Pub Date : 2023-01-01DOI: 10.1177/11795735231195756
Lakshman Arcot Jayagopal, Afsaneh Shirani, Kelly Cawcutt, Jie Chen, Ana Yuil-Valdes, Rana Zabad
A 56-year-old Caucasian man was diagnosed with definite neurosarcoidosis after he presented with progressive bilateral lower extremity weakness and dysesthesia. He was started on a combination immunosuppressant regimen of dexamethasone, methotrexate and infliximab. Two months into treatment with immunosuppressants, he developed devastating disseminated aspergillosis which clinically stabilized with aggressive antifungal treatment however had a protracted radiological course despite prolonged anti-fungal treatment for over two years. Interestingly, he remained in remission from neurosarcoidosis off immunosuppression during the same period. This case emphasizes need for vigilance for fungal infections in patients treated with combination immunosuppressive therapy particularly TNF-α inhibitors such as infliximab.
{"title":"Disseminated Aspergillosis in a Patient With Neurosarcoidosis: Persistent Contrast Enhancement in CNS Despite Prolonged Antifungal Treatment: A Case Report.","authors":"Lakshman Arcot Jayagopal, Afsaneh Shirani, Kelly Cawcutt, Jie Chen, Ana Yuil-Valdes, Rana Zabad","doi":"10.1177/11795735231195756","DOIUrl":"https://doi.org/10.1177/11795735231195756","url":null,"abstract":"<p><p>A 56-year-old Caucasian man was diagnosed with definite neurosarcoidosis after he presented with progressive bilateral lower extremity weakness and dysesthesia. He was started on a combination immunosuppressant regimen of dexamethasone, methotrexate and infliximab. Two months into treatment with immunosuppressants, he developed devastating disseminated aspergillosis which clinically stabilized with aggressive antifungal treatment however had a protracted radiological course despite prolonged anti-fungal treatment for over two years. Interestingly, he remained in remission from neurosarcoidosis off immunosuppression during the same period. This case emphasizes need for vigilance for fungal infections in patients treated with combination immunosuppressive therapy particularly TNF-α inhibitors such as infliximab.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/31/2d/10.1177_11795735231195756.PMC10423447.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10295285","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231151830
Samuel W Cramer, Robert A McGovern, Clark C Chen, Michael C Park
We examined the efficacy of vagal nerve stimulation (VNS) for patients suffering from medically intractable epilepsy. Four randomized controlled trials (RCTs - 3 adult RCTs and 1 pediatric RCT) were identified in our comprehensive literature search. Across the 4 studies, high frequency VNS stimulation (frequency >20 Hz) consistently achieved a greater seizure frequency reduction (23.4-33.1%) relative to low frequency VNS stimulation (1 Hz, .6-15.2%). We identified 2 RCTs examining whether the parameters of stimulation influenced seizure control. These studies reported that VNS achieved seizure control comparable to those reported by the first 4 RCTs (22-43% seizure frequency reduction), irrespective of the parameters utilized for VNS stimulation. In terms of VNS associated morbidity, these morbidities were consistently higher in adults who underwent high frequency VNS stimulation (eg dysphonia 37-66%, dyspnea 6-25.3%). However, no such differences were observed in the pediatric population. Moreover, <2% of patients withdrew from the RCTs/prospective studies due to intolerable symptoms. To provide an assessment of how the risks and benefits of VNS impact the patient experience, 1 study assessed the well-being of enrolled patients (as a secondary end point) and found VNS was associated with an overall improvement in well-being. Consistent with this observation, we identified a prospective, non-randomized study that demonstrated improved quality of life for epilepsy patients managed with VNS and best medical practice relative to best medical practice alone. In aggregate, these RCT studies support the efficacy and benefit of VNS as a neuro-modulatory platform in the management of a subset of medically refractory epilepsy patients.
