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Clinical and Radiographic Improvement Following Steroid Therapy in Subacute Post-Traumatic Ascending Myelopathy. 亚急性创伤后上行性脊髓病接受类固醇治疗后的临床症状和影像学改善
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-04 DOI: 10.1212/WNL.0000000000210050
Olumide Emmanuel Adegunna, Anuj Rastogi, Nathan Chan Smyth, Daniel M Mandell, Alfonso Fasano
{"title":"Clinical and Radiographic Improvement Following Steroid Therapy in Subacute Post-Traumatic Ascending Myelopathy.","authors":"Olumide Emmanuel Adegunna, Anuj Rastogi, Nathan Chan Smyth, Daniel M Mandell, Alfonso Fasano","doi":"10.1212/WNL.0000000000210050","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210050","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142576701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Teaching Video NeuroImage: Noogenic Epilepsy With Orofacial Reflex Myoclonus: A Rare Distinct Electroclinical Syndrome. 神经影像教学视频:原发性癫痫伴口面部反射性肌阵挛:一种罕见的独特电临床综合征。
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-04 DOI: 10.1212/WNL.0000000000210045
Jayakumari Nandana, Karamala Yalapalli Manisha, Prasannakumar Surabhi, Surendran Veena, Kiren George Koshy, Ramshekhar N Menon, Ashalatha Radhakrishnan
{"title":"Teaching Video NeuroImage: Noogenic Epilepsy With Orofacial Reflex Myoclonus: A Rare Distinct Electroclinical Syndrome.","authors":"Jayakumari Nandana, Karamala Yalapalli Manisha, Prasannakumar Surabhi, Surendran Veena, Kiren George Koshy, Ramshekhar N Menon, Ashalatha Radhakrishnan","doi":"10.1212/WNL.0000000000210045","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210045","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142576704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Lance-Adams Syndrome: It Really Comes From the Cortex! 兰斯-亚当斯综合症:它真的来自大脑皮层!
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-05 DOI: 10.1212/WNL.0000000000210138
Jonathan C van Zijl, Martijn Beudel
{"title":"Lance-Adams Syndrome: It Really Comes From the Cortex!","authors":"Jonathan C van Zijl, Martijn Beudel","doi":"10.1212/WNL.0000000000210138","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210138","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142582712","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Optimizing Anti-Myelin-Associated Glycoprotein and IgM-Gammopathy Testing for Neuropathy Treatment Evaluation. 优化神经病变治疗评估中的抗髓鞘相关糖蛋白和 IgM-伽玛病检测。
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-05 DOI: 10.1212/WNL.0000000000210000
Christopher J Klein, James D Triplett, David L Murray, Amy P Gorsh, Shahar Shelly, Divyanshu Dubey, Marcus V Pinto, Stephen M Ansell, Michael P Skolka, Grace Swart, Michelle L Mauermann, John R Mills
{"title":"Optimizing Anti-Myelin-Associated Glycoprotein and IgM-Gammopathy Testing for Neuropathy Treatment Evaluation.","authors":"Christopher J Klein, James D Triplett, David L Murray, Amy P Gorsh, Shahar Shelly, Divyanshu Dubey, Marcus V Pinto, Stephen M Ansell, Michael P Skolka, Grace Swart, Michelle L Mauermann, John R Mills","doi":"10.1212/WNL.0000000000210000","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210000","url":null,"abstract":"<p><strong>Background and objectives: </strong>Patients with typical anti-myelin-associated glycoprotein (anti-MAG) neuropathy have IgM-gammopathy, mimic distal chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and are treatment resistant. Anti-MAG patients go unrecognized when IgM-gammopathy is undetected or with atypical phenotypes. We investigated an optimal anti-MAG titration cutoff for excluding CIDP and the impact of IgM-gammopathy detection on neuropathy treatment evaluation without anti-MAG antibodies.</p><p><strong>Methods: </strong>European Academy of Neurology/Peripheral Nerve Society 2021 guidelines were used to assess patients with neuropathy using anti-MAG Bühlmann titration units (BTU) and IgM-gammopathy with Mass-Fix (mass spectrophotometry) and serum protein immunofixation electrophoresis (SPIEP). The immunotherapy outcome was reviewed by inflammatory neuropathy cause and treatment (INCAT) and summated compound muscle action potential (CMAP) nerve conduction changes.