Archives of neurology最新文献

{"title":"Stroke from acute cervical internal carotid artery occlusion: treatment results and predictors of outcome.","authors":"Raymond C S Seet,&nbsp;Eelco F M Wijdicks,&nbsp;Alejandro A Rabinstein","doi":"10.1001/archneurol.2012.2569","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2569","url":null,"abstract":"<p><strong>Background: </strong>Previous studies have not distinguished patients with acute cervical internal carotid artery (ICA) occlusions from those with intracranial occlusions and often consider them together in the same cohort.</p><p><strong>Objectives: </strong>To evaluate the outcomes of patients with stroke from acute cervical ICA occlusion treated with intravenous thrombolysis or primary endovascular procedures and to identify early predictors of functional recovery among these patients.</p><p><strong>Design: </strong>Retrospective study.</p><p><strong>Setting: </strong>Academic hospital.</p><p><strong>Patients: </strong>We studied patients with ischemic stroke who received intravenous thrombolysis or endovascular treatment for acute cervical ICA occlusion at St Mary's Hospital, Mayo Clinic, Rochester, Minnesota. We evaluated the associations of vascular risk factors, severity of stroke, arterial recanalization, presence of tandem occlusions, and collateral distal flow with functional recovery at 90 days after stroke.</p><p><strong>Main outcome measures: </strong>Favorable functional recovery (Modified Rankin Scale score, 0-2).</p><p><strong>Results: </strong>We identified 21 patients (median age, 67 years; median National Institutes of Health Stroke Scale score at presentation, 13), of whom 13 patients received intravenous thrombolysis and 8 patients underwent primary endovascular treatment. Three patients who received intravenous thrombolysis underwent rescue endovascular treatment. Favorable functional recovery (Modified Rankin Scale score, 0-2) was observed in 7 patients who received intravenous thrombolysis and in 1 patient who underwent primary endovascular treatment. Good collateral distal flow and intracranial tandem occlusions were observed in 6 patients and 12 patients, respectively. Good collateral distal flow, observed more frequently in cigarette smokers, was associated with favorable functional recovery (odds ratio, 20; 95% CI, 2-242; P = .02).</p><p><strong>Conclusions: </strong>Intravenous thrombolysis should be administered as first-line treatment in patients with early acute cervical ICA occlusion. Treatment benefits are accentuated in patients with better collateral circulation.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2569","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30929535","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":"Families with Parkinson disease and cancer.","authors":"Walter A Rocca","doi":"10.1001/archneurol.2012.2664","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2664","url":null,"abstract":"W E ARE REACHING THE CONVERgence of 2 important lines of research on the etiology of Parkin-son disease (PD). The first line involves the accumulation of evidence on the familial aggregation of multiple neurode-generative diseases, including PD. The second line involves the accumulation of evidence on the association of PD with a decreased risk of some types of cancer and an increased risk of some other types of cancer, primarily melanoma. Along the first line of research, first-degree relatives of patients with younger-onset PD were found to have an increased risk not only of PD or parkinsonism but also of essential tremor, cognitive impairment or dementia, anxiety disorders, and depressive disorders, compared with relatives of controls. 1-3 A reciprocal familial aggre-gation of other neurodegenerative disorders, including PD, was also found among relatives of patients with amyo-trophic lateral sclerosis or with dementia. 