Archives of neurology最新文献

{"title":"Diffusion tensor imaging of vascular parkinsonism: structural changes in cerebral white matter and the association with clinical severity.","authors":"Han-Cheng Wang,&nbsp;Jung-Lung Hsu,&nbsp;Alexander Leemans","doi":"10.1001/archneurol.2012.633","DOIUrl":"https://doi.org/10.1001/archneurol.2012.633","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the white matter (WM) microstructure using diffusion tensor imaging in patients with vascular parkinsonism (VP) and specific fiber tract involvement with respect to clinical severity.</p><p><strong>Design: </strong>Diffusion measures (fractional anisotropy and mean diffusivity) were calculated from diffusion tensor images of patients with VP and control subjects. We performed global-, voxel-, and tract-based analyses to compare WM microstructural properties between groups.We further correlated findings with Unified Parkinson's Disease Rating Scale scores and modified postural instability gait difficulty (PIGD) scores to identify most relevant tract involvement.</p><p><strong>Setting: </strong>Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.</p><p><strong>Participants: </strong>Twelve patients with VP and 12 age-matched healthy controls without VP.</p><p><strong>Results: </strong>In the VP group, the left thalamus, right frontal subcortical WM, and left anterior limb of the internal capsule had a significantly lower regional fractional anisotropy compared with the control group. The bilateral frontal subcortical WM showed a significantly higher regional mean diffusivity. The diffusion metrics in these regions were significantly correlated with the modified PIGD score part III, and the sum of modified PIGD scores parts II and III. Tract-based analysis showed a group difference in mean fractional anisotropy and mean diffusivity for multiple fiber bundles, but only diffusion measures of fiber tracts from the bilateral frontal lobe that pass through the anterior limb of internal capsule and tracts of the genu of the corpus callosum showed significant correlation with these scores.</p><p><strong>Conclusions: </strong>Disruption of the microstructural organization of frontal lobe WM is associated with the severity of VP. Our findings are in accordance with the frontal lobe disconnection hypothesis for gait problems and reinforce the paradigm that the involvement of fibers related to the prefrontal cortex is crucial for the core features of VP.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.633","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30784044","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}

{"title":"Callosal disconnection syndrome in a patient with corpus callosum hemorrhage: a diffusion tensor tractography study.","authors":"Min Cheol Chang,&nbsp;Sang Seok Yeo,&nbsp;Sung Ho Jang","doi":"10.1001/archneurol.2012.48","DOIUrl":"https://doi.org/10.1001/archneurol.2012.48","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.48","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30784258","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}

{"title":"TOMM40 association with Alzheimer disease: tales of APOE and linkage disequilibrium.","authors":"Rita J Guerreiro,&nbsp;John Hardy","doi":"10.1001/archneurol.2012.1935","DOIUrl":"https://doi.org/10.1001/archneurol.2012.1935","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.1935","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30815522","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}

{"title":"Cellular immune suppression in paraneoplastic neurologic syndromes targeting intracellular antigens.","authors":"Dana Orange,&nbsp;Mayu Frank,&nbsp;Suyan Tian,&nbsp;Athanasios Dousmanis,&nbsp;Ronen Marmur,&nbsp;Noreen Buckley,&nbsp;Salina Parveen,&nbsp;Jerome J Graber,&nbsp;Nathalie Blachère,&nbsp;Robert B Darnell","doi":"10.1001/archneurol.2012.595","DOIUrl":"https://doi.org/10.1001/archneurol.2012.595","url":null,"abstract":"<p><p>BACKGROUND Tumor treatment is the mainstay of therapy for paraneoplastic neurologic disorders (PNDs), but it is only effective in some cases and other treatment options are limited. OBJECTIVE To evaluate the short-term use of a combination of prednisone and tacrolimus for acute neurologic worsening in PND in which intracellular antigens are targeted. DESIGN Retrospective single-center case series of patients with PND treated with tacrolimus. SETTING The Rockefeller University Hospital, a research hospital in New York, New York. PATIENTS Twenty-six patients with PND with high titer (≥1:1000) anti-HuD, anti-Yo, or anti-CRMP5 autoantibodies were enrolled. Patients were referred from Memorial Sloan Kettering Cancer Center or self-referred. Two patients discontinued intervention owing to adverse events. INTERVENTIONS Patients were treated with tacrolimus, 0.15-0.30 mg/kg per day, in 2 divided oral doses with 60 mg per day of oral prednisone, tapered off during 1 to 4 weeks. MAIN OUTCOME MEASURES The primary outcome measure was median survival. Neurologic examinations before and after treatment as well as adverse events are described. RESULTS Median survival time was 52 months from time of diagnosis. Some patients experienced neurologic improvement that was functionally meaningful. The incidence of adverse events was similar to that generally reported with tacrolimus. CONCLUSIONS A short course of prednisone and tacrolimus to target central nervous system T cells in patients with PND with acute neurologic decline in which intracellular antigens are targeted was well tolerated and warrants further study. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00378326.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.595","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30600827","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}

