Abstract:
OBJECTIVE:To evaluate the frequency and types of change in "self" seen in frontotemporal dementia (FTD) and to determine the relative involvement of the nondominant and dominant frontal and temporal brain regions in FTD patients with or without changes in a sense of self using neuropsychology tests and neuroimaging. BACKGROUND:The self has been defined as "the total, essential, or particular being of a person" involving "the essential qualities distinguishing one person from another." Some suggest that the frontal lobes play a dominant role in maintaining the self. FTD affects anterior frontal and temporal areas and can be associated with a loss of self. METHODS:Seventy-two consecutive FTD patients were evaluated with neuropsychiatric, neuropsychologic, and behavioral measures. Patients were imaged with MRI and SPECT. Charts were reviewed by a social psychologist to determine patients who exhibited a dramatic change in their self as defined by changes in political, social, or religious values. The brain areas with the most severe atrophy or hypoperfusion on neuroimaging were noted. RESULTS:Seven of 72 patients exhibited a dramatic change in self. In six of the seven, the selective dysfunction involved the nondominant frontal region. In contrast, only one of the other 65 patients without selective nondominant frontal dysfunction showed a change in self. CONCLUSIONS:FTD patients with asymmetric loss of function in the nondominant frontal lobe often exhibit a diminished maintenance of previously learned self-concepts despite intact memory and language. Normal nondominant frontal function is important for the maintenance of the self.
journal_name
Neurologyjournal_title
Neurologyauthors
Miller BL,Seeley WW,Mychack P,Rosen HJ,Mena I,Boone Kdoi
10.1212/wnl.57.5.817subject
Has Abstractpub_date
2001-09-11 00:00:00pages
817-21issue
5eissn
0028-3878issn
1526-632Xjournal_volume
57pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::Spinal muscular atrophy (SMA) is the most common fatal neuromuscular disease of infancy. SMA type I is the most severe and mortality is usually due to respiratory failure. In type II the disability is of later onset and less severe, and prognosis has improved primarily due to supportive care. Type III is the mildest f...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000183282.10946.c7
更新日期:2005-11-08 00:00:00
abstract::Juvenile-onset dystonia that improves after levodopa may occur in both dopa-responsive dystonia (DRD) and juvenile parkinsonism (JP), clinically similar conditions with different prognoses and management goals. The authors show normal striatal uptake of the dopamine transporter ligand FP-CIT with SPECT in a clinically...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.56.2.266
更新日期:2001-01-23 00:00:00
abstract::Seronegative (SN) patients with myasthenia gravis (MG) have clinical and electrophysiologic features similar to those of seropositive (SP) patients, and they respond to the same therapeutic measures. However, because SN patients lack detectable (by standard radioimmunoassays) serum antibodies to acetylcholine receptor...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.48.6.1638
更新日期:1997-06-01 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:OBJECTIVE:To examine the long-term cognitive trajectories of individuals with normal cognition at baseline and distinct amyloid/tau/neurodegeneration (ATN) profiles. METHODS:Pooling data across 4 cohort studies, 814 cognitively normal participants (mean baseline age = 59.6 years) were classified into 8 ATN groups usin...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000007248
更新日期:2019-04-02 00:00:00
abstract:OBJECTIVES:Patients surviving retinopathy-positive cerebral malaria (CM) are at high risk for the development of epilepsy, developmental disabilities, and behavioral abnormalities. We aimed to establish whether retinopathy-negative CM is also a risk factor for these outcomes. METHODS:Between 2005 and 2007, survivors o...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31826aacd4
更新日期:2012-09-18 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::Four patients with presumed Tolosa-Hunt syndrome ultimately proved to have a parasellar tumor. All four had manifestations of a cavernous sinus syndrome, normal radiologic and medical investigations, and response to steroid therapy. The Tolosa-Hunt syndrome is a diagnosis of exclusion; many other lesions can simulate ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.36.2.198
更新日期:1986-02-01 00:00:00
abstract:OBJECTIVE:To investigate whether neuronal activity-regulated pentraxin (Narp) colocalizes with hypocretin (Hcrt or orexin) in the normal human brain and to determine if Narp staining is lost in the narcoleptic human brain. BACKGROUND:Human narcolepsy is characterized by a loss of the peptide hypocretin in the hypothal...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000175219.01544.c8
更新日期:2005-10-25 00:00:00