4,5 These studies suggest that some genetic or environmental factors may cluster in some families and may cause several distinct neurologic or psychiatric diseases. Along the second line of research, several studies have shown an association of PD with a decreased risk of some types of cancers and an increased risk of some other types of cancers. Although the literature concerning PD and most cancers remains controversial, 6,7 a consistent association between PD and increased risk of melanoma has been reported. In a recent review of the literature, 6 the association was found to be significant when a diagnosis of PD preceded a diagnosis of melanoma but not when a diagnosis of melanoma preceded a diagnosis of PD, and the association was stronger for men than for women. Another study 8 showed that a positive family history of melanoma among first-degree relatives was a risk factor for PD after adjustment for known environmental risk factors. However, that study 8 did not find a significant association of family history of other types of cancers (such as colorectal, lung, prostate, or breast cancer) with the risk of PD. In this issue of Archives of Neurology, Kareus and colleagues 9 report new findings from the Utah statewide linkage database suggesting that PD is associated with mela-noma at the individual level and also across first-, second-, and third-degree relatives. Thus, relatives of patients with PD had an increased risk of melanoma. In separate analyses , relatives of patients with melanoma also had an increased risk of PD, suggesting common underlying causes of both …","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2664","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30879510","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":"Usefulness of positron emission tomography with fludeoxyglucose f 18 and with carbon 11-tagged methionine in the diagnosis of hippocampal lesions.","authors":"Sara Ortega-Cubero,&nbsp;Pablo D Domínguez,&nbsp;Carlos Caicedo,&nbsp;Javier Arbizu,&nbsp;Pau Pastor","doi":"10.1001/archneurol.2012.304","DOIUrl":"https://doi.org/10.1001/archneurol.2012.304","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.304","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30911944","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":"Shared predispositions of parkinsonism and cancer: a population-based pedigree-linked study.","authors":"Seth A Kareus,&nbsp;Karla P Figueroa,&nbsp;Lisa A Cannon-Albright,&nbsp;Stefan M Pulst","doi":"10.1001/archneurol.2012.2261","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2261","url":null,"abstract":"<p><p>OBJECTIVE To use a statewide population-based genealogic database to evaluate the relationship between Parkinson disease (PD) and cancer subtypes. DESIGN Using a computerized genealogy for the Utah pioneers and their descendants linked to a statewide cancer registry and statewide death certificates, we estimated relative risks for cancer in individuals with PD listed on their death certificate, and in their first-degree, second-degree, and third-degree relatives. SETTING Utah Cancer Registry. PARTICIPANTS Approximately 2.3 million individuals in the Utah genealogic resource, including death certificates of 2998 individuals with PD listed as a cause of death from 1904 to 2008 and information on 100 817 individuals with a cancer diagnosis in the Utah Cancer Registry. RESULTS Melanoma and prostate cancer were the only cancers observed in significant excess among PD cases; colorectal, lung, pancreas, and stomach cancers were observed in deficit. A significantly increased risk for prostate cancer was observed in the PD population as well as among their relatives. A reciprocal significantly increased risk for PD was also found in the 22 147 prostate cancer cases and their relatives. A significantly elevated risk for melanoma was found in the Utah PD population as well as in their relatives. A reciprocal significantly increased relative risk for PD was found in 7841 Utah melanoma cases and their relatives. CONCLUSIONS Our study identified a novel association between PD and prostate cancer, which extended to first-degree, second-degree, and third-degree relatives. We also confirmed the reported risk association for melanoma in patients with PD; we extended the finding to include a significantly increased risk in relatives. These results strongly support a genetic link. This conclusion is further strengthened by observation of the reciprocal relationship, an increased risk for PD in relatives of individuals with melanoma or prostate cancer.