{"title":"Clinical Benefit of Vagus Nerve Stimulation for Epilepsy: Assessment of Randomized Controlled Trials and Prospective Non-Randomized Studies.","authors":"Samuel W Cramer, Robert A McGovern, Clark C Chen, Michael C Park","doi":"10.1177/11795735231151830","DOIUrl":"https://doi.org/10.1177/11795735231151830","url":null,"abstract":"<p><p>We examined the efficacy of vagal nerve stimulation (VNS) for patients suffering from medically intractable epilepsy. Four randomized controlled trials (RCTs - 3 adult RCTs and 1 pediatric RCT) were identified in our comprehensive literature search. Across the 4 studies, high frequency VNS stimulation (frequency >20 Hz) consistently achieved a greater seizure frequency reduction (23.4-33.1%) relative to low frequency VNS stimulation (1 Hz, .6-15.2%). We identified 2 RCTs examining whether the parameters of stimulation influenced seizure control. These studies reported that VNS achieved seizure control comparable to those reported by the first 4 RCTs (22-43% seizure frequency reduction), irrespective of the parameters utilized for VNS stimulation. In terms of VNS associated morbidity, these morbidities were consistently higher in adults who underwent high frequency VNS stimulation (eg dysphonia 37-66%, dyspnea 6-25.3%). However, no such differences were observed in the pediatric population. Moreover, <2% of patients withdrew from the RCTs/prospective studies due to intolerable symptoms. To provide an assessment of how the risks and benefits of VNS impact the patient experience, 1 study assessed the well-being of enrolled patients (as a secondary end point) and found VNS was associated with an overall improvement in well-being. Consistent with this observation, we identified a prospective, non-randomized study that demonstrated improved quality of life for epilepsy patients managed with VNS and best medical practice relative to best medical practice alone. In aggregate, these RCT studies support the efficacy and benefit of VNS as a neuro-modulatory platform in the management of a subset of medically refractory epilepsy patients.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4e/92/10.1177_11795735231151830.PMC9841854.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9100304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231167868
Wilson J Xu, Giuseppe Barisano, Daniel Phung, Brendon Chou, Soniya N Pinto, Alexander Lerner, Nasim Sheikh-Bahaei
Migraine is a complex and common disorder that affects patients around the world. Despite recent advances in this field, the exact pathophysiology of migraine is still not completely understood. Structural MRI sequences have revealed a variety of changes to brain parenchyma associated with migraine, including white matter lesions, volume changes, and iron deposition. This Review highlights different structural imaging findings in various types of migraine and their relationship to migraine characteristics and subtypes in order to improve our understanding of migraine, its pathophysiologic mechanisms, and how to better diagnose and treat it.
{"title":"Structural MRI in Migraine: A Review of Migraine Vascular and Structural Changes in Brain Parenchyma.","authors":"Wilson J Xu, Giuseppe Barisano, Daniel Phung, Brendon Chou, Soniya N Pinto, Alexander Lerner, Nasim Sheikh-Bahaei","doi":"10.1177/11795735231167868","DOIUrl":"https://doi.org/10.1177/11795735231167868","url":null,"abstract":"<p><p>Migraine is a complex and common disorder that affects patients around the world. Despite recent advances in this field, the exact pathophysiology of migraine is still not completely understood. Structural MRI sequences have revealed a variety of changes to brain parenchyma associated with migraine, including white matter lesions, volume changes, and iron deposition. This Review highlights different structural imaging findings in various types of migraine and their relationship to migraine characteristics and subtypes in order to improve our understanding of migraine, its pathophysiologic mechanisms, and how to better diagnose and treat it.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/15/72/10.1177_11795735231167868.PMC10108417.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9383918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Ischemic stroke is a leading cause of morbidity and mortality worldwide. One possible predictor is the use of biomarkers especially neurofilament light chain (NFL).
Objectives: To explore whether NFL could predict clinical outcome and hemorrhagic transformation in moderate to severe stroke.
Design: Single center prospective cohort study.
Methods: Fifty-one moderate to severe ischemic stroke patients were recruited. Blood NFL was obtained from patients at admission (First sample) and 24-96 hours later (Second sample). NFL was analyzed with the ultrasensitive single molecule array (Simoa). Later, we calculated incremental rate NFL (IRN) by changes in NFL per day from baseline. We evaluated National Institute of Health stroke scale (NIHSS), modified Rankins score (mRs), and the presence of hemorrhagic transformation (HT).
Results: IRN was found to be higher in patients with unfavorable outcome (7.12 vs 24.07, P = .04) as well as Second sample (49.06 vs 71.41, P = .011), while NFL First sample was not significant. IRN had a great correlation with mRS (r = .552, P < .001). Univariate logistic regression model showed OR of IRN and Second sample to be 1.081 (95% CI 1.016-1.149, P = .013) and 1.019 (1.002-1.037, P = .03), respectively. Multiple logistic regression model has shown to be significant. In receiver operating analysis, IRN, Second sample, combined IRN with NIHSS and combined Second sample with NIHSS showed AUC (.744, P = .004; 0.713, P = .01; 0.805, P < .001; 0.803, P < .001, respectively). For HT, First sample and Second sample had significant difference with HT (Z = 2.13, P = .033; Z = 2.487, P = .013, respectively).