</p><p><strong>Results: </strong>Seven hundred and fifty-two patients (average age: 63.8 years, female: 31%) were identified over 30 months: (1) typical anti-MAG neuropathy (n = 104); (2) atypical anti-MAG neuropathy (n = 13); (3) distal or sensory-predominant CIDP (n = 25), including 7 without IgM-gammopathy; (4) typical CIDP (n = 47), including 36 without IgM-gammopathy; (5) axonal IgM-gammopathy-associated neuropathy (n = 104); and (6) IgM-gammopathy-negative, anti-MAG-negative axonal neuropathies (n = 426); and (7) without neuropathy (n = 33) anti-MAG negative. IgM-gammopathy was evaluated by Mass-Fix (n = 493), SPIEP (n = 355), or both (n = 96). Mass-Fix detected 4 additional IgM-gammopathies (3%, 4/117) among patients with anti-MAG antibodies and 7 additional patients (2%, 7/376) without anti-MAG not detected by SPIEP testing. Immunotherapy follow-up was available in 123 (mean: 23 months, range: 3-120 months) including 47 with CIDP (28 without IgM-gammopathy) and 76 non-CIDP (5 without IgM-gammopathy, 45 anti-MAG positive). Treatments included IVIG (n = 89), rituximab (n = 80), and ibrutinib or zanubrutinib (n = 24). An optimal anti-MAG-positive cutoff was identified at ≥1,500 BTU (78% sensitivity, 96% specificity) and at ≥10,000 BTU (74% sensitivity, 100% specificity) for typical anti-MAG neuropathy. Improvements in INCAT scores (<i>p</i> < 0.0001) and summated CMAP (<i>p</i> = 0.0028) were associated with negative anti-MAG (<1,500 BTU, n = 78) and absence of IgM-gammopathy (n = 34). Among 47 patients with electrodiagnostically confirmed CIDP, all anti-MAG negative, the presence of IgM-gammopathy (n = 19) also correlated with a worse treatment response (INCAT scores <i>p</i> = 0.035, summated CMAP <i>p</i> = 0.049).</p><p><strong>Discussion: </strong>A cutoff of 10,000 BTU seems optimal for typical anti-MAG neuropathy while ≥1,500 BTU reduces the likelihood of immune-treatable CIDP. Mass-Fix improves IgM-gammopathy detection in anti-MAG and othe","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142582877","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Lumipulse-Measured Cerebrospinal Fluid Biomarkers for the Early Detection of Alzheimer Disease. 用于早期检测阿尔茨海默病的 Lumipulse 测量脑脊液生物标记物。
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-04 DOI: 10.1212/WNL.0000000000209866
Michelle Safransky, Jenna R Groh, Kaj Blennow, Henrik Zetterberg, Yorghos Tripodis, Brett Martin, Jason Weller, Breton M Asken, Gil D Rabinovici, Wendy Wei Qiao Qiu, Ann C McKee, Thor D Stein, Jesse Mez, Rachel L Henson, Justin Long, John C Morris, Richard J Perrin, Suzanne E Schindler, Michael L Alosco
{"title":"Lumipulse-Measured Cerebrospinal Fluid Biomarkers for the Early Detection of Alzheimer Disease.","authors":"Michelle Safransky, Jenna R Groh, Kaj Blennow, Henrik Zetterberg, Yorghos Tripodis, Brett Martin, Jason Weller, Breton M Asken, Gil D Rabinovici, Wendy Wei Qiao Qiu, Ann C McKee, Thor D Stein, Jesse Mez, Rachel L Henson, Justin Long, John C Morris, Richard J Perrin, Suzanne E Schindler, Michael L Alosco","doi":"10.1212/WNL.0000000000209866","DOIUrl":"10.1212/WNL.0000000000209866","url":null,"abstract":"<p><strong>Background and objectives: </strong>CSF biomarkers of Aβ42 and phosphorylated tau (p-tau181) are used clinically for the detection of Alzheimer disease (AD) pathology during life. CSF biomarker validation studies have largely used clinical diagnoses and/or amyloid PET imaging as the reference standard. The few existing CSF-to-autopsy studies have been restricted to late-stage AD. This CSF-to-autopsy study investigated associations between CSF biomarkers of AD and AD neuropathologic changes among brain donors who had normal cognition at the time of lumbar puncture (LP).</p><p><strong>Methods: </strong>This was a retrospective study of brain donors from the National Alzheimer's Coordinating Center who had normal cognition at the time of LP and who had measurements of CSF Aβ42 and p-tau181 performed with Lumipulse assays. All brain donors were from Washington University Knight ADRC. Staging of AD neuropathologic change (ADNC) was made based on National Institute on Aging-Alzheimer's Association criteria. For this study, participants were divided into 2 categories: \"AD-\" (no AD/low ADNC) and \"AD+\" (intermediate/high ADNC). Accuracy of each biomarker for discriminating AD status was evaluated using area under the curve (AUC) statistics generated using predicted probabilities from binary logistic regressions that controlled for age, sex, <i>APOE ε4</i>, and interval between LP and death.