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2261","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30878272","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":"Substitution Monotherapy With Levetiracetam vs Older Antiepileptic Drugs: A Randomized Comparative Trial.","authors":"Tahir Hakami,&nbsp;Marian Todaro,&nbsp;Slave Petrovski,&nbsp;Lachlan Macgregor,&nbsp;Dennis Velakoulis,&nbsp;Meng Tan,&nbsp;Zelko Matkovic,&nbsp;Alexandra Gorelik,&nbsp;Danny Liew,&nbsp;Raju Yerra,&nbsp;Terence J O'Brien","doi":"10.1001/archneurol.2012.2203","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2203","url":null,"abstract":"<p><p>OBJECTIVE To determine whether patients who fail their first antiepileptic drug (AED) have better neuropsychiatric and quality-of-life (QOL) outcomes if substituted to levetiracetam monotherapy compared with a second older AED. DESIGN Randomized comparative trial. Participants with partial epilepsy who had failed monotherapy with phenytoin sodium, carbamazepine, or valproate sodium were randomized to substitution monotherapy with levetiracetam or a different older AED. Assessments were performed at baseline, 3 months, and 12 months using questionnaires measuring neuropsychiatric, QOL, seizure control, AED adverse effects, and neurocognitive outcomes. SETTING Epilepsy service of a teaching hospital. PATIENTS Fifty-one patients were randomized to levetiracetam and 48 were randomized to a second older AED (25 to valproate and 23 to carbamazepine). MAIN OUTCOME MEASURES Proportions showing improvements in depression (on the Hospital Anxiety and Depression Scale) and QOL scores (on the 89-item Quality of Life in Epilepsy Inventory) at 3 months. RESULTS There were no differences between the groups in depression scores at 3 months (improvement in 17 of 43 patients [39.5%] in the levetiracetam group and 15 of 44 patients [34.1%] in the older AED group; P = .60), but a greater proportion of the older AED group improved on the 89-item Quality of Life in Epilepsy Inventory compared with the levetiracetam group (27 of 38 patients [71.1%] vs 21 of 43 patients [48.8%], respectively; P = .04). The QOL, anxiety, and AED adverse effects scores were improved in both groups at 3 and 12 months after randomization. CONCLUSIONS Substitution monotherapy in a patient experiencing ongoing seizures or tolerability issues is associated with sustained improvements in measures of QOL, psychiatric, and adverse events outcomes. Patients switched to levetiracetam do not have better outcomes than those switched to a second older AED. TRIAL REGISTRATION anzctr.org.au Identifier: ACTRN12606000102572.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2203","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30879504","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":"Sex, stroke, and atrial fibrillation.","authors":"Robert G Hart,&nbsp;John W Eikelboom,&nbsp;Lesly A Pearce","doi":"10.1001/archneurol.2012.2691","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2691","url":null,"abstract":"<p><strong>Context: </strong>Stroke is a serious complication associated with atrial fibrillation (AF). Women with AF are at higher risk of stroke compared with men. Reasons for this higher stroke risk in women remain unclear, although some studies suggest that undertreatment with warfarin may be a cause.</p><p><strong>Objective: </strong>To compare utilization patterns of warfarin and the risk of subsequent stroke between older men and women with AF at the population level.</p><p><strong>Design, setting, and patients: </strong>  Population-based cohort study of patients 65 years or older admitted to the hospital with recently diagnosed AF in the province of Quebec, Canada, 1998-2007, using administrative data with linkage between hospital discharge, physicians, and prescription drug claims databases.</p><p><strong>Main outcome measures: </strong>  Risk of stroke.</p><p><strong>Results: </strong>The cohort comprised 39 398 men (47.2%) and 44 115 women (52.8%). At admission, women were older and had a higher CHADS2 (congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, prior stroke or transient ischemic attack) score than men (1.99 [SD, 1.10] vs 1.74 [SD, 1.13], P &lt; .001). At 30 days postdischarge, 58.2% of men and 60.6% of women had filled a warfarin prescription. In adjusted analysis, women appeared to fill more warfarin prescriptions compared with men (odds ratio, 1.07 [95% CI, 1.04-1.11]; P &lt; .001). Adherence to warfarin treatment was good in both sexes. Crude stroke incidence was 2.02 per 100 person-years (95% CI, 1.95-2.10) in women vs 1.61 per 100 person-years (95% CI, 1.54-1.69) in men (P &lt; .001). The sex difference was mainly driven by the population of patients 75 years or older. In multivariable Cox regression analysis, women had a higher risk of stroke than men (adjusted hazard ratio, 1.14 [95% CI, 1.07-1.22]; P &lt; .001), even after adjusting for baseline comorbid conditions, individual components of the CHADS2 score, and warfarin treatment.