Abstract:
OBJECTIVE:Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinicopathologic entities. The antemortem prediction of the underlying pathologic lesions is reputed to be difficult. This study sought to characterize correlations between 1) the different clinical variants of primary progressive language and speech disorders and 2) the pathologic diagnosis. METHODS:The latter was available for 18 patients having been prospectively monitored in the Lille Memory Clinic (France) between 1993 and 2008. RESULTS:The patients were diagnosed with progressive anarthria (n = 5), agrammatic progressive aphasia (n = 6), logopenic progressive aphasia (n = 1), progressive jargon aphasia (n = 2), typical semantic dementia (n = 2), and atypical semantic dementia (n = 2). All patients with progressive anarthria had a tau pathology at postmortem evaluation: progressive supranuclear palsy (n = 2), Pick disease (n = 2), and corticobasal degeneration (n = 1). All patients with agrammatic primary progressive aphasia had TDP-43-positive FTLD (FTLD-TDP). The patients with logopenic progressive aphasia and progressive jargon aphasia had Alzheimer disease. Both cases of typical semantic dementia had FTLD-TDP. The patients with atypical semantic dementia had tau pathologies: argyrophilic grain disease and corticobasal degeneration. CONCLUSIONS:The different anatomic distribution of the pathologic lesions could explain these results: opercular and subcortical regions in tau pathologies with progressive anarthria, the left frontotemporal cortex in TDP-43-positive frontotemporal lobar degeneration (FTLD-TDP) with agrammatic progressive aphasia, the bilateral lateral and anterior temporal cortex in FTLD-TDP or argyrophilic grain disease with semantic dementia, and the left parietotemporal cortex in Alzheimer disease with logopenic progressive aphasia or jargon aphasia. These correlations have to be confirmed in larger series.
journal_name
Neurologyjournal_title
Neurologyauthors
Deramecourt V,Lebert F,Debachy B,Mackowiak-Cordoliani MA,Bombois S,Kerdraon O,Buée L,Maurage CA,Pasquier Fdoi
10.1212/WNL.0b013e3181c7198esubject
Has Abstractpub_date
2010-01-05 00:00:00pages
42-9issue
1eissn
0028-3878issn
1526-632Xpii
WNL.0b013e3181c7198ejournal_volume
74pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::The visual-motor adaptation to lateral displacement of vision by prism glasses was studied in normal individuals and patients with cerebellar dysfunction, Parkinson's disease, right or left cerebral hemisphere lesions, Alzheimer's disease, or Korsakoff's syndrome. Adaptation was analyzed in two phases, the return to n...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.33.6.766
更新日期:1983-06-01 00:00:00
abstract:OBJECTIVE:To evaluate the validity of data derived from magnetic source imaging (MSI) regarding cerebral dominance for language in patients with intractable seizure disorder. METHOD:The authors performed functional imaging of the receptive language cortex using a whole-head neuromagnetometer in 26 consecutive epilepsy...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.53.5.938
更新日期:1999-09-22 00:00:00
abstract::A 14-year-old boy survived for 7 years after the initial diagnosis and treatment of acute lymphocytic leukemia. Neurologic deterioration occured repeatedly throughout his complicated clinical course but it was most severe and only partially reversible following orally administered pyrimethamine. The neuropathologic le...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.27.7.609
更新日期:1977-07-01 00:00:00
abstract::Article abstract-The authors analyzed whether the pituitary gland enlarges in intracranial hypotension syndrome by studying 11 consecutive patients. Initial MRI scans showed pituitary gland enlargement with a convex superior margin. Follow-up MRI studies revealed that the size of the gland invariably diminished in all...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.55.12.1895
更新日期:2000-12-26 00:00:00
abstract::CSF does not normally contain motile cells within flagella or cilia. When motile cells are seen in the CSF, they may be incorrectly assumed to be amebae. We studied a patient with a traumatic mucocele, in whom motile ciliated respiratory epithelial cells were seen in the CSF and within a frontal lobe brain abscess. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.10.1487
更新日期:1985-10-01 00:00:00
abstract:OBJECTIVE:To generate a national multiple sclerosis (MS) prevalence estimate for the United States by applying a validated algorithm to multiple administrative health claims (AHC) datasets. METHODS:A validated algorithm was applied to private, military, and public AHC datasets to identify adult cases of MS between 200...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000007035
更新日期:2019-03-05 00:00:00
abstract::We studied a family in which the father and 7 of 10 children had episodes of Bell's palsy. Five of the eight affected family members also had ocular motor palsies. Facial nerve and blink reflex studies in four affected siblings demonstrated asymmetrically reduced amplitude of evoked responses without delayed conductio...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.37.8.1369
更新日期:1987-08-01 00:00:00
abstract::Restless legs syndrome (RLS) is a common neurologic disorder whose prevalence has been estimated at 4 to 10% of the general population. Although its pathophysiology remains unknown, dopaminergic mechanisms are believed to play a central role. Furthermore, dopaminergic drugs have shown therapeutic efficacy in various l...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.61.6_suppl_3.s49