</p><p><strong>Results: </strong>The average age at LP was 79.3 years (SD = 5.6), and the average age at death was 87.1 years (SD = 6.5). Of the 49 brain donors, 24 (49%) were male and 47 (95.9%) were White. 20 (40.8%) had autopsy-confirmed AD. The average interval from LP until death was 7.76 years (SD = 4.31). CSF p-tau181/Aβ42 was the optimal predictor of AD, having excellent discrimination accuracy (AUC = 0.97, 95% CI 0.94-1.00, <i>p</i> = 0.003). CSF p-tau181 alone had the second-best discrimination accuracy (AUC = 0.92, 95% CI 0.84-1.00, <i>p</i> = 0.001), followed by CSF Aβ42 alone (AUC = 0.92, 95% CI 0.85-1.00, <i>p</i> = 0.007), while CSF t-tau had the numerically lowest discrimination accuracy (AUC = 0.87, 95% CI 0.76-0.97, <i>p</i> = 0.005). Effects remained after controlling for prevalent comorbid neuropathologies. CSF p-tau181/Aβ42 was strongly associated with CERAD ratings of neuritic amyloid plaque scores and Braak staging of NFTs.</p><p><strong>Discussion: </strong>This study supports Lumipulse-measured CSF Aβ42 and p-tau181 and, particularly, the ratio of p-tau181 to Aβ42, for the early detection of AD pathophysiologic processes.</p><p><strong>Classification of evidence: </strong>This study provides Class II evidence that Lumipulse measures of p-tau181/Aβ42 in the CSF accurately discriminated cognitively normal participants with and without Alzheimer disease neuropathologic change.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11540457/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142576702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Association of Sleep Disturbances With Prevalent and Incident Motoric Cognitive Risk Syndrome in Community-Residing Older Adults. 在社区居住的老年人中,睡眠障碍与运动性认知风险综合征的发病率和发病率之间的关系。
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-06 DOI: 10.1212/WNL.0000000000210054
Victoire Leroy, Emmeline Ayers, Dristi Adhikari, Joe Verghese
{"title":"Association of Sleep Disturbances With Prevalent and Incident Motoric Cognitive Risk Syndrome in Community-Residing Older Adults.","authors":"Victoire Leroy, Emmeline Ayers, Dristi Adhikari, Joe Verghese","doi":"10.1212/WNL.0000000000210054","DOIUrl":"10.1212/WNL.0000000000210054","url":null,"abstract":"<p><strong>Background and objectives: </strong>There is growing evidence that sleep disturbances are associated with cognitive impairment risk, but their association with the incidence of motoric cognitive risk syndrome (MCR)-a predementia syndrome characterized by slow gait speed and cognitive complaints-is unknown. We aimed to examine the association of sleep disturbances, overall and specific subtypes, with (1) incident and (2) prevalent MCR in older adults.</p><p><strong>Methods: </strong>Community-residing adults aged 65 years and older without dementia were recruited from population lists and included in Central Control of Mobility and Aging, a prospective cohort study, in Albert Einstein College of Medicine, Bronx, NY. We included participants with available data for MCR and Pittsburgh Sleep Quality Index (PSQI). MCR was defined as cognitive complaints reported on standardized questionnaires and slow gait speed as recorded on an electronic treadmill and was adjudicated at baseline and annual follow-up visits. Participants were divided into \"good\" sleepers (≤5) and \"poor\" sleepers (>5) based on an established PSQI cut score. Among participants without MCR at baseline, Cox proportional hazard models adjusted for (1) age, sex, and education and (2) further for comorbidity index, Geriatric Depression Scale score, and global cognitive score were used to examine the association of baseline sleep disturbances with MCR incidence. Association between poor sleep quality and prevalent MCR at baseline in the overall population was explored using multivariate logistic regression analysis.</p><p><strong>Results: </strong>445 participants were included (56.9% women, mean age: 75.9 years [75.3; 76.5]). In MCR-free participants at baseline (n = 403), 36 developed incident MCR over a mean follow-up of 2.9 years. Poor sleepers had a higher risk of incident MCR (HR = 2.7 [1.2; 5.2]) compared with good sleepers, but this association was not significant after adjustment for depressive symptoms (adjusted hazard ratio [aHR] = 1.6 [0.7-3.4]). Among the 7 PSQI components, only sleep-related daytime dysfunction (excessive sleepiness and lower enthusiasm) showed a significant risk of MCR in fully adjusted models (aHR = 3.3 [1.5-7.4]). Prevalent MCR was not associated with poor sleep quality (OR [95% CI] = 1.1 [0.5-2.3]).