abstract:OBJECTIVE:To determine the incidence of spinocerebellar ataxia (SCA) types 1, 2, 3, 6, and 7 and Friedreich's ataxia (FA) among a large panel of ataxia families. BACKGROUND:The ataxias are a clinically and genetically heterogeneous group of neurodegenerative diseases that variably affect the cerebellum, brainstem, and...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.51.6.1666
更新日期:1998-12-01 00:00:00
abstract::Wolfram syndrome is an autosomal recessive disorder beginning in childhood that consists of four cardinal features: optic atrophy, diabetes mellitus, diabetes insipidus, and neurosensory hearing loss. Aside from these features, the clinical picture is highly variable and may include other neurologic abnormalities such...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.42.6.1220
更新日期:1992-06-01 00:00:00
abstract::Seventy-one neonates were observed for the relationship between phenobarbital plasma concentrations and elimination of seizures. Sixty neonates (85%) had seizures controlled by phenobarbital alone. Effective plasma concentrations were 10.1 to 46.4 mg per liter. Although 36 neonates had seizures controlled by phenobarb...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.32.12.1401
更新日期:1982-12-01 00:00:00
abstract::This study was undertaken to determine the amount and histologic appearance of the persistent thymic tissue removed post mortem from 20 patients over 60 years of age with myasthenia gravis. One patient died several days after thymectomy. No recognizable thymic tissue on gross examination was seen in any patient. On mi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.25.3.294
更新日期:1975-03-01 00:00:00
abstract::The biochemical hallmark of adult Refsum disease (ARD) is an isolated deficiency in the breakdown of phytanic acid. This usually results from a PHYH gene defect, although some cases have been found to carry a PEX7 defect. We describe the phenotype of such a patient, indistinguishable from that of classic ARD. Hence, w...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000255960.01644.39
更新日期:2007-02-27 00:00:00
abstract::Trigeminal neuralgia is an uncommon but troublesome symptom of multiple sclerosis that can be refractory to conventional treatments. Misoprostol, a long-acting prostaglandin E1 analogue, relieved pain in six of seven patients who had failed to respond to conventional pharmacologic therapy. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.45.6.1097
更新日期:1995-06-01 00:00:00
abstract::Rats with unilateral lesions of the substantia nigra were treated with L-dopa 25 mg/kg subcutaneously 30 minutes after treatment with carbidopa 25 mg/kg intraperitoneally. In the ipsilateral striatum, dopamine levels achieved a peak of 1.57 +/- 0.63 micrograms/g (control 0.19 +/- 0.05 micrograms/g) at 20 minutes and d...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.8.1105
更新日期:1984-08-01 00:00:00
abstract::Use of neuroleptic medications is common among elderly patients. Using multivariate models, the authors found that use of antipsychotic medications, age, and disability were strong predictors of developing self-reported Parkinson disease (PD) in the subsequent year among 1992 to 2000 Medicare Current Beneficiary Surve...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000203348.13337.89
更新日期:2006-03-28 00:00:00
abstract:OBJECTIVE:To determine whether longitudinal cognitive decline is associated with increased risk of sleep disturbance in older, nondemented, community-dwelling women. METHODS:We studied 2,474 women (mean age 68.9 years) who were part of a prospective study started in 1986; women with baseline or follow-up evidence of p...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000265814.69163.da
更新日期:2007-07-17 00:00:00
abstract:OBJECTIVE:To investigate the possible association of persistent enterovirus (EV) infection with the development of ALS. BACKGROUND:Although ALS is a clinically well-defined motor neuron disease, little is known about the etiology and pathogenesis of the sporadic cases. Among the different causes that have been hypothe...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.54.1.20
更新日期:2000-01-11 00:00:00
abstract::We review adjunctive ketogenic diet (KD) and modified Atkins diet (MAD) treatment of refractory epilepsy in adults. Only a few studies have been published, all open-label. Because of the disparate, uncontrolled nature of the studies, we analyzed all studies individually, without a meta-analysis. Across all studies, 32...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/WNL.0000000000001004
更新日期:2014-11-18 00:00:00
abstract::We assessed the severity and temporal profile of distant neuromuscular effects from a single dose (280 units) of botulinum neurotoxin injected into neck muscles for torticollis. We performed single-fiber EMG studies on the biceps brachii of six patients to measure jitter (20 pairs) and fiber density on the initial tre...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.38.11.1780
更新日期:1988-11-01 00:00:00
abstract::We performed proton magnetic resonance spectroscopic imaging (1H-MRSI) in patients with motor neuron disease (MND) to evaluate the distribution and extent of cortical neuron damage or loss as reflected by decreased N-acetyl (NA) to creatine (Cr) resonance intensity ratios. We examined premotor (superior frontal gyrus)...