{"title":"Identification of dominant human aquaporin 4 T-cell epitopes: observations in neuromyelitis optica with implications for other autoimmune disorders.","authors":"Carsten Korth","doi":"10.1001/archneurol.2012.1561","DOIUrl":"https://doi.org/10.1001/archneurol.2012.1561","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.1561","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30731900","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}

{"title":"Epidemiology of neuromyelitis optica in the United States: a multicenter analysis.","authors":"Maureen A Mealy,&nbsp;Dean M Wingerchuk,&nbsp;Benjamin M Greenberg,&nbsp;Michael Levy","doi":"10.1001/archneurol.2012.314","DOIUrl":"https://doi.org/10.1001/archneurol.2012.314","url":null,"abstract":"<p><strong>Background: </strong>Rare diseases require integrated multicenter clinical networks to facilitate clinical research. Neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSDs) are uncommon neuroinflammatory syndromes that are distinct from multiple sclerosis and associated with NMO-IgG, a serologic antibody against aquaporin 4.</p><p><strong>Objective: </strong>To develop a national multicenter NMO clinical consortium and report initial demographic, clinical, and radiographic features of a cohort of patients with NMO/NMOSD in the United States.</p><p><strong>Design: </strong>Review of medical records from patients undergoing evaluation during a 5-year period. We used uniform diagnostic criteria and clinical, laboratory, and neuroimaging definitions to describe the cohort.</p><p><strong>Setting: </strong>Three academic medical centers.</p><p><strong>Patients: </strong>One hundred eighty-seven patients with NMO/NMOSD.</p><p><strong>Results: </strong>Of the 187 patients included in the analysis, 86 had NMO-IgG-seropositive NMO; 40, NMO-IgG-seronegative NMO; and 61, NMO-IgG-seropositive NMOSD. Altogether, 29.4% of our patients were initially misdiagnosed with multiple sclerosis. The average age at onset of NMO/NMOSD was 41.1 years with a strong female predilection, similar to other autoimmune disorders. Nonwhite patients constituted 52.4% of the cohort. The hallmark of NMO and NMOSD is recurrent longitudinally extensive transverse myelitis, but patients with NMO tend to initially present with optic neuritis.</p><p><strong>Conclusions: </strong>A national multicenter consortium to study NMO/NMOSD is feasible and facilitates accurate clinical diagnosis. This network establishes a foundation for determining disease prevalence, translational research, and clinical trials.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.314","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30717114","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}

{"title":"McLean Course in Electrodiagnostic Medicine.","authors":"Jaya R Trivedi","doi":"10.1001/archneurol.2012.1705","DOIUrl":"https://doi.org/10.1001/archneurol.2012.1705","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.1705","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31494612","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}

{"title":"Fatal varicella-zoster virus vasculopathy associated with adalimumab therapy.","authors":"Wonki Baek,&nbsp;Sang-Gon Lee,&nbsp;Young Seo Kim,&nbsp;Jae-Hoon Kim,&nbsp;Jae-Bum Jun,&nbsp;Hyun Young Kim","doi":"10.1001/archneurol.2011.3741","DOIUrl":"https://doi.org/10.1001/archneurol.2011.3741","url":null,"abstract":"<p><strong>Objective: </strong>To describe the case of a patient who had been receiving adalimumab for rheumatoid arthritis and died of varicella-zoster virus vasculopathy with multifocal cerebral hemorrhage.</p><p><strong>Design: </strong>Case report.</p><p><strong>Setting: </strong>Hanyang University Hospital, Seoul, Republic of Korea.</p><p><strong>Patient: </strong>A 66-year-old woman with adalimumab therapy for rheumatoid arthritis followed by stuporous mental changes.</p><p><strong>Results: </strong>Magnetic resonance imaging scans showed multifocal parenchymal lesions and hemorrhage in the brainstem and supratentorial areas. Polymerase chain reaction analysis of the cerebrospinal fluid was positive for varicella-zoster virus. The patient died of multifocal vasculopathy despite intensive antiviral and antibacterial medication.</p><p><strong>Conclusions: </strong>Varicella-zoster virus multifocal vasculopathy with encephalitis may be associated with adalimumab therapy. Clinicians should be aware of the possibility of fatal varicella-zoster virus vasculopathy with encephalitis in patients undergoing adalimumab therapy for rheumatoid arthritis.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2011.3741","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30716163","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}