</p><p><strong>Discussion: </strong>Overall poor sleep quality was associated with incident MCR, but not with prevalent MCR. Specifically, older adults with sleep-related daytime dysfunction are at increased risk of developing MCR. Further studies are needed to validate mechanisms of this relationship.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11540459/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142591272","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Association of Cerebrovascular Reactivity With 1-Year Imaging and Clinical Outcomes in Small Vessel Disease: An Observational Cohort Study. 脑血管反应性与小血管疾病 1 年成像和临床结果的关系:一项观察性队列研究
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-05 DOI: 10.1212/WNL.0000000000210008
Emilie Sleight, Michael S Stringer, Una Clancy, Carmen Arteaga-Reyes, Daniela Jaime Garcia, Angela C C Jochems, Stewart Wiseman, Maria Valdes Hernandez, Francesca M Chappell, Fergus N Doubal, Ian Marshall, Michael J Thrippleton, Joanna M Wardlaw
{"title":"Association of Cerebrovascular Reactivity With 1-Year Imaging and Clinical Outcomes in Small Vessel Disease: An Observational Cohort Study.","authors":"Emilie Sleight, Michael S Stringer, Una Clancy, Carmen Arteaga-Reyes, Daniela Jaime Garcia, Angela C C Jochems, Stewart Wiseman, Maria Valdes Hernandez, Francesca M Chappell, Fergus N Doubal, Ian Marshall, Michael J Thrippleton, Joanna M Wardlaw","doi":"10.1212/WNL.0000000000210008","DOIUrl":"10.1212/WNL.0000000000210008","url":null,"abstract":"<p><strong>Background and objectives: </strong>In patients with cerebral small vessel disease (SVD), impaired cerebrovascular reactivity (CVR) is related to worse concurrent SVD burden, but less is known about cerebrovascular reactivity and long-term SVD lesion progression and clinical outcomes. We investigated associations between cerebrovascular reactivity and 1-year progression of SVD features and clinical outcomes.</p><p><strong>Methods: </strong>Between 2018 and 2021, we recruited patients from the Edinburgh/Lothian stroke services presenting with minor ischemic stroke and SVD features as part of the Mild Stroke Study 3, a prospective observational cohort study (ISRCTN 12113543). We acquired 3T brain MRI at baseline and 1 year. At baseline, we measured cerebrovascular reactivity to 6% inhaled CO<sub>2</sub> in subcortical gray matter, normal-appearing white matter, and white matter hyperintensities (WMH). At baseline and 1 year, we quantified SVD MRI features, incident infarcts, assessed stroke severity (NIH Stroke Scale), recurrent stroke, functional outcome (modified Rankin Scale), and cognition (Montreal Cognitive Assessment). We performed linear and logistic regressions adjusted for age, sex, and vascular risk factors, reporting the regression coefficients and odds ratios with 95% CIs.</p><p><strong>Results: </strong>We recruited 208 patients of whom 163 (mean age and SD: 65.8 ± 11.2 years, 32% female) had adequate baseline CVR and completed the follow-up structural MRI. The median increase in WMH volume was 0.32 mL with (Q1, Q3) = (-0.48, 1.78) mL; 29% had a recurrent stroke or incident infarct on MRI. At 1 year, patients with lower baseline cerebrovascular reactivity in normal-appearing tissues had increased WMH (regression coefficient: B = -1.14 [-2.13, -0.14] log<sub>10</sub> (%ICV) per %/mm Hg) and perivascular space volumes (B = -1.90 [-3.21, -0.60] log<sub>10</sub> (%ROIV) per %/mm Hg), with a similar trend in WMH. CVR was not associated with clinical outcomes at 1 year.</p><p><strong>Discussion: </strong>Lower baseline cerebrovascular reactivity predicted an increase in WMH and perivascular space volumes after 1 year. CVR should be considered in SVD future research and intervention studies.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11540458/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142582711","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Teaching NeuroImage: Use of Arterial Spin Labeling to Differentiate Ictal Phenomenon From Tumor Progression in Grade 3 Astrocytoma. 神经影像教学:使用动脉自旋标记法区分 3 级星形细胞瘤的椎管内现象和肿瘤进展。