Conclusion: NFL was found to correlate and predict clinical outcome. In addition, it was found to correlate with HT.
背景:缺血性脑卒中是世界范围内发病率和死亡率的主要原因。一个可能的预测指标是生物标志物的使用,尤其是神经丝轻链(NFL)。目的:探讨NFL能否预测中重度脑卒中患者的临床转归和出血转化。设计:单中心前瞻性队列研究。方法:选取51例中重度缺血性脑卒中患者。患者入院时(第一样本)和24-96小时后(第二样本)采集血液NFL。采用超灵敏单分子阵列(Simoa)对NFL进行分析。随后,我们通过从基线开始每天NFL的变化来计算增量率NFL (IRN)。我们评估了美国国立卫生研究院卒中量表(NIHSS)、改良Rankins评分(mRs)和出血性转化(HT)的存在。结果:不良结局患者的IRN较高(7.12 vs 24.07, P = 0.04),第二样本的IRN较高(49.06 vs 71.41, P = 0.011),而第一样本的IRN无统计学意义。IRN与mRS有显著相关性(r = .552, P < .001)。单因素logistic回归模型显示,IRN和Second样本的OR分别为1.081 (95% CI 1.016 ~ 1.149, P = 0.013)和1.019 (1.002 ~ 1.037,P = 0.03)。多元逻辑回归模型已显示出显著性。在受试者操作分析中,IRN、第二样本、IRN联合NIHSS和第二样本联合NIHSS显示AUC()。744, p = .004;0.713, p = 0.01;0.805, p < 0.001;0.803, P < 0.001)。对于HT,第一样本和第二样本与HT有显著性差异(Z = 2.13, P = 0.033;Z = 2.487, P = 0.013)。结论:发现NFL与临床预后相关并预测其预后。此外,它被发现与HT相关。
{"title":"Neurofilament light is associated with clinical outcome and hemorrhagic transformation in moderate to severe ischemic stroke.","authors":"Wanakorn Rattanawong, Tatchaporn Ongphichetmetha, Thiravat Hemachudha, Poosanu Thanapornsangsuth","doi":"10.1177/11795735221147212","DOIUrl":"https://doi.org/10.1177/11795735221147212","url":null,"abstract":"<p><strong>Background: </strong>Ischemic stroke is a leading cause of morbidity and mortality worldwide. One possible predictor is the use of biomarkers especially neurofilament light chain (NFL).</p><p><strong>Objectives: </strong>To explore whether NFL could predict clinical outcome and hemorrhagic transformation in moderate to severe stroke.</p><p><strong>Design: </strong>Single center prospective cohort study.</p><p><strong>Methods: </strong>Fifty-one moderate to severe ischemic stroke patients were recruited. Blood NFL was obtained from patients at admission (First sample) and 24-96 hours later (Second sample). NFL was analyzed with the ultrasensitive single molecule array (Simoa). Later, we calculated incremental rate NFL (IRN) by changes in NFL per day from baseline. We evaluated National Institute of Health stroke scale (NIHSS), modified Rankins score (mRs), and the presence of hemorrhagic transformation (HT).</p><p><strong>Results: </strong>IRN was found to be higher in patients with unfavorable outcome (7.12 vs 24.07, <i>P</i> = .04) as well as Second sample (49.06 vs 71.41, <i>P</i> = .011), while NFL First sample was not significant. IRN had a great correlation with mRS (r = .552, <i>P</i> < .001). Univariate logistic regression model showed OR of IRN and Second sample to be 1.081 (95% CI 1.016-1.149, <i>P</i> = .013) and 1.019 (1.002-1.037, <i>P</i> = .03), respectively. Multiple logistic regression model has shown to be significant. In receiver operating analysis, IRN, Second sample, combined IRN with NIHSS and combined Second sample with NIHSS showed AUC (.744, <i>P</i> = .004; 0.713, <i>P</i> = .01; 0.805, <i>P</i> < .001; 0.803, <i>P</i> < .001, respectively). For HT, First sample and Second sample had significant difference with HT (Z = 2.13, <i>P</i> = .033; Z = 2.487, <i>P</i> = .013, respectively).</p><p><strong>Conclusion: </strong>NFL was found to correlate and predict clinical outcome. In addition, it was found to correlate with HT.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f1/59/10.1177_11795735221147212.PMC9827527.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10529562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231181467
Dafne Dain Gandelman Horovitz, Maria Angelica de Faria Domingues de Lima, Lais de Carvalho Pires, Abelardo de Queiroz Campos Araujo, Fernando Regla Vargas
IRF2BPL gene variants have recently been associated to developmental disability and epilepsy in children and movement disorders in adults. So far, only few cases have been reported; here we present four novel cases identified by exome sequencing, while investigating developmental delay, adult-onset cerebellar ataxia or regression.