</p><p><strong>Conclusion: </strong>Among older patients admitted with recently diagnosed AF, the risk of stroke was greater in women than in men, regardless of warfarin use.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2691","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30927805","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":"The (Mis)diagnosis of Creutzfeldt-Jakob Disease.","authors":"Richard J Caselli","doi":"10.1001/2013.jamaneurol.1","DOIUrl":"https://doi.org/10.1001/2013.jamaneurol.1","url":null,"abstract":"","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/2013.jamaneurol.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30929931","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":"β-amyloid dynamics in human plasma.","authors":"Yafei Huang,&nbsp;Rachel Potter,&nbsp;Wendy Sigurdson,&nbsp;Tom Kasten,&nbsp;Rose Connors,&nbsp;John C Morris,&nbsp;Tammie Benzinger,&nbsp;Mark Mintun,&nbsp;Tim Ashwood,&nbsp;Mats Ferm,&nbsp;Samantha L Budd,&nbsp;Randall J Bateman","doi":"10.1001/archneurol.2012.18107","DOIUrl":"https://doi.org/10.1001/archneurol.2012.18107","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate dynamic changes in human plasma β-amyloid (Aβ) concentrations, evaluate the effects of aging and amyloidosis on these dynamics, and determine their correlation with cerebrospinal fluid (CSF) Aβ concentrations.</p><p><strong>Design: </strong>A repeated plasma and CSF sampling study.</p><p><strong>Setting: </strong>The Washington University School of Medicine in St Louis, Missouri.</p><p><strong>Participants: </strong>Older adults with amyloid deposition (Amyloid+), age-matched controls without amyloid deposition (Amyloid-), and younger normal controls (YNCs) were enrolled for the study.</p><p><strong>Main outcome measures: </strong>Hourly measurements of plasma Aβ were compared between groups by age and amyloidosis. Plasma Aβ and CSF Aβ concentrations were compared for correlation, linear increase, and circadian patterns.</p><p><strong>Results: </strong>Circadian patterns were observed in plasma Aβ, with diminished amplitudes with aging. Linear increase of Aβ was only observed for CSF Aβ in the YNC and Amyloid- groups, but not in the Amyloid+ group. No linear increase was observed for plasma Aβ. No significant correlations were found between plasma and CSF Aβ concentrations.</p><p><strong>Conclusions: </strong>Plasma Aβ, like CSF, demonstrates a circadian pattern that is reduced in amplitude with increasing age but is unaffected by amyloid deposition. However, we found no evidence that plasma and CSF Aβ concentrations were related on an hourly or individual basis.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.18107","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31113489","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":"Impact of delirium on the course of Alzheimer disease.","authors":"Myron F Weiner","doi":"10.1001/archneurol.2012.2703","DOIUrl":"https://doi.org/10.1001/archneurol.2012.2703","url":null,"abstract":"<p><strong>Background: </strong>Delirium is characterized by acute cognitive impairment. We examined the association of delirium with long-term cognitive trajectories in older adults with Alzheimer disease (AD).</p><p><strong>Methods: </strong>We evaluated prospectively collected data from a nested cohort of hospitalized patients with AD (n = 263) in the Massachusetts Alzheimer Disease Research Center patient registry between January 1, 1991, and June 30, 2006 (median follow-up duration, 3.2 years). Cognitive function was measured using the information-memory-concentration (IMC) section of the Blessed Dementia Rating Scale. Delirium was identified using a validated medical record review method. The rate of cognitive deterioration was contrasted using random-effects regression models.