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-04 DOI: 10.1212/WNL.0000000000210048
Anza Zahid, Nithisha Thatikonda, Prachi Dubey, Ivo W Tremont-Lukats
{"title":"Teaching NeuroImage: Use of Arterial Spin Labeling to Differentiate Ictal Phenomenon From Tumor Progression in Grade 3 Astrocytoma.","authors":"Anza Zahid, Nithisha Thatikonda, Prachi Dubey, Ivo W Tremont-Lukats","doi":"10.1212/WNL.0000000000210048","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210048","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142576703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome. 兰斯-亚当斯综合征肌阵挛起源的多模态评估
IF 7.7 1区 医学
Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-05 DOI: 10.1212/WNL.0000000000209994
Geoffroy Vellieux, Emmanuelle Apartis, Paul Baudin, Manuel Alejandro Del Río Quiñones, Diane Villemonte de la Clergerie, Aurélie Kas, Vincent Navarro
{"title":"Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome.","authors":"Geoffroy Vellieux, Emmanuelle Apartis, Paul Baudin, Manuel Alejandro Del Río Quiñones, Diane Villemonte de la Clergerie, Aurélie Kas, Vincent Navarro","doi":"10.1212/WNL.0000000000209994","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209994","url":null,"abstract":"<p><strong>Background and objectives: </strong>Lance-Adams syndrome (LAS), or chronic posthypoxic myoclonus, is a long-term disabling neurologic disorder occurring in survivors of anoxia. The cortical or subcortical origin of this myoclonus is unclear. We aimed to identify the neuroanatomical origin of myoclonus in LAS.</p><p><strong>Methods: </strong>We conducted a cross-sectional study and investigated patients diagnosed with LAS from the Department of Neurology of Pitié-Salpêtrière Hospital, using multimodal neurologic explorations: EEG with quantitative analyses, polygraphic EMG recording of myoclonus, coupled EEG-EMG analyses with jerk-locked back averaging, and <sup>18</sup>fluorodeoxyglucose PET/CT imaging.</p><p><strong>Results: </strong>All 18 patients had action multifocal or generalized myoclonus. Eleven patients also presented seizures, mainly generalized tonic-clonic seizures. For 8 patients, myoclonus decreased after seizures for a variable duration, from 1 day to 2 weeks. Epileptiform discharges were identified over the central median region (n = 14), with a maximal amplitude on the Cz (65 ± 20 µV, n = 12) and Fz (107 µV, n = 1) electrodes, and a significantly increased frequency during non-rapid eye movement sleep stages 1 (12 ± 8.5 events/minute, <i>p</i> = 0.004, n = 9) and 2 (11 ± 8.8 events/minute, <i>p</i> = 0.016, n = 7) compared with wake (5.5 ± 5.4 events/minute). The duration of the cortical and muscular events was significantly and positively correlated (ρ = 0.58, <i>p</i> < 0.001, n = 9). Action myoclonic jerks with a duration of <50 ms were confirmed in all patients, with a fast-descending corticospinal way organization with a mean biceps brachii-first interossei dorsalis delay of 9.8 ± 1 ms (n = 8). A central cortical transient preceding the muscular jerks was identified (n = 14), with a mean latency of -31.9 ± 2.9 ms for the tibialis anterior muscle (n = 7). A regional metabolism decrease was observed in the precentral cortex, supplementary motor area, paracentral lobule (n = 6), and postcentral cortex and precuneus (n = 5). This metabolism decrease was bilateral in the precentral cortex for 83% of the patients and in the postcentral cortex for 100%. Hypometabolism in the precentral, supplementary motor, and postcentral areas was confirmed with a voxelwise analysis (<i>p</i> < 10<sup>-3</sup>, n = 6).</p><p><strong>Discussion: </strong>Our findings, based on a large cohort of patients with LAS, strongly suggest a cortical myoclonus, originating within the motor cortex and related to epileptiform mechanisms.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142582861","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hughlings Jackson's Second Thoughts on Mental States in Epilepsy. 休林-杰克逊对癫痫精神状态的再思考。
IF 7.7 1区 医学
Neurology Pub Date : 2024-11-26 Epub Date: 2024-10-21 DOI: 10.1212/WNL.0000000000209959
Andrew J Larner, Michael Swash
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