{"title":"Neurological Phenotypes of <i>IRF2BPL</i> Gene Variants: A Report of Four Novel Variants.","authors":"Dafne Dain Gandelman Horovitz, Maria Angelica de Faria Domingues de Lima, Lais de Carvalho Pires, Abelardo de Queiroz Campos Araujo, Fernando Regla Vargas","doi":"10.1177/11795735231181467","DOIUrl":"https://doi.org/10.1177/11795735231181467","url":null,"abstract":"<p><p><i>IRF2BPL</i> gene variants have recently been associated to developmental disability and epilepsy in children and movement disorders in adults. So far, only few cases have been reported; here we present four novel cases identified by exome sequencing, while investigating developmental delay, adult-onset cerebellar ataxia or regression.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/84/cc/10.1177_11795735231181467.PMC10280516.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10291251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231195775
Diogo G Corrêa, Eelco van Duinkerken, João Gabriel D Farinhas, Valéria C Pereira, Emerson L Gasparetto, Soniza V Alves-Leon, Fernanda Cristina R Lopes
Background: Changes in brain connectivity occur in patients with multiple sclerosis (MS), even in patients under disease-modifying therapies. Using magnetic resonance imaging (MRI) to asses patients treated with disease-modifying therapies, such as natalizumab, can elucidate the mechanisms involved in clinical deterioration in MS.
Objectives: To evaluate differences in resting-state functional connectivity among MS patients treated with natalizumab, MS patients not treated with natalizumab, and controls.
Methods: Twenty-three MS patients being treated with natalizumab were retrospectively compared with 23 MS patients who were naïve for natalizumab, and were using first-line medications (interferon-β and/or glatiramer acetate), and 17 gender- and age-matched control subjects. The MS patient groups were also matched for time since diagnosis and hyperintense lesion volume on FLAIR. All participants underwent brain MRI using a 3 Tesla scanner. Independent component analysis and dual regression were used to identify resting-state functional connectivity using the FMRIB Software Library.
Results: In comparison to controls, the MS patients treated with natalizumab presented decreased connectivity in the left orbitofrontal cortex, in the anterior cingulate and orbitofrontal cortex network. The patients not treated with natalizumab presented increased connectivity in the secondary visual, sensorimotor, and ventral attention networks in comparison to controls.Compared to patients treated with natalizumab, the patients not using natalizumab presented increased connectivity in the left Heschl's gyrus and in the right superior frontal gyrus in the ventral attention network.
Conclusion: Differences in brain connectivity between MS patients not treated with natalizumab, healthy controls, and patients treated with natalizumab may be secondary to suboptimal neuronal compensation due to prior less efficient treatments, or due to a compensation in response to maladaptive plasticity.
