value of microembolus detection by TCD in stroke treatment practice.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135193583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Larisa A. Dobrynina, Maria M. Tsypushtanova, Аlla A. Shabalina, Kamila V. Shamtieva, Angelina G. Makarova, Viktoria V. Trubitsyna, Elina T. Bitsieva, Aleksandra A. Byrochkina, Anastasia A. Geints
Introduction. Cerebral small vessel disease (CSVD) as well as the Alzheimer's disease (AD) and their comorbidities are the most common causes of cognitive impairments (CIs).
Objective: to evaluate the predictive power of the biochemical neurodegeneration markers in patients with CSVD and AD.
Materials and methods. We assessed the following neurodegeneration markers in 68 patients with CSVD (61.0 8.6 years; 60.3% males), 17 patients with AD (65.2 8.3 years; 35.3% males), and 26 healthy volunteers (59.9 6.7 years; 38.5% males): neuron-specific enolase (NSE), glial fibrillary acid protein (GFAP), neurofilament light polypeptide (NEFL) in blood (for all patients) and in cerebrospinal fluid (CSF; in patients with CSVD and AD). We assessed the predictive power of those markers with ROC analysis.
Results. As compared to the control group, serum GFAP in patients with CSVD showed its predictive power at 0.155 ng/ml (sensitivity 74%; specificity 70%). Serum NEFL 0.0185 ng/ml (sensitivity 82%; specificity 96%) and NSE 4.95 g/ml (sensitivity 77%; specificity 71%) showed their predictive power in patients with AD. CSF GFAP 1.03 ng/ml (sensitivity 84%; specificity 88%), CSF NSE 19.10 g/ml (sensitivity 88%; specificity 91%), serum NEFL 0.021 ng/ml (sensitivity 71%; specificity 76%), serum NSE /CSF NSE ratio 0.273 ng/ml (sensitivity 87%; specificity 88%) help differentiate CSVD from AD.
Conclusions. We found that serum GFAP can be a useful diagnostic marker in patients with CSVD, while serum NEFL and serum NSE can help identify the AD. In addition, CSF GFAP and CSF NSE as well as serum NEFL and serum NSE/CSF NSE can help differentiate CSVD from AD. We can use those markers in clinical and research practice to identify the vascular and neurodegenerative causes of CIs and their comorbidities, which is of a great importance in developing specific treatment and predicting the course of the disease.
{"title":"Biochemical markers of neurodegeneration in patients with cerebral small vessel disease and Alzheimer's disease","authors":"Larisa A. Dobrynina, Maria M. Tsypushtanova, Аlla A. Shabalina, Kamila V. Shamtieva, Angelina G. Makarova, Viktoria V. Trubitsyna, Elina T. Bitsieva, Aleksandra A. Byrochkina, Anastasia A. Geints","doi":"10.54101/acen.2023.3.3","DOIUrl":"https://doi.org/10.54101/acen.2023.3.3","url":null,"abstract":"Introduction. Cerebral small vessel disease (CSVD) as well as the Alzheimer's disease (AD) and their comorbidities are the most common causes of cognitive impairments (CIs).
 Objective: to evaluate the predictive power of the biochemical neurodegeneration markers in patients with CSVD and AD.
 Materials and methods. We assessed the following neurodegeneration markers in 68 patients with CSVD (61.0 8.6 years; 60.3% males), 17 patients with AD (65.2 8.3 years; 35.3% males), and 26 healthy volunteers (59.9 6.7 years; 38.5% males): neuron-specific enolase (NSE), glial fibrillary acid protein (GFAP), neurofilament light polypeptide (NEFL) in blood (for all patients) and in cerebrospinal fluid (CSF; in patients with CSVD and AD). We assessed the predictive power of those markers with ROC analysis.
 Results. As compared to the control group, serum GFAP in patients with CSVD showed its predictive power at 0.155 ng/ml (sensitivity 74%; specificity 70%). Serum NEFL 0.0185 ng/ml (sensitivity 82%; specificity 96%) and NSE 4.95 g/ml (sensitivity 77%; specificity 71%) showed their predictive power in patients with AD. CSF GFAP 1.03 ng/ml (sensitivity 84%; specificity 88%), CSF NSE 19.10 g/ml (sensitivity 88%; specificity 91%), serum NEFL 0.021 ng/ml (sensitivity 71%; specificity 76%), serum NSE /CSF NSE ratio 0.273 ng/ml (sensitivity 87%; specificity 88%) help differentiate CSVD from AD.
 Conclusions. We found that serum GFAP can be a useful diagnostic marker in patients with CSVD, while serum NEFL and serum NSE can help identify the AD. In addition, CSF GFAP and CSF NSE as well as serum NEFL and serum NSE/CSF NSE can help differentiate CSVD from AD. We can use those markers in clinical and research practice to identify the vascular and neurodegenerative causes of CIs and their comorbidities, which is of a great importance in developing specific treatment and predicting the course of the disease.","PeriodicalId":36946,"journal":{"name":"Annals of Clinical and Experimental Neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135193672","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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