</p><p><strong>Results: </strong>Fifty-six percent of patients with AD developed delirium during hospitalization. The rate of cognitive deterioration before hospitalization did not differ significantly between patients who developed delirium (1.4 [95% CI, 0.7-2.1] IMC points per year) and patients who did not develop delirium (0.8 [95% CI, 0.3-1.3] IMC points per year) (P = .24). After adjusting for dementia severity, comorbidity, and demographic characteristics, patients who had developed delirium experienced greater cognitive deterioration in the year following hospitalization (3.1 [95% CI, 2.1-4.1] IMC points per year) relative to patients who had not developed delirium (1.4 [95% CI, 0.2-2.6] IMC points per year). The ratio of these changes suggests that cognitive deterioration following delirium proceeds at twice the rate in the year after hospitalization compared with patients who did not develop delirium. Patients who had developed delirium maintained a more rapid rate of cognitive deterioration throughout a 5-year period following hospitalization. Sensitivity analyses that excluded rehospitalized patients and included matching on baseline cognitive function and baseline rate of cognitive deterioration produced essentially identical results.</p><p><strong>Conclusions: </strong>Delirium is highly prevalent among persons with AD who are hospitalized and is associated with an increased rate of cognitive deterioration that is maintained for up to 5 years. Strategies to prevent delirium may represent a promising avenue to explore for ameliorating cognitive deterioration in AD.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archneurol.2012.2703","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30913099","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":"Differential diagnosis of Jakob-Creutzfeldt disease.","authors":"Ross W Paterson,&nbsp;Charles C Torres-Chae,&nbsp;Amy L Kuo,&nbsp;Tim Ando,&nbsp;Elizabeth A Nguyen,&nbsp;Katherine Wong,&nbsp;Stephen J DeArmond,&nbsp;Aissa Haman,&nbsp;Paul Garcia,&nbsp;David Y Johnson,&nbsp;Bruce L Miller,&nbsp;Michael D Geschwind","doi":"10.1001/2013.jamaneurol.79","DOIUrl":"https://doi.org/10.1001/2013.jamaneurol.79","url":null,"abstract":"<p><strong>Objectives: </strong>To identify the misdiagnoses of patients with sporadic Jakob-Creutzfeldt disease (sCJD) during the course of their disease and determine which medical specialties saw patients with sCJD prior to the correct diagnosis being made and at what point in the disease course a correct diagnosis was made.</p><p><strong>Design: </strong>Retrospective medical record review.</p><p><strong>Setting: </strong>A specialty referral center of a tertiary academic medical center.</p><p><strong>Participants: </strong>One hundred sixty-three serial patients over a 5.5-year period who ultimately had pathologically proven sCJD. The study used the subset of 97 patients for whom we had adequate medical records.</p><p><strong>Main outcome measures: </strong>Other diagnoses considered in the differential diagnosis and types of medical specialties assessing patients with sCJD.</p><p><strong>Results: </strong>Ninety-seven subjects' records were used in the final analysis. The most common disease categories of misdiagnosis were neurodegenerative, autoimmune/paraneoplastic, infectious, and toxic/metabolic disorders. The most common individual misdiagnoses were viral encephalitis, paraneoplastic disorder, depression, vertigo, Alzheimer disease, stroke, unspecified dementia, central nervous system vasculitis, peripheral neuropathy, and Hashimoto encephalopathy. The physicians who most commonly made these misdiagnoses were primary care physicians and neurologists; in the 18% of patients who were diagnosed correctly at their first assessment, the diagnosis was almost always by a neurologist. The mean time from onset to diagnosis was 7.9 months, an average of two-thirds of the way through their disease course.</p><p><strong>Conclusions: </strong>Diagnosis of sCJD is quite delayed. When evaluating patients with rapidly progressive dementia with suspected neurodegenerative, autoimmune, infectious, or toxic/metabolic etiology, sCJD should also be included in the differential diagnosis, and appropriate diagnostic tests, such as diffusion brain magnetic resonance imaging, should be considered. Primary care physicians and neurologists need improved training in sCJD diagnosis.</p>","PeriodicalId":8321,"journal":{"name":"Archives of neurology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/2013.jamaneurol.79","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31113488","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}