更新日期:2003-09-23 00:00:00
abstract::While intracranial tuberculomas have become uncommon in industrial nations, 12 patients with signs and symptoms of an intracranial mass lesion were recently found to have tuberculomas. Clinical findings suggestive of tuberculosis were frequently subtle or absent. Five patients did not have extracranial tuberculosis. T...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.28.3.256
更新日期:1978-03-01 00:00:00
abstract::We have demonstrated the reliability of a noninvasive method for successfully localizing the intracranial origin of the EEG. The dipole localization method (DLM) is a computer-assisted, mathematical method based on electrical field theory and is similar to localization methods currently used by electrocardiologists. I...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.12.1702
更新日期:1985-12-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to assess the relation of retinal microvascular signs and incident stroke and stroke mortality in an older population. METHODS:The authors took retinal photographs on baseline participants (3,654 patients aged 49+ years) of the Blue Mountains Eye Study (1992 to 1994). They asses...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000179177.15900.ca
更新日期:2005-10-11 00:00:00
abstract:OBJECTIVE:To determine the surgical outcome and factors of predictive value in patients undergoing reoperation for intractable partial epilepsy. METHODS:The authors retrospectively studied the operative outcome in 64 consecutive patients who underwent reoperation for intractable partial epilepsy. Demographic data, res...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000147476.86575.a7
更新日期:2004-12-28 00:00:00
abstract::The antimigraine efficacy of many drugs may be mediated less through their primary modes of action than through the common pathway of serotonergic transmission stabilization. Migraine treatment may be symptomatic, acute/abortive, or prophylactic. Because oral drug absorption is impaired during attacks, parenteral agen...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:
更新日期:1993-06-01 00:00:00
abstract::Patients, clinicians, and hospitals have undergone monumental changes during the coronavirus disease 2019 (COVID-19) pandemic. This crisis has forced us to consider the obligations that we neurologists have to our individual patients as well as the greater community. By returning to our fundamental understanding of th...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000009744
更新日期:2020-07-28 00:00:00
abstract::First-degree relatives of narcoleptic subjects (probands) may have sleep pathology related to the transmission of the disorder through their family members. The authors examined four groups: probands (n = 96), first-degree relative (n = 337), environmental reference (n = 85), and general population (n = 6,694) groups....
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000229930.68094.48
更新日期:2006-08-22 00:00:00
abstract:OBJECTIVE:To investigate the role of intragenic deletions of ALDH7A1 in patients with clinical and biochemical evidence of pyridoxine-dependent epilepsy but only a single identifiable mutation in ALDH7A1. METHODS:We designed a custom oligonucleotide array with high-density probe coverage across the ALDH7A1 gene. We pe...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001883
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVE:Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks. METHODS:The patient population consisted of a consec...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001697
更新日期:2015-06-16 00:00:00
abstract::Current concepts suggest that avoidance of pulsatile stimulation of dopamine receptors in Parkinson's disease (PD) can prevent the onset of dyskinesia. In MPTP-treated primates, repeated administration of levodopa or other short-acting dopamine agonist drugs leads to the onset of marked involuntary movements. In contr...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.62.1_suppl_1.s47
更新日期:2004-01-13 00:00:00
abstract:OBJECTIVE:To devise a neurophysiologic strategy to select X-linked Charcot-Marie-Tooth neuropathy syndrome (CMTX) families for connexin 32 mutation screening. BACKGROUND:Once the common chromosome 17 DNA duplication (CMT1A syndrome) has been excluded, clinical features are not sufficiently distinctive to select which ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.51.5.1412
更新日期:1998-11-01 00:00:00
abstract:OBJECTIVE:We aimed to analyze lesion patterns of lacunae-sized infarctions in the perforating arterial territory in terms of shape and to determine whether the particular pattern of conglomerated beads shape affected early neurologic deterioration. METHODS:We consecutively included acute ischemic stroke patients with ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e318253d62f
更新日期:2012-05-01 00:00:00
abstract::Four men with evidence of hemiatrophy resulting from early-life hemispheral injury developed unilateral hemiparkinsonism on the side of the hemiatrophy. The parkinsonism began in the fourth decade and has remained unilateral for 5 to 35 years. In all four patients, the parkinsonism was slowly progressive, but the clin...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.5.625
更新日期:1981-05-01 00:00:00
abstract:BACKGROUND:Because of the relatively poor results of frontal lobe epilepsy (FLE) surgery, identification of prognostic factors for surgical outcome is of great importance. METHODS:To identify predictive factors for FLE surgery, we analyzed the data of 61 patients (mean age at surgery 19.2) who had undergone presurgica...