{"title":"Influence of natalizumab on resting-state connectivity in patients with multiple sclerosis.","authors":"Diogo G Corrêa, Eelco van Duinkerken, João Gabriel D Farinhas, Valéria C Pereira, Emerson L Gasparetto, Soniza V Alves-Leon, Fernanda Cristina R Lopes","doi":"10.1177/11795735231195775","DOIUrl":"https://doi.org/10.1177/11795735231195775","url":null,"abstract":"<p><strong>Background: </strong>Changes in brain connectivity occur in patients with multiple sclerosis (MS), even in patients under disease-modifying therapies. Using magnetic resonance imaging (MRI) to asses patients treated with disease-modifying therapies, such as natalizumab, can elucidate the mechanisms involved in clinical deterioration in MS.</p><p><strong>Objectives: </strong>To evaluate differences in resting-state functional connectivity among MS patients treated with natalizumab, MS patients not treated with natalizumab, and controls.</p><p><strong>Design: </strong>Single-center retrospective cross-sectional study.</p><p><strong>Methods: </strong>Twenty-three MS patients being treated with natalizumab were retrospectively compared with 23 MS patients who were naïve for natalizumab, and were using first-line medications (interferon-β and/or glatiramer acetate), and 17 gender- and age-matched control subjects. The MS patient groups were also matched for time since diagnosis and hyperintense lesion volume on FLAIR. All participants underwent brain MRI using a 3 Tesla scanner. Independent component analysis and dual regression were used to identify resting-state functional connectivity using the FMRIB Software Library.</p><p><strong>Results: </strong>In comparison to controls, the MS patients treated with natalizumab presented decreased connectivity in the left orbitofrontal cortex, in the anterior cingulate and orbitofrontal cortex network. The patients not treated with natalizumab presented increased connectivity in the secondary visual, sensorimotor, and ventral attention networks in comparison to controls.Compared to patients treated with natalizumab, the patients not using natalizumab presented increased connectivity in the left Heschl's gyrus and in the right superior frontal gyrus in the ventral attention network.</p><p><strong>Conclusion: </strong>Differences in brain connectivity between MS patients not treated with natalizumab, healthy controls, and patients treated with natalizumab may be secondary to suboptimal neuronal compensation due to prior less efficient treatments, or due to a compensation in response to maladaptive plasticity.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/44/f0/10.1177_11795735231195775.PMC10433731.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10667927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231160036
Cassie Liu, Joseph Carmicheal, Michael J Baine, Chi Zhang
Pineal parenchymal tumor of intermediate differentiation (PPTID) is a rare, primary tumor of the pineal gland. Due to its rarity, there is no consensus on optimal therapeutic strategies or standard characterization of the tumor's behavior. Here, we report 2 new cases of PPTID and an extensive review of the literature involving the use and extent of radiation therapy. Patient 1 is a 54-year-old male who presented with PPTID and drop metastases in the spinal cord, received cranial spinal irradiation (CSI), and experienced recurrence 3.5 years after treatment. Stereotactic body radiation therapy (SBRT) helped the patient into remission for 9 months. Patient 2 is a 32-year-old male with a local PPTID at presentation who went on to receive surgical resection followed by focused adjuvant radiation therapy to the pineal tumor bed. He then presented 6 years after treatment with extensive disseminated recurrence and died due to leptomeningeal disease (LMD) about 4 years after recurrence. The available literature on PPTID is limited and reported cases of LMD with ongoing follow-up in PPTID are scarce. Our report adds to the current known PPTID cases, contributing to the information available regarding prognosis and treatment response. Although an optimal therapeutic strategy for PPTID still cannot be determined, data from the literature suggest that utilizing radiation therapy in patients with low-risk disease and gross total resections as well as the use of upfront CSI have the potential to improve patient progression and survival outcomes.
{"title":"Radiation therapy for pineal parenchymal tumor of intermediate differentiation: A case series and literature review.","authors":"Cassie Liu, Joseph Carmicheal, Michael J Baine, Chi Zhang","doi":"10.1177/11795735231160036","DOIUrl":"https://doi.org/10.1177/11795735231160036","url":null,"abstract":"<p><p>Pineal parenchymal tumor of intermediate differentiation (PPTID) is a rare, primary tumor of the pineal gland. Due to its rarity, there is no consensus on optimal therapeutic strategies or standard characterization of the tumor's behavior. Here, we report 2 new cases of PPTID and an extensive review of the literature involving the use and extent of radiation therapy. Patient 1 is a 54-year-old male who presented with PPTID and drop metastases in the spinal cord, received cranial spinal irradiation (CSI), and experienced recurrence 3.5 years after treatment. Stereotactic body radiation therapy (SBRT) helped the patient into remission for 9 months. Patient 2 is a 32-year-old male with a local PPTID at presentation who went on to receive surgical resection followed by focused adjuvant radiation therapy to the pineal tumor bed. He then presented 6 years after treatment with extensive disseminated recurrence and died due to leptomeningeal disease (LMD) about 4 years after recurrence. The available literature on PPTID is limited and reported cases of LMD with ongoing follow-up in PPTID are scarce. Our report adds to the current known PPTID cases, contributing to the information available regarding prognosis and treatment response. Although an optimal therapeutic strategy for PPTID still cannot be determined, data from the literature suggest that utilizing radiation therapy in patients with low-risk disease and gross total resections as well as the use of upfront CSI have the potential to improve patient progression and survival outcomes.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ac/97/10.1177_11795735231160036.PMC10026104.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9174566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231151818
Amreen Farooqui, Yoram A Roman Casul, Varun Jain, Nandakumar Nagaraja
Background: Mild stroke has variable outcomes, and there is an ongoing debate regarding whether the administration of thrombolytics improves outcomes in this subgroup of stroke patients. Having a better understanding of the features of mild stroke may help identify patients who are at risk of poor outcomes.