journal_title:Neurology
pub_type: 临床试验,杂志文章
doi:10.1212/wnl.44.10.1933
更新日期:1994-10-01 00:00:00
abstract::The association of APOE genotypes with cerebral microbleeds (CMBs) was examined on the basis of the location of CMBs in 414 patients who were admitted primarily because of stroke. With respect to possession of the epsilon2 or epsilon4 allele, the adjusted odds ratio was 1.94 (1.05 to 3.58) for lobar CMBs but 1.21 (0.6...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000183311.48144.7f
更新日期:2005-11-08 00:00:00
abstract:BACKGROUND:Over 50% of persons with idiopathic REM sleep behavior disorder (RBD) will develop Parkinson disease (PD) or dementia. At present, there is no way to predict who will develop disease. Since polysomnography is performed in all patients with idiopathic RBD at diagnosis, there is an opportunity to analyze if ba...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181ca0166
更新日期:2010-01-19 00:00:00
abstract::We studied 10 patients with transient global amnesia (TGA). Routine electroencephalograms (EEGs) were normal in six; two showed mild temporal lobe abnormalities, one had bitemporal delta activity with rare sharp waves, and one showed bioccipital rhythmic theta activity. Nasopharyngeal (NP) recordings were obtained in ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.6.869
更新日期:1979-06-01 00:00:00
abstract:OBJECTIVES:To identify gene mutations in patients with dystroglycanopathy and prove pathogenicity of those mutations using an in vitro cell assay. METHODS:We performed whole-exome sequencing on 20 patients, who were previously diagnosed with dystroglycanopathy by immunohistochemistry and/or Western blot analysis. We a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001162
更新日期:2015-01-20 00:00:00
abstract::Most gliomas in neurofibromatosis type 1 (NF1) are pilocytic astrocytomas (PAs) of the optic pathway occurring in young children. However, some individuals develop gliomas that lack the typical NF1-associated clinical features or radiographic appearance. We identified 17 atypical presentations from a review of 100 pat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000240076.31298.47
更新日期:2006-10-24 00:00:00
abstract:OBJECTIVE:To critically re-evaluate cases diagnosed as adult neuronal ceroid lipofuscinosis (ANCL) in order to aid clinicopathologic diagnosis as a route to further gene discovery. METHODS:Through establishment of an international consortium we pooled 47 unsolved cases regarded by referring centers as ANCL. Clinical a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000002943
更新日期:2016-08-09 00:00:00
abstract:OBJECTIVES:To provide information on the effect of the coronavirus disease of 2019 (COVID-19) pandemic on people with epilepsy and provide consensus recommendations on how to provide the best possible care for people with epilepsy while avoiding visits to urgent care facilities and hospitalizations during the novel cor...
journal_title:Neurology
pub_type: 共识发展会议,杂志文章
doi:10.1212/WNL.0000000000009632
更新日期:2020-06-09 00:00:00
abstract:OBJECTIVE:To test the hypothesis that ApoE isoforms affect mitochondrial structure and function that are related to cognitive impairment in Alzheimer disease (AD), we systematically investigated the effects of ApoE isoforms on mitochondrial biogenesis and dynamics, oxidative stress, synapses, and cognitive performance ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000009582
更新日期:2020-06-09 00:00:00