{"title":"Primary progressive aphasia and transient global amnesia.","authors":"Ricardo Nitrini,&nbsp;Mirna Lie Hosogi-Senaha,&nbsp;Paulo Caramelli","doi":"10.1001/archneurol.2012.1647","DOIUrl":"https://doi.org/10.1001/archneurol.2012.1647","url":null,"abstract":"W e read with great interest the article by GraffRadford and Josephs describing 3 patients with recurrent episodes of transient global amnesia (TGA) who developed primary progressive aphasia (PPA). In the First Brazilian Symposium on Frontotemporal Dementia in 2007, we described 2 patients with semantic dementia, one of the variants of PPA, who had recurrent episodes of TGA. The first patient, a 66-yearold woman, had 2 episodes of TGA 7 and 6 years before her presentation, with a 2-year history of naming impairment. The second patient, a 75-year-old man, had experienced 3 episodes of TGA 1 year before his presentation, with a 3-year history of naming and reading difficulties. Both patients had never had seizures, and their electroencephalograms did not show epileptic activity. Magnetic resonance imaging revealed severe left temporal lobe atrophy, which was more prominent in the anterior part but also involved the hippocampal formation, in both cases. In the first patient, TGA occurred before the onset of PPA, while in the second patient, language impairment started 2 years before the TGA episodes. In the cases described by Graff-Radford and Josephs, TGA preceded PPA in 2 patients, whereas the reverse occurred in 1. We think our data reinforce the hypothesis that this is not a chance association. Further research is needed to elucidate whether recurrent TGA may predispose to PPA or whether the presence of left temporal or frontal lobe pathology renders patients more susceptible to the disturbance that causes TGA.","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.1647","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30894775","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}

{"title":"Characterization of a family with c9FTD/ALS associated with the GGGGCC repeat expansion in C9ORF72.","authors":"Rodolfo Savica,&nbsp;Anahita Adeli,&nbsp;Prashanthi Vemuri,&nbsp;David S Knopman,&nbsp;Mariely Dejesus-Hernandez,&nbsp;Rosa Rademakers,&nbsp;Julie A Fields,&nbsp;Jennifer Whitwell,&nbsp;Clifford R Jack,&nbsp;Val Lowe,&nbsp;Ronald C Petersen,&nbsp;Bradley F Boeve","doi":"10.1001/archneurol.2012.772","DOIUrl":"https://doi.org/10.1001/archneurol.2012.772","url":null,"abstract":"<p><strong>Background: </strong>The hexanucleotide repeat in the chromosome 9 open reading frame 72 (C9ORF72) gene was recently discovered as the underlying genetic cause of many families with frontotemporal dementia (FTD) and/or amyotrophic lateral sclerosis (ALS) linked to chromosome 9 (c9FTD/ALS). We report the clinical, neuropsychologic, and neuroimaging findings of a family with the C9ORF72 mutation and clinical diagnoses bridging the FTD, parkinsonism, and ALS spectrum.</p><p><strong>Objective: </strong>To characterize the antemortem characteristics of a family with c9FTD/ALS associated with the GGGGCC repeat expansion in C9ORF72.</p><p><strong>Design: </strong>Clinical series.</p><p><strong>Setting: </strong>Tertiary care academic medical center. PATIENTS The members of a family affected by the mutation with features of FTD and/or ALS.</p><p><strong>Main outcome measures: </strong>Clinical, neuropsychologic, and neuroimaging assessments.</p><p><strong>Results: </strong>All 3 examined subjects had the hexanucleotide expansion detected in C9ORF72. All had personality/behavioral changes early in the course of the disease. One case had levodopa-unresponsive parkinsonism, and 1 had ALS. Magnetic resonance imaging showed symmetric bilateral frontal, temporal, insular, and cingulate atrophy.</p><p><strong>Conclusions: </strong>This report highlights the clinical and neuroimaging characteristics of a family with c9FTD/ALS. Further studies are needed to better understand the phenotypical variability and the cliniconeuroimaging-neuropathologic correlations.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.772","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30647803","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}