Objective: The objective of this study is to evaluate the association of clinical and imaging-based small vessel disease features (white matter hyperintensities and cerebral microbleeds) with stroke severity and clinical outcomes in patients with mild stroke.
Methods: In this retrospective study, mild stroke was defined as a National Institute of Health stroke scale (NIHSS) score <5. Clinical, laboratory and imaging data were compared between patients with mild stroke versus non-mild stroke (NIHSS≥5). Multivariate logistic regression analysis was performed to identify predictors of mild stroke and poor discharge outcome.
Results: Among 296 patients included in the study, 131 patients (44%) had mild stroke. On multivariate analysis, patients with mild stroke were three times more likely to have sensory symptoms [odds ratio (OR) = 2.9; 95% confidence interval (CI) = (1.2-6.8)] and four times more likely to have stroke due to small vessel disease (OR = 3.7; 95%CI = 1.4-9.9). Among patients with mild stroke, higher age (OR = 1.1; 95%CI = 1.02-1.1), presence of cerebral microbleed (OR = 4.5; 95%CI = 1.5-13.8), vertigo (OR = 7.3; 95%CI = 1.2-45.1) and weakness (OR = 5.0; 95%CI = 1.2-20.3) as presenting symptoms were more likely to have poor discharge outcome.
Conclusion: Sensory symptoms and stroke due to small vessel disease are more common in mild stroke than non-mild stroke. Among patients with mild stroke, presence of cerebral microbleeds on imaging and symptoms of muscle weakness are associated with poor discharge outcome. Larger studies are needed to assess the impact of cerebral microbleed on mild stroke outcomes and risk stratify the benefit of thrombolytics in this group.
{"title":"Standard clinical and imaging-based small vessel disease parameters associated with mild stroke versus non-mild stroke.","authors":"Amreen Farooqui, Yoram A Roman Casul, Varun Jain, Nandakumar Nagaraja","doi":"10.1177/11795735231151818","DOIUrl":"https://doi.org/10.1177/11795735231151818","url":null,"abstract":"<p><strong>Background: </strong>Mild stroke has variable outcomes, and there is an ongoing debate regarding whether the administration of thrombolytics improves outcomes in this subgroup of stroke patients. Having a better understanding of the features of mild stroke may help identify patients who are at risk of poor outcomes.</p><p><strong>Objective: </strong>The objective of this study is to evaluate the association of clinical and imaging-based small vessel disease features (white matter hyperintensities and cerebral microbleeds) with stroke severity and clinical outcomes in patients with mild stroke.</p><p><strong>Methods: </strong>In this retrospective study, mild stroke was defined as a National Institute of Health stroke scale (NIHSS) score <5. Clinical, laboratory and imaging data were compared between patients with mild stroke versus non-mild stroke (NIHSS≥5). Multivariate logistic regression analysis was performed to identify predictors of mild stroke and poor discharge outcome.</p><p><strong>Results: </strong>Among 296 patients included in the study, 131 patients (44%) had mild stroke. On multivariate analysis, patients with mild stroke were three times more likely to have sensory symptoms [odds ratio (OR) = 2.9; 95% confidence interval (CI) = (1.2-6.8)] and four times more likely to have stroke due to small vessel disease (OR = 3.7; 95%CI = 1.4-9.9). Among patients with mild stroke, higher age (OR = 1.1; 95%CI = 1.02-1.1), presence of cerebral microbleed (OR = 4.5; 95%CI = 1.5-13.8), vertigo (OR = 7.3; 95%CI = 1.2-45.1) and weakness (OR = 5.0; 95%CI = 1.2-20.3) as presenting symptoms were more likely to have poor discharge outcome.</p><p><strong>Conclusion: </strong>Sensory symptoms and stroke due to small vessel disease are more common in mild stroke than non-mild stroke. Among patients with mild stroke, presence of cerebral microbleeds on imaging and symptoms of muscle weakness are associated with poor discharge outcome. Larger studies are needed to assess the impact of cerebral microbleed on mild stroke outcomes and risk stratify the benefit of thrombolytics in this group.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1c/d7/10.1177_11795735231151818.PMC9843637.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9116980","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/11795735231156349
Vishal Mehta, Akhya Sharma, Divya Jyoti, Rathod Prabhakar, Ritesh Kumar, Rishi T Guria, Chandra B Sharma
Background: Early neurological deterioration (END) is a common occurrence in ischemic stroke and contributes significantly to poor outcomes. Although multiple factors that predict END have already been identified, the role of fibrinogen - a key component of the coagulation pathway, is controversial.