journal_title:Neurology
pub_type: 临床试验,杂志文章
doi:10.1212/wnl.54.7.1470
更新日期:2000-04-11 00:00:00
abstract:OBJECTIVE:To examine whether prior statin use affects outcome and intracranial hemorrhage (ICH) rates in stroke patients receiving IV thrombolysis (IVT). METHODS:In a pooled observational study of 11 IVT databases, we compared outcomes between statin users and nonusers. Outcome measures were excellent 3-month outcome ...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0b013e31822c9135
更新日期:2011-08-30 00:00:00
abstract::Three children with cerebral oligodendrogliomas causing partial complex or generalized seizures presented with completely normal neurologic examinations. CT showed low-density, nonenhancing surface lesions. Although these CT features are usually associated with infarcts or cysts, neoplasm was suspected because of irre...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.33.6.806
更新日期:1983-06-01 00:00:00
abstract:OBJECTIVE:Dominant, left anteromedial temporal lobe resection (AMTLR) for seizure control carries risks to verbal episodic memory and visual object naming. Consistent with traditional thinking, verbal memory decline is considered a consequence of hippocampal removal and naming decline has been attributed to lateral tem...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181dd40f0
更新日期:2010-05-11 00:00:00
abstract::Mammalian spinal cord neurons were grown in dissociated cell culture and used to study the effects of the anticonvulsant barbiturates phenobarbital and mephobarbital, and the anesthetic barbiturates pentobarbital, secobarbital, and 1,3-dimethyl-butylethyl barbituric acid on amino acid responses and neuronal membrane p...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.4.432
更新日期:1979-04-01 00:00:00
abstract::The objective of this study was to use high-resolution MRI techniques to determine whether children with Down syndrome exhibit decreases in hippocampal and amygdala volumes similar to those demonstrated in recent studies of adults with this condition. When corrected for overall brain volumes, amygdala volumes did not ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.56.7.972
更新日期:2001-04-10 00:00:00
abstract:OBJECTIVE:To describe neural autoantibody profiles and outcomes in patients with neurologic autoimmunity associated with immune checkpoint inhibitor (ICI) cancer immunotherapy. METHODS:In this retrospective descriptive study, 63 patients with ICI-related neurologic autoimmunity were included: 39 seen at the Mayo Clini...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000010632
更新日期:2020-10-27 00:00:00
abstract::We present a new benign eating disorder associated with lesions involving parts of the right anterior cerebral hemisphere. This "gourmand" syndrome describes a preoccupation with food and a preference for fine eating. Two exemplary case reports illustrate this new syndrome. Analysis of the clinical and anatomical data...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.48.5.1185
更新日期:1997-05-01 00:00:00
abstract:OBJECTIVE:To test the diagnostic accuracy of the horizontal head impulse test (h-HIT) of vestibulo-ocular reflex (VOR) function in distinguishing acute peripheral vestibulopathy (APV) from stroke. Most patients with acute vertigo, nausea/vomiting, and unsteady gait have benign APV (vestibular neuritis or labyrinthitis)...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000314685.01433.0d
更新日期:2008-06-10 00:00:00