Objective: To assess the role of fibrinogen in predicting END and poor hospital outcome in patients with acute ischemic stroke.
Methods: 141 patients with acute ischemic stroke were analyzed in this prospective observational study from a single tertiary-care hospital in East India. END was defined as a worsening of ≥2 points on the National Institutes of Health Stroke Scale (NIHSS) within 7 days of admission. A score of 3-5 on the Modified Rankin Scale (mRS), a stroke recurrence event or death during hospital stay was considered poor hospital outcome. We performed univariate analysis using age, sex, body-mass index (BMI), hypertension, diabetes, NIHSS scores, stroke etiology, blood glucose and lipid parameters and plasma fibrinogen to develop a logistic regression model to establish the independent predictors of END and poor outcome.
Results: Age (Odds Ratio (OR) 1.034 [95% CI 1.001-1.069], P = .046), NIHSS score at admission (OR 1.152 [95% CI 1.070-1.240], P < .001) and fibrinogen (OR 1.011 [95%CI 1.006-1.015], P < .001) were independent predictors of END in patients with acute ischemic stroke. Factors independently associated with poor outcome were NIHSS score at admission (OR 1.257 [95% CI 1.150-1.357], P < .001), fasting plasma glucose (OR 1.007 [95% CI 1.001-1.013], P = .020), and fibrinogen [OR 1.004 [95% CI 1.000-1.007], P = .038).
Conclusion: The significant role of fibrinogen in determining neurological worsening and subsequent poor outcomes in patients with acute ischemic stroke may help in early prognostication and guided therapeutic interventions.
背景:早期神经功能恶化(END)在缺血性脑卒中中很常见,是导致预后不良的重要因素。虽然预测END的多种因素已经被确定,但纤维蛋白原(凝血途径的关键成分)的作用仍存在争议。目的:探讨纤维蛋白原在预测急性缺血性脑卒中患者预后及预后不良中的作用。设计:单中心前瞻性观察研究。方法:在这项前瞻性观察研究中,对东印度一家三级医院的141例急性缺血性卒中患者进行了分析。END定义为入院后7天内美国国立卫生研究院卒中量表(NIHSS)恶化≥2分。在改良兰金量表(mRS)得分为3-5分时,住院期间中风复发或死亡被认为是不良的医院预后。我们使用年龄、性别、身体质量指数(BMI)、高血压、糖尿病、NIHSS评分、卒中病因、血糖和血脂参数以及血浆纤维蛋白原进行单变量分析,建立logistic回归模型,建立END和不良预后的独立预测因子。结果:年龄(比值比(OR) 1.034 [95%CI 1.001-1.069], P = 0.046)、入院时NIHSS评分(OR 1.152 [95%CI 1.070-1.240], P < 0.001)和纤维蛋白原(OR 1.011 [95%CI 1.006-1.015], P < 0.001)是急性缺血性卒中患者END的独立预测因素。与预后不良独立相关的因素是入院时NIHSS评分(OR 1.257 [95% CI 1.150-1.357], P < .001)、空腹血糖(OR 1.007 [95% CI 1.001-1.013], P = .020)和纤维蛋白原[OR 1.004 [95% CI 1.000-1.007], P = .038)。结论:纤维蛋白原在判断急性缺血性脑卒中患者神经系统恶化及其预后不良中的重要作用可能有助于早期预后和指导治疗干预。
{"title":"Fibrinogen as a Predictor of Early Neurological Deterioration in Acute Ischemic Stroke - Evidence From the Indian Population.","authors":"Vishal Mehta, Akhya Sharma, Divya Jyoti, Rathod Prabhakar, Ritesh Kumar, Rishi T Guria, Chandra B Sharma","doi":"10.1177/11795735231156349","DOIUrl":"https://doi.org/10.1177/11795735231156349","url":null,"abstract":"<p><strong>Background: </strong>Early neurological deterioration (END) is a common occurrence in ischemic stroke and contributes significantly to poor outcomes. Although multiple factors that predict END have already been identified, the role of fibrinogen - a key component of the coagulation pathway, is controversial.</p><p><strong>Objective: </strong>To assess the role of fibrinogen in predicting END and poor hospital outcome in patients with acute ischemic stroke.</p><p><strong>Design: </strong>Single-centre prospective observational study.</p><p><strong>Methods: </strong>141 patients with acute ischemic stroke were analyzed in this prospective observational study from a single tertiary-care hospital in East India. END was defined as a worsening of ≥2 points on the National Institutes of Health Stroke Scale (NIHSS) within 7 days of admission. A score of 3-5 on the Modified Rankin Scale (mRS), a stroke recurrence event or death during hospital stay was considered poor hospital outcome. We performed univariate analysis using age, sex, body-mass index (BMI), hypertension, diabetes, NIHSS scores, stroke etiology, blood glucose and lipid parameters and plasma fibrinogen to develop a logistic regression model to establish the independent predictors of END and poor outcome.</p><p><strong>Results: </strong>Age (Odds Ratio (OR) 1.034 [95% CI 1.001-1.069], <i>P</i> = .046), NIHSS score at admission (OR 1.152 [95% CI 1.070-1.240], <i>P</i> < .001) and fibrinogen (OR 1.011 [95%CI 1.006-1.015], <i>P</i> < .001) were independent predictors of END in patients with acute ischemic stroke. Factors independently associated with poor outcome were NIHSS score at admission (OR 1.257 [95% CI 1.150-1.357], <i>P</i> < .001), fasting plasma glucose (OR 1.007 [95% CI 1.001-1.013], <i>P</i> = .020), and fibrinogen [OR 1.004 [95% CI 1.000-1.007], <i>P</i> = .038).</p><p><strong>Conclusion: </strong>The significant role of fibrinogen in determining neurological worsening and subsequent poor outcomes in patients with acute ischemic stroke may help in early prognostication and guided therapeutic interventions.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a4/02/10.1177_11795735231156349.PMC9909079.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10766275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
New-onset refractory status epilepticus (NORSE) is a rare and devastating condition and the prognosis is often poor, with half to two-thirds of survivors experiencing drug-resistant epilepsy, residual cognitive impairment, or functional disability, and the mortality rate is 16% to 27% for adults. We describe a patient with cryptogenic NORSE and favorable recovery from drug-resistant super-refractory SE after the use of intravenous lidocaine. The patient experienced fever and presented with refractory generalized tonic-clonic seizures. The cause was not found by performing extensive examinations, including cell surface autoantibodies and rat brain immunohistochemistry evaluations. The refractory SE with unresponsiveness to multiple anti-epileptic and prolonged sedative medications, which are necessary for prolonged mechanical ventilation, were ameliorated by additive treatment with intravenous lidocaine initiating at 1 mg/kg/h and maintaining at 2 mg/kg/h for 40 days, which led to freedom from intravenous sedative medication and mechanical ventilation. The patient was able to return to school. Lidocaine may be an optional treatment for cryptogenic NORSE.
{"title":"Lidocaine as a potential therapeutic option for super-refractory status epilepticus: A case report.","authors":"Mayu Sugata, Hiroshi Kataoka, Yuto Uchihara, Daisuke Shimada, Kazuaki Atagi, Michitaka Nakamura, Makoto Hara, Makoto Kawahara, Kazuma Sugie","doi":"10.1177/11795735231200740","DOIUrl":"https://doi.org/10.1177/11795735231200740","url":null,"abstract":"<p><p>New-onset refractory status epilepticus (NORSE) is a rare and devastating condition and the prognosis is often poor, with half to two-thirds of survivors experiencing drug-resistant epilepsy, residual cognitive impairment, or functional disability, and the mortality rate is 16% to 27% for adults. We describe a patient with cryptogenic NORSE and favorable recovery from drug-resistant super-refractory SE after the use of intravenous lidocaine. The patient experienced fever and presented with refractory generalized tonic-clonic seizures. The cause was not found by performing extensive examinations, including cell surface autoantibodies and rat brain immunohistochemistry evaluations. The refractory SE with unresponsiveness to multiple anti-epileptic and prolonged sedative medications, which are necessary for prolonged mechanical ventilation, were ameliorated by additive treatment with intravenous lidocaine initiating at 1 mg/kg/h and maintaining at 2 mg/kg/h for 40 days, which led to freedom from intravenous sedative medication and mechanical ventilation. The patient was able to return to school. Lidocaine may be an optional treatment for cryptogenic NORSE.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f9/5c/10.1177_11795735231200740.PMC